Pance: Neurology 2

Front 1

Migraine description

Back 1

severe headache, most often unilateral associated w/ visual or sensory symptoms (aura)—present most often before the head pain

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Migraine epidemiology

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MC in ♀; strong genetic component (F/hx); onset: adolescent, early adult, perimenopause

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Migraine c/b

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hormonal, emotional/physical stress, lack of sleep, certain foods, ETOH, OCP, insomnia, barometric pressure change and hunger

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Migraine presentation

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unilateral, dull or throbbing head pain, anorexia, N/V; photophobia, phonophobia, blurred vision; aura (precedes episode); sx follow same pattern except side

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Migraine labs

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Clinical dx: tests determined by individual presentation (r/o other causes)

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Migraine imaging

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Not necessary w/ hx of migraine and nl neuro exam

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Migraine tx

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5 steps: (1) dx (2) assess disability (3) pt education:↑risk of stroke w/smoking & OCP use (4)individual mgmt (5) stratified care

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Migraine Rx

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Prophylaxis: BB, CCB, TCA, anticonvulsants; Abortive: (1)Selective serotonin receptor (5-HT1) agonists (triptans):Sumatriptan; (2)Ergot alkaloids: Dihydroergotamine (DHE-45, Migranal)

Front 9

Cluster HA description

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aka: histamine headache; Severe unilateral, periorbital HA; ♂>♀; onset: middle age ♂; occurs daily x several months then goes into remission

Front 10

Cluster HA triggers

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ETOH, stress, Glare, certain foods

Front 11

Cluster HA presentation

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ipsilateral nasal congestion, Rhinorrhea, lacrimation, redness of eye, Horner's syndrome

Front 12

Cluster HA Dx

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H&P; plus exclusion of intracranial pathology

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Cluster HA tx

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#1) Oxygen, MEDS: Sumatriptan(Imitrex), dihydgoergotamine (Migranal), butorphanol (stadol)

Front 14

Tension HA description

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Generalized HA described as band-like or vice-like pain

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Tension HA c/b

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Both muscular and psychogenic factors; chronic tension HA > 5 yrs d/t hippocampus atrophy resulting from chronic stress

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Tension HA presentation

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Pressing or tightening (nonpulsatile quality)band-like pain in neck & back of head, Frontal-occipital location, Bilateral and occipitonuchal or bifrontal pain, duration of 30 min to 7 days; photophobia; NO N/V; NEVER focal neurologic deficits

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Tension HA dx

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Clinical

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Tension HA tx

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High-flow oxygen, NSAIDs; hot or cold packs, massage, improvement of posture, trigger point injections, occipital nerve blocks, stretching, and relaxation techniques

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HEADACHE KEYS

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(1) MIGRAINE: ♀, unilateral; painful, throbbing, nausea, photophobia; (2) CLUSTER: ♂, unilateral, occurs daily for period of time > remission; (3) TENSION: neck & head pain, stress, band-like or vice-like
Seizure Disorders
Seizure description - transient disturbance of cerebral function d/t abnormal paroxysmal neuronal discharge in the brain c/b cortical hyperexcitability

Front 20

Epilepsy (recurrent seizure) Etiology

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Idiopathic or constitutional; onset: 5-20y/o (may start later); No specific cause identified

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SYMPTOMATIC Epilepsy c/b

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PEDIATRIC: congenital / perinatal injury; METABOLIC:ETOH, Uremia, Hypo/hyper-glycemia; TRAUMA; TUMOR & space occupying lesions; VASCULAR: elderly(≥60y); DEGENERATIVE: Alzheimer's; INFECTIOUS: HIV/AIDS, Meningitis, Herpes, Syphilis, Cerebral Cysticercosis

Front 22

Seizure onset

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5-20 y/o

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Seizure CATEGORIES

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(1) PARTIAL seizures: a)Simple and b) Complex; (2) GENERALIZED seizures: a) Absence (petit mal); b) Atypical absences; c) Myoclonic d) Tonic-clonic (grand mal); (3) Status Epilepticus

Front 24

PARTIAL Seizure KEY s/sx

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*AURA(transient abnormality); PARTIAL= restricted to part of one cerebral hemisphere a)SIMPLE partial: remain conscious/one limb tonic or clonic → may extend to other regions (Jacksonian March) and b) COMPLEX partial = impaired consciousness → may be preceded by/accompanied or f/b AURA

