Question | Answer |
ALS: Dx | UMN & LMN sx in 3 regions; UMN: clinical; LMN: EMG; CK nml or high |
ALS Tx | Riluzole; cough assist device, chest PT, BiPAP; PEG feeding tube; |
PLS: path | degeneration of lateral corticospinal tract |
PLS clin findings | usu legs before arms; leg weak/stiff; spasticity; ave duration >8 yrs; many pts develop LMN sx & transition to ALS |
PLS: dx | EMG, MRI (br & spcord), LP, evoked potls; B12, Lyme, RPR, long chain fatty acids |
PMA | pure LMN dz; slower progress than ALS; often spares bulbar mx; CK very high |
Kennedy dz | X-linked rec, TNR; mutation in androgen receptor; sx onset (wide age range); facial fasciculations, weakness mouth/tongue; dysphagia, limb weakness; gynecomastia, DM, oligospermia |
Disorders of neuromx transmission (NMJ): | MG, LEMS, botulism |
MG: path | Acquired autoimmune: Ach receptor insufficiency |
MG S/S | fluctuating mx weakness (worse w/reps); asymmetric ptosis; tired face, difficulty chewing/swallowing; SOB |
MG Dx | Tensilon test; Ach receptor & MuSK Abs; Repetitive nerve stim; Single fiber EMG; CT Chest to exclude thymoma |
MG Tx | Symptomatic (Cholinesterase inhibitors); Firstline: steroids (alt immunosupp: CellCept, Cyclosporine); thymectomy |
MG exacerbations | d/t infxn, PG, meds; may worsen resp sx; tx w/ plasmapheresis or IVIg, supportive respiratory care |
MG: drugs to avoid | NM blockers; quinine; macrolides, FQ; botox; beta/CCB; IV contrast |
LEMS | usu paraneoplastic (autoimm rare); proximal weakness / autonomic sx (dry mouth); hypo/absent reflexes; voltage gated Ca channel Abs; facilitation w/ RNS |
Botulism MOA | irreversible blockade of Ach release |
Botulism S/S | Diplopia, ptosis, dilated pupils; facial / resp weakness; descending paralysis; Autonomic dysfunction |
Botulism Tx | supportive; horse serum antitoxin? (months to recover) |
MD classified by: | distribution, inheritance and clinical features |
MD: labs/studies | ultrastructural protein abnormalities; mx bx: mx fiber necrosis |
Duchenne | onset at 3-5 yrs; calf pseudohypertrophy; loss of ambulation by teens; cardiomyopathy; death in 20s |
Duchenne/Becker: Dx | Mx bx; genetic testing (need complete sequencing); CK sometimes >10,000; FH |
Duchenne/Becker Tx | Prednisone (age 5) for Duchenne; Supportive (orthotics, resp, cardiac, PT/OT); cardiac TP in Becker |
FSHD | auto dominant; asymmetric; eye or mouth closure weakness: may sleep w/ eyes open; pectoral & biceps/triceps atrophy (but normal deltoid); also scap winging; no fx on life exp; orthotics & counseling |
FSHD Dx | genetic testing (95%); FH; mx bx rarely helpful |
Emery Dreifuss | Humeroperoneal / scapuloperoneal weakness w/ early contractures (ankle neck elbow); teen onset (very thin); scap winging/deltoid sparing; x linked or auto dom; cardiac conduction block/arrhythmia (need pacemaker) |
Most common MD's | Duchenne/Becker; myotonic; FSHD |
Myotonic dystrophy | auto dom; any age; TNR |
Myotonic dystrophy S/S | tenting upper lip; distal weakness; frontal bald; cataracts; conduction block; DM, infertile; cognitive impairment; percussion myotonia |
Allelic vs non-allelic heterogeneity | allelic: 1 gene causes >1 condition; non-allelic: clinical syndrome is d/t >1 gene |
Autosomal dominant | 1:1 M:F; multi generations; M-to-M transmission seen; variable expression (important); late-onset neurodegenerative dz's |
Autosomal dominant disorders | Huntington, NF1/NF2, spinocerebellar ataxias, familial Alz, CMT |
NF1: other manifestations: | hydrocephalus, seizures, learning disabilities, short, lack of GH, precocious puberty, Renal Artery stenosis |
Auto recessive | >1 affected each generation; M:F 1:1; consanguinity; carriers usu asx; inborn errors of metabolism |
Auto recessive disorders | PKU, Tay-Sachs, MSUD, Friedreich's, Wilson, homocystinuria, sickle cell |
Wilson Dz | hepatolenticular degen (impaired ceruloplasmin synth); presents in teen years (hepatitis) |
Wilson S/S | tremor, dysarthria, slow, hoarse, chorea, psych |
Wilson: dx | high ceruloplasmin & copper; low copper on liver bx; Kayser Fleischer rings |
Wilson: tx | reduce copper intake; chelate; transplant / |
X-linked recessive | F-toM trans; M=affected, F=carrier; |
X-linked rec dz | Duchenne/Becker MD; Kennedy; adrenoleukodystrophy; Menkes (kinky hair); Lesch-Nyhan; Fragile X |
Duchenne/Becker Dx/Tx | genetic testing, elevated CK, EMG; Tx: supportive, corticosteroids, PT, ortho, cardiopulmonology |
X-linked dom | F-to-F transmission (lethal to males); Rett; Aicardi; Lissencephaly 2 |
Rett dz | 6-18 mos; live to 40s; autism; cardiac & scoliosis |
Mitochondrial dz | multi generations; trans by F only; 1:1 M:F affected; MERRF, MELAS, LHAN, Kearns-Sayre |
MELAS | d/t pt mutation of transfer DNA from leucine; <40 yo; lactic acidosis; HA, stroke, seizure, short; progressive dementia; Dx high serum pyruvate & lactate, stroke lesions; no tx |
