Neuro Flashcards

Question

Frontotemporal Dementia (Pick’s Disease) definition, sx, dx, tx

Answer 1

Localized brain degeneration of the frontotemporal lobes May progress globally sx Marked changes in social behavior, personality, & language (aphasia) are early signs Eventual executive & memory dysfunction (dementia w/ advanced disease) – onset earlier than Alzheimer’s (6th decade) Behavioral changes: disinhibition or socially inappropriate behaviors, apathy, hyperorality (binge-eating, changes in food preferences, putting large amounts of food in their mouths), compulsive ritualistic behaviors, loss of sympathy/empathy dx Histology: pick bodies – round or oval aggregates of Tau protein seen on silver-staining of the cortex

Answer 2

Progressive dementia characterized by the diffuse presence of Lewy bodies (abnormal neuronal protein deposits) in comparison to Parkinson disease, where the Lewy bodies are localized sx Core features that occur early: visual hallucinations, episodic delirium (cognitive fluctuations), Parkinsonism & REM sleep disorder Dementia late finding Delusions, sensitivity to antipsychotic drugs, autonomic dysfunction (orthostatic hypotension) dx Histology: cortical Lewy bodies (abnormal deposition of alpha-synuclein proteins) tx of Parkinsonism sxs may worsen the neuropsychiatric sxs & vice versa

Answer 3

Autosomal dominant inherited d/o of unknown etiology Incidence increases w/ age sx Intentional tremor: postural bilateral action tremor MC affecting the upper extremities & head (hands, forearms, head, neck, or voice) *worsened w/ intentional movement & adrenergic activity (emotional stress, anxiety) *improved w/ ETOH; slight improvement w/ rest PE: *on finger to nose testing, tremor increase at the end of approaching the target or holding a position against gravity *no other significant neurological findings (cogwheel phenomenon may be seen in some) dx Dx of exclusion based on hx, family hx, & physical after r/o other causes tx Treatment usually not needed Propranolol may help if severe or situational Second line: primidone Third line: alprazolam Refractory: thalamotomy

Answer 4

Acquired autoimmune demyelinating polyradiculopathy of the peripheral nervous system PATHO: autoantibody attacks the myelin sheath of the peripheral nerves after an infection Etiologies: *increased incidence w/ Campylobacter jejuni (MC) or other antecedent GI or respiratory infections *CMV, EBV, HIV, mycoplasma infections, immunizations, post-surgical sx *symmetric ascending weakness & sensory changes (paresthesias, pain) – distal lower extremities first *may develop weakness of the respiratory muscles & bulbar muscles (swallowing difficulties) PE: *LMN signs: ↓ DTRs, flaccid paralysis, weakness *sensory deficits; cranial nerve palsies (CN VII) *autonomic dysfunction: tachycardia, arrhythmias, hypotension or HTN, breathing difficulties dx Electrophysiologic studies *decreased motor nerve conduction velocities & amplitude CSF analysis *high protein w/ a normal WBC count PFTs tx *plasmapheresis, IVIG first line *mechanical ventilation

Answer 5

Autosomal dominant neurodegenerative disorder PATHO: *trinucleotide repeats on chromosome 4 sx 3 hallmark manifestations: mood, movement, memory *behavioral and mood changes *chorea (rapid involuntary movements) *dementia *gait abnormalities, ataxia, incontinence, facial grimacing dx Clinical sxs + family hx + genetic confirmation CT/MRI *cerebral & striatal (caudate nucleus & putamen) atrophy tx No cure – usually fatal within 15-20yrs Tetrabenazine for dyskinesia or chorea Typical & atypical antipsychotics BDZs

Answer 6

~1/3 of all primary intracranial neoplasms are glial cell origin & 1/3 are meningiomas: Some other types of primary tumors are vestibular schwannomas, pituitary adenomas, neurofibromas, & CNS lymphomas Most glial cell tumors are malignant & they can be divided into one of several types Astrocytoma – comes in 4 grades w/ worsening prognosis Grade 4 – Glioblastoma – MC primary malignant brain tumor; poor prognosis Ependymoma – primary glial cell tumor that occurs in the ependymal cells that line the ventricles & spinal canal Medulloblastoma – MC primary malignant brain tumor in children; long-term survival w/ treatment is about 70% MC sources of intracranial metastasis 🡪 lung, breast, GI tract – these metastatic brain tumors are 3x MC than primary intracranial tumors MC CNS tumors in children 1) Astrocytomas 2) Medulloblastomas 3) Ependymomas MC CNS tumors in adults Gliomas – particularly glioblastoma, anaplastic astrocytoma, low-grade astrocytoma, pilocytic astrocytoma, oligodendroglioma Meningiomas Pituitary adenoma Oligodendroglioma (relative rare) Hemangioblastoma Schwannomas Primary CNS lymphomas MC manifestation 🡪 increased ICP HA, N/V, seizures Irritability, lethargy, changes in behavior Gait & balance disorders