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Seizure Aura description

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perceptual disturbance: MC = motor, somatosensory, autonomic, psychic sx; AURA can spread through multiple regions continuously or discontinuously, on the same side or to both sides; VISUAL: bright light/blobs, scintillating scotoma, size/shape distortions; AUDITORY: buzzing, heightened hearing sensitivity, auditory hallucinations; OTHER: smells/tastes, Déjà-vu, nausea, numbness, weakness, sudden anxiety/fear/foreboding, overheating(diaphoretic); aphasia/slurred speech; confusion (forgetting how to do task/comprehend)

Front 26

Generalized Seizure classification

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Absence (petit mal):Typical or Atypical; Myoclonic seizures; Tonic-clonic (grand-mal)

Front 27

Absence (petit mal) Seizure KEY s/sx

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h/o staring spells, may miss words; not responsive during seizure; have no memory of what happened during the attack; generally unaware that seizure occurred; begins in childhood > stops by age 20 > can progress to other types of seizures

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Absence (petit mal) Seizure other s/sx

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may be clonic, tonic or atonic (loss of postural tone); autonomic components (enuresis); or accompanying automatisms

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Atypical absences Seizure KEY s/sx

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often occur in the setting of developmental delay or mental retardation; more GRADUAL ONSET AND TERMINATION than typical absence seizure; more MARKED CHANGES IN TONE may occur

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Myoclonic Seizure KEY s/sx

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Single or multiple myoclonic jerks

Front 31

Tonic-clonic (grand-mal)Seizure KEY s/sx

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(1)TONIC phase: Sudden loss of consciousness, rigidity and arrest of respiration (lasting < 1 min); (2) CLONIC phase: jerking occurs (2-3min); (3)Flaccid coma > consciousness > sleep: Variable duration

Front 32

Tonic-clonic (grand-mal)Seizure other s/sx

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may be accompanied by tongue biting, incontinence, aspiration; commonly f/b postictal confusion variable in duration

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Status Epilepticus Seizure description

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life-threatening neurologic disorder: acute, prolonged epileptic crisis

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Status Epilepticus Seizure c/b

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an exacerbation of a preexisting seizure disorder, the initial manifestation of a seizure disorder, or an insult other than a seizure disorder

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Atonic Seizure

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Drop attacks

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Seizure labs

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CBC, blood glucose, LFT, renal function

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Seizure imaging

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CT scan, MRI, EEG

Front 38

GENERALIZED/ABSENCE Seizure EEG

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generalized spikes w/ assoc slow waves

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PARTIAL-SIMPLE Seizure EEG

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focal rhythmic discharge at start of seizure / may have no ictal activity

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PARTIAL-COMPLEX Seizure EEG

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interictal spikes or spikes associated w/ slow waves in temporal or fronto-temporal areas

Front 41

GENERALIZED tonic clonic (grand mal) or PARTIAL (simple, complex) focal Seizure Tx

Back 41

phenytoin (Dilantin); carbamazepine(Tegretol); valproic acid (Depakote); phenobarbital;

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GENERALIZED absence Seizure Tx

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ethosuximide; valproic acid (Depakote); clonazepam (Klonopin)

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GENERALIZED myoclonic Seizure Tx

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valproic acid (Depakote); clonazepam (Klonopin)

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STATUS EPILEPTICUS Generalized convulsive disorder description

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a common, life-threatening neurologic disorder that is essentially an acute, prolonged epileptic crisis > can be an exacerbation of a preexisting seizure disorder, the initial manifestation of a seizure disorder, or an insult other than seizure disorder

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STATUS EPILEPTICUS Generalized convulsive disorder c/b

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seizures are sustained by excess excitation and reduced inhibition; Failure of inhibitory processes is thought to be the major mechanism leading to status epilepticus

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STATUS EPILEPTICUS Generalized convulsive disorder presentation

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Focal/unilateral paresthesias or numbness; Focal visual changes(flashing lights); Focal visual obscuration or focal colorful hallucinations; Olfactory or gustatory hallucinations; Atypical rising abdominal sensations

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STATUS EPILEPTICUS Generalized convulsive disorder labs

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blood culture & UA w/ fever; Lumbar puncture (after neuroimaging r/o cerebral herniation) if a CNS infxn suspected; CBC, blood glucose, LFT, renal function

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STATUS EPILEPTICUS Generalized convulsive disorder tx

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EMERGENCY: can cause permanent brain damage secondary to hyperthermia, circulatory collapse or excitotoxic neuronal damage > tx ABCs; manage hyperthermia; BREAK w/ lorazepam (Ativan) or diazepam (Valium), give phenytoin (Dilantin)

