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What is Dementia / Neurocognitive Disorders?
A progressive, acquired decline in one or more cognitive domains (memory, executive function, language, visuospatial) severe enough to impair independent daily functioning, without altered consciousness.
What is the most common cause of dementia and its hallmark pathology?
Alzheimer disease; characterized by beta-amyloid plaques and neurofibrillary tau tangles with early short-term memory loss.
How does vascular dementia typically present compared to Alzheimer disease?
Stepwise decline with focal neurologic deficits and a history of cerebrovascular disease, rather than the gradual, smooth progression of Alzheimer.
What features distinguish dementia with Lewy bodies?
Fluctuating cognition, recurrent visual hallucinations, spontaneous parkinsonism, and REM sleep behavior disorder; antipsychotics cause severe sensitivity reactions.
What characterizes frontotemporal dementia (Pick disease)?
Early personality/behavioral changes and language dysfunction with relatively preserved memory, typically presenting before age 65.
What are first-line pharmacologic treatments for Alzheimer dementia?
Cholinesterase inhibitors (donepezil, rivastigmine, galantamine) for mild-moderate disease; add memantine (NMDA antagonist) for moderate-severe disease.
What reversible causes of cognitive decline should be excluded in a dementia workup?
B12 deficiency, hypothyroidism, neurosyphilis, normal pressure hydrocephalus, depression (pseudodementia), and medication effects.
What is Delirium?
An acute, fluctuating disturbance of attention and awareness caused by an underlying medical condition, representing a medical emergency.
What are the cardinal features distinguishing delirium from dementia?
Delirium has acute onset, fluctuating course, impaired attention, and altered level of consciousness; dementia is chronic with preserved alertness early.
What are common precipitating causes of delirium (mnemonic-friendly)?
Infection (especially UTI/pneumonia), medications/anticholinergics, metabolic derangements, hypoxia, dehydration, urinary retention/constipation, and substance withdrawal.
What is the cornerstone of delirium management?
Identify and treat the underlying cause, reorientation, sleep-wake normalization, and removal of offending medications; antipsychotics only for severe agitation.
Which delirium subtype is most often missed and carries worse prognosis?
Hypoactive delirium, presenting with lethargy and decreased responsiveness rather than agitation.
What is Multiple Sclerosis (MS)?
A chronic autoimmune demyelinating disease of the CNS characterized by lesions disseminated in space and time.
What is the classic demographic and presentation of MS?
Young women (20-40) with relapsing-remitting neurologic deficits such as optic neuritis, internuclear ophthalmoplegia, sensory changes, and weakness.
What is the most common initial presenting symptom of MS?
Optic neuritis—painful monocular vision loss with an afferent pupillary defect.
What is the diagnostic gold standard imaging finding in MS?
MRI showing periventricular white matter plaques (Dawson fingers); CSF may show oligoclonal bands and elevated IgG.
What is Uhthoff phenomenon and Lhermitte sign in MS?
Uhthoff: worsening symptoms with heat/exercise; Lhermitte: electric shock sensation down the spine with neck flexion.
How are acute MS exacerbations treated?
High-dose IV corticosteroids (methylprednisolone); plasma exchange for steroid-refractory severe relapses.
What disease-modifying therapies are used for long-term MS management?
Interferon-beta, glatiramer acetate, natalizumab, ocrelizumab, fingolimod, and dimethyl fumarate to reduce relapse frequency.
What is Myasthenia Gravis?
An autoimmune disorder caused by antibodies against postsynaptic acetylcholine receptors at the neuromuscular junction, producing fatigable muscle weakness.
What is the hallmark clinical feature of myasthenia gravis?
Fatigable weakness that worsens with activity/throughout the day, classically with ptosis and diplopia (ocular symptoms first).
What antibody and imaging studies confirm myasthenia gravis?
Anti-acetylcholine receptor antibodies (most specific); anti-MuSK antibodies; chest CT/MRI to evaluate for thymoma.
What bedside and electrodiagnostic tests support myasthenia gravis?
Ice pack test improving ptosis; repetitive nerve stimulation showing decremental response; single-fiber EMG (most sensitive).
