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Neurology High Yield notes for step 1

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USMLE notes for neurology step 1
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  Neurology High Yield ( http://www.neuroanatomy.ca/module_list.html ) Ischemic Brain Disease    IRREVERSIBLE DAMAGE after 5 minutes    Most vulnerable Hippocampus pyramidal, cerebellum purkinjie cells , watershed areas    Risks: diabetes, smoking,  ATRIAL FIBRILLATION , cocaine    Transient attack: less than 24 hours neurological deficit    Types or stroke 1.   Thrombotic/atherosclerotic: MOST COMMON -pale liquifactive necrosis (NO REFERFUSION) -causes reactive Gliosis cystic cavity -usually MCA therefore at periphery 2.   Embolic -Hemorrhagic red infarct (DUE TO REPERFUSION) -usually atrial fibrillation or atherosclerotic plaques -MCA 3.   Lacunar Infarct -small perforating vessels (at BASAL GANGLIA  ) hyaline arteriosclerosis -Causes: diabetes, HTN 4.   Hemorrhagic stroke -from aneurysm - CharcotBouchard microaneursym at basal ganglia of lenticulostriate arteries -from reperfusion of ischemic 5.   Watershed Infarct -prolonged HYPOTENSION -damage usually bilateral deep cortical areas btw border of two vessels -ACA- MCA Top -MCA- PCA Bottom Artery Deficient MCA -Aphasia (Wernicke or brocas), -Neglect syndrome (if right parietal, so ignore left side) -hemiparesis weakness ( arm and face)  -homonymous hemianopsia (temporal more common, so superior quadrant) ACA - LEG paresis  -hemiplegia (total paralysis) Ischemic stroke Hemorrhagic stroke Diminished blood flow Leads to Gliosis Watershed infarct Hemorrhage aneurysm Embolic Reperfusion  urinary incontinence PCA -homonymous hemianopsia with macular sparing  (since MCA give blood) Basilar Affect pons ( cranial nerves, coma, apnea, vertigo) Lacunar stroke -Pure motor ( internal capsule) -Pure sensory (thalamus) but the smell intact (since only sensory that bypass) -dysarthria -Ataxic hemiparesis (ipsilateral clumpsy Imaging:    ACUTE do CT to see if hemorrhagic (dark on CT after 24 hours)    MRI shows up bright in 3-30 min and remain bright 10 days Histology    12-24 H : RED neurons    24-72: necrosis + PMN    3 -5 day : macrophage    1-2 week : reactive gliosis + vascular proliferation    >2 week: glial scar Treatment    tPA for occlusive (give within 3 hours)    warfarin/ aspirin for embolic once hemorrhage has been ruled out  Alzheimers    Diffuse cortical atrophy (widen sulci, narrow gyri, expand ventricles, ex vacuo hydrocephalus )    Nucleus basalis of Meynert ( low ACH)    MOST COMMON cause of dementia ( 2 nd  is stroke)    Causes 1.   Apolipoprotein E 2protective ( “   2 serve and protect”) 2.   ‘’ E4 increases risk 3.   Down Syndrome, chromosome 21 has APP (amyloid precursor protein) * will stain with Congo red -usually get it before 40 4.   Presenilin -Presenilin 1 (chromosome 14) - ‘’ 2 (chromosome 1)    Clinical diagnosis (confirmed on autopsy)    Histology    Senile/ neuritic plaques : ABETA-amyloid (beta cleavage of APP) EXTRACELLULAR -normal cleavage if ALPHA    Neurofibrillary tangles INTRACELLULAR  in neurons -HYPER phosphorylated Tau proteins which are microtubule associated  - amount of tangles CORRELATED WITH DEGREE OF DEMENTIA      Death: infection, penumonia   Diseases of myelination Guillain- Barre Syndrome/ Acute inflammation Demylinating polyradiculopathy (PNS and motor + autonomic)    Endoneurium and Schwann cells attacked = autoimmune demyliantion    1-3 weeks post GI infection OR RESPIRATORY (campylobacter jejuni) or herpes    SYMMETRIC Ascending paralysis     MOTOR AND AUTONOMIC      Physical exam:    Decrease or absent reflex    Facial paralysis if ascended to face     Autonomic dysfunction  : cardiac and blood pressure fluctuation    Need to give respiratory support    Lumbar puncture: high protein, normal cell count ( albuminocytologic dissociation)  -this high protein is also seen in intracranial cancer Progressive Multifocal Leukoencephalopathy( PML) (CNS )    Demylination by destruction of oligodendrocytes    Associated with JC virus reactivation in AIDS patients ( polyomavirus,ds DNA)      Dementia and neurological deficits with RAPID PROGRESSION      Typically fatal    Histology: widespread demylination, astrogliosis, lymphohistiocytosis    * associated with Natalizumab ( drug against alpha-integrin used in Crohns and MS ) *MS also demylination oligodendrocytes Usually seen on one side more   Acute Disseminated /post infectious Encephalomyelitis    Multifocal PERIVENULAR  inflammation and demyelination immune activation -after infection : chickenpox, measles -Vaccination: rabies, smallpox    Similar to Multiple Sclerosis + constitutional (fever etc) -mental status change, seizure coma * usually in children and adolescents  (but anyone can get it) Metachromic Leukodystrophy ( CNS and PNS, myelin accumulate in lysosome)      Autosomal recessive lysosomal disease due to  ARYLSULFATASE A  deficiency . cant metabolise sphingolipids    Impaired myelin sheath    Diverse psychiatric and neurological problems    Presents at different times depends on the form inherited -infantile -juvenile -Adult Krabbe Disease    A.R. lysosomal storage disease due to deficiency of GALACTOCEREBROSIDE    See globoid cells    Peripheral neuropathy, developmental delay, optic atrophy AdrenoLeukoDystrophy    X linked disrupted metabolisim of VERY LONG chain Fatty acids    Build up Fatty acids in adrenal gland, testes, nervous system    Coma/ death, and adrenal crisis occur( not enough cortisol ) Charcot- Marie- Tooth Disease/ Hereditary motor and sensory neuropathy    Defective protein for structure and function of peripheral nerves or myelin    Onset less than 30 years old    Slow progressive weakness of distal limbs with loss of proprioception and vibration    Autosomal Dominant usually. Associated with Scoliosis and foot deformity (HGH ARCH OR FLAT)    PRESENT FOOT DROP, and loss of foot and hands motor and sensory first Central Pontine Myelinolysis    Demylination in the pons : loss of corticospinal and corticobulbar tracts    Due to rapid correction of HYPONATREMIA    Seen in malnourished or alcoholics    Locked in presentation    Often fatal

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