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379 Cards in this Set
- Front
- Back
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what are the non specefic neuronal changes in response to injury or illness? Describe each.
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central or axonal chromatolysis - swollen with eccentric nucleus and powdery nissl. Simple chromatolysis - swollen central nu. Acute neuronal injury - ischemic necrosis, eosinophilic cytoplasm with contracted dark nu (red dead neurons). Neuronal atrophy and degeneration - smaller with small flattened or deformed nu. Pigmentary degeneration- unduly large amounts of lipofuscin. ferrugination - mineral encrustation of injured cell and processes.
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what are the semi-specefic neuronal changes?
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large irregular neurons as seen in tuberous sclerosis. Intraneuronal storage - large bulbous neurons containing abnormally large amounts of normal or abnormal substances and eccentrically located nuclei. Neurofibrillary degeneration (tangles) - paired helical neurofilament protein (typical of Alzheimers). Interneuronal cytoplasmic inclusion bodies which have many types indicating certain diseases and will be discussed on a later card.
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what are the types of neuronal inclusion bodies and what disease do each of them indicate?
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granulovacular bodies are seen in alzheimer's. Lewy bodies are eosinophilic bodies that push neuromelanin to the side and are typical of parkinson's and lewy body dementia. Pick bodies are round to oval and mildly basophilic, with large accumulations of neurofilaments and microtubules as seen in Pick's disease. Lafora bodies are polyglucosans which are large spherical homogenous or laminated formations (myoclonic epilepsy). Negri bodies are sharp rounded or oval eosinophilic bodies containing small internal basophilic granules and are seen in rabies
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what are three abnormal dendritic changes?
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stellate bodies (swollen dendrite), reduction in arborization (branching), and severly distorted orientation of the dendritic tree.
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what are the four results of axonal injury?
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irregular swelling and fragmentation. Wallerian degenerations from cutting the axon and the tissue bundleing up. Spheroids which are accumulations of granulomous tissue with the axon still attached. A torpedo is a large mass of tissue balled up in the axon.
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what are the functions of astrocytes?
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physical and biochemical support. Electrical insulation orf receptor surfaces of neurons. Establishment and maintenance of the blood brain barrier. Lay down glial scar tissue. Move molecules to and from BV's to other CNS cells. May help process info with neurons.
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describe the process of and types of gliosis.
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gliosis is scar formation in the CNS by astrocytes. They become enlarged and increase in number and are called gemistocytes during this process. If a specefic tract is damaged, the pattern of gliosis is organized and called isomorphic. If damage is gross, then random gliosis or anisomorphic gliosis occurs.
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what is clasmatodendrosis?
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injury to the astrocyte itself, it becomes swollen and granular
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when can granulation tissue and collagenous scars form in the CNS?
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when the astrocytes die in response to severe damage, fibroblasts form scars from the BV's. (Occurs in surgical wounds and abscesses)
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an enlarged pale rounded or oval nu with invisible cytoplasm in an astrocyte indicates what?
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liver disease - alzheimer II astrocyte
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an enlarged pale rounded or oval nu with visible cytoplasm in an astrocyte indicates what?
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liver disease - alzheimer I astrocyte
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formations/bodies on the astrocytic process indicates and are called what?
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long standing gliosis, some tumors, and rare congenital diseases. Rosenthal fibers
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describe oligodendrocyte and myelin sheath reactions to injury.
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cells will swell and look like a fried egg with perinuclear halo. Myelin will swell and break into shorter segments called elipsoids. Demyelination may occur.
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what are the ependymal cells reaction to injury?
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cell loss mainly, ependymal granulation to fill the holes. Can lead to hydrocephalus if the aqueduct is gliosed.
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describe the microglial response to injury and the various shapes they may take.
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injury makes their processes retract, the cells then proliferate (satelliteosis) the nuclei then elongate and cells become mobile to move to the site of injury. Monocytes from the vessels also join the fight. Both cell types become macs or histiocytes and may be called gitter or compound granule cells. Rod cells are the elongated microglia seen in response to slow injury. Lamellar cells AKA glial nodules are seen in response to viruses, ricketsias, and protozoas.
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what herniations result from increased intracranial pressure?
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subfalcin AKA cingulate gyrus herniation which may compress the vessels. Uncal or uncinate transtentorial herniation, and cerebellar tonsillar herniation
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what are the three types of CNS edema?
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vasogenic (fluid in the extracellular space - can cause seperation of myelin), cytotoxic (excess intracellular water), and interstitial (in non communicating hydrocephalus when excess fluid crosses the ependymal lining).
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differentiate between communicating and non communicating hydrocephalus.
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communicating is in the subarachnois space or arachnoid villi while non-communicating is within the ventricular system
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slow and insidious hydrocephalus in adults may result in what?
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dementia, gait disturbances, and incontinence
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what is the gross pathology of enlarged ventricles called?
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hydrocephalus ex vacuo
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during gestation, what are the important periods and associated anamolies?
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1-4 wks: dysraphic effects in which the brain or cord does not develop or maybe outside of the skull or spine. 4-7 weeks: major parts of the brain do not separate or form (secondary cleavage stuff). 7-28 wks: dysplasias can occur which are defects in cell migration and differentiation. Last half of fetal life deals with formation of sulci and gyri and myelinization. After birth myelinization and glial proliferation are important
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what is the area cerebrovasculosa?
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deorganized neural tissue, ependyma, and thin walled vessels where the brain should be in anencephaly
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what are the caudal neural tube defects?
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spinal dysraphism (defect of posterior vertebrae), rachischisis (absent posterior vertebrae and cord and nerves are exposed or non-existant) myelomeningoceles, meningeocele, and spina bifida occulta (small defect in vertebral arch)
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dysraphic birth defects may be detected by what? They are usually associated with what?
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raised AFP levels. Folate deficiency
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forebrain defects are associated with what?
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maternal diabetes, alcohol and other drugs, rubella, and toxoplasmosis
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the least serious form of holoprosencephaly is what? What chromosomal abnormality Is holoprosencephaly associated with?
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arrhinencephally, trisomy 13.
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what are four dysplasias (migrational defects) during development?
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agyria, pachgyria (fewer gyri - most severe), polymicrogyria (cobblestone appearance with four layer cortex histologically), and neuronal heterotopias.
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microcepaly and megalencephaly are results of what?
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forebrain defects
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describe arnold chiari malformation.
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low tentorium, malformed midline cerebellum with extension of vermis through enlarged foramen magnum, lumbar meningomyelocele, and hydrocephalus
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describe the findings of dandy walker.
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enlarged posterior fossa, absent cerebellar vermis, and large midline cyst lined by ependyma
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describe the four common cord defects.
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dimyelia is 2 complete cords. Diastematomyelia is division of the cord into two side by side structures. Hydromyelia is expansion of the central canal and syringomyelia is a cleft in the cord. Note hydromyelia and syringomyelia are often in the cervical region.
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what are the gross findings on a down syndrome pt's brain?
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shortened anterior to posterior diameter and simple gyri pattern
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what are the phakomatoses?
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neurofibromatosis, tuberous sclerosis, von-hippel lindau
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differentiate between type I and type II neurofibromatosis.
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I is peripheral and II is central
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what are the clinical manifestations of tuberous sclerosis?
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seizures, MR, cutaneous angiofibromas, cortical tubers, subependymal hamartomas, cysts in the liver, kidney and pancreas, renal angiomyolipomas, retinal glial hamartomas, and pulm/cardiac myomas
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describe von hippel-lindau.
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hemangioblastomas of cerebellum and other CNS and eye locations. Cysts/cystadenomas of the pancreas, liver, kidney, and epidydimis. Increased incidence of renal cell carcinoma
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this condition is characterized by massive destruction of the brain except for some small clinical hallmarks after it has formed.
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hydranencephaly
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fetal alcohol syndrome signs and symptoms include…
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MR, microcephaly, growth retardation, many facial disfigurements…
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intraparenchymal hemmorhages occur when?
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in a premie less than 30 wks
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periventricular leukomalacia occurs when?
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in a premie older than 30 wks
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perinatal (term) ischemic lesions look like what?