TNR: mode of inheritance | can be multiple modes of inheritance |
TNR dz | Huntington, Fragile X, myotonic dys; Kennedy; spinocerebellar ataxia; Friedreich |
Alz dx/tx: | dx of exclusion (neuropsych eval); MRI/CT: hippocampal atrophy; amyloid on PET (Pittsburgh B); LP: inc tau, dec amyloid-beta 42; tx: cholinesterase inhib |
Alz: APOE gene | E2: protective vs Alz; E4: inc risk of dev Alz |
Alz dz: main reason for genetic counseling is: | info only |
Parkinson dz | usu unilateral onset; resting tremor, slow, cogwheel rigidity, festinating gait, masked facies, postural instability |
Parkinson: TRAP = | Tremor, Rigidity, Akinesia, Postural instability |
Pathological hallmark of Parkinson: | Lewy body (alpha-synuclein is main component) |
Most common genetic form of Parkinson: | PARK8 |
Ulnar neuropathy | stretch / compress ulnar n.; cubital tunnel or Guyon canal (FCU mx may be spared); d/t pressure, bone spurs, cysts; sensory precede motor sx |
Ulnar neuropathy: provoking factors | Elbow Flexion (Cubital), Wrist Extension (Guyon's); Nighttime |
Ulnar neuropathy: Dx | Hx; EMG/NCS can help find site of lesion |
Ulnar neuropathy: Tx | modify activity; extensor splint at night; NSAIDs; surgery (nerve transposition or ligament release); No C'steroids |
Radial neuropathy: etiology | axilla (crutches); Saturday night palsy; handcuffs; humerus fx |
Radial neuropathy: Dx | Motor>sensory; weakness in extension & arm ext rotation; forearm atrophy; xray shoulder/humerus |
Meralgia paresthetica: patho | stretch/compress lat fem cutaneous n. |
Meralgia paresthetica: RF | obesity; DM; PG; hip hyperextension; lumbar lordosis |
Meralgia paresthetica: Dx | pain, paresthesia, numb; outer thigh; usu unilateral (relieved by sitting); no motor sx |
Meralgia paresthetica: Tx | often self-ltg; hydrocortisone injxn; nerve transposition |
Fem neuropathy: RF | lithotomy posn (inguinal lig); DM; retroperitoneal neoplasm/hematoma; pelvic fx; fem art cath (n. trauma) |
Fem neuropathy: Dx | Quads atrophy/weakness; sensory impairment anteromedian thigh; decreased patellar DTR; EMG/NCS; CT/MRI |
Fem neuropathy: Tx | Tx etiology; splints/braces; PT |
Sciatic n. palsy: Dx | weakness w/ leg flexion, dorsiflexion & foot eversion (drop foot); hamstring & ankle DTR dec/absent; sensory loss posterior thigh/leg/foot; tingling/burning/lanceting pain (worse w/standing, cough) |
Sciatic n. palsy: Dx tests | EMG/NCS (distinguish from peroneal neuropathy); xray |
Sciatic n. palsy: Tx | Tx etiology; behave change; anti-inflam; PT; surg |
Peroneal n. palsy: Dx | weak dorsiflexion (foot drop) & eversion; sensory loss/ paresthesia: anterolateral calf & foot dorsum; EMG/NCS? Tx sim to sciatic n. palsy |
CN VII palsy (Bell): etiology | idio; HIV, sarcoid, Lyme, tumor; HSV infxn? RF = PG, DM |
CN VII palsy: Dx | abrupt; facial paralysis (some upper sparing); drooping corner of mouth; ptosis/ forehead smooths out; ear pain; dysgeusia; hyperacusis |
CN VII palsy: to distinguish btw peripheral & central lesion: | peripheral: complete paralysis forehead mx; central: partial sparing forehead mx |
CN VII palsy: prognosis | 60% spont resolve; 10% perm disfigurement/dysfn; best indicator of severity = progress first 2-3 days; worst: complete palsy at onset, advanced age, hyperacusis, severe initial pain |
CN VII palsy: Tx | prednisone; artificial tears/eye patch; No Surg |
CMT: genetics | usually auto dom |
CMT: patho | Type I: demyelinating; II: axonal; Motor>Sensory; Lower > Upper; childhood/young; slow progression |
CMT I vs CMT II: Dx | CMT II: less mx wasting/ secondary weakness; less common postural tremor/arm involvement; NO peripheral n. hypertrophy |
CMT Dx: | H&P; DNA testing; Nerve/mx bx (confirmatory); EMG/NCS |
CMT: EMG/NCS | CMT I: segmental demyelination; reduced motor & sensory conduction velocity; CMT II: axonal loss; normal/sl dec motor conduction, dec SNAPs; chronic partial denervation in affected mx |
Dejerine-Sottas Dz (CMT III): patho | phytanic acid disturbance; prog demyelinating neuropathy; infancy/kids |
Dejerine-Sottas Dz: Dx | weakness, ataxia; sensory loss; DTR: global hyporeflexia; high CSF pro; EMG/NCS: dec motor velocity, sensory conduction |
Dejerine-Sottas Dz: Tx | Supportive; plasmapheresis; dietary restriction |
Refsum dz: patho | Progressive Demyelinating Neuropathy; Early Childhood |
Refsum dz: Dx | weakness, ataxia; sensory loss; DTR: global hyporeflexia; retinitis pigmentosa; CSF protein normal; nerve bx; EMG/NCS: dec motor velocity, sensory conduction; Tx supportive |
Systemic-metabolic neuropathies include: | DM; uremia; alcoholic & nutrition def; paraproteinemias |
DM neuropathy | sensory precedes motor; lower ext precedes upper; Hx: autonomic sx?; NCS nml / mildly slow |