Answer 7

*Systemic viral infection within the CNS restricted to the meninges, ependyma, & subarachnoid space MC Etiologies: enteroviruses (esp. Coxsackievirus B & Echovirus), HSV-2, HIV Less Common: mumps, VZV, lymphocytic choriomeningitis virus *Acute onset *Less severe than bacterial, usually w/o alterations in consciousness sx Cardinal SXS: fever, HA, nuchal rigidity *general malaise, myalgia, N/V, photophobia, diarrhea, rash PE: absence of abnormal neurologic findings +/- transiently increased DTRs Clinical Considerations by Viral Cause: Enteroviruses: summer, fall (MCC overall) 1) Echovirus: maculopapular rash 2) Coxsackie B: myocarditis, pericarditis, pleurodynia HIV: year-round, mono-like syndrome HSV-2: year-round, +/- polyradiculitis dx CSF pattern: lymphocytic pleocytosis, mildly elevated protein, normal glucose Other: viral PCR, culture to r/o bacterial LP: opening pressure: normal appearance: normal WBC: 10-500 glucose: normal or slightly decreased protein: normal or slightly increased tx Self-limited, only supportive tx *analgesics, antiemetics, IVF HIV: antiretroviral therapy HSV: acyclovir

Answer 8

MCC: S. pneumoniae (gram+ diplococci), Neisseria meningitides (gram- diplococci), Haemophilus influenzae B (Hib) *Acute onset sx Classic SXS: severe HA, fever, stiff neck (nuchal rigidity), AMS *Almost all pts will have 2/4 classic sxs Other sxs: N/V, photophobia Infants: fever, irritability, poor feeding, bulging fontanelles *generally do not have stiff neck +Kernig: inability/reluctance to allow full knee extension when hip flexed 90o +Brudzinski: spontaneous hip flexion during passive neck flexion N. meningitides: petechial rash on trunk, legs, & mucous membranes** dx CT before LP if: immunocompromised, hx of CNS disease, new-onset seizure, papilledema, abnormal level of consciousness, focal neurological deficit CSF pattern: neutrophilic pleocytosis, elevated protein, low glucose Other: CSF gram stain & culture, PCR LP: opening pressure: increased at 18-30 appearance: cloudy WBC: >1000, heavily increases glucose: <40 heavily decreased protein: increased >200 tx Everyone *vancomycin + (ceftriaxone or cefotaxime) Empiric ABX: <1mo: ampicillin + gentamicin >50y: vancomycin + ampicillin + 3rd gen cephalosporin (ceftriaxone or cefotaxime)

Answer 9

*Hematogenous spread of infection from lungs or LN to brain parenchyma, forming small tubercules that rupture into the subarachnoid space/ventricles in the initial weeks following airborne acquisition *Subacute onset MC Complications: stroke, seizures, hydrocephalus, hyponatremia, vision loss, transverse myelitis sx Early Prodromal Phase, 1-3wks: insidious onset of malaise, fatigue, HA, low-grade fever, & personality change Meningitic Phase: typical meningitis sxs + protracted HA, vomiting, lethargy, confusion, varying degrees of cranial nerve palsies & long-tract signs (spasticity, ↑ reflexes, +Babinski) Paralytic Phase: occurs later as pace of illness rapidly quickens *confusion leads to stupor, coma, seizures, & often hemiparesis *death within 5-8wks if untreated dx DX: CSF NAAT, AFB smear/culture, LAM antigen CT/MRI: +/- hydrocephalus, basilar exudates, periventricular infarcts, parenchymal tuberculomas LP opening pressure: increased 18-30 tx Intensive Phase: RIP + 4th agent x2mo *Rifampin *Isoniazid *Pyrazinamide Options for 4th agent: ethambutol, streptomycin, levofloxacin, ethionamide Continuation Phase: *Rifampin + Isoniazid x7-10mo Adjunctive steroids x6-8wks w/ taper *Dexamethasone or prednisolone

Answer 10

**IMMUNOSUPPRESSION MCC: Cryptococcus neoformans *Opportunistic infection in HIV pts, CD4+ <200 *Insidious onset *Meningoencephalitis (brain parenchyma involved) sx MC: HA (develops over 1-2wks), almost always present *Meningeal signs, fever, & AMS may be minimal or absent Other s/sxs: cough, dyspnea, skin rash, visual/hearing loss Meningeal inflammation can lead to cranial neuropathies, deep infarcts, & communicating hydrocephalus which may cause rapid changes in mental status (often improve w/ large-volume LP to relieve ICP), may require ventricular shunt DX: CSF culture, antigen (CrAg) India ink stain: round encapsulated yeast MRI/CT: hydrocephalus common LP: opening pressure: increased 18-30 appearance: clear or turbid WBC: <50 glucose: decreased or normal protein: normal or increased tx Induction Therapy: *Amphotericin B + Flucytosine x2wks Consolidation Therapy: *Fluconazole 400-800mg QD x8wks Maintenance Therapy: *Fluconazole 200mg QD long-term

Answer 11

*autoimmune, inflammatory demyelinating disease of the CNS of idiopathic origin *associated w/ axon degeneration of white matter (brain & spinal cord) *MC in women & young adults 20-40yrs, colder climates; associated w/ HLA-DR2 3 MAIN TYPES: *Relapsing-Remitting (MC!): episodic exacerbations *Progressive Disease: progressive decline w/o acute exacerbations *Secondary Progressive: relapsing-remitting pattern that becomes progressive sx Sensory disturbances: *pain, paresthesias Motor deficits: *weakness, gait & balance problems Visual disturbances: *diplopia, optic neuritis *trigeminal neuralgia Uhthoff’s Phenomenon 🡪 worsening of sxs w/ heat PE: Upper motor neuron signs *spasticity, upward Babinski, hyperreflexia, muscle rigidity Lhermitte’s sign 🡪 neck flexion causes lightening-shock type pain radiating from the spine down the leg Cerebellar: Charcot’s neurologic triad *nystagmus, staccato speech, intentional tremor Spinal cord sxs: bladder, bowel, or sexual dysfunction dx Mainly clinical – at least 2 distinct episodes of CNS deficits MRI w/ gadolinium *hyperintense white matter plaques Lumbar puncture *↑ IgG & oligoclonal bands tx Acute exacerbations: *IV glucocorticoids *plasmapheresis if unresponsive Prevention of relapse & progression: *Beta-interferon, Glatiramer

Answer 12

*autoimmune peripheral nerve disorder due to autoantibodies against the acetylcholine receptor on the muscles, leading to weakness *strong association w/ an abnormal thymus gland (e.g., hyperplasia or thymoma) 75%; HLA-B8 & DR3 PATHO: autoantibodies against acetylcholine postsynaptic receptor at the neuromuscular junction cause decreased skeletal muscle neuromuscular transmission w/ muscle recovery after a period of rest sx Ocular weakness Generalized weakness Weakness is worsened w/ repeated muscle use & throughout the day *diplopia & ptosis *bulbar muscle weakness *respiratory muscle weakness May lead to respiratory failure = myasthenic crisis dx *acetylcholine receptor antibodies initial test of choice *electrophysiology testing *chest imaging (CXR, CT, MRI) 🡪 thymus gland *Edrophonium (Tensilon) test: improvement after admin *ice pack test: improvement of ocular sxs after ice pack admin for 10min tx Long term 🡪 pyridostigmine or neostigmine first line Myasthenic crisis or severe: *plasmapheresis *IVIG Thymectomy

Answer 13

Movement d/o due to idiopathic loss of dopaminergic neurons in the substantia nigra PATHO: - loss of dopaminergic neurons leads to failure of acetylcholine inhibition in the basal ganglia (acetylcholine is an excitatory CNS NT, dopamine is inhibitory) - also affects dopamine’s ability to initiate movement Onset 45-65yrs MC dx Triad: resting tremor + bradykinesia + muscle rigidity Resting tremor: often the first symptoms; “pill rolling” tremor of the hand *worse at rest & w/ emotional stress *improves w/ voluntary activity, intentional movement, & sleep *usually confined to 1 limb or 1 side for years before it becomes generalized Bradykinesia: slowness of voluntary movement & ↓ automatic movements (e.g., lack of swinging of arms while walking, shuffling gait) Rigidity: ↑ resistance to passive movement (cogwheel, flexed posture) *festination = increasing speed while walking Face involvement: relatively immobile face (fixed facial expressions), widened palpebral fissure *Myerson’s sign: tapping the bridge of the nose repetitively causes a sustained blink Postural instability: usually a late finding; pull test – standing behind the pt & pulling the shoulders causes the pt to fall or take steps backwards dx Clinical Post-mortem histology: cytoplasmic inclusions (Lewy bodies) & loss of pigment cells seen in the substantia nigra tx Levodopa-carbidopa – most effective tx Dopamine agonist: bromocriptine, pramipexole, ropinirole Anticholinergics (trihexyphenidyl, benztropine) Amantadine – increases dopamine MAOB inhibitors (selegiline, rasagiline) COMT inhibitors (entacapone, tolcapone) Deep brain stimulation is extremely effective for rigidity & tremors in select pts