What is first-line symptomatic treatment for myasthenia gravis?
Pyridostigmine (acetylcholinesterase inhibitor); immunosuppression (steroids, azathioprine) and thymectomy for definitive management.
What is a myasthenic crisis and how is it treated?
Respiratory failure from severe weakness; treat with IVIG or plasmapheresis plus respiratory support—not cholinesterase inhibitors acutely.
How is myasthenia gravis distinguished from Lambert-Eaton syndrome?
Myasthenia worsens with use; Lambert-Eaton (associated with small cell lung cancer) improves with repeated use and shows incremental EMG response.
What is Parkinson Disease?
A progressive neurodegenerative disorder from dopaminergic neuron loss in the substantia nigra, producing a characteristic movement disorder.
What is the classic clinical tetrad of Parkinson disease (mnemonic: TRAP)?
Resting Tremor (pill-rolling), Rigidity (cogwheel), Akinesia/bradykinesia, and Postural instability.
How does the Parkinsonian tremor differ from essential tremor?
Parkinson tremor occurs at rest and improves with movement; essential tremor occurs with action/posture and improves at rest.
What non-motor features are common in Parkinson disease?
Masked facies, micrographia, shuffling gait, anosmia, constipation, REM sleep behavior disorder, and depression.
What is first-line pharmacologic treatment for Parkinson disease?
Carbidopa-levodopa (most effective); dopamine agonists (pramipexole, ropinirole) often preferred in younger patients to delay levodopa.
What is a feared long-term complication of levodopa therapy?
Dyskinesias and motor fluctuations ("on-off" phenomenon) with chronic use.
What is Essential Tremor?
A common movement disorder characterized by a bilateral action/postural tremor, often familial, that improves with alcohol.
How does essential tremor classically present?
Symmetric tremor of the hands worsened by sustained posture or action, frequently involving the head and voice, with a positive family history.
What is the first-line treatment for essential tremor?
Propranolol (beta-blocker) or primidone (anticonvulsant).
What clue in the history strongly suggests essential tremor over Parkinson?
Transient improvement of tremor with small amounts of alcohol and absence of bradykinesia or rigidity.
What is Amyotrophic Lateral Sclerosis (ALS)?
A progressive neurodegenerative disease causing combined upper and lower motor neuron degeneration with preserved sensation and cognition.
What is the hallmark combination of findings in ALS?
Both upper motor neuron signs (spasticity, hyperreflexia, Babinski) and lower motor neuron signs (atrophy, fasciculations, weakness) without sensory loss.
What functions are characteristically spared in ALS?
Sensation, extraocular movements, bowel/bladder function, and cognition (in most cases).
What is the only FDA-approved medication shown to prolong survival in ALS?
Riluzole (a glutamate inhibitor); edaravone may slow functional decline.
What is the usual cause of death in ALS?
Respiratory failure due to diaphragmatic and respiratory muscle weakness.
What is Migraine?
A primary recurrent headache disorder characterized by moderate-to-severe, often unilateral throbbing pain with associated features.
What are the characteristic features of migraine (mnemonic: POUND)?
Pulsatile quality, One-day duration (4-72 hrs), Unilateral, Nausea/vomiting, Disabling intensity; often with photophobia and phonophobia.
What distinguishes migraine with aura?
Reversible visual, sensory, or speech symptoms (e.g., scintillating scotoma) preceding or accompanying the headache.
What is the first-line abortive treatment for acute migraine?
NSAIDs or triptans (sumatriptan); antiemetics as adjuncts; triptans contraindicated in coronary/vascular disease.
When is migraine prophylaxis indicated and what agents are used?
When attacks are frequent/disabling; options include beta-blockers (propranolol), topiramate, amitriptyline, and CGRP antagonists.
What is Tension Headache?
The most common primary headache, presenting as a bilateral, band-like, non-pulsatile pressure without nausea or significant photophobia.
How does tension headache classically present?
Bilateral, mild-to-moderate, pressing/tightening "band-like" pain not aggravated by routine activity and lacking migrainous features.
What is the first-line treatment for tension headaches?
NSAIDs or acetaminophen for acute episodes; amitriptyline for prophylaxis of chronic tension headache.