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injured depths of sulci, AKA thinned gliotic gyri or ulegyria
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what are the four ways infection can reach the CNS?
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hematogenous (most common), direct implantation, local extension, and via the PNS
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what types of meningitis exist besides aseptic and acute pyrogenic?
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CSF infection, pachymeningitis (dura inflammation), leptomeningitis (pia and arachnoid inflammation), pleocytosis (increased WBC in the CSF)
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what are the common organisms (bacs) that give meningitis to neonates? Infants and children? Adolescents/young adults, and the elderly?
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E. coli and GROUP B STREP. S.pneumoniae. N. meningitidis. S. PNEUMONIAE, L. monocytogenes
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what are two common signs of acute bacterial meningitis?
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kerning sign (pain on extension of knee when hip is flexed) and Brudziski's sign (spontaneous flexion of knees and hips when neck is flexed)
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What does CSF look like when there is bac meningitis?
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has bacteria and increased WBC (90,000 at peak) which are mainly neutrophils, increased pressure and protein, decreased glucose
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what are the complications of acute pyrogenic meningitis?
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phlebitis can lead to hemmorrhagic infarct of the brain. Leptomeningeal fibrosis may lead to hydrocephalus
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describe the features of aseptic meningitis
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most common viral disease of the CNS, clinical features are simillar to acute pyrogenic but are less severe, mainly affects children and young adults. CSF has much more lymphocytes than neutro's, slightly increased protein, and normal glucose.
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what are the most common organisms in CNS abscesses?
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staph and strep
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what are the predisposing conditions that may lead to a CNS abcsess
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bacterial endocarditis, cyanotic congenital heart disease, chronic pulmonary disease, sepsis, ENT infections
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what kind of scar tissue is seen surrounding a CNS abscess?
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fibrous
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what is a subdural empyma?
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infectious spread from adjacent tissue such as a sinus infection
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tuberculosis of spinal columns is called what?
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Pott's disease
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tuberculosis and mycobacterioses in the CNS may present these which are discrete and large confluent granulomas with central caseous necrosis with surrounding granulomatous rxn.
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tuberculoma
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complications of tuberculosis and mycobacterioses can cause what complications?
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arachnoid fibrosis which can lead to hydrocephalus and obliterative endarteritis which can also lead to an infarct
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what are the three types of neurosyphilis?
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meningovascular neurosyphilis, paretic, and tabes dorsalis
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describe the features of meningovascular neurosyph.
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CSF has normal pressure, few lymphos, increased protein, and serology +. Chronic meningitis also exists with or without obliterative endarteritis
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Describe paretic neurosyph
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dementia, dementia, dementia, plus pupils that accommodate but do not react to light. CSF has serology
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describe tabes dorsalis
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sensory nerves are attacked, thus locomotor ataxia, decreased pain, absent DTR's. CSF shows lymphos, increased protein and serology, histo reveals meningitis around the dorsal root, wallerian degeneration with loss of axons and myelin in dorsal roots and posterior columns.
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what are the chronic bacterial infections of the CNS besides neurosyph, tuberculosis, and mycobacterioses?
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lyme disease
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describe the histopathology associated with viral encephalitis as well as the inclusion bodies seen with individiual viruses.
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perivascular and parenchymal mononuclear infiltrates, glial nodules and neuronophagia (oligos eating dead neurons). Herpes has small intranuclear eosinophilic bodies. Rabies has cytoplasmic negri bodies. Polyoma has oligodendroglial nuclear ground glass, subacute sclerosing panencephalitis (measles) has basophilic intranuclear with halo. Cytomegalovirus has eosinophilic nuclear and cytoplasmic inclusions in astrocytes and neurons with cellular enlargement
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describe arthropod borne viral encephalitis including the clinical features, CSF, and the most important virus.
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eastern equine encephalitis is the most important. Clinical is confusion, delirium, seizures, coma. CSF is initially neutros then lymphos, increased protein and normal sugar.
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describe encephalitis from herpes simplex virus 1.
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alterations in mood, memory, then somnolence and coma. PCR based virus detection in CSF. Necrosis in the inferomedial temporal lobes and inferior frontal lobe. Histo has intranuclear eosinophilic inclusions. Treat with antivirals. Comon in children/YA
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this virus causes liquefication of cerebrum and cerebellum of 50% of neonated born to mothers with active infections.
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herpes simplex virus 2
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describe who gets cytomegalovirus encephalitis.
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fetus will have periventricular necrosis and calcification leading to brain destruction and microcephaly. The immunosuppressed will have subacute encephalitis with microglial nodules especially in gray matter with or without inclusions.
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describe poliomyelitis
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fecal oral infection. Flacid paralysis, muscle wasting, and hyporeflexia. Acute can lead to respiratory paralysis or myocarditis. Anterior horn cells may be lost via neuronophagia
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describe rabies clinical symptoms, path, and virus spread.
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local parasthesias around wound, fever, malaise, headache. Agitation, stupor, coma, death, extraordinary excitability of throat. Brain stem and basal ganglia encephalitis and can travel, negri bodies in pyramidal neurons.
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Most HIV meningoencephalitus infections contain what on histo?
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giant cells, usually see macs/monos that get in there and bring the virus there
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progressive multifocal leukoencephalopathies kill what cells and are spread by what virus?
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oligodendrocytes and spread by polyoma virus
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describe the gross and histopatholgy of PML.
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white matter destruction. Lipin laiden macs. No myelin on axons. Few oligodendroglia. Edge of infection has oligodendroglia with amphophillic viral inclusions and bizarre giant astrocytes.
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describe spongiform encephalopathies.
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AKA creutzfeldt Jakob. Prion is infectious agent. Rapidly progressive dementia, kills in 6months. Histo shows intracytoplasmic membrane bound vacuoles and astrocytosis.
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what fungal species cause meningitis? Vasculitis? And parenchymal infection?
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cryptococcus, aspergillus and mucor, candida and cryptococcus
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the CSF of a cryptoccocal infected pt will be positive for what two specefic tests?
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india ink preperation and cryptococcal antigen
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when should you do a spinal tap?
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is MD - infection (most important), subarachnoid hemorrhage, malignancy of CNS, and demyelinating disease
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how much CSF can normally be removed? When is this not true and why?
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up to 20 mL unless opening pressure is over 200 or pressure drops a lot after taking a bit of CSF. This could mean or lead to cerebellar herniation and death or block in spinal canal.
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what is examined in the CSF?
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4 tubes one for chemistry, one for critters, one for cell count, and one for cytology
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when are emergent LP's indicated?
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suspected meningitis, CNS leukemia, and subarachnoid hemorrhage.
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what are common LP complications? Significant complications?
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post puncture headache. Cerebellar tonsil herniation, introduction of infectious agents, progression of paralysis in patients with spinal cord tumors.
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gross examination of CSF is performed to determine what?
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xanthochromia
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what does CSF of traumatic taps look like as opposed to pathologic hemmorrhage?
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color will clear between tubes 1 and 3. It is not xanthrochromic if examined quickly. IT contains no hemosiderin laden macrophages or cells that have ingested RBC's. Has no fibrin degradation products.
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what is tyndall's effect?
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white snowy effect when the CSF is held at a 90 degree angle indicating high WBC, RBC, micro orgs, elevated proteins, etc.
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increased CSF viscosity indicates what?
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metastatic mucin producing adenocarcinomas, cryptococcal meningitis, and nucleus pulposus injury.
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clot formation in CSF occurs when…?
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when things in the protein result in high CSF protein
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when should microscopic examination of the CSF be done?
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within the hour of the tap
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what are the normal number of mononuclear cells in CSF in an adult, infant and neonate?
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less than 6, 19, and 30
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how do you correct for the number of white blood cells in the CSF if they are increased due to hemmorhage?
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subtract 1 WBC for each 700 RBC's
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what is the cause of the majority of cases of increased neurtophils in the CSF?
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meningitis (usually bac, but can be fungal or viral) persistant increase may be due to non infectious causes or unusual orgs
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what are the causes of increased lymphos in the CSF?
