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51 Cards in this Set
- Front
- Back
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Essential Tremor
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6-12 Hz tremor primarily affecing arms/hands
- worsens as time goes on - increases when anxious - accentuated with intentional movement - eventually can progress to tremor of head and voice |
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Poliomyelitis
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poliovirus - a PICORNAvirus
- FECAL-ORAL - replicates in oropharynx and small intestine before spreading through bloodstream to CNS - destruction of ANTERIOR HORN (Ventral Horn) of spinal cord --> LMN destruction Sx's: - malaise, ha, fever, nausea, abd pain, sore throat (flu-like_ - signs of LMN lesions - muscle weakness, atrophy, FASCICULATIONS, fibrillation, hyporeflexia findings: - CSF w/lymphocytic pleocytosis with slight elevation of protein (no change in CSF glucose) - virus recovered from stool or throat Tx: Pleconanl - inhibits penetration of picornavirus into cell |
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MS
- epi - path - CSF findings - course of illness - presentation - Tx |
- increasing prevalence with increasing distance from equator
- women in 20's and 30s - HLA DR2 - mainly a LMN problem - periventricular plaques (areas of OLIGODENDROCYTE LOSS and REACTIVE GLIOSIS!!!) - inflammation and myelin breakdown with hyperplasia and hypertrophy of astrocytes such that plaques become gliotic scar TYPE IV HSTY! Th1 activates Macrophage to cause demyelination - preservation of axons Labs - increased protein IgG in CSF - increased leukocytes in CSF - increased proteins in CSF - increased myelin basic protein - normal glucose - relapsing-remitting course - pts present with OPTIC NEURITIS MLF Syndrome (internuclear opthalmoplegia) HEMIPARESIS HEMISENSORY Sx's Bladder/bowel incontinence NYstagmus Intention Tremor!!! Scanning speech Tx: IFN-B or immunosupressant tx |
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Progressive multifocal leukoencephalopathy
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PML
- associated with JC virus (a papovavirus) - seen in 2-4% of AIDS pts (reactivation of latent viral infection) - reactivation of latent viral infection - inclusions in oligodendrocytes - abnormal myelin production - abnormal T2 signal in white matter Cx: hemiparesis, ataxia, homonymous hemianopsia, abnormal white matter - GIANT OLIGODENDROCYTES WITH EOSINOPHILIC INCLUSIONS |
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Acute disseminated (post-infectious) encephalomyelitis
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another form of abnormal myelination disease state
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Metachormatic leukodystrophy
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AR lysosomal storage disease
- arylsulfatase A deficiency |
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Guillan Barre syndrome
(acute idiopathic polyneuritis) |
inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (sensory less severe than motor)
- causes symmetric ascending muscle weakness beginning in distal lower extremities - facial paralysis in 50% of cases - autonomic function may be severely affected (cardiac irregularities, HTN, or HYPOtension) - almost all patients survive - most recover completely after weeks-months - Findings: elevated CSF protein normal cell count elevated protein --> papilledema associated with infections --> autoimmune attack of peripheral myelin due to MOLECULAR MIMICRY (eg - C.jejuni or herpesvirus infection), inoculations, and stress, but no definitive link to pathogens ***Respiratory support is critical until recovery - additional treatment - plasmapheresis, IV Igs |
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Charcot Marie Tooth Disease
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most common inherited neuropathy
- damage to deep peroneal nerve - wasting of muscles in anterior compartment of lower limb - child falls (legs have "inverted bottle" appearance) - foot drop - flat feet |
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subacute sclerosing panencephalitis
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direct infection of oligodendrocytes
- associated with Rueobella (measles) virus |
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Central pontine myelinolysis
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due to rapid IV correction of hyponatremia
- seen in alcoholics with hyponatremia |
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Partial seizures
- simple - complex |
SIMPLE PARTIAL
- consciousness intact - motor, sensory, autonomic, psychic COMPLEX PARTIAL - impaired consciousness NOTE: Partial seizures can secondarily generalize |
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Generalized Seizures
- absence - myoclonic - tonic clonic - tonic - atonic |
Generalized seizures are diffuse
Absence (petit mal) - blank stare - - treat with ETHOSUXIMIDE Myoclonic - quick, repetitive jerks - Tx with Valproic acid Tonic-clonic (grand mal) - Tx with phenytoin, carbamazepine, valproic acid Tonic - stiffening Atonic - "drop" seizures - falls to floor, commonly mistaken for fainting Status Epilepticus - Tx: Benzodiazepines in acute setting - Prophylaxis with Phenytoin |
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Epilepsy
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a disorder of recurrent sizures (febrile seizures are not epilepsy
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Causes of seizures by age:
- children - adults - elderly |
children:
- genetic - infection - trauma - congenital - metabolic adults - tumors - trauma - stroke - infection Elderly - stroke - tumor - trauma - metabolic - infection |
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Epidural hematoma
- and what it can lead to |
rupture of middle meningeal artery (branchi of maxillary)
- often secondary to fracture of temporal bone - lucid interval - CT: "BICONCAVE DISK" not crossing suture lines *** can lead to UNCAL HERNIATION: medial temoral lobe (especially uncus) herniates thorough tentorial notch - CN III compression - middle cerebellar penduncle compression --> hemiplegia because of impingement on corticospinal tracts |