Answer 14

MOA: *converted to dopamine once it crosses BBB *carbidopa reduces amount of levodopa needed & also reduces peripheral conversion of levodopa into dopamine, reducing the adverse effects of levodopa Indications: most effective tx for sxs of PD

Answer 15

MOA: directly stimulates dopamine receptors; less motor adverse effects than levodopa but not as effective Indications: can be used as first ling agents in younger pts (<65yrs) to delay the use of levodopa

Answer 16

MOA: anticholinergic (antimuscarinic) that blocks the excitatory effects of acetylcholine Indications: most useful as monotherapy in younger pts (<70yrs) w/ tremor as the predominant sxs (w/o significant bradykinesia or gait disturbance) - does not improve bradykinesia - adjunctive tx for severe tremor despite levodopa or dopamine agonists

Answer 17

MOA: increases presynaptic dopamine release & inhibits dopamine reuptake Indications: low-potency antiparkinsonian therapy that can help early on w/ mild sxs - improves long-term levodopa-induced dyskinesia

Answer 18

Focal (Partial): abnormal neuronal discharge from one discrete section of one hemisphere Generalized: simultaneous neuronal discharge of both hemispheres (diffuse brain involvement) Status Epilepticus: seizure lasting ≥5min or >1 seizure within a 5min period w/o recovery between episodes Auras: sensory/autonomic/motor sxs of which the pt is aware of; may precede/accompany or follow seizures Automatisms: lip smacking, manual picking, patting, coordinated motor movements Type I: Focal Seizures 1. w/ Retained Awareness (Simple) *no alteration in consciousness 2. w/ Loss of Awareness (Complex) *altered consciousness, automatisms (lip-smacking) *postictal state: confusion & loss of memory Type II: Generalized Seizures 1. Absence (Petit Mal) *brief lapse of consciousness, staring episodes w/ pauses *eyelid twitching, lip smacking; NO POSTICTAL 2. Atonic (Drop Attacks) *sudden loss of muscle tone 3. Tonic *extreme rigidity 🡪 LOC 4. Clonic *repetitive rhythmic jerking; often associated w/ postictal state 5. Myoclonic *no LOC *sudden, brief, sporadic involuntary twitching 6. Tonic Clonic (Grand Mal): LOC 🡪 tonic 🡪 clonic 🡪 postictal *Tonic Phase: LOC 🡪 rigidity (usually <60sec) 🡪 clonic phase *Clonic Phase: repetitive, rhythmic jerking (lasts <2-3min) *Postictal Phase: flaccid coma/sleep; variable duration

Answer 19

Status Epilepticus: seizure lasting ≥5min or >1 seizure within a 5min period w/o recovery between episodes Etiologies: *structural abnormalities *infections (meningitis, encephalitis) *metabolic abnormalities *medications *toxins Diagnostics: neuroimaging once stabilized Management: *place pt in left lateral decubitus position *BDZs first line (lorazepam) *second line: phenytoin or fosphenytoin *third line: phenobarbital (refractory)

Answer 20

Labs: CBC, electrolytes, glucose, renal & liver function, RPR *↑ prolactin & lactic acid immediately after seizures CT 🡪 first seizure EEG 🡪 BEST TEST Management: *absence (Petit Mal): Ethosuximide 1st line; valproic acid 2nd line *Grand Mal: valproic acid, phenytoin, carbamazepine, lamotrigine *Myoclonic: valproic acid, clonazepam *Febrile: phenobarbital

Answer 21

Reflex-Mediated: *vasovagal *situational: micturition, defecation, swallowing, coughing *carotid sinus syndrome Orthostatic: *medication-related: diuretics, vasodilators, antidepressants *volume depletion: hemorrhage, GI loss *autonomic failure Cardiac: *tachyarrhythmias Ventricular tachycardia Supraventricular tachycardia *bradyarrhythmias Sinus node dysfunction AV block *structural disease Aortic stenosis, hypertrophic cardiomyopathy Cardiac tamponade, prosthetic valve dysfunction Congenital coronary abnormalities, cardiac mass/tumor *cardiopulmonary/vascular Pulmonary embolus Severe pulmonary HTN Aortic dissection

Neuro Flashcards

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