Front 49

Alzheimer's Dz description

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steady incurable, long course of progressive memory loss w/cognitive and behavioral impairment that markedly interferes with social and occupational functioning

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Alzheimer's Dz onset

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6th or 7th decade of life

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Words associated w/ Alzheimer's dz

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intracellular neruofibrillary tangles (beta-amyloid tau protein); extracellular neuritic plaques (senile plaques)

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Alzheimer's Dz c/b

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plaques develop in the hippocampus (helps to encode memories), and in other areas of the cerebral cortex used in thinking and making decisions; unknown if plaques cause AD or if a by-product of AD

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Alzheimer's Dz D/dx

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depression (MC elderly problem) can mimic early stage Alzheimer's (20% of cases > should be r/o)

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Alzheimer's Dz presentation

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short term memory loss; inability to learn & retain new information; language/word finding problems; repetitive statements, confusion w/location of familiar places, trouble handling money/paying bills/ADL; compromised judgment; respond to memory loss w/irritability, hostility and agitation; ↑anxiety, restlessness, agitation, anxiety, tearfulness, wandering(get lost), ↑risk for falls; (sundowners), hallucinations, delusions, suspiciousness, paranoia

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Alzheimer's Dz ENDSTAGE presentation

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totally incontinent, unable to swallow & eat, coma, death, usually from infection

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Alzheimer's Dz labs

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to r/o other causes of dementia: CBC, electrolytes, SMA, thyroid function, B12, folate, VDRL, UA

Front 57

Alzheimer's Dz imaging

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CT or MRI to r/o treatable causes of progressive cognitive decline (chronic subdural hematoma/normal-pressure hydrocephalus) or if H&P suggests a mass

Front 58

Alzheimer's Dz dx

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clinical H&P(usually at mild stage using s/sx); Folstein Mini-Mental Status Examination; Barthel scale for activities of daily living (85% dx w/H&P and standard neuro exam)

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Alzheimer's Dz prevention

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no proven modalities: healthy lifestyles can reduce risk = exercise & diet (Mediterranean)

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Alzheimer's Dz tx

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Drugs that enhance cholinergic neurotransmission: Aricept (donepezil), Cognex(tacrine), Exelon (rivastigmine), Razadyne (galantamine) NOTE: anticholinergic drugs may cause confusion
Movement Disorders
Multiple Sclerosis description - immune-mediated inflammatory disease that attacks myelinated axons in the central nervous system, destroying the myelin and the axon in variable degrees and producing significant physical disability within 20-25 years

Front 61

Multiple Sclerosis HALLMARK

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symptomatic episodes that occur months or years apart and affect different anatomic locations

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Multiple Sclerosis onset

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mid 30's; ♀>♂

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Words associated w/ Multiple Sclerosis

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Multifocal demyelination of white matter; relapse → remitting and progressive

Front 64

Multiple Sclerosis c/b

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inflammatory process c/b immunologic d.o. → humoral (B-cell activation); earliest steps in lesion formation = breakdown of blood-brain barrier d/t enhanced expression of adhesion molecules on surface of lymphocytes & macrophages

Front 65

Multiple Sclerosis presentation

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sensory loss(parathesias/early); spinal cord sx(motor); muscle cramping 2° to spasticity; bladder/bowel/sexual dysfunction; Charcot triad: dysarthria, ataxia, and tremor(Cerebellar sx); optic neuritis; trigeminal neuralgia; Facial myokymia (irregular twitching of the facial muscles);diplopia on lateral gaze;

Front 66

Multiple Sclerosis labs

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lumbar puncture: CSF: IgG > 13%; ↑lymphocytes, ↑protein; ↑oligoclonal bands, ↑myelin basic protein

Front 67

Multiple Sclerosis dx

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MRI +/- gadolinium (shows plaques); Evoked potentials (ID subclinical lesions); lumbar puncture: CSF: IgG > 13%; ↑lymphocytes, ↑protein; ↑oligoclonal bands, ↑myelin basic protein

Front 68

Multiple Sclerosis tx

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Corticosteroids = mainstay; Interferon-B:↓frequency and relapses (delay disability); IV gamma globulins (control relapses); regular exercise (↑conditioning); avoid overwork, fatigue and excess heat

Front 69

Multiple Sclerosis Rx

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amitriptyline, carbamazepine and narcotic analgesics

Front 70

Essential Tremor description

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MC movement disorder characterized by a slowly progressive postural and/or kinetic tremor, usually affecting both upper extremities

Front 71

Essential Tremor c/b

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syndrome of unknown etiology; maybe autosomal dominant; begins at any age (exacerbated by emotional stress)

