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112 Cards in this Set
- Front
- Back
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What is Dementia?
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Dec Cognitive Ability
Dec memory Dec Fxn Intact consciousness |
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4 Main Causes of Dementia?
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Alzheimer's disease
Pick's disease Lewy Body dementia Creutzfeldt-Jakob disease |
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Pathogenesis of Alzheimer's?
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Widespread, accelerated cortical atrophy w/ dec ACh
Senile plaques w/ beta-amyloid core Neurofibrillary tangles (tau protein) |
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Who is at increased risk of developing Alz's?
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Down syndrome pts
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How much of Alz's is familial?
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10%
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Genetics of familial alzheimer's?
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Genes on 1, 14, 19
APOE4 allele Auto Dom and Ch 21 (p-App gene) |
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Complication w/ alzheimer's?
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may cause amyloid angiopathy--->intracranial hemorrhage
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Sx's of Pick's Disease?
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Dementia
Aphasia Parkinsonian aspects |
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Morphological changes w/ Pick's?
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Pick bodies = intracellular, aggregated tau protein
Frontotemporal lobe atrophy |
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What is spared in Pick's?
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Parietal Lobe
Posterior 2/3 of superior temporal gyrus |
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What is Lewy Body Dementia?
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Parkinsonism w/ dementia and hallucinations
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What causes Lewy Body Dementia?
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alpha-synuclein defect
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What is Creutzfeldt-Jakob disease?
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rapidly progressive (wks-->mos) dementia w/ myoclonus, spongiform ctx
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Associated CJD cause?
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prions
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2nd most common cause of dementia?
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Multiple Infarcts
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Other causes of dementia?
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Syphilis
HIV B12 def Wilson's |
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What is Multiple Sclerosis?
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Autoimmune inflammation and demyelination of CNS (brain and SC)
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How do MS pts present?
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Optic neuritis (sudden loss of vision)
MLF syndrome (internuclear ophthalmoplegia) Hemiparesis Hemisensory sx's Bladder/bowel incontinence |
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Course of MS?
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relapse remitting
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Who does MS normally affect?
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Whites
Women 20s and 30s |
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Clinical Findings in MS pts?
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Inc IgG (protein) in CSF
Periventricular plaques (oligodendrocyte loss and reactive gliosis) w/ preserved axons |
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What are Charcot's classic sx's of MS?
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Scanning Speech
Intention tremor Incontinence Internuclear Ophthalmoplegia Nystagmus |
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Rx for MS?
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beta-Interferon or immunosuppressant therapy
Symptomatic Rx for incontinence, spasticity, pain |
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What is Guillain-Barre syndrome?
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Inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (sensory effect less severe than motor), causing symmetric, ascending muscle weakness beginning in distal lower extremities
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What do some Guillain-Barre pts experience?
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50% get facial paralysis
Autonomic fxn can be severely affected---> cardiac irregularities, HTN or hyoptension |
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Prognosis w/ Guillain-Barre?
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almost all pts survive
majority recover completely after weeks to months |
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Possible etiologic association?
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Infections---> autoimmune attack of peripheral myelin dur to molecular mimicry, inoculations, and stress
no definite link to pathogens |
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Clinical Findings w/ Guillain-Barre
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Inc CSF protein w/ normal cell count
Inc Protein---> Papilledema |
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Rx for Guillain-Barre?
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Resp support is critical
Plasmapharesis IV immuno globulins |
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Other de- or dys-myelinating diseases?
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Progressive Multifocal Leukoencephalopathy (PML)
Acute Disseminated (postinfectious) Encephalomyelitis Metachromatic leukodystrophy Charcot-Marie-Tooth Disease |
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What is PML?
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Demyelination of CNS due to destruction of oligodendrocytes
Associated w/ JC virus and seen in 2-4% of AIDS pts |
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Px for PML?
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rapidly progressive
usually fatal |
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What is Acute disseminated encephalomyelitis?
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multifocal perivenular inflammation and demyelination after infection (chicken pox, measles) or certain vaccinations (rabies, smallpox)
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What is Metachromatic Leukodystrophy? inheritance? pathogenesis?
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Auto Rec lysosomal storage disease, most commonly due to arylsulfatase A def.