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mostly viral meningitis, sometimes partially treated bac or fungal meningitis, TB, or syph. Non meningitis cuases are MS, SSPE, sarcoid, vasculitis, drugs, guillain barre
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what are the causes of increased eosinophils in the CSF?
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eosinophilic meningitis, fungal (coccidiodes), parasitic CNS infection, allergic reactions
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what causes increased monocytes in the CSF?
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part of mixed cell rxn, lack diagnostic speceficity
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a mixed cell pattern without neutrophils in the CSF usually means what?
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early viral or syphilitic meningoencephalitis
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increased macs in the CSF usually means what? What types are seen and when?
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hemorrhages. Erythrophages 12 to 14 hours after. Siderophages 2 to 3 days later and have brown hemosiderin and they may persist fo weeks
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what 3 tumor cells are found in the CSF (from most often to least often)?
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leukemia, metastatic neoplasms (melanoma), primary CNS malignancies
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For what organisms do the following preperations/stains of CSF test: gram stain, acid fast/auramine rhodamine stains, india ink, phase contrast +/- acridine orange, darkfield??
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meningitis (this is the single best test for rapid Dx of bac, fungal or parasites), TB, cryptococcus, amebic meningitis, syphillis
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what are the componenets of a micro-org exam?
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gram stain, cultures, possible latex agglutination (bacterial antigen, good for partially treated meningitis)
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what test is done to confirm the presence of neurosyph?
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VDRL test
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viral meningitis is essentially a diagnosis of exclusion but these tests may be done for specefic etiology.
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viral culture, RT-PCR, immunofluorescence for ag, brain biopsy for herpes encephalitis, serologic methods
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Abnormalities in the CSF for HIV include what?
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lymphopleocytosis, increased IgG, oligoclonal bands, opportunistic infections
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what tests can be done for fungal meningitis?
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india ink and mucicarmine stains, latex agglutuniation for cryptococcall antigens (most sensitive), culture (takes weeks to grow)
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what tests can be done for TB meningitis?
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acid fast stain is not very sensitive, fluorescent auramine-rhodamine stain is better, cultures are sensitive but need lotsa CSF
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elevated CSF protein indicates what?
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increased permeability of BBB, CSF circulation defects, increased IgG synth, increased blood-CSF permeability
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low CSF may indicate what?
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removal of large volumes of CSF, leaks, increased intracranial pressure, hyperthyroidism. Seen in young children
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albuminocytologic dissociation is what and indicates what?
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increased CSF protein with normal cells, indicates Guillain Barre
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hypoglycorrhacia is what and may indicate what?
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low CSF glucose, can mean hypoglycemia, decreased transport in CNS, increased use in CNS, WBC's, micro-orgs
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What tumors does CEA mark for? HCG and AFP?
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metastatic adenocarcinoma. Germ cell tumor
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where are MS lesions in the CNS often found?
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periventricularly around vessels, but no area is spared
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differentiate recent plaques versus old plaques of MS.
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recent are pink, soft, swollen and ill defined while older ones are firm, gray, sunken, and sharply demarkated
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describe the characteristics of early MS plaques and the edges of chronic plaques.
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axons spared, macs, perivascular, glial hypertrophy, adjacent white matter hypercellularity and oligodendrocytic proliferation
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describe the microscopic appearance of a chronic inactive MS plaque.
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sharply demarkated with fibrillary gliosis, depletion of axons, oligodendros absent in the center, few lipid laden macs
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describe an MS shadow plaque.
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ill defined, pale staining with thin myelinated fibers, mild increase in oligodendros, moderate gliosis, foamy macs occasionally
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Describe the acute MS variant.
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acute/subacute onset of headache, vomitting, brain stem signs, spinal cord and optic nerve involvement then eventual corticospinal involvement and death in 10 months
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describe neuromyelitis optica, an MS variant.
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coincidence of retrobulbar neuritis and signs of demyelinating disease in the cord
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describe the MS variant known as Balos AKA concentric sclerosis.
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bands of severly demyelinated tissue alternate in a concentric pattern with rings or arcs of well preserved myelin
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this variant of MS is usually seen in children, has a 3 yr course, and is characterized by giant plaques.
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schilder's disease
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these diseases are characterized by either postvaccinial or postinfectious widespread demyelination that is monophasic with headaches, vomitting, coma and can be fatal.
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acute disseminated encephalomyelitis
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where are the preferred demyelination sights in acute disseminated encephalomyelitis?
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perivascular
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what is the name and characteristics of the hyper acute form of acute disseminating encephalomyelitis?
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weston hurst: pree teens/YA, necrotizing hemorrhagic leukoencephalitis
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the rapidly progressive quality of dysmyelinating diseases usually results in what?
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demntia, quadriplegia, sensory deprivation with possible blidness
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describe the pathogenesis of the dysmyelinating disease metachromatic leukodystrophy.
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deficiency in Aryl aulfatase A leading to the accumulation of galactosyl sulfatide in white matter, peripheral nerves, and epithealial cells of other tissues
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describe the pathogenesis of the dysmyelinating disease globoid cell leukodystrophy.
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AKA krabbe's disease which is a defect in galactocerecroside beta galactosidase shunts galactocerebroside to a pathway that results in the buildup of psychosine thus injuring oligodendrocytes. Broken down myelin builds up in perivascular globoid cells and multinucleated histiocytes often surround BV's
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describe the pathogenesis of the dysmyelinating disease adrenoleukodystrophy.
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XLR, peroxisome malfunction, accumulation of cholesterol that can't be degraded, adrenal failure, usually juveniles.
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describe the pathogenesis of the dysmyelinating disease fibrinoid leukodystrophy (alexander's disease)
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mutation in glial fibrillary acid protein, diffusely demyelinated brain, rosenthal fibers
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describe the pathogenesis of the dysmyelinating disease canavan's disease AKA spongy sclerosis.
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def in apartoacylase. Large brains with spongy changes in deep cortex and adjacent white matter.
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describe the dysmyelinating disease pelizaeus merzbacher disease.
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possible myelination arrest. Widespread demyelination with sparing around BV's in leopard skin pattern
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describe the recovery of a wallerian degeneration.
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distal to transection, the myelin and axon degenerate, schwann cells will then proliferate (bands of bungner) and then part of the axon proximal to the cut will search for the group of schwann cells and regeneration may occur
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regeneration or recovery of wallerian degeneration depends upon what?
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if lesion is distal or proximal, age of pt, closeness of severed ends, degree of adjacent soft tissue injury
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where do demyelinating diseases usually begin their destruction in the PNS and what nerves are usually targeted?
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at the nodes, large diameter motor axons and spinal roots
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multiple demyelination and remyelination in the PNS results in what kind of nerve pattern?
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onion bulbs
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this condition implies metabolic derangement of the whole nueron and what is an example?
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axonal degeneration, diabetic neuropathy
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describe the cause and process of a neuronpathy and give an example.
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morphologic or biochemical changes in the cell body cause degeneration of entire cell and processes that is generally permanent. ALS
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what are common causes of distal axonopathies?
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exogenous toxins, many metabolic and hereditary diseases
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distinguish between neuropraxia, axonotmesis, and neurotmesis.
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myelin disruption, axonal disruption, entire nerve disruption
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give an example of something that would cause a focal AKA mono neuropathy.
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carpal tunnel
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what types of lesions can cause focal neuropathies?
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physical injury, ishcemic, infiltration (cancer, infection, auto immune)
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what are the positive and negative symptoms of a neuropathy?
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pos is dysethesia (burning from light touch) and parasthesia (tingling) while neg is numbness
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an axonopathy will have what effects on an EMG?
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polyphasic, motor unit dropout, and denervation (fibrilations and positive waves)
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what lab studies should be done if axonopathy is considered?
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CBC, metabolic panel, thyroid function test, B12 levels, folate levels, autoimmune screening, syph test
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myelinopathies may cause what EMG changes?
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no denervation, but polyphasics and motor unit drop out may exist.
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what peripheral neuropathy is the most common or prototypical hereditary neuropathy? What are associated symptoms?