What is Cluster Headache?
A primary headache disorder featuring severe, unilateral periorbital pain occurring in cyclical clusters with autonomic symptoms.
What are the classic features of cluster headache?
Excruciating unilateral periorbital pain lasting 15-180 minutes with ipsilateral lacrimation, rhinorrhea, ptosis, and miosis; patients are restless/agitated.
What is the demographic and timing pattern of cluster headache?
Middle-aged men, attacks occurring at the same time daily (often nocturnal), clustering over weeks with remission periods.
What is the acute treatment for cluster headache?
High-flow 100% oxygen and subcutaneous sumatriptan.
What is used for cluster headache prophylaxis?
Verapamil (first-line); prednisone as a bridge during cluster periods.
What is Idiopathic Intracranial Hypertension (IIH)?
Elevated intracranial pressure without an identifiable structural cause, classically in young obese women, risking vision loss.
What is the classic presentation of IIH?
Obese woman of childbearing age with headache, papilledema, transient visual obscurations, pulsatile tinnitus, and possible CN VI palsy.
What medications are associated with IIH?
Vitamin A/isotretinoin, tetracyclines, and growth hormone.
What diagnostic findings confirm IIH?
Normal neuroimaging (or empty sella/flattened globe) with elevated opening pressure on lumbar puncture and normal CSF composition.
What is the treatment for IIH?
Weight loss and acetazolamide (reduces CSF production); serial LPs or shunting/optic nerve sheath fenestration if vision is threatened.
What is Hydrocephalus?
An abnormal accumulation of cerebrospinal fluid within the ventricles causing ventricular enlargement and often increased intracranial pressure.
What is the difference between communicating and non-communicating hydrocephalus?
Communicating: impaired CSF reabsorption with open ventricular pathways; non-communicating (obstructive): blockage of CSF flow within the ventricular system.
What is the classic triad of normal pressure hydrocephalus (NPH)?
"Wet, wobbly, wacky"—urinary incontinence, gait apraxia (magnetic gait), and dementia; gait disturbance usually first.
How is NPH treated and what test predicts response?
Ventriculoperitoneal (VP) shunting; large-volume lumbar puncture improving gait predicts shunt responsiveness.
What is Brain Abscess / Epidural Abscess?
A focal, encapsulated collection of pus within the brain parenchyma or epidural space, usually from contiguous or hematogenous infectious spread.
What is the classic clinical triad of a brain abscess?
Headache, fever, and focal neurologic deficit; seizures and signs of increased ICP may also occur.
What imaging finding suggests a brain abscess?
A ring-enhancing lesion on contrast CT or MRI with surrounding edema.
What is the management of a brain abscess?
Empiric broad-spectrum antibiotics (e.g., vancomycin + metronidazole + ceftriaxone) plus surgical aspiration/drainage; avoid LP if mass effect.
What is the hallmark presentation of a spinal epidural abscess (classic triad)?
Back pain, fever, and neurologic deficit; Staphylococcus aureus is the most common organism and MRI with contrast is the diagnostic test of choice.
What is Encephalitis?
Inflammation of the brain parenchyma, most often viral, producing altered mental status with focal neurologic findings.
How does encephalitis differ clinically from meningitis?
Encephalitis prominently features altered mental status, seizures, and focal deficits (brain dysfunction), whereas meningitis features meningeal signs with preserved cognition early.
What is the most common cause of sporadic fatal encephalitis and its hallmark?
Herpes simplex virus (HSV-1), with characteristic temporal lobe involvement on MRI/EEG.
What is the treatment for suspected HSV encephalitis?
Empiric IV acyclovir started immediately—do not wait for confirmatory PCR.
What CSF findings are typical in viral encephalitis?
Lymphocytic pleocytosis, normal or mildly elevated protein, normal glucose, and positive viral PCR (e.g., HSV).
What is Meningitis?
Inflammation of the meninges surrounding the brain and spinal cord, most commonly from infectious causes.
What is the classic clinical triad of bacterial meningitis?
Fever, nuchal rigidity, and altered mental status; headache and photophobia are also common.