Build up of sulfatides--->impaired production of myelin sheath |
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What is Charcot-Marie-Tooth disease?
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Group of progressive, hereditary nerve disorders related to defective production of proteins involved in structure and fxn of peripheral nerves or myelin sheath
aka hereditary motor and sensory neuropathy (HMSN) |
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Different between Partial and generalized seizures?
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partial = 1 area of brain
generalized = diffuse |
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Most common origin of partial seizures?
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Mesial Temporal Lobe
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Types of Partial Seizures?
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Simple Partial (consciousness intact)
Complex Partial (impaired consciousness) |
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Types of Generalized Seizures?
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Absence (petit mal)-blank stare
Myoclonic - quick, repetitive jerks Tonic-Clonic (grand mal) - alternating stiffening and movement Tonic - stiffening Atonic - drop seizures, mistaken for fainting |
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What is epilepsy?
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Disorder of recurrent seizures (not febrile)
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Causes of seizures in kids?
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Genetic
infection (febrile) trauma congenital metabolic |
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Causes of seizures in adults?
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tumors
trauma stroke infection |
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Causes of seizures in elderly?
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Stroke
tumor trauma metabolic infection |
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What is a migraine?
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4-72 hours of unilateral, pulsating pain w/
Nausea Photophobia Phonophobia +/- aura before HA (visual, sensory, or speech sx's) |
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Cause of migraines?
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Irritation of CN V and release of Substance P, CGRP, and vasoactive peptides
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What is a Tension HA?
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> 30 minutes of bilateral steady pain
No photo or phono-phobia No Aura |
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What is a Cluster HA?
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Repetitive brief HA's
Unilateral, periorbital pain Associated w/: ipsilateral lacrimation Rhinorrhea Horner's syndrome |
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Who are cluster HA's common in?
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MALES
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What is Vertigo?
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the illusion of movement, not to be confused w/ dizziness or lightheadedness
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Types of Vertio?
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peripheral
central |
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Which type is more common?
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peripheral
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Etiology of Peripheral Vertigo?
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Inner ear etiology:
semicircular canal debris vestibular nerve infection Meniere's disease |
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Testing for Peripheral Vertigo?
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Positional testing--->delayed horizontal nystagmus
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Etiology of Central Vertigo?
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BS or Cerebellar lesion (vestibular nuclei, posterior fossa tumor)
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Testing for Central Vertigo?
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Positional Testing--->immediate nystagmus in any direction and can change directions
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4 Neurocutaneous Disorders?
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Sturge-Weber Syndrome
Tuberous Sclerosis Neurofibromatosis Type 1 (von recklinghausen) von Hippel-Lindau Disease |
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What is Sturge-Weber Syndrome?
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Congenital disorder w/ port-wine stains and ipsilateral leptomeningeal angioma
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Possible Sx's w/ Sturge-Weber?
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Glaucoma
Seizures Hemiparesis MR |
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Characteristics of Tuberous Sclerosis?
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Hamartomas in:
CNS Skin organs Cardiac Rhabdomyoma Renal Angiomyolipoma Subependymal Giant Cell Astrocytoma MR Seizures Hypopigmented "ash leaf spots" Sebaceous adenoma Shagreen patch |
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Inheritance of Tuberous Sclerosis?
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Auto Dom
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Neurofibromatosis Type 1 Sx's?
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Cafe-au-lait spots
Lisch nodules Neurofibromas on skin |
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What are lisch nodules?
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pigmented iris hamartomas
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Inheritance of NF type 1?
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Auto Dom
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Sx's of von Hippel Lindau Disease
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Cavernous Hemangiomas in skin, mucosa, and organs
Renal Cell Carcinoma Hemangioblastoma in retina, BS, and Cerebellum |
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Inheritance of von Hippel Lindau Disease
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Auto Dom
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What % of brain tumors are primary?
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50%
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Primary Brain Tumor location based on age?
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Adults are supratentorial
Children are infratentorial |
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Most common primary brain tumor?
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Glioblastoma multiforme (grade IV astrocytoma)
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Px for Glioblastoma multiforme?
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Bad, < 1 year life expectancy
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Location for Glioblastoma multiforme
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Cerebral hemispheres
Can cross corpus callosum (butterfly glioma) |
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Markers for Glioblastoma multiforme?