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carcot marie tooth disease. It is AD and also shows pes cavus (high arched feet) and scoliosis
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aside from fixing the underlying cause of a neuropathy (diabetes, drug induced, etc.) how can the positive symptoms be treated?
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anticonvulsants (block tingling), topical ointmants like xylocaine, analgesics, central pain modifiers like SSRI's
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what are the clinical manifestations of sturge-weber syndrome?
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port wine stain of the forehead and upper eyelid from birth (trigem distribution) (possibly bilateral and in trunk and extremeties), leptomeningeal angioma ipsilateral to the nevus, but neurologica symptoms including seizures, hemiparesis, visual field defects are ipsilateral. Glaucoma most likely will be present ipsilaterally and cognitive impairments may exist.
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what would radiologic findings of the brain be in one with sturge weber?
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tram tracks or calcifications in the ipsilateral cortex.
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what is the management for sturge weber?
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anticonvulsants, hemisphorectomy, pulsed dye laser therapy, monitor intraoccular pressure
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what is a tumor manifestation of neurofibromatosis type 1 that can lead to blindness?
|
optic glioma
|
|
|
what gene is defective in neurofibromatosis type 1 and what is the result of this?
|
neurofibromin which is a tumor suppressor gene that inhibits ras, thus ras will be turned on…
|
|
|
describe neurofibromatosis type 2.
|
subcutaneous schwanomas, cataracts, retinal hamartomas, optic nerve meningiomas, flat hairy pigmented skin lesions, complete deafness due to 8th nerve tumors, loss of facial nerve function, poor balance, weakness
|
|
|
describe the genetics of neurofibromatosis type 2
|
AD, tumor suppressor gene called merlin that is a cell membrane related protein
|
|
|
what skin lesions are seen in tuberous sclerosis?
|
ash leaf spot is the most common, facial angiofibromas, shagreen patch, periungual fibromas
|
|
|
what tumor manifestations does tuberous sclerosis have?
|
skin, eyes, cardiac, kidneys and brain
|
|
|
what are a common CNS tumor type in tuberous sclerosis?
|
subependymal tumors
|
|
|
what is the management procedure for tuberous sclerosis?
|
genetic counseling, treat epilepsy if it exists, surgical intervention for hydroceph, monitor involvement in the kidney, heart and eye
|
|
|
what are the common tumors of von hippel lindau?
|
hemangioblastomas of the cerebellum are the most common, renal cell carcinomas, pancreatic cystadenomas, epidiymal cystadenomas, renal angiomas
|
|
|
what are the genetics for von hippel lindau?
|
VHL gene which is a tumor suppressor, AD transmission
|
|
|
what is the management for von hippel lindau disease?
|
surgical removal of posterior fossa tumor, laser photocoagulation of retinal lesions, genetic counseling
|
|
|
what are the clinical features of incontinentia pigmenti?
|
alopecia, hypodontia, bulbous and pigmented rash, retinal vascular abnormalities, cataracts, ptosis, intellectually normal
|
|
|
what are the genetics of incontinentia pigmenti?
|
XLD, NEMO gene deletion, deals with NF-kb
|
|
|
what are the neurological findings of ataxia-telangiectasia?
|
progressive ataxia, intention tremor, choreoathetosis, segmental myoclonus, dystonia of fingers, neck and limbs, nystagmus and praxia of voluntary gaze, peripheral nerve and cord involvement, dysarthria
|
|
|
what are the systemic manifestations of ataxia telangiectasia?
|
recurrent sinopulmonary infections, deficiency of IgA and IgG, cellular sensitivity to ionizing radiation, increased risk of malignancy to the lymphoreticular system
|
|
|
what are the skin manifestations of ataxia telengiectasia?
|
telangiectasias in and around the eye, nose, ears, neck, blepharitis (inflammation of eyelids), premature aging in skin and hair.
|
|
|
when ataxia telangiectasia is suspected, what labs are done?
|
alpha fetal protein, carcinoembryonic antigen, decreased IgA, E, G, G2, chromosomal breakages
|
|
|
what are the current theories on developing MS?
|
it is believed to be genetic and environmental. T cell (Th1) mediated autoimmune disease that develops in genetically susceptable ppl following some yet unidentifiable infection
|
|
|
Specefically in the CNS, where do MS plaques usually form?
|
on the venule side of a vessel.
|
|
|
describe the immune response and scar formation in MS lesions?
|
T cells exit the BBB on venule side and enter perivenular parenchyma along with monocytes that become macs and then eat the myelin. Oligodendrocyte function is disrupted. The inflammation ceases due to some endogenous CNS immune activity (Th2 response, increased cortisol) and then astrocytes swell and gliosis occurs.
|
|
|
Lack of what HLA markers are associated with MS?
|
HLA type II
|
|
|
what is the typical ocular manifestation of MS?
|
optic neuritis
|
|
|
the most common MS symptom is what?
|
fatigue
|
|
|
what are some clinical manifestations of MS?
|
any cortical signs, trigeminal neuralgia, lhermitte's sign (pain down arm or spine when bend neck forward), paroxymal phenom, other pain syndromes
|
|
|
what are the four clinical patterns of MS?
|
relapsing remitting (most common, have CNS issues, then go back to baseline), secondary progressive (relapse occurs and then they go back to something a bit worse than their baseline with each relapse), primary progressive has no relapses/attacks, they just progressively get worse), progressive relapsing (worst one, they progress quickly and develop many plaques, usually in ICU, could die from plaques affecting vital centrers).
|
|
|
the most important differential to rule out in MS is what?
|
HIV
|
|
|
the purpose of muscle satellite cells is what?
|
to help regenerate muscle
|
|
|
describe type 1 muscle fibers.
|
fat red ox, more myoglobin, mito and ox enzymes, slow twitch
|
|
|
describe type 2 muscle fibers.
|
white fast twitch, more glycogen for anaerobic glycolsis, more powerful, these guys get bigger when you work out
|
|
|
what are the effects of the atpase stain on muscles? Reverse atpase stain?
|
normal is at ph 9.4 and causes the type 2's to be dark, while reverse at ph 4.3 makes them lighter.
|
|
|
what does the non specefic esterase stain show?
|
atrophic muscle fibers, macs, endplates, note this might be abnormal in myotonic dystrophy
|
|
|
what does the NADH TR stain show?
|
shows distribution of organelles in the muscle fiber, can show atrophic fibers, mito accumulations, sarcoplasmic masses and target fibers
|
|
|
what does the alkaline phosphatase stain show?
|
regenerating muscle fibers, connective tissue in inflammatory myopathy
|
|
|
what does the pas stain show?
|
basement membrane and glycogen
|
|
|
what type of muscle atrophy is diagnostic of denervation?
|
large group atrophy
|
|
|
atrophy and hypertrophy are more common in what muscle fiber type?
|
type 2
|
|
|
muscle fiber splitting is most commonly seen in what?
|
myopathy
|
|
|
multiple internal nuclei of muscle fibers is characteristic of what condition?
|
myotonic dystrophy
|
|
|
describe the process of muscle degeneration, necrosis, and regeneration.
|
injury leads to hyaline and or granular changes in fiber and creatine kinase leaks out (with other muscle protein), myophagocytosis occurs via macs, regeneration occurs via proliferation of satellite cells, the regenerating fibers are large and basophilic with prominent nucleoli. necrosis heals by fibrosis
|
|
|
what do ring muscle fibers indicate?
|
a myopathic change and characteristic of myotonic dystrophy
|
|
|
what architectural defect in individual muscle fibers is typical of recent denervation/neurogenic disease?
|
target fibers
|
|
|
moth eaten muscle fibers indicate what pathology?
|
non specefic myopathy
|
|
|
partial denervation of the muscle will result in what process?
|
denervated fibers become covered with nicotinic Ach receptors which induce nearby nerves to sprout new buds and innervate them, leads to fiber type grouping
|
|
|
vacuoles seen in muscle fibers may indicate what?
|
muscular dystrophy, polymyositis, metabolic and toxic myopathies
|
|
|
sarcoplasmic masses and nemaline rods in muscle fibers indicate what?