What are Kernig and Brudzinski signs?
Kernig: pain/resistance with knee extension while hip flexed; Brudzinski: involuntary hip/knee flexion when neck is flexed—both suggest meningeal irritation.
What are the most common bacterial causes of meningitis by age?
Neonates: Group B Strep, E. coli, Listeria; adults: Streptococcus pneumoniae and Neisseria meningitidis; elderly/immunocompromised: add Listeria.
What CSF findings indicate bacterial meningitis?
Elevated opening pressure, neutrophilic pleocytosis, high protein, and low glucose.
What is the empiric treatment for bacterial meningitis in adults?
Dexamethasone plus vancomycin and ceftriaxone; add ampicillin if Listeria is a concern; do not delay antibiotics for CT/LP.
What is Intracranial Neoplasms?
Primary or metastatic tumors within the cranial cavity that produce symptoms via mass effect, edema, or increased intracranial pressure.
What are common presenting symptoms of a brain tumor?
Progressive headache (worse in morning/with Valsalva), new-onset seizures, focal deficits, and signs of increased ICP (nausea, papilledema).
What is the most common source of brain metastases?
Lung cancer (most common overall); also breast, melanoma, renal, and colorectal—metastases are more common than primary brain tumors in adults.
What is the most common primary malignant brain tumor in adults and its prognosis?
Glioblastoma multiforme—aggressive with poor prognosis; shows a "butterfly" pattern crossing the corpus callosum.
What is the most common primary benign intracranial tumor in adults?
Meningioma—often asymptomatic, dural-based, and more common in women.
What is Guillain-Barré Syndrome (GBS)?
An acute immune-mediated demyelinating polyneuropathy causing ascending symmetric weakness, often following an infection.
What is the classic presentation of GBS?
Ascending symmetric weakness with areflexia, often 1-3 weeks after a respiratory or GI infection (notably Campylobacter jejuni).
What CSF finding is characteristic of GBS?
Albuminocytologic dissociation—elevated protein with normal white cell count.
What is the most life-threatening complication of GBS and how is it monitored?
Respiratory failure from diaphragmatic weakness and autonomic dysfunction; monitor with serial spirometry (forced vital capacity, negative inspiratory force).
What is the treatment for GBS?
IVIG or plasmapheresis; corticosteroids are NOT effective.
What is Bell Palsy / Neuralgia?
Bell palsy is an acute idiopathic unilateral facial nerve (CN VII) paralysis; trigeminal neuralgia is paroxysmal facial pain along CN V.
How is Bell palsy distinguished from a central (stroke) facial palsy?
Bell palsy (peripheral CN VII) affects the entire half of the face including the forehead; central lesions spare the forehead.
What is the treatment for Bell palsy?
Oral corticosteroids (prednisone) within 72 hours; add antivirals for severe cases; eye protection/lubrication to prevent corneal damage.
What characterizes trigeminal neuralgia and its first-line treatment?
Brief, severe, electric shock-like unilateral facial pain triggered by light touch/chewing; first-line treatment is carbamazepine.
What is Peripheral Neuropathy?
Damage to peripheral nerves causing sensory, motor, or autonomic dysfunction, classically in a length-dependent "stocking-glove" distribution.
What is the most common cause of peripheral neuropathy in developed countries?
Diabetes mellitus (distal symmetric polyneuropathy).
What are common reversible/treatable causes of peripheral neuropathy to screen for?
B12 deficiency, hypothyroidism, alcohol use, uremia, and medication toxicity (e.g., chemotherapy).
What medications treat painful diabetic neuropathy?
Gabapentin, pregabalin, duloxetine, or tricyclic antidepressants; plus optimizing glycemic control.
What is Mononeuropathies?
Dysfunction of a single peripheral nerve, most often from compression, entrapment, or trauma.
What is the most common mononeuropathy and its presentation?
Carpal tunnel syndrome (median nerve)—numbness/tingling in the thumb, index, middle, and radial half of the ring finger, worse at night.
What exam findings and tests support carpal tunnel syndrome?
Positive Tinel and Phalen signs, thenar atrophy in advanced cases; nerve conduction studies confirm the diagnosis.