Charcot Arthropathy is a complication of: | DM peripheral neuropathy |
Charcot Arthropathy | d/t Joint Subluxation, Periarticular fx; rocker bottom foot; pain, swelling, ulceration |
Uremia: Dx | Symmetric sensory-motor; lower ext > upper; distal > proximal; severity correlates with degree of renal insufficiency |
EtOH/Nutritional deficiency | cobalamin (B12) def; axonal > myelin; slow progression |
EtOH/Nutritional deficiency: Dx | Distal symmetric Polyneuropathy; sensory precedes motor; Lower ext precedes upper; cramps, painful paresthesias, tenderness; CNS Sx often precede PNS; mental status change; myelopathy; optic neuropathy |
AIDP (GBS): patho | Progressive Demyelinating; prob immune-mediated; Axonal Subtypes (AMAN; AMSAN) |
AIDP (GBS): Dx: | H&P; high CSF pro (2-3 wks post onset); EMG/NCS: demyelination with delayed conduction (3-4 wks) |
AIDP (GBS): Dx: MOTOR | Weakness; Symmetric; Proximal before Distal; Lower ext before Upper; Advanced: Resp mx Compromise, CN Involvement |
AIDP (GBS): Dx: SENSORY | Paresthesias; Loss of Sensation; Distal before Proximal; DTR: Global hyporeflexia or areflexia; ANS: tachycardia, cardiac irreg; BP changes, pulmonary dysfn, loss of rectal tone |
AIDP (GBS): Tx | Anti-inflam; plasmapheresis; IVIg; mech ventilation |
Leprosy: 2 Types: | tuberculoid (multifocal) & lepromatous (symmetrical) |
Most common causative of meningitis in adults | S. pneumo |
When do LP? | suspect meningitis; not if suspect abscess |
Glucose depressed: usually: | bac mening, or TB or fungal |
Meningitis general S/S | HA, fever, neck / back stiffness, neuro impairment |
Meningitis presentation | acute (hrs - days), usu w/ fever, HA, stiff neck, lethargy; usu without focal sx |
Typical purulent meningitis orgs | N. meningitidis, S. pneumoniae, or H. influenzae |
Chronic meningitis: orgs | TB; fungal |
Encephalitis presentation | diffuse infxn; confusion, lethargy, often seizures; CSF may be nml |
Increased WBC in CSF indicates: | inflammation (not necessarily infection) |
Pneumococcal meningitis Tx | ceftriaxone (if GP diplococci seen, add Vanc, pending cx) |
N. meningitidis | petechial rash; GN diplococci; often assoc w/DIC |
Meningococcal meningitis: nasal carriage eliminated with: | Rifampin (alt: cipro or ceftriaxone) |
Meningococcal meningitis: DOC | Acqueous Pen G |
H flu meningitis | less common in adults; in setting of otitis or sinusitis; DOC ceftriaxone |
Tuberculous meningitis | usu gradual onset; listlessness & irritability; CN palsies; active TB elsewhere; CSF inc WBC (100-150), mostly lymphs; abnml CXR; acid-fast normal |
Abscess: common orgs | streptococcus, staphylococcus or anaerobes |
Ring enhancing lesion is usually: | abscess or tumor |
Abscess tx | prolonged IV Abx, surg drainage; monitor tx w/ serial scans; if <2 cm poss medical tx only |
Rabies S/S | (> 10 days) delirium, painful swallowing, rage alternating with calm |
Rabies incubation period | >10 days (usu 3-7 wks) (infected animal dies within 5-7 days) |
Herpes encephalitis | more common in elderly; often medial temporal lobes; tx most pts w/ clin syndrome of viral encephalitis empirically for HSE |
CJD Sx | usu later in life; rapidly progressive dementia, myoclonus, ataxia & somnolence; epileptiform pattern on EEG |
MS epidemiology | F>M; 350K in US (1M world); onset 20-40 yo; most present w/relapsing form; prevalence increases with distance from equator |
MS S/S | Optic neuritis; Transverse myelitis; Paresthesias; Ataxia; Weakness or incoordination; Spasticity; Cognitive impairment |
MS: MRI findings | multiple characteristic lesions or plaques: periventricular or subcortical U-fibers, corpus callosum lesions |
MS: CSF findings | evidence of oligoclonal bands or increased IgG index |
MS: types of dx criteria | Schumacher; Poser: Macdonald |
MS: Goals of tx | Tx whole dz; slow accumulation of disability; Reduce relapse rate & CNS inflam (lesions) & progression of brain atrophy (shrinkage); improve pt QOL (including cognitive) |
MS Tx options | IFN; glatiramer; poss immunosupp adjuncts for aggressive/ breakthrough; corticosteroids (methylprednisolone) for relapse |
Majority of malignant gliomas are: | grade IV tumors (GBM or gliosarcomas) |
Gliomas include: | astro; oligo; ependymoma; glial cells: support |
Grade 1 Glioma = | Pilocytic Astrocytomas |
Grade 2 Glioma = | Astrocytomas, Oligodendrogliomas |
Grade 3 Glioma = | Anaplastic Astrocytomas, Anaplastic Oligodendrogliomas |
Grade 4 Glioma = | GBM; Gliosarcoma |
Diffuse astrocytomas include: | Low-grade infiltrative Astro (WHO II); anaplastic astro (WHO III); Glioblastoma (WHO IV) |
Circumscribed astrocytomas include: | Pilocytic Astro (WHO grade I); Pleomorphic xanthoastrocytoma (PXA); Subependymal giant cell Astro (SEGA) |
Well-differentiated Astro: | 10-15% of Astros; 35 yo; 5-10 yr surviv; cerebral hemi / cortex; slow growing; often undergo malig progression to AA or GBM |
High-grade gliomas (III/IV): | invade via white matter tracts, cross via corpus callosum; |
Primary characteristic of a grade IV glioma = | necrosis with vascular proliferation |
Anaplastic astro | High-Grade; 1/3 of Astros; 45 yo; 3 yr surviv; usu in cerebral white matter; relatively fast-growing; Frequent progression to GBM |
GBM | 50% of Astros; >60 yo; rare <30; 1 yr surviv; usu in cerebral hemi white matter; spreads rapidly & diffusely; Ring of tissue around necrotic core; Highly vascular |
Oligodendroglioma | Usu presents at low-grade (II) stage; young / middle-aged; grade II >10 yr surviv; Fried-egg cells |
Most common primary brain neoplasm = | GBM |
Untreated GBMs: growth | double in size in 14 days |
Brain tumor: genl clin presentation | HA; seizures; Cognitive dysfn; Focal neuro deficits; N/V; Sx endocrine dysfn; Visual sx; Sx from plateau waves (ICP changes) |
Headache in brain tumor | 20% of pts present w/HA (more w/ post fossa tumor); usu d/t inc ICP; Progressive increase in HA freq & severity; Classic HA on waking or HA that wakens one from sleep |
Seizure in brain tumor | 35% of pts present with this; often the sx that precedes dx; focal or secondarily generalized |
Probably most common problem in pts w/brain tumors = | cognitive dysfn |
Cognitive dysfn in brain tumor: | Frontal personality; Memory problem; Depression |
Cognitive dysfn: Left hemispheric tumors: | language dysfunction |
Cognitive dysfn: Right hemispheric tumors: | problems with visual perception & scanning |
Brain tumor: Focal neurologic deficits | Hemiplegia; Hemiparesis; Ataxia; Nystagmus; Can mimic stroke |
N/V more common in: | post fossa tumors |
Endocrine sx: | hypothyroid; dec libido |
Visual symptoms include: | Contralateral flashing lights; Visual field loss; Diplopia |
Symptoms from plateau waves | Transitory episodes of altered consciousness & visual disturbances |
Brain tumor: eval & dx | H&P; CT +/- MRI; EEG; LP; PET |
Factors of better prognosis: | Young; High KPS; Lower pathological grade |
Less significant prognostic predictors: | Long duration of sx; Absence of mental status changes; Cerebellar location; Small preop tumor size; Completeness of surgical resection |
BCNU AE | fatigue, low blood counts, pulmo fibrosis |
Tx for high grade gliomas | Primary tx = surg resection, RTx, CTx; most sig prognostic factors: extent of surg resection, age, & performance status |
First treatment modality for high grade glioma | Surgery |
Goals of surgery: | Confirm patho dx; improve sx rapidly; reduce number of ca cells requiring tx (removes hypoxic core of the tumor; relatively resistant to radiation & inaccessible to CTx) |
Std tx for WHO III &IV gliomas: | Radiation tx (role of RT in WHO II gliomas is controversial) |
RTx Modalities: | Whole brain (ltd use now in gliomas); Focal RTx: Conventional high-dose (59.4 Gy x 42 days; 2 cm border around tumor area) or IMRT; Stereotactic (gamma-knife) |
RTx injury | Acute (fever, neuro def) or delayed encephalopathy (edema, MRI enhance); tx w/c'steroids; focal cerebral necrosis (mos-yrs): tx w/steroids & hyperbaric |
Most frequent delayed effect of RTx | Diffuse cerebral injury; cortical dysfunction, progressive dementia, gait disturbance; WM destruction; MRI: lg ventricles, wide sulci, basal ganglia calcifn, hyperintense T2 |
Std of care: high grade gliomas | Resection; RTx & 42 days temozolomide; then (if stable dz), adjuvant CTx |
Stupp study result: | temozolomide added to RTx improves survival |
CTx MOA & AE | targets DNA replication; AE: Myelosuppression; N/V; Fatigue; Constipation or diarrhea; DI |
CTx: passage across BB depends on: | Molecule Size; Lipid solubility; Ionization state |
CTx DI: | Dex (closing BBB); phenobarbital (dec nitrosourea efficacy); anti-epileptics (affect CTx metabm) |
Low grad glioma: Tx | Bx (confirm dx & r/o high grade); seizures only: defer tx til progresses; if progressive sx: resect +/- CTx (+ RTx only if refractory) |
VEGF | higher the VEGF, worse the prognosis; anti-VEGF Ab's effective in xenografts |
Tumor blood vessels | dept on growth factor; leaky & fragile; less supported by pericytes; disorganized, twisted, variable pressure; inconsistent nutrient delivery to tumor tissue |
Second leading COD in brain tumor pts: | thromboembolic complications |
Meningioma | slow growing, benign; attached to dura mater; 13-26% of primary IC tumors; RF: prior radiation; often Asx; visual complications; DDx: neurofibromatosis; Firstline tx: Surgery |
Ependymoma: worse prognosis: | <3 yo |
Ependymomas within brain: locations | Infratentorial > supratentorial (2x) |