|
non specefic myopathy, but large number of rods may indicate rod body myopathy
|
|
|
abnormal accumulation of mitochondria in muscle fibers shows up as what pathologically?
|
ragged red fibers
|
|
|
what is a congenital disease in which there is loss of motor neurons to the muscles of infants and is seen as small rounded and normal size muscle fibers of both types with hypertrophic type 1 fibers?
|
infantile spinal muscular atrophy
|
|
|
this group of myopathies are described as hereditary progressive non inflammatory degenerative muscle disease.
|
muscular dystrophy
|
|
|
describe the genetics of duchenne's MD.
|
XLR resulting in absence of dystrophin gene. 1/3 are spontaneous mutations, 1/3 are inherited, 1/3 are spontaneous mutations of mother
|
|
|
clinically, what is seen in duchenne's MD?
|
marked increase in creatine kinase from birth, gower maneuver, mostly pelvic and shoulder weakness, pseudohypertrophy of the calves, die early 20's - usually from resp failure and/or cardiac arrythmias and failure, occasionally in females, note that creatine kinase is elevated in 75% of carriers.
|
|
|
describe the early and late pathological changes seen in duchenne's MD.
|
increased numbers of internal nuclei, degeneration of fibers, necrosis and then myophagocytosis, some regerneration, but then fibrosis with fibrofatty replacement and overall muscle atrophy
|
|
|
the most common adult form of MD is what?
|
myotonic dystrophy
|
|
|
describe the genetics of myotonic dystrophy.
|
AD with anticipation (due to CTG repeat that gets longer with each generation) thus an earlier and more debilitating disease as generations go by… gene is AMP dependent myotonin protein kinase
|
|
|
what are the clinical features of myotonic distrophy?
|
slow progressive muscle wasting, myotonia, can have heart issues and smooth muscle issues, bladness, gonad deterioration
|
|
|
what are the pathological changes seen in myotonic dystrophy?
|
atrophy of type 1 fibers, hypertrophy of type 2 fibers (until late), numerous internal nuclei, ring fibers
|
|
|
what are the clinical manifestations of congenital myopathy?
|
hypotonia (floppy baby), decreaed DTR's and muscle bulk, non progressive, normal creatine kinase
|
|
|
what are three congenital myopathies?
|
central core disease, rod (nemaline) myopathy, central nuclear myopathy
|
|
|
what are the three inflammatory myopathies and what muscles do they each affect?
|
dermatomyositis and polymyositis affect the proximal muscles while inclusion body myositis affects the distal muscles.
|
|
|
what are the main clinical features of the infflammatory myopathies?
|
pain, weakness and increased creatine kinase
|
|
|
what is the pathogenesis of dermatomyositis?
|
complement mediated cytotoxic autoantibodies directed against muscle microvasculature.
|
|
|
what are the clinical manifestations of dermatomyositis?
|
skin will have heliotrope rash of upper eyelids and periorbital edema, gotron's sign of elevated red patches on knuckles, elbows, etc, some hyperpigmentation, increased risk of carcinoma esp in lungs
|
|
|
what are the pathological features of dermatomyositis?
|
BV issues, necrosis and phagocytosis of muscle fibers, inflammatory infiltrates, perifascicular atrophy
|
|
|
what is the pathogenesis of polymyositis and inclusion body myositis?
|
direct damage to muscles by cytotoxic T cells
|
|
|
what are the age ranges of ppl affected by dermatomyositis, polymyositis, and inclusion body myositis?
|
adults and children, over 20, over 50
|
|
|
what other organ disease has been associated with myostenia gravis, and in some cases removal of this organ and disease will cure myastenia gravis?
|
thymus: thymoma, thymic hyperplasia
|
|
|
what sex is normally affected in myastenia gravis?
|
2F:1M
|
|
|
what are the pathological manifestations of myastenia gravis?
|
muscle end plates will have simplifications of secondary folds, focal aggregates of lymphocytes, possible atrophy of type 2 fibers
|
|
|
what is the immunological difference between lambert eaton and myastenia gravis?
|
LE has ab against ca channels while myastenia gravis is ab against Ach receptors
|
|
|
what is the etiology of familial periodic paralysis?
|
abnormal potassium fluxes through the ion channels causing periodic paralysis… an ion channel myopathy
|
|
|
what are three metabolic diseases that effect muscle?
|
glycogen storage, lipid myopathy, rhabdomyolysis
|
|
|
describe a cavernous angioma.
|
large vascular spaces in the brain with fibrous walls, most are asymptomatic, few will bleed, seizures and defecits may occur
|
|
|
what are the causes of aneurysms?
|
developmental defects (berry), atherosclerosis, hypertension (charcot bouchard aneurysms), bacteria (mycotic), trauma
|
|
|
what is the pathogenesis of berry aneurysms?
|
failure of muscular coat to interdigitate across the bifurcation point of an artery
|
|
|
what are some features of atherosclerotic aneurysms?
|
usually in the major cerebral vessels and usually compresses or thromboses rather than rupturing
|
|
|
describe pathogenesis and results of a mycotic aneurism.
|
septic emboli usually from infected heart valve lodges in the branch of an artery leading to destruction of the wall and aneurysm. Intracerebral or subarachnoid hemorrhage, meningitis, or abscess may occur
|
|
|
what causes 60% of subarachnoid hemorrhages and what is the chronic sequelae?
|
aneurysm. Leads to blockage of CSF and communicating hydrocephalus
|
|
|
atherosclerosis in brain vessels can lead to what kind of aneurysm?
|
charcot bouchard microaneurysm
|
|
|
hypertensive hemorrhage can present as what three clincal scenarios?
|
basal ganglia (most common - weakness predominates), pontine (unconsciousness and fast death), and cerebellar (abrupt ataxia and occipital headache and vomiting, may compress medulla leading to death
|
|
|
what areas of the CNS and specefic cells of the CNS are vulnerable to ischemia and hypoxia?
|
cerebral cortex (esp hippo), depths of sulci, basal ganlia, purkinje cells. Neurons, oligodendros, epedymal, astrocytes, microglial cells
|
|
|
what are four types of infarcts?
|
arterial embolus, arterial thrombosis, lacunar infarcts, superior sagittal sinus thrombosis
|
|
|
describe the changing pathology of ischemia in the brain over time (long ass answer)?
|
first 6 hours nothing changes; w/I 2 days it will be soft and pale and swollen, necrosis and fragmented axons with neutros, RBC leak, endothelial swelling; 2-10 days marked edema, micro shows more macs, less neutros, more lymphos, reactive astros begin appearing, vascular proliferations; 10 days to weeks, progressive liquefication and cavitation, micro shows more macs and astros, macs begin to resolve as necrotic tissue is removed; weeks to months, cystic cavity with cobwebs of gliovascular strands, possibly dome macs and hemosiderin
|
|
|
epidural hemorrhage as a result of trauma is usually due to what?
|
fracture of the temporal bone thus injuring the middle meningeal artery
|
|
|
what is the usual pathogenesis of a subdural hematoma?
|
in older ppl, brain is smaller, so it moves easier and farther in the cranium thus the bridging veins can bust and bleed into the subdural space. Bleeding usually stops after 50ml leak bc the veins will be compressed at thrombosis will occur, thus this is not as dangerous as a epidural hemorrhage
|
|
|
what are the pathologic and clinical effects of subdural hematomas?
|
adjacent brain structures are compressed, but does not get into subarachnoid space, granulation tissue forms and an inner dura forming outer membrane, this can calcify or remain static or bleed again later. Clinical presentation includes headaches, contralateral weakness, seizures, cognitive dysfunction, all wi 2 days of bleed.
|
|
|
if a subarachnoid hemorrhage exists, what does this indicate?
|
probably not the only lesion
|
|
|
define a concussion.
|
head trauma causes functional paralysis of neurons in brain, neuro dysfunction for a bit, but no major structural damage
|
|
|
an acceleration injury to the head is what? What side of the brain will have a contussion?
|
something accelerates into the head resulting in a coup contusion, on the same side as the impact
|
|
|
this type of hemorrhage results from tearing of the brain.
|
parenchymal hemorrhage
|
|
|
healed bullet wounds and other open craniocerebral injuries result in what type of scar and may result in what else?
|
collagenous scar which may lead to seizure center
|
|
|
degenerative neurological diseases were not originally associated with inflammation; what new evidence refutes this?
|
the presence of many microglial cells in these lesions
|
|
|
what degenerative diseases predominantly effect the cortex?
|
alzheimers, dimentia with lewy bodies, creutzfield jacob disease, corticobasal degeneration, frontotemporal dimentias including pick's disease and chrom 17 linked frontotemporal dementia and parkinsonism.
|
|
|
what degenerative diseases predominantly effect the basal ganglia?
|
huntington's, parkinsons, dementia with lewy bodies, parkinson's plus syndromes including progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration and chrom 17 fronaltemporal lobe dimentia
|
|
|
what degenerative disorders primarily effect the spinal cord, cerebellum, and brainstem?
|
fredereich's ataxia, multiple system atrophy, inherited spinocerebellar atrophies.
|
|
|
what degenerative disorders affect upper and lower motor neurons primarily
|
ALS
|
|
|
what degenerative disease are characterized by neurofibrillary tangles microscopically?
|
alzheimers and progressive supranuclear palsy
|
|
|
what are the tauopathies? Why is alzheimers not a tauopathy?
|
chrom 17 frontotemporal dimentia, progressive nuclear palsy, corticobasal degeneration and Pick's disease all have mutated tau proteins while alzheimer's is an accumulation of normal protein
|
|
|
what is the biochemical pathogenesis of the amyloid plaques in alzheimers?
|
beta amyloid is a spliced product of the membrane protein amyloid precursor protein. APP is spliced abnormally in alzheimers thus leading to accumulation of beta amyloid, thus alzheimer's is an amyloidopathy.
|
|
|
aside from amyloid plaques, what other pathologic changes are noted in alzheimers?
|
tau containing neurites (swollen unmyelinated axons) and microglia in "mature" plaques, neurite like structures called neuropil are found outside of the plaques
|
|
|
what types of microscopic lesions are seen in the prionopathy creutzfeld jacob disease?
|
amyloid plaques simillar to alzheimers, without neurites typically, note the amyloid here is composed of prion protein, spongiform change is also characteristic and is a sever vacuolization of the neuropil
|
|
|
what is the name of eosinophilic cytoplasmic inclusions that have a target like appearance and what conditions do they indicate?
|
lewy bodies indicate parkinson's and dementia with lewy bodies
|
|
|
what protein is found in lewy bodies and what other lesions contain this protein?
|
alpha synuclein. In senile alzheimers plaques, inclusions in multiple system atrophy, also found in swollen neurites in the basal ganglia of parkinsons and dementia with lewy bodies
|
|
|
what are the synucleinopathies?
|
parkinsons, dementia with lewy bodies, and multiple system atrophy
|
|
|
what inclusion bodies result from an AD mutation with CAG repeats? Nuclear or cytoplasmic? What conditions do they indicate?
|
polyglutamate inclusiosn are intranuclear and intra cytoplasmic and are found in huntington's and many of the spinocerebellar atrophies
|
|
|
these round, tau containing, cytoplasmic inclusions characteristic of a specefic frontotemporal dementia are called what?
|
pick's bodies
|
|
|
balloon like neurons are seen in what neurodegenerative diseases?
|
tauopathies
|
|
|
granulovacuolar degeneration occurs in what conditions?
|
alzheimers and progressive supranuclear palsy
|
|
|
what neurodegenerative lesions may occur in normal elderly ppl without displaying symptoms (or many symptoms) of these lesions?
|
NFT's, granulovacuolar degeneration, and senile plaques without abnormal neurites, 10% of this population has lewy bodies in the LC
|
|
|
how is the number of functional synapses in pt's with alzhheimers determined?
|
staining for the protein synaptophysin
|
|
|
what is the pathological hallmark of huntington's disease?
|
atrophy within the caudate and putamen
|
|
|
opthalmoplegia, axial symptoms, and poor response to meds are all characteristics of what disorder?
|
progressive supranuclear palsy
|
|
|
variable presentations affecting different systems resulting in parkinsonism, ataxia, or autonomic responses are due to what?
|
multiple system atrophy
|
|
|
assymetrical symptoms, apraxia, alien hand, poor response to meds, and focal parietal atrophy are all characteristic of what?
|
corticobasal degeneration
|
|
|
discus alcoholic amblyopia.
|
dimness of vision esp in one eye, scotomata (blind or dark spot in visual field), loff of central optic nerve myelinated fibers.
|
|
|
what are some consequences of chronic alcoholism?
|
amblyopia, dementia, cerebellar degeneration, peripheral neuropathy, and marchiafava-bignami
|
|
|
what is the classic pathological finding of the brain when toxin is responsible for death?
|
edema and diffuse petechial hemorrhages
|
|
|
describe marchiafava-bignami syndrome.
|
malnourished italians who drank crude red wine, acute is convulsions leading to death and coma, subacute is dementia. Pathology reveals necrotic and areas of demyelination, esp the corpus callosum
|
|
|
describe the pathogenesis of central pontine myelinolysis.
|
lesion in the pons grey and white matter, throught to be related to anelectrolyte imbalance esp overly rapid correction of hyponatremia esp if pt is chronically hyponatremic
|
|
|
describe the clinical manifestations of central pontine myelinolysis.
|
locked in syndrome and quadriparesis
|
|
|
what are the clinical manifestations of methanol poisoning? Pathological?
|
drowsiness, headache, nausea, vomiting, ab pain, visual disturbances, dyspnea. Cerebral edema if fast death, if slow death necrosis of basal gangliaand internal capsule, optic disc degeneration may also occur
|
|
|
what are the clinical manifestations of ethylene glycol poisoning? Pathological?
|
CNS dysfunction - appear drunk, seizure, movement dos, stupor then coma. Calcium oxylate crystals in and around meninges and petechial hemorrhages.
|
|
|
in order of increasing hemoglobin saturation, what are the symptoms of CO poisoning?
|
headache and dizziness, impaired vision and hearing and mentation, coma and convulsions, death
|
|
|
what is a classic dx of CO poisoning post mortem?
|
cherry red skin
|
|
|
long term CO poisoning pathological effects include what?
|
necrosis of globus pallidus
|
|
|
what are the CNS toxicities of methotrexate?
|
if put in subarachnoid space will get chemical meningitis, transverse myelitis, meningoencephalitis. If given intraventricularly will have periventricular necrosis, delayed encephalopathies also may occur
|
|
|
what are the neurotoxicities of cisplain?
|
hearing loss and retinopathy, peripheral neuropathy (mainly sensory and autonomic)
|
|
|
what are the neurotoxicities of vincristine?
|
peripheral sensory, motor, and autonomic neuropathies
|
|
|
what are the toxicities of arsenic?
|
ab pain, renal failure, shock, encephalopathy, neuropathy, diarrhea, cerebral edema and petechiae
|
|
|
how does lead cause toxicity and what are its toxic effects?
|
inhibits oxphos, peripheral neurop (pure motor), encephalopathy, irritability, seizures, altered consciousness, increased intracranial pressure
|
|
|
what are the results of chronic inorganic mercury poisoning?
|
loss of granular cell layer and purkinje cell layer thus bizzare behavior and intention tremor and shakes
|
|
|
what are the results of organic mercury poisoning?
|
visual and hearing loss, incoordination, sensory impairment, cerebral and cerebellar atrophy
|
|
|
how does cyanide poison the CNS? What are acute causes of death and pathological changes?
|
poisons cellular respiration by binding to cytochrome oxidase, respiratory arrest, edema and subarachnoid hemmorrhage
|
|
|
toxicity of organochlorines leads to what?
|
muscle twitching, convulsion and coma
|
|
|
toxicity of organophosphates leads to what?
|
muscle weakness, GI and opthalmic issues, respiratory issues, seizures, tachy or bradycardia, note this is all due to its cholinesterase inhibiting activity
|
|
|
describe the clinical and pathological manifestations of neuronal ceroid lipofuscinosis.
|
psychomotor retardation or degeneration, blindness or seizures, atrophy of brain with ceroid in neurons or macs
|
|
|
describe the clinical and pathological manifestations of hepatic encephalopathy.
|
tremor, dysarthria, cerebellar ataxia, choreoathetosis, liver flap, delirium , seizures and coma; alzheimers type II astrocytes
|
|
|
what syndrome is associated with too much aspirin? Clinical and pathological manifestations?
|
reye's syndrome. Retching, edema of brain, young ppl, elevated ammonia, steatosis of liver, also coma, convulsions
|
|
|
thiamine (B1) defeciencies are most commonly seen in who?
|
alcoholics
|
|
|
what are the clinical and pathological manifestations of wernicke's encephalopathy (due to B1 def)?
|
confusion, ataxia, small pupils, opthalmoplegia, nystagmus, peripheral neurop, can lead to stupor/coma. Hemmorhages, edema, demyelination, chronic will have discolored mammies, gliosis
|
|
|
what is the cause and manifestations of korsakoff's psychosis?
|
retrograde amnesia, impaired ability to acquire new info, confabulation all due to thiamine def.
|
|
|
what are the issues seen with thiamine defeciency (B1)?
|
peripheral neurop, korsakoff's psychosis, wernicke's encephalopathy
|
|
|
What changes are seen in B12 (cobalamin) def?
|
subacute combined degeneration AKA cord disease (mainly loss of dorsal columns, also motor issues), mental function changes (megaloblastic madness due to megaloblastic anemia), peripheral neurop
|
|
|
describe a parkinsonian tremor
|
resting, slow, distal involvement
|
|
|
describe a familial essential tremor.
|
intention and postural, fast, proximal involvement
|
|
|
describe a hyperadrenergic tremor.
|
intention and postural, fast, proximal involvement
|
|
|
describe a cerebellar tremor.
|
intension, slow, proximal involvement
|
|
|
what seems to account for the hypokinetic features of the akinetic rigid syndrome as well as hyperkinesia
|
shifts in balance between activity in direct and indirect basal ganglia pathways
|
|
|
activation of the direct pathway in basal ganglia tends to do what?
|
disinhibit thalamocortical activity
|
|
|
activation of the indirect pathway in basal ganglia tends to do what?
|
inhibit thalamocortical activity
|
|
|
when is it appropriate to use the term dementia with lewy bodies?
|
when dementia occurs within one year of onset of parkinsonism
|
|
|
parkinsons affects what sex more often?
|
2M:1W
|
|
|
of the known genetic mutations for parkinsons, what do they all have in common?
|
all of the proteins deal with ubiquinilization
|
|
|
how might mitochondria affect the etiology of parkinsons?
|
oxidative stress and apoptosis may have something to do with destruction of the substantia nigra, mitochondria are central in each of these processes
|
|
|
treatments for parkinsons?
|
LDOPA, MAOI-B, COMT inhibiter, anticholinergics, pallidotomy, electrical stimulation of the subthalamic nu
|
|
|
in general the younger the onset of huntingtons, then …
|
more bradykinesia, rigidity, greater severity of dementia, and less prominent the hyperkinetic features
|
|
|
this is an AR disorder of copper metabolism associated with involuntary movements, mental deterioration, and personality change.
|
wilson's disease or hepatolenticular degeneration
|
|
|
what are the major pathological features of wilson's disease?
|
custic degeneration of the basal ganglia and assymptomatic cirrhosis of the liver
|
|
|
kayser fleischer rings are what and are associated with what?
|
disposition of copper around the limbus of the cornea, associated with wilson's disease.
|
|
|
what exams are used to make a diagnosis of wilson's disease?
|
serum ceruloplasmin levels, urinary copper levels, and the slit lamp exam (SC levels are low, urine copper is high)
|
|
|
treatment for wilsons?
|
penicillamine and a low copper diet
|
|
|
what are four examples of primary dystonia?
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idiopathic torsion dystonia (young onset and severe), spasmodic torticollis (wry neck), blepharospasm (involuntary eyelid closure), and writer's cramp
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how is tourette's syndrome distinguished from multiple motor tics?
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by the presence of one or more vocal tics
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what other disorders is tourette's associated with; how is it treated?
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OCD, ADHD, neuroleptics if necessary
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what drugs can induce dyskinesias?
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LDOPA and neuroleptics
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where are most CNS tumors located in children? In adults?
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infratentorially. Supratentorially
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adult astrocytomas are found where? Children?
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cerebral hemis, pons or cerebellum
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describe diffuse (less malignant) astrocytomas. Anaplastic (more malignant) astrocytomas.
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may be seen as only firm areas, paler than usual. Lareger with pale or soft areas and hemorrhages
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what is the prognosis of diffuse atrocytomas? Anaplastic?
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5+ years, 18-36 months
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describe a pilocytic astrocytoma.
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simillar to hamartoma, gross- usually in cerebellum and looks like a nodule in wall of a large cyst. Histopath: looks like diffuse astrocytoma with bipolar cells and rosenthal fiber microcysts
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describe glioblastoma multiformes.
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50% of gliomas, males predominate ~ 50 years old, cerebral hemis, firm to soft white or yellow with areas of necrosis and hemorrhage, histo shows highly anaplastic tumor pleomorphic and mitotic cells, endothelial proliferation, prognosis is very poor (8-10 mos)
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descrbe oligodendrogliomas.
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5-15% of gliomas, seen in cerebral white matter, gross shows gray/pink fleshy, soft, with possible cysts, hemorrhages, and calcifications; histo shows fried egg cells, calcification, some will have astrocytoma that could shorten the prognosis; prognosis is 5-10yrs
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describe ependyomas
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only 5% of gliomas, most are in posterior fossa thus pt's present with symptoms of hydrocephalus, in adults they are frequently found in the spinal cord and can present with cord compression symptoms; usually in children and young adults (those with neurofibromatosis type 2); gross is gray and fleshy near ventricle, cysts in the cerebral hemis; histo shows epithelial cells making canals, papillary branches, and cellular shapes (ependymal rosettes); prognosis is similar to astrocytomas, average survival is about 4 years
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describe the choroid plexus papilloma.
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1-2% of intracranial neoplasms; young males; benign; symptoms are usually from hemorrhage into papilloma or from tumor causing hydroceph
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describe cerebral neuroblastoma.
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usually in frontal lobes, below age of 10, soft white to pink red masses with small cells with scant cytoplasm, usually occurs in adrenal glands
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describe a medulloblastoma
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arise in cerebellum, most in children and midline, some in adults and are lateral, males, midline can cause fourth ventricle obstruction, histo shows carrot shaped nuclei and little cytoplasm with mitosis; prognosis with surgery, craniospinal irridation and chemo will give 50-75% ten year survival.
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what are the two CNS tumors of neuronal origin?
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gangliocytoma and ganglioglioma
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describe CNS hemangioblastomas.
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lateral lobes of the cerebellu; cystic, red, hemorrhagic; histo:small vascular channels lined by endotehlium, foamy histiocyte like cells; associated with hemangioendothelioblastoma of the eye AKA von hippel-lindau; prognosis is good if entire tumor is surgically removed.
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describe meningiomas.
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adults, females; gross gray white tumors attached to dura, does not invade the brain, can compress it, calcification, may penetrate the bone; histo: syncytial or meningotheliomatous, fibrous, transitional, psammomatous, hemangiopericytoma (more aggressive); rare malignant transformation
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describe primary lymphomas of the CNS.
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rare, B lymphs, immunocompromised pts; gross- diffuse infiltration or multiple nodules; histo- large cell lymphoma, infiltrates and expands BV walls; can go to dura; prognosis is poor ~18mos
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describe metastatic tumors of the CNS
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majority from bronchiogenic or breast carcinomas, can hit the meninges and look like meningitis; gross - spherical canters at gray white junction and sharply demarcated; hemorrhagic and/or necrotic; multiple usually; histo of the primary tumor
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this tumor can be intraccranial, spinal, or peripheral; if it is intercranial, it usually affects CN VIII.