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Stain Astrocytes for GFAP
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Histo of Glioblastoma multiforme
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"Pseudopalisading" pleomorphic tumor cells
Border central areas of necrosis and hemorrhage |
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2nd most common Primary Brain Tumor?
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Meningioma
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common locations for Meningiomas?
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Convexities of hemispheres and parasagittal region
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Origin of meningiomas?
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arachnoid cells external to brain
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Px for meningiomas/
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resectable
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Histo of Meningiomas?
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Spindles cells concentrically arranged in a whorled pattern
Psammoma bodies (laminated calcifications) |
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3rd most common brain tumor?
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Schwannoma
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Common Location for Schwannoma?
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Localized to CN VIII-->acoustic schwannoma
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Rx for Schwannoma
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resectable
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Disease association w/ Schwannoma?
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Bilateral Schwannomas seen in NF Type 2
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Where are Oligodendrogliomas normally found?
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most often in Frontal lobes
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Histo of Oligodendrogliomas?
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Chicken-wire capillary pattern
Fried-egg appearance of oligodendrocytes (who are often calcified in this tumor) |
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Most common type of Pituitary Adenoma?
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prolactinoma
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Presenting sx's for Pituitary Adenoma?
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Bitemporal hemianopia
Hyper or Hypo-pituitarism |
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5 Primary Brain Tumors of Childhood (maybe peak incidence)?
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Pilocytic (low-grade) Astrocytoma
Medulloblastoma Ependymoma Hemangioblastoma Craniopharyngioma |
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Location and Appearance of Pilocytic astrocytoma?
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Well circumscribed
Most often in posterior fossa May be supratentorial |
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Px and Marker for Pilocytic astrocytoma
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Benign
Good Px GFAP positive |
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Histo of Pilocytic astrocytoma?
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Rosenthal fibers
eosinophilic, corkscrew fibers |
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What is a Medulloblastoma?
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Highly malignant cerebellar tumor
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Origin of Medulloblastoma?
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Form of Primitive Neuroectodermal Tumor (PNET)
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possible complication w/ Medulloblastoma
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Can compress 4th ventricle---> hydrocephalus
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Histo of Medulloblastoma
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Rosettes or perivascular pseudorosette pattern
Radiosensitive |
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Most common location of Ependyoma?>
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4th ventricle (hydrocephalus)
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Px w. Ependyoma
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poor
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Histo of Ependyoma
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perivascular pseudorosettes
Rod shaped blepharoplasts (basal ciliary bodies) found near nucleus |
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Usual location w/ Hemangioblastoma?
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Most often cerebellar
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Disease association w/ Hemangioblastoma
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von Hippel-Lindau when found w/ retinal angiomas
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Possible complication w/ Hemangioblastoma
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can produce EPO---> secondary polycythemia
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histo of Hemangioblastoma
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foamy cells and high vascularity
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What is a craniopharyngioma?
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Benign childhood tumor often confused w/ pituitary adenoma (can also cause bitemporal hemiopia)
most common childhood supratentorial tumor |
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Origin of craniopharyngioma?
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Rathke's pouch
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common morphological change for craniopharyngioma?
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calcification is common (tooth enamel like)
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4 Types of Herniation Syndromes?
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1. Cingulate herniation under falx cerebri
2. Downward transtentorial (central) herniation 3. Uncal Herniation 4. Cerebellar tonsillar herniation into foramen magnum |
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Effects of Cingulate herniation?
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Can compress anterior cerebral artery
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General worst-case effect of herniations?
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Coma and Death from compression of BS
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Clinical Sx's of Uncal Herniation?
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Ipsilateral dilated pupil/ptosis
Contralateral homonymous hemianopia Ipsilateral paresis Duret Hemorrhages |
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What are Duret Hemorrhages?
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Paramedian artery rupture
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What causes the Ipsilateral mydriasis/ptosis?
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Stretching of CN III (innervates levator palpebrae)
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What causes the contralateral homonymous hemianopia?
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Compression of ipsilateral posterior cerebral artery
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What causes the ipsilateral paresis?
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Compression of contralateral crus cerebri (kernohan's notch)
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What causes the Duret Hemorrhages?
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Caudal displacement of brain stem
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