Ependymoma | Usu slow growing; kids & YA; young adults; 4% all BT; from walls of ventricles or spinal canal (obstructive hydrocephalus); 90% are in brain, 10% in spinal cord (often adults) |
Medulloblastoma | Malig, invasive embryonal cerebellar tumor; kids & YA; 70-80% 5-yr survival; Most arise in vermis (4th ventricle involvement) |
Medulloblastoma: metastases | Metastasize via CSF pathways (Drop Mets); Rare mets to bone, lymph nodes, other extracranial sites |
Medulloblastoma: Sig LT toxicities of tx: | Cognitive decline; Psychomotor / growth retardation; Hormonal deficits |
Primary CNS lymphomas | Usu present deep in brain parenchyma; usu multifocal (diffuse lg cell B-cell lymphoma); Survival w/o tx <1 yr; Steroid tx & Methotrexate CTx |
Primary CNS lymphomas: incidence increased in: | Immunodef pts; AIDS; Organ TP; older pt |
Std of care: Grade I | Surgery +/- RT |
Std of care: Grade II | Surgery; Observe; If progression: CTx |
Std of care: Grade III | Surgery; RT with temozolomide; 12 cycles of temozolomide |
Std of care: Grade IV | Surgery; RT with temozolomide; 52 weeks rotational CTx |
Emergent eval of stroke | CBC/plt; PT/ PTT; Lytes, glu, renal; ECG/ markers of cardiac ischemia; Brain CT or MRI |
Ischemic stroke patho | atheroembolic (50%); cardioembolic (30%) |
Hemorrhagic stroke patho | parenchymal ICH (10-15%); subarachnoid (5-10%) |
Most common parenchymal ICH: | Hypertensive intracerebral hemorrhage |
Primary ICH presentation | HA, N/V; progressive hemiparesis & hemisensory def; HTN (on hx and on PE) |
Primary hypertensive ICH: typical locations | Thalamus; Basal Ganglia; Pons; Cerebellum |
ICH presentation: Thalamus/ Basal Ganglia | Contralateral Motor/ Sensory Deficit; Aphasia, Neglect; Depressed LOC with mass effect, IVC extension |
ICH presentation: Cerebellum | Ipsilateral Ataxia; Depressed LOC |
ICH presentation: Pons | Vertigo, Diplopia; Crossed signs; Depressed LOC |
Amyloid angiopathy stroke: patho | Blood vessel degeneration; Dementia; Lobar hemorrhage |
Amyloid angiopathy: presentation | Dementia; Episodic worsening; No h/ o HTN; poss acute limb weakness; BP 130/80 mmHg (less severe than in ICH); stroke d/t cerebral microhemorrhages |
Venous infarction presentation | OCP/SMK; HA; aphasia, weakness |
Most common cause of subarachnoid bleed | aneurysm |
Subarachnoid bleed: RF | HTN; SMK; heavy EtOH; genetics (polycystic kidney dz; Ehlers-Danlos; if first-degree rel w/this stroke) |
SAH stroke presentation | Abrupt severe HA; meningismus; depressed LOC; nonfocal neuro exam |
Atheroembolic Stroke characterized by: | Single vascular territory; Warning signs; Stepwise progression |
Atheroembolic presentation | Hx HTN, CAD; transient language disturbance; transient weakness; Normal head CT; Doppler US: high grade stenosis (e.g., L ICA) |
Ant cerebral art infarct: likely fx: | contralateral leg (motor > sensory) |
MCA infarct: likely fx: | face/arm more than leg/vision; poss aphasia |
Vertebrobasilar art infarct: fx: | Midbrain: 3d nerve nuclei; ipsilateral ptosis; eye deviated outward (bc CN VI is fine, but III is affected); crossed signs: pt has CNIII probs on one side and sensory probs on opp side of body |
Subcortical infarct fx: | face = arms = legs |
Cortical infarct fx: | gradation btw face, arms, & legs |
Lacunar syndromes | Pure Motor Stroke; Pure Sensory Stroke; Ataxic Hemiparesis; Clumsy Hand Dysarthria |
Atheroembolic stroke: additional eval | Neuroimaging; Carotid US; MRA; CTA; Catheter angiography |
Cardioembolic stroke presentation | h/o A fib; aphasia; hemiparesis/hemisensory deficit affecting face and arm; Carotid US normal (no brain lg vessel prob) |
Cardioembolic: dx | Maximal deficit at onset; Multiple vascular territories; Cardioembolic source; Hemorrhagic infarction (Wedge shaped infarct towards cortical surface) |
Cardioembolic: poss sources | A fib; Cardiomyopathy; Acute MI; Valvular heart dz |
Cardioembolic stroke: additional eval | pulse; EKG; 24-48 hr EKG; TTE (microcavitation); TEE |
TIA | Acute focal neuro def; S/S resolve within 24 hr; No rad evidence of infarction; Ischemic etiology; 11% risk of stroke within 3 mo; 63% of strokes occur within the first wk, 85% within first month |
Modifiable RF for first stroke | *HTN*; A fib; carotid stenosis; DM; hyperlipidemia; prior stroke/TIA |
Lifestyle mods affecting BP | wt reduction; DASH diet; sodium reduction; exercise; moderate EtOH consumption |
Stroke comorbid RF's | CHD, CHF, DM, stroke |
Prevention of A fib RF: | Low risk (0-1) ASA; mod (2) ASA or warfarin (but AE/bleed risks); high risk (>2) warfarin |
Asx carotid stenosis: eval | Carotid bruit; Doppler US; MRA, CTA |
Stroke prevention DM pts | glucose ctrl: no fx on stroke/macrovascular comp; tight BP ctrl (<130/80) effective; statins |
Stroke prevention: hyperlipidemia | chol reduction w/statins |
