Neurology Flashcards
cerebrum
CORTEX:
fx in higher brain processes like thought/action, controls all voluntary activity (w/ help of the cerebellum)
-Frontal: reasoning, problem solve, parts of speech, mvmt, emo
-Parietal: perception/recognition of stimuli, orientation, mvmt
-Temporal: memory, perception/recognition of auditory stimuli and speech
-Occipital: visual processing
BASAL GANGLIA:
voluntary motor mvmt, coordination, cognition, emotion
LIMBIC SYSTEM:
thalamus, hypothalamus, amygdala and hippocampus
-Amygdala: located in temporal lobe, involved in memory, emo and fear
-Hippocampus: learning, memory; converts short-term into long-term memory
diencephalon
THALAMUS:
- located deep in the forebrain
- processes nearly all sensor and motor info
- relays info to/from cortex
HYPOTHALAMