Neurology Flashcards
(143 cards)
What is Lewy Body Dementia?
Dementia + >2 of the following: visual hallucinations, Parkinsonism, fluctuating cognition, and REM sleep behavior disorder; cognitive symptoms may precede or appear along with parkinsonian features; repeated falls and sleep disturbance are characteristic
Cognitive impairment would develop in this condition before or at the same time as Parkinsonism, not years later
Characterized by fluctuating cognition w/ pronounced variations in attention and alertness and bizarre visual hallucinations that are well formed and detailed
Cognitive decline, then Parkinsonism with severe sensitivity to potent dopamine antagonists
Micro: eosinophilic intracytoplasmic inclusion representing accumulation of alpha synuclein protein
Tx: carbidopa-levodopa for parkinsonism, cholinesterase inhibitors for cognitive impairment, 2nd generation antipyschotics if symptoms persist
What is Frontotemporal dementia?
Characterized by early personality changes (e.g., euphoria, disinhibition, apathy), compulsive behaviors (e.g., peculiar eating habits, hyper-orality) and impaired memory; behavioral changes precede memory impairment
Presents around ag 60, autosomal dominant, progresses rapidly compared to AZ
Tau proteins, Pick bodies
Visual-spatial functions usually remain intact
Frontotemporal atrophy on neuroimaging
Manifests around age 60, demonstrably early than AD
What is Myasthenia gravis?
Autoantibody-mediated degradation of Ach receptors at neuromuscular junction
Epidemiology: women - 2nd to 3rd decade, men - 6th to 8th decade
Involved site: Ach receptor in postsynaptic membrane
Features: fluctuating and fatigable muscle weakness that is worse later in the day - ocular (ptosis, diplopia), bulbar (dysphagia, dysarthria), facial, neck and limb muscles; respiratory muscles (myasthenic crisis)
Causes of exacerbation: medication (abx-fluoroquinolones, aminoglycosides; anesthetics-neuromuscular blocking agents; cardiac meds-beta blockers, procainamide; other-MgSulfate, penicillamine; tapering of immunosuppressive meds) pregnancy/childbirth; surgery (especially thymectomy); infection
Dx: bedside- edrophonium (tensilon) test, ice pack test (cold temp improves muscle strength by inhibiting breakdown of Ach at NMJ); Ach receptor antibodies (highly specific), CT scan of chest to evaluate for thymoma
Tx: AchE inhibitors (eg pyridostigmine), +/- immunotherapy (eg corticosteroids, azathioprine), thymectomy
Ptosis only. pupillary response is spared.
What is Lambert-Eaton syndrome?
Approx. 50% of cases are assoc. w/ an underlying malignancy, mostly small cell lung cancer
Involved site: presynaptic membrane voltage-gated calcium channels
Features: proximal muscle weakness (eg standing from a chair, combing hair, putting dishes in overhead cabinets), autonomic dysfunction (eg dry mouth), cranial nerve involvement (eg ptosis), diminished or absent deep-tendon reflexes
Tx: guanidine or 3,4-diaminopyridine to increase presynaptic Ach levels; refractory - IV Ig or oral immunosuppressants (eg corticosteroids, azathioprine)
What is dermatomyositis/polymyositis?
Involved site: muscle fiber injury
Features: symmetrical and more proximal muscle weakness; interstitial lung disease, esophageal dysmotility, Raynaud phenomenon; polyarthritis; skin findings (eg gottron papules, heliotrope rash) in dermatomyositis
Preserved reflexes, elevated muscle enzymes (eg creatine kinase)
What is a CT with contrast?
Useful in identifying structural abnormality or mass lesions (eg primary or metastatic cancer, abscess, infarct)
Not helpful for identifying intracranial hemorrhage.
What is a glucocorticoid-induced myopathy?
Complication of chronic corticosteroid use
Presentation: muscle atrophy and painless proximal muscle weakness, which is more prominent in the LE
No muscle inflammation or tenderness
Normal CK and ESR
Improves when offending med is discontinued
What is polymyalgia rheumatica?
Muscle pain and stiffness in shoulder and pelvic girdle; normal muscle strength
Tenderness w/ decreased ROM at shoulder, neck and hip
Responds rapidly to glucocorticoids
Normal CK
Elevated ESR
What is cerebral palsy?
Non-progressive motor dysfunction
RF: Prematurity*, low birth weight; premature infants are more likely to have periventricular leukomalacia (white matter necrosis from ischemia/infection) and intraventricular hemorrhage (germinal matrix bleeding due to fragile vasculature and unstable cerebral blood flow) assoc. w/ CP
Presentation: delayed motor milestones, hypertonia, hyperreflexia; comorbid seizures, intellectual disability; equinovarus deformity (feet point down and inward); commando crawl (commonly seen in CP due to contractures of the lower limbs)
Dx: MRI of brain, +/- EEG, +/- genetic/metabolic testing
Mgmt: physical, occupational, speech therapies; nutritional support, antispastic medications
What is acute angle closure glaucoma?