ASA recommended for 10-yr stroke risk of: | 6-10% |
Ischemic stroke: Tx | tPA (within 4.5 hrs of sx onset); head CT w/o evidence of hemorrhage/complicating lesion |
tPA absolute CI (<3 hr) | CT: bleed/comp (AVM); BP >185 or >110; recent stroke/ICH; bleed elsewhere; anticoag use; plt <100K; h/o seizure preceding stroke |
tPA CI (3-4.5 hr) | >85 yo; NIH-SS >25; h/o both stroke/DM |
If pt not tPA candidate: Tx: | poss endovascular tx; MERCI clot retriever? |
Acute stroke mgmt | Temperature; Fluids/Glucose; BP; Antithrombotic agents |
Acute ischemic stroke: tx BP? | No (drop in MAP can drop CBF, make things worse) |
Secondary stroke prevention | Plt antiaggregants (ASA); Anticoagulants; BP; Lipid lowering; Endarterectomy |
Antihypertensives & stroke risk | each 10 mmHg drop in BP = 28% decrease stroke risk |
Action tremor: | Most common; arise when attempting to maintain a fixed position; frequency is 7-13 Hz; Essential or physiologic |
Essential tremor = | resembles slower freq physio tremor; essential or familial; common in later life; extremities, head (titubation), or voice |
Drug causes of physiologic tremor | Amphetamines, theophylline, lithium and valproate. |
Essential tremor Tx | Firstline: Beta-blocker (CI in COPD; asthma; DM; bradycardia; AV conduction probs); Primidone: effective but sedating; Tranquilizers (benzo) for anxiety related; EtOH: temporary suppression |
Ataxic (intention) tremor | Absent at rest/start of movement; dysmetria |
Ataxic tremor: Causes: | Dz of cerebellum / connections (MS; tumors; infarct; ethanol-induced cerebellar degeneration) |
Ataxic tremor: Tx: | Meds usu ineffective; Weights; surgical lesions of ventrolateral thalamus may be effective in severe cases |
Athetosis: | slow writhing purposeless movements usu involving hands, tongue & face; usu in kids w/ cerebral palsy or result of kernicterus or hypoxia (poss later in life d/t hypoxia) |
Chorea: Sx | Involuntary, irregular jerky movements; can cause continuous movements. |
Chorea: may be due to: | untreated strep infxn (Sydenham chorea), PG (chorea gravidarum) or Huntington dz |
Tics | onset 2-13 yrs; tx Haldol/pimozide |
Hemiballismus | Violent flinging movements; Unilateral hemiballismus usu d/t infarct in contralateral subthalamic nucleus of Luys |
Myoclonus = | Shock-like contraction of a group of mx; irreg in rhythm/amplitude; may involve restricted grp mx or be generalized. |
Myoclonus may result from: | anoxic damage, spinal cord injury, uremia, hepatic encephalopathy or other rare neuro syndromes |
Myoclonus Tx | Clonazepam, Valproate (Both have limited efficacy) |
Dystonia: | Maintenance of a persistent extreme posture in one or more joints. |
Generalized dystonia: | dystonia musculorum deformans; a rare hereditary dystonia |
Focal dystonias: | torticollis (dystonia of the neck), writer's cramp & blepharospasm (dystonia of the mx's involving eye closure). |
Dystonia: Tx | Botox injxn increasingly common & helpful; Surgical tx (torticollis) also available; Medical tx (benzos, anticholinergics, neuroleptics) generally unsatisfactory |
Parkinson dz: epi | 500,000 in US; 58 yo |
Parkinson dz: Sx | Rest tremor/3-6 hz; pill rolling; cogwheeling; rigidity; bradykinesia; difficulty initiating movement; masked facies; stooped posture, shuffling; disturbance of postural reflexes; diminished eye blinking rate |
Parkinson dz: Patho | Degen nigrostriatal pathway, raphe nuclei, locus ceruleus & motor nucleus of vagus; dramatic loss of DA-containing neurons |
L-DOPA | 80% of pts improve; Sinemet treats akinesia & is less effective in treating tremor. |
L-DOPA MOA | precursor of DA (which cannot cross BBB; L-DOPA can) |
Dopamine agonists MOA | Act like DA at DA receptor; may allow for reduction in dose of Sinemet required & may decrease on-off probs |
Amantidine MOA | May increase DA release from nerve terminals; weakly effective |
Anticholinergic drugs MOA: | May be more effective for tx of tremor; AE = confusion, constipation, dry mouth, urinary retention |
Selegiline MOA | inhibits monoamine oxidase type B |
Cabergoline MOA | inhibits COMT |
Huntington Dz presentation | AD, varying age of onset; Sx often begin w/ psych disorder (immaturity, impulsivity, depression); later apathy & dementia; Chorea, Athetosis; butterfly ventricles (caudate atrophy) |
Huntington chorea due to: | DA excess state |
Huntington Tx | DA receptor blockers such as neuroleptics (haloperidol, chlorpromazine); DA reserve depleters (reserpine; no longer used); future: tetrabenazine? Tx often unsatisfactory |
Wilson dz | Auto rec; onset 2nd-3rd decades; first sx: hepatic dysfn/cirrhosis; tremor/ bradykinesia, dysarthria, dysphagia; limbs rigid, facial mx fixed empty smile; arms: wing-beating tremor; Kayser-Fleischer (deep cornea) |
Wilson: labs | Serum ceruloplasmin (Cu carrying pro) very low; urine Cu high |