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subdural hematoma
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rupture of BRIDGING VEINS
- venous bleeding (less pressure) - delayed onset of sx's - seen in elderly individuals - alcoholics - blunt trauma - shaken baby predisposing factors - brain atrophy - shaking - whiplash CT: CRESCENT SHAPED HEMORRHAGE THAT CROSSES SUTURE LINES |
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Subarachnoid hemorrhage
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ruptured aneurysm (usually berry aneurysm)
- or ArterioVenous Malformation!!! - WHOL!!! - bloody or yellow (xanthochromic) spinal tap |
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parenchymal hematoma
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due to
- HTN - amyloid angiopathy - DM - tumor |
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Berry aneurysms
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occur at bifurcation in circle of willis
- most common site is bifurcation of ACA - rupture (most common complication) leads to hemorrhage/subarachnoid hemorrhage - associated with adult polycystic kidney disease Ehlers-Danlos syndrome Marfan's syndrome other risk factors - age - HTN - smoking - race (higher risk in blacks) charcot-Bouchard microaneurysms - associated wtih chronic HTN - affects small vessels - often in lenticulostriate arteries |
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layers passed through during a lumbar puncture
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skin
superficial fascia (camper's fascia, scarpa's fascia) deep fascia supraspinous ligament interspinous ligament interlaminar space epidural space dura arachnoid subarachnoid |
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hydrocephalus
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normal pressure (communicating) hydrocephalus
- enlarged ventricles with nl opening pressure on lubar puncture - classic triad: Dementia, Gait problems, Urinary incontinence - due to impaired absorption of CSF by arachnoid granulations Obstructive (noncommunicating) hydrocephalus - caused by structural blockage of CSF circulation within the ventricular system (eg - stenosis of Aqueduct of Sylvius) - dev from mesencephalon of midbrain |
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Sturge-Weber syndrome
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- congenital disorder w/ port-wine stains
- ipsilateral leptomeningeal angioma Cx: - glaucoma - seizures - hemiparesis - mental retardation |
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Tuberous sclerosis
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AD
- hamartomas (astrocyte proliferations) in CNS, skin, organs - cardiac rhabdomyoma - renal angiomyolipoma - subependymal giant cell astrocytoma - MR - seizures - ash leaf spots - subcutaneous adenoma (facial angiofibromata, hypopigmented patches of skin) - shagreen patch |
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Neurofibromatosis 1
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AD
- cafe-au-lait spots - Lisch nodules (iris - pigmented hamartomas) - neurofibromas in skin - long arm CHROM 17! NF2 - bilateral acoustic neuroma - NF2 gene on chromosome 22 - juvenile cataracts NF - also associated with Wilm's tumor and kyphoscoliosis |
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von Hippel Lindau disease
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AD
- disorder with cavernous hemangiomas in skin, mucosa, organs - RCC, hemangioblastoma in retina, brain stem, cerebellum |
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Empty sella syndrome
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radiographically, pituitary is missing from sella turcica
- cuased by herniation of arachnoid through diaphragm sella - most patients have enough remaining pituitary to prevent hypopituitarism |
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ring enhancing lesion on CT
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= brain abscess often
- 60% are related to otitis media - cerebellar abscesses are specifically associated with otitis media - sinusitis and otitis media can cause frontal and temporal lobe abscesses |
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effx of tumor filling 4th ventricle
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bilateral papilledema (swelling of optic disk due to increased ICP)
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Primary brain tumors
- likelihood of metastases - cause of presentation - location of adult tumors - location of childhood tumors |
- clinical presentation is due to mass effect (seizures, dementia, focal lesions)
- primary brain tumors rarely undergo mets - majority of adult primary tumors are SUPRATENTORIAL - majoirty of childhood primary tumors are INFRATENTORIAL NOTE: 1/2 of adult brain tumors are mets - usually presenting at the gray-white jtn |
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GBM (grade IV astrocytoma)
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most common primary brain tumor
- most grave - <1yr life expectancy - found in cerebral hemispheres - can cross corpus callosum - "butterfly glioma" - stain astrocytes for GFAP PATHOLOGY: - pseudopalisading - pleomorphic tumor cells - border central areas of necrosis and hemorrhage |
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Meningioma
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- 2nd most common primary brain tumor
- most often occurs in convexities of hemispheres and parasagittal region - arises from ARACHNOID CELLS external to brain - RESECTABLE! PATHOLOGY: - spindle cells concentrically arranged in a whorled pattern - PSAMMOMA bodies (laminated calcifications) |
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Schwannoma
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3rd most common primary brain tumor
- Schwann cell origin - often localized to CN VIII - acoustic schwannoma - RESECTABLE associated with NF2 |
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Oligodendroglioma
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relatively rare
slow-growing - RARELY resectable - survival of 7-10 years PATHOLOGY: - most often in frontal lobes - chicken-wire capillary pattern - "fried egg" cells - round nuclei with clear cytoplasm - often calcified in oligodendroglioma |