Front 72

Essential Tremor presentation

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Tremor begins in one upper extremity and then affects both; initially intermittent w/emotional activation > then persistent; may affect the head, voice, jaw, lips, and face; suppressed by manual tasks; amplitude variable (worse w/emotion, hunger, fatigue, temperature extremes); resolves when body part relaxes & w/sleep; ETOH improves sx

Front 73

Essential Tremor PE

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Muscle tone and reflexes are normal; there is no bradykinesia or rigidity

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Essential Tremor labs

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electrolytes, Thyroid function; BUN, Cr, LFT, Serum ceruloplasmin (Wilson dz)

Front 75

Essential Tremor imaging

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CT (normal); MRI (r/o MS/Wilson); SPECT (DaTSCAN) r/o Parkinson dz

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Essential Tremor tx

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beta-blockers: propranolol; Anticonvulsants: primidone (if propanolol fails), topiramate; Second-generation antipsychotics: clozapine; Antidepressants: mirtazapine

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Essential Tremor surgery indication

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pts w/disabling, medically refractory upper extremity tremor > procedures of choice: STEREOTACTIC THALAMOTOMY and THALAMIC VENTRALIS INTERMEDIUS NUCLEUS (deep brain stimulation)

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Parkinson's dz description

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neurologic disorder affecting ~1% of individuals ≥ 60 years causing progressive disability that can be slowed, but not halted, by treatment

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Parkinson's dz c/b

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2 major neuropathologic findings: (1) loss of pigmented dopaminergic neurons of the substantia nigra pars compacta and (2) the presence of Lewy bodies and Lewy neurites

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PRIMARY Parkinson's dz etiology

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Dopaminergic cells are lost in the nigrostriatal system > causes imbalance between the dopamine and acetylcholine in the corpus striatum

Front 81

Parkinson's dz onset

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45-65yrs; ♂=♀; occurs in all ethnic groups

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Parkinson's dz HALLMARK presentation

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Resting=pill-rolling tremor; Rigidity = no tremor; LEAD-PIPE and COGWHEEL-like rigidity; ↓facial expression(mask-like); characteristic gait abnormality

Front 83

Parkinson's dz s/sx

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↓dexterity, ↓arm swing (1st=involved side); soft voice; loss of atonia during REM; ↓sense of smell; autonomic dysfunction (eg, constipation, sweating abnormalities, sexual dysfunction, seborrheic dermatitis);
weakness, malaise, lassitude, depression or anhedonia, slowness in thinking

Front 84

Parkinson's dz onset of motor signs

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MC = asymmetric resting tremor in an upper extremity; bradykinesia, rigidity, and gait difficulty; Axial posture flexed w/short strides

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Parkinson's dz imaging

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CT scan and MRI findings = unremarkable; No laboratory or imaging study required in pts w/typical presentation

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Parkinson's dz dx

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Clinical diagnosis: requires 2 of 3 cardinal signs: (1) Resting tremor (2) Rigidity (3) Bradykinesia

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Parkinson's dz tx

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Levodopa/carbidopa: gold standard; initial treatment of early disease = Monoamine oxidase (MAO)–B inhibitors; dopamine agonists: ropinirole, pramipexole; 2nd line (tremor only) Anticholinergics: trihexyphenidyl, benztropine

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Parkinson's dz surgery

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Deep brain stimulation(Surgical procedure of choice);  destruction of brain tissue; Reversible; adjusted as the disease progresses; Bilateral procedures performed without a significant increase in adverse events

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Huntington's dz description

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incurable, adult-onset, autosomal dominant inherited disorder associated w/cell loss within a specific subset of neurons in the basal ganglia and cortex

Front 90

Huntington's dz c/b

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Marked neuronal loss in deep layers of the cerebral cortex and other regions: globus pallidus, thalamus, subthalamic nucleus, substantia nigra, and cerebellum (varying degrees of atrophy)

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Huntington's dz presentation

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HALLMARK: involuntary movements, dementia, and behavioral changes;

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Huntington's dz labs

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Measurement of the bicaudate diameter (ie, the distance between the heads of the 2 caudate nuclei) by CT scan or MRI is a reliable marker of HD

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Huntington's dz imaging

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No single imaging necessary or sufficient for diagnosis

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Huntington's dz tx

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chorea: clonazepam or diazepam; valproic acid; dopamine-depleting agents, such as reserpine or tetrabenazine; bradykinesia and rigidity may benefit from treatment w/levodopa or dopamine agonists