Wilson: patho | CNS changes: brownish discoloration of some basal ganglia; proliferation of protoplasmic astrocytes (Alzheimer Type II cells) |
Wilson: Tx | Should begin before neuro Sx onset; early tx prevents neuro sequela devt; eat low Cu foods; sulfurated potash with meals (prevent Cu absorption); chelator (d-penicillamine) to remove absorbed Cu |
Wilson dz: Tx: foods to avoid | liver; chocolate; mushrooms; shellfish; nuts |
Tardive dyskinesia: cause | Iatrogenic d/t LT neuroleptic tx (esp Haldol; also atypicals, poss metoclopramide, amphetamines, L-dopa); may be result of DA receptor supersensitivity |
Tardive dyskinesia: Sx | Abnormal BLM (tongue thrusting & chewing); head movements (turning & bobbing); poss also abnormal limb movements; Sx can fluctuate & may take mos-yrs to resolve (sometimes permanent) |
Tardive dyskinesia: Tx | stop the offending drug; many tx tried w/ marginal success |
Tardive dyskinesia: most successful tx: | achieved with DA-depleting agents (tetrabenazine); also Vitamin E? |
Excess DA results in: | dyskinesia and chorea |
Dopamine deficiencies cause: | Parkinsonian-like symptoms |
Action of acetylcholine & DA: | Act in opposite directions; adding DA is equivalent to blocking acetylcholine |
Migraine dx criteria | ≥2 of: (Unilateral; Pulsating; Mod/ severe intensity; fx: avoidance of routine physical activity); 1 of: (N/V; Photophobia an& phonophobia) |
5 phases of migraine | Prodrome; aura; HA; termination; postdrome |
Severe episodic HA with cerebellar sx = | basilar migraine |
Menstrual migraine tx | triptans given acutely; NSAIDs; OCP |
Chronic migraine: dx | CDH ≥3 months; >8 d/mo x 3 mo; |
Sinus HA vs migraine | sinus usu continuous (not intermittent); TTP over sinuses; tx w/ Abx |
CDH | ≥ 15 d/mo; primary or secondary (usu considered primary); ≥1 migraine/wk = RF for dev CDH |
CTTH: | CDH meeting TTH criteria |
NDPH: | CDH dev within 3 days of sx onset, last ≥ 3 mo |
MOH = | CDH assoc w/regular overuse for >3 month of one or more acute meds; try bridge tx; initiate preventive agent as analgesic is withdrawn |
Med overuse indicators: | Simple analgesics: >3 d/wk; Triptans/ combo analgesics: >2 d/wk; Opioids/ergotamine: >2 d/wk |
Hemicrania continua | Daily, continuous, strictly unilateral primary HA; assoc w/ cranial autonomic features (miosis, ptosis, eyelid edema, lacrimation, nasal congestion or rhinorrhea) |
Hemicrania continua: dx & tx | dx: responds to indomethacin |
Paroxysmal hemicrania | ≥ 20 frequent attacks (2-30 min); Pain severe & strictly unilateral, orbital, supraorbital, or temporal; Parasymp ipsilateral activation; Responds only to indomethacin |
SUNCT | Short-lasting; Unilateral; Neuralgiform HA, with Conjunctival injection & Tearing; rare; M>F (>50 yo) |
SUNCT sx | burning, stabbing, throbbing; seconds to 4 min; 5-6 per hr; SBP may rise |
Trigeminal autonomic cephalgias include: | SUNCT; cluster; trigeminal neuralgia |
Cluster HA | M>F (older than migraine); severe, unilateral, nasal congestion, injected conjunctiva, ipsilateral sweating; at night (wakes pt) |
HA red flags (SNOOP) | Systemic sx; secondary RF; neuro sx; onset sudden; older pt; Progression/prior HA hx; Pattern changes |
Neuroimaging not needed when: | No focal neuro findings; Pt has stable pattern of recurrent HA; No h/o seizures |
Consider neuroimaging when: | Neuro exam abnormal; progressively worsening HA; new persistent HA; new, rapid onset HA (thunderclap headache); HA does not respond to standard tx |
Thunderclap HA (non-SAH) | dx after exclude SAH; peaks in 1 min, lasts 1 hr-10 days |
Migraine tx considerations | Pt age; current health status; coexistent illnesses; migraine type |
Use LP only after: | normal CT obtained & platelet count is normal |
LP should be performed if: | Neuroimaging is normal or suggests dz that must be dx by measuring cerebrospinal fluid (CSF) pressure, cell count, and chem |
Pt needs preventive med (as well as abortive) if: | >8 HA / month |
Defn of trigger | causes HA more than 50% of the time within 24 hr |
EEG 3/sec spike and wave = | Absence |
Most common cause of tonic-clonic in pts (onset < 30 yo) is: | idiopathic epilepsy |
Clonic seizures: | usu in childhood; impaired consciousness, followed by asymmetric bilateral jerking |
Most common seizure type: | complex partial |
complex partial: | 10-30 yo; common post-head trauma; 50% abnml CT/MRI; 50% mesial temporal sclerosis; 20% hamartoma |
complex partial: 30-60 yo: | brain tumor? |
complex partial: >60 yo: | stroke? |
Seizure: labs | Glucose; lytes; AED levels; LP if poss meningitis; EtOH/tox if susp; ABG if susp hypoxia; poss CXR, CT, MRI |
Status epilepticus: does not apply to: | continuous simple partial seizures |
EtOH withdrawal seizure: | onset 6-48 hr after last drink; often primary generalized, often have Todd's |