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Pituitary adenoma
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prolactin-secreting is most common form
- bitermporal hemoanopia (due to pressure on optic chiasm) and hyper or hypo pituitarism are sequelae - Rathke's pouch |
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Summary list of main adult tumors
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Adult brain tumors:
- mets, Pee & SMOG - Pituitary Adenoma - Schwannoma - Meningioma - Oligodendroglioma - GBM (astrocytoma grade IV) - |
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pilocytic (low-grade astrocytoma)
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usually well-circ
- most often in POSTERIOR FOSSA - benign - good prognosis (unlike ependymoma which has poor prognosis) PATHOLOGY: - Rosenthal fibers - eosinophilic corkscrew fibers |
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Medulloblastoma
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highly malignant CEREBELLAR tumor
- a form of primitive neuroectodermal tumor - can compress 4th ventricle --> hydrocephalus PATHOLOGY: - rosettes or perivascular pseudorosette pattern cells - radiosensitive |
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Ependymoma
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tumors of 4th ventricle
- can cause hydrocephalus - poor prognosis (unlike pilocytic astro which has good prog) PATHOLOGY: - perivascular pseudorosettes - rod-shaped blepharoplasts (basal ciliary bodies) found near nucleus |
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Hemangioblastoma
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often CEREBELLAR
- associated with von-Hippel-Lindau syndrome - can produce ERYTHROPOIETIN --> secondary polycythemia PATHOLOGY: - foamy cells with high vascularity!!! |
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craniopharyngioma
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benign childhood tumor
- confused with pituitary adenoma (can cause bitemporal hemianopia) - most common childhood supratentorial tumor - derived from remnants of Rathke's pouch - calcification is common (tooth-enamel like) |
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pinealoma
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tumor of pineal gland
- compresses vertical gaze center - inability to look upward - pineal gland maufactures melatonin from 5HT - inadqueate supply of melatonin causes INSOMNIA |
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Schwannoma
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neurolemoma
- benign tumor of scwann cells - CN V and VIII may be involved CN VIII suggests acoustic neuroma - neurofibromatosis - Tinnitus (ringing in ears) - sensorineural hearing loss - microscopy shows alt light and dark areas - can get CNV sensory changes |
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Arnold Chiari malformations I & II
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1. Small posterior fossa
2. downward displacement of cerebellum 3. medulla deformity 4. associated with tonsilar herniation Arnold-Chiari I - low-lying cerebellum obstructs CSF flow and compresses medulla - cerebellar tonsils descend through posterior foramen magnum - frequently asymptomatic - correctable with surgery Arnold-Chiari II - cerebellar vermis and medulla descend through foramen magnum - FATAL - associated with Meningomyelocele (SC and meninges outside vertebral column) |
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Dandy-Walker malformation
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large posterior fossa (as opposed to Arnold-Chiari which is small posterior fossa)
- ABSENT CEREBELLUM with cyst in its place |
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cranial nerve and cerebellar lesion - discuss laterality of lesion with each of the following lesions:
1. CN XII 2. V motor 3. X 4. XI |
1. tongue deviates TOWARD the side of the lesion
2. jaw deviates TOWARD the side of the lesion 3. uvula deviates AWAY from the side of the lesion 4. weakness in turning head to CONTRALATERAL side of lesion - shoulder drop on side of lesion |
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result of UMN lesion to face - lesion of motor cortex or connection b/t cortex and facial nucleus
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CONTRALATERAL paralysis LOWER FACE only
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facial LMN lesion
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IPSILATERAL paralysis of UPPER and LOWER FACE
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Bells palsy
- describe location of lesion - effects of lesion in terms of motor impairment - causes - associated disease states |
complete destruction of the facial nucleus or the branchial efferent branches (facial nerve proper)
- peripheral ipsilateral facial paralaysis with inability to close ipsilateral eye NOTE: ONLY LOWER FACE IS AFFECTED, since upper face has contralateral and ipsilateral innervation by CN VII - can occur idiopathically - gradual recovery in most cases seen as complicaiton in - AIDS - Lyme disease - Sarcoidosis - Tumors - Diabetes Cx: mouth drooling, inability to close eye, inabilty to wrinkle forehead, loss of blink reflex, hyperacusis |
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carotid body tumor
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secretes catecholamines (like a pheochromocytoma)
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discuss sequelae
1. cingulate herniation under falx cerebri 2. downward transtentorial (central) herniation |
1. can compress ACA
coma and death result when these herniations compress brain stem! |
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uncal herniation - name the case for each sign:
1. ipsilateral dilated pupil/ptosis 2. contralateral homonymous hemoanopsia 3. ipsilateral paresis 4. dureh hemorrhages - paramedian artery rupture |
1. stretching of CN III - remember CN III does efferent limb of pupillary light reflex which is constricting reaction
2. compression of ipsilateral PCA 3. compression of contralateral cruz cerebri (Kernohan's notch) - a "false localizing" sign 4. caudal displacement of brain stem |