Due to sudden narrowing or closure of the anterior angle chamber
Typically occurs as a response to pupillary dilation from meds (eg anticholinergics such as tolterodine, trihexyphenidyl; sympathomimetics, and decongestants) or other stimuli (eg dim light)
Anticholinergics cause mydriasis, which may precipitate acute ACG
More common in women (esp >40yo), Asian and Inuit populations, individuals with farsightedness
Presentation: rapid onset of severe eye pain, halos around lights; affected eye will appear injected, pupil will be dilated and poorly responsive to light; tearing and headache w/ subsequent nausea and vomiting as IOP increases
Untreated acute ACG can lead to severe and permanent vision loss w/in 2-5h of symptom onset
What is Guillain-Barré syndrome?
Pathophys: immune-mediated demyelinating polyneuropathy of peripheral nerve fibers, preceding GI (campylobacter) or respiratory infection Presentation: paresthesia, neuropathic pain; symmetric, ascending weakness; decreased/absent deep-tendon reflexes; autonomic dysfunction (eg arrhythmia, ileus, orthostatic hypotension, urinary retention, lack of sweating); respiratory compromise Bulbar sxs (dysarthria) Dx: clinical; supportive findings (CSF fluid: albuminocytologic dissociation = increased protein, NORMAL WBCs; abnormal electromyography and nerve conduction) Mgmt: monitor autonomic and respiratory function; IV Ig or plasmapheresis
What is CSF analysis normally?
OP: 100-180/200-300 mm H2O (8-15mm Hg)
WBC 0-5
Glucose 40-70
Protein <40
What is CSF analysis in bacterial meningitis?
WBC >1000 (PMNs)
Glucose <40
Protein >250
What is CSF analysis in tuberculous meningitis?
Similar to bacterial CSF findings:
WBC 100-500
Glucose <45
Protein 100-500
What is CSF analysis in viral meningitis?
WBC 10-500
Glucose 40-70
Protein <150 (Normal to high)
What is CSF analysis in GBS?
WBC 0-5
Glucose 40-70
Protein 45-1000
What is Lesch-Nyhan syndrome?
Pathophys: X-linked recessive, deficiency of hypoxanthine-guanine phophoribosyltransferase, hypoxanthine and uric acid accumulation
Presentation: delayed milestones and hypotonia in infancy; early childhood - intellectual disability, extrapyramidal sxs (dystonia, chorea), pyramidal sxs (spasticity, hyperreflexia), self-mutilation; biting fingers and tongue, writhing movements, neprholithiasis; gouty arthritis in late, untreated disease
What is subclavian steal syndrome?
Due to stenosis or occlusion of proximal subclavian artery, leading to reversal of blood flow in ipsilateral vertebral artery
Pts are often asymptomatic but may have sxs of upper extremity ischemia (eg pain, fatigue, paresthesias) or vertebrobasilar insufficiency (eg dizziness, ataxia, dysequilibrium) that are worsened by upper extremity exercise
Lower brachial SBP (>15mm Hg) in affected arm, systolic bruit in supraclavicular fossa on affected side
4th heart sound may be present due to LVH from systolic HTN
What is cavernous sinus thrombosis?
B/c facial/ophthalmic venous system is valveless, uncontrolled infection of the skin, sinuses, and orbit can spread to the cavernous sinus
Headache = most common symptom; low grade fever and periorbital edema usually occur several days later secondary to impaired venous flow in the orbital veins
Vomiting = result of intracranial HTN and fundoscopy may reveal papilledema
Unilateral sxs of HA, binocular palsies, periorbital edema, hypoesthesia, or hyperesthesia can rapidly become bilateral b/c CN 3, 4, 5.1, 6 pass through cavernous sinus
What is Sturge Weber syndrome?
Neurocutaneous disorder characterized by capillary malformation in CN 5.1, 2 distribution
Pathophys: mutation in GNAQ gene
Presentation: Port wine stain (CN 5.1, 2 distribution), leptomeningeal capillary-venous malformation, seizures +/- hemiparesis, intellectual disability, visual field defects, glaucoma (common)
Dx: MRI of brain w/ contrast
Mgmt: laser therapy to reduce appearance of port wine stain, antiepileptic drugs, IOP reduction
What is infant botulism?
Pathogenesis: ingestion of c. botulinum spores from environmental dust
Highest in California, Utah, and Pennsylvania
Ingestion of raw honey is also a risk factor
Presentation: descending flaccid paralysis; bulbar palsies (ptosis, sluggish pupillary response to light, poor suck and gag reflexes), constipation and drooling
Treatment: human-derived botulism immune globulin
What is foodborne botulism?
Pathogenesis: ingestion of preformed c. botulinum toxin
Presentation: descending flaccid paralysis
Treatment: equine-derived botulism antitoxin
What is CNS lymphoma in an HIV patient?
Presence of EBV DNA in the CSF is specific for this condition
Dx: MRI reveals a weakly ring-enhancing mass that is usually solitary and periventricular
What is psychogenic nonepileptic seizure?
A type of conversion disorder commonly misdiagnosed as a seizure disorder.
Presentation: forceful eye closure, side to side head or body movements, rapid alerting or reorienting, memory recall of the event, lack of post-ictal confusion
Not assoc. w/ abnormal cortical activity
Dx: video-EEG (gold standard) demonstrating lack of epileptiform activity