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schwanoma
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describe the clinical findings with schwannoma
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ringing in the ear if on CN VIII or sensory involvement if on cord
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what type of spinal cord tumors are usually metastatic?
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extradural
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what are three common intradural, extramedullary tumors of the spine?
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neurofibromas, meningiomas, schwannomas
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what are three common peripheral nerve tumors?
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schwannoma, neurofibroma, malignant peripheral sheath tumor
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slowly progressive dementia indicates what? Stepwise progression? Fluctuating confusion and early hallucinations?
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alzheimers, vascular dementia, dementia with lewy bodies
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this group of patients often complain of memory problems. The other group often do not know they are having memroy problems.
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depressed patients, demented patients
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what are the metabolic and vitamin deficiency disorders that may cause dementias?
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hypothyroidism, hepatic encephalopathy, renal failure, cushings, wilsons, B12 def, thiamine def, hypercalcemia, hyponatremia, CO2 narcosis
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what infections can cause dementia?
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syph, viral encephalitis, TB meningitis, cryptococcal meningitis, creutzfieldt-jacob
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what non infectious inflammatory diseases can cause dimentia?
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lupus, vasculitidities with CNS involvement, sarcoidosis, MS
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what features with dementia make a diagnosis of alzheimers unlikely?
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sudden onset, focal neurological findings, or incoordination, gait disturbances, seizures early on in the course
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describe genetic risk factors for alzheimers.
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mutations in the amyloid precursor protein and presenilins are risk factors for early onset. ApoE4 is a risk factor for both early and late onset
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what are two symptomatic treatments for alzheimers?
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cholinesterase inhibitors and glutamate (NMDA calcium channel) inhibitors
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what is the caveat in terms of ischemic changes and dementia?
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a patient can have white matter ischemic changes without other features that would suggest vascular dementia, they may have no stroke and no dimentia or another type of dimentia
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if dementia develops within one year of onset of parkinsonism, what is the diagnosis?
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dementia with lewy bodies.
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what types of neuroleptics should be treated with DLB and why?
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atypicals like clozapine bc the typicals will worsen the parkinsons
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this dementia presents as changes in drive, personality, executive functions, and language (if left hemi is affected) as well as frontal release signs.
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picks disease.
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how would one distinguish frontotemporal dementia from parkinsons and why?
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by pathology, alzheimers may present with same symptoms as frontotemporal dementia
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what are the clinical signs and symptoms of normal pressure hydrocephalus?
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cognitive impairment, urinary incontinence, and a frontal lobe gait disorder in the settings of communicating hydrocephalus.
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what are the types of primary ischemic stroke?
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thrombotic (most common), embolic, sino-venous thrombosis, vascular dissection causing thrombus, vasculopathy (vasculitis or moyamoya)
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primary intracerebral hemorrhages are commonly associated with what? What not so commonly?
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primary hypertension; bleeding diathesis, cerebral amyloid angiopathy, vascular anomaly or neoplasm
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what are common causes of subarachnoid hemorrhages?
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AVM and cerebral aneurysm rupture
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what are the symotoms of carotid distribution TIA?
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amaurosis fugax (transient monocular blindness), hemiparesis, hemisensory deficit, speech disturbance if dominant hemi is involved.
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what are the symptoms of the vertebrobasilar distribution TIA?
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cross findings (left face, right body), bilateral vision loss, diplopia, ataxia, dysarthria, quadriparesis, can have vertigo and syncope
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what are the warnings of an aneurysm rupture? Tests?
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atypical or severe headache with or without syncope and stiff neck. CT may not pick it up, so do spinal tap
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during stroke, an elevated temperature raises questions about what?
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infective endocarditis
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an irregular pulse and stroke may indicate what?
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atrial fibrilation
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discuss treating hypertension during an ischemic stroke? Hemorrhagic stroke?
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it may be protective in ischemic stroke but needs to be treated in hemorrhagic stroke
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what should be done when a patient presents with symptoms of stroke?
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asses the fundi to look for evidence of intracranial pressure or retinal emboli, auscultate for carotid bruit, auscultate the heart listening for an irregular heart rhythm or murmur, look for systemic disease and connective tissue disorder, neuro exam
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what will CT help you evaluate in pt presenting with stroke?
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if it is hemorrhagic you will see blood, if it looks normal the stroke is probably ischemica and TPA will likely be useful.
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sentinel headache is the most common primary presenting feature of what?
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aneurymal subarachnoid bleed.
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what pathologies are often associated with severe headache and stroke?
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cerebral vasculitis, AVM, temporal arteritis, meningitis with vessel involvement, cerebrovascular dissection from trauma
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when evaluating stroke in the post partum period, what is the likely diagnosis?
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venous thrombosis
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what type of severe headache is more common in women? Men?
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migraine; cluster
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describe cluster headaches.
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sharp, burning and or throbbing pain in the periorbital region associated with red eye, ipsilateral nasal congestion and horner's syndrome. It tends to be episodic or clustering
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what are the prophylactic treatments for migraines
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beta blockers, calcium channel blockers, tricyclics, anticonvulsants, B12
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describe migraine pathogenesis.
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felt to be related to dysfunction of brainstem or thalamic nuclei involved in sensory pathways through modulation of craniovascular afferents
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describe headache due to brain tumors.
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chronic nagging headache that can be relieved with mild analgesics, but worsens with time and is often associated with some focal neurologic defect
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what are the types of primary ischemic stroke?
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thrombotic (most common), embolic, sino-venous thrombosis, vascular dissection causing thrombus, vasculopathy (vasculitis or moyamoya)
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primary intracerebral hemorrhages are commonly associated with what? What not so commonly?
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primary hypertension; bleeding diathesis, cerebral amyloid angiopathy, vascular anomaly or neoplasm
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what are common causes of subarachnoid hemorrhages?
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AVM and cerebral aneurysm rupture
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what are the symotoms of carotid distribution TIA?
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amaurosis fugax (transient monocular blindness), hemiparesis, hemisensory deficit, speech disturbance if dominant hemi is involved.
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what are the symptoms of the vertebrobasilar distribution TIA?
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cross findings (left face, right body), bilateral vision loss, diplopia, ataxia, dysarthria, quadriparesis, can have vertigo and syncope
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what are the warnings of an aneurysm rupture? Tests?
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atypical or severe headache with or without syncope and stiff neck. CT may not pick it up, so do spinal tap
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during stroke, an elevated temperature raises questions about what?
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infective endocarditis
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an irregular pulse and stroke may indicate what?
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atrial fibrilation
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discuss treating hypertension during an ischemic stroke? Hemorrhagic stroke?
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it may be protective in ischemic stroke but needs to be treated in hemorrhagic stroke
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what should be done when a patient presents with symptoms of stroke?
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asses the fundi to look for evidence of intracranial pressure or retinal emboli, auscultate for carotid bruit, auscultate the heart listening for an irregular heart rhythm or murmur, look for systemic disease and connective tissue disorder, neuro exam
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what will CT help you evaluate in pt presenting with stroke?
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if it is hemorrhagic you will see blood, if it looks normal the stroke is probably ischemica and TPA will likely be useful.
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sentinel headache is the most common primary presenting feature of what?
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aneurymal subarachnoid bleed.
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what pathologies are often associated with severe headache and stroke?
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cerebral vasculitis, AVM, temporal arteritis, meningitis with vessel involvement, cerebrovascular dissection from trauma
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when evaluating stroke in the post partum period, what is the likely diagnosis?
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venous thrombosis
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what type of severe headache is more common in women? Men?
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migraine; cluster
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describe cluster headaches.
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sharp, burning and or throbbing pain in the periorbital region associated with red eye, ipsilateral nasal congestion and horner's syndrome. It tends to be episodic or clustering
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what are the prophylactic treatments for migraines
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beta blockers, calcium channel blockers, tricyclics, anticonvulsants, B12
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describe migraine pathogenesis.
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felt to be related to dysfunction of brainstem or thalamic nuclei involved in sensory pathways through modulation of craniovascular afferents
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describe headache due to brain tumors.
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chronic nagging headache that can be relieved with mild analgesics, but worsens with time and is often associated with some focal neurologic defect
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