Tx after single seizure: | If a structural lesion or recognized abnormal EEG |
Remission: | usu within 3 yrs of first seizure; prolonged remission in 60% of such pts |
Factors against remission | FH; psych comorbid; febrile seizure hx; more seizures; age |
Awareness vs arousal | awareness: high level fn (resides diffusely in cerebral cortex); arousal: primitive (brainstem) |
For LOC to occur: | Both cerebral hemispheres damaged OR brainstem lesion |
Coma: causes | Cerebral infarction 10%; Cerebral hemorrhage 20%; Metabolic causes 50% (Drug ingestion >50%; Hypoglycemia 5-10%); Psych 2% |
Coma: sudden onset d/t: | Cardiac arrest; SAH; 2nd to aneurysm; Brainstem infarct or hemorrhage; Bicerebral hemispheric infarction |
Coma: onset over min-hrs d/t: | usually drug overdose; also hypoxia; hypoglycemia; SAH; hydrocephalus; AVM; meningitis; metab |
Head trauma sx | Battle (mastoid); raccoon eyes (orbital) CSF rhinorrhea/ otorrhea (basilar) |
Roth spots | sx of septic emboli; on funduscopic exam & btw toes |
Hollenhorst plaque | chol emboli from carotid |
Coma: sensation: may see: | Purposeful withdrawal bilaterally; absent response Unilaterally; facial Grimace; posturing |
Decorticate posturing: | hemispheric or diencephalic dysfn d/t destructive lesions or metabolic abnormality; hands come up (response to stimuli) but do not localize |
Decerebrate posturing: | midbrain or upper pons dysfunction on a structural or metabolic basis; wrists flex (response to stimuli), not localizing |
Cheynes-Stokes: | Bilateral hemispheric lesions; most commonly seen in non-neurologic disorders (CHF); crescendo-decrescendo |
Central neurogenic hyperventilation: | Commonly metabolic cause (Sepsis; DKA) |
Apneustic: | Rare, but usually associated with pontine infarction; pt breathes in, holds breath 15-20 sec, breathes out |
Ataxic breathing (Biot's respiration) | Damage to the medullary respiratory centers; breathing slows; long breathless pause; then inhales; this is often premorbid |
Most common reason for noting unreactive pupils: | an inadequate light source |
Reactive pupils = | midbrain is intact. |
Intact pupillary responses in unresponsive pt w/ absent EOM & corneal responses: | metabolic abnormality (e.g., hypoglycemia) or drug ingestion (e.g., barbiturate) |
Midposition (3-5mm) nonreactive pupils = | midbrain damage. |
Blown Pupil | unilaterally dilated, nonreactive pupil: sx of CN III (oculomotor nerve) compression (Aneurysm, Mass Lesion); dilated nonreactive pupil may also be caused by DM & some drugs (esp atropine, scopolamine) |
Small, reactive pupils: seen in: | pontine damage (infarct or hemorrhage) or with some drug use (opiates, pilocarpine). |
Bilateral midposition unreactive pupils: | hypothermia |
Eye deviation occurs in what direction? | toward a unilateral hemispheric lesion and away from a unilateral brainstem lesion. |
Functional testing of Eye Movements is done by: | oculo-cephalic reflex (Doll's head) or oculo-vestibular reflex (ice water calorics) |
Oculo-cephalic reflex (Doll's head): CI | if there is a question of cervical spine injury |
Oculo-cephalic reflex: Abnormal response = | absent or asymmetric eye movement: destructive lesion at midbrain or pontine level; poss also deep barbiturate poisoning |
Oculo-vestibular reflex: Normal response (conscious pt): | Tonic (sustained) deviation of eyes toward stimulated side, w/ quick phase of nystagmus toward the opposite side |
Oculo-vestibular reflex: Response in comatose pt w/ intact brainstem: | Tonic deviation of eyes, but no nystagmus |
Oculo-vestibular reflex: Response in comatose pt w/ brainstem dysfn: | Loss of tonic deviation w/ stimulation of one, or both ears; if there is no tonic deviation there can be no fast response |
Oculo-vestibular response does not: | distinguish between metabolic and structural causes of coma |
Corneal sensation: | carried by CN V (Trigeminal); test with cotton swab pressed gently onto cornea; abnormal reponse suggests a pontine lesion |
Coma: labs | GLUCOSE, lytes, renal, Ca, PO4, ABG, CBC, tox screen; CXR, imaging (after stabilization) |
GCS: 3 tests | Eye, verbal, motor; range: 3-15; <8 means coma |
GCS: eye | 1: no eye opening; 2: open in response to pain; 3: in response to voice; 4: open spontaneously |
GCS: verbal | 1: None; 2: Incomprehensible sounds; 3: Inappropriate words; 4: confused; 5: oriented |
GCS: Motor | 1: no response; 2: extension to pain; 3: flexion in response to pain; 4: withdraws from pain; 5: localizes to pain; 6: obeys commands |
Brain death | no purposeful movements, pupil responses, EOM, corneal reflexes; spont resp / movements; DTRs may be present |
Coma & sleep | Almost all coma pts will wake up to some degree; most develop a sleep-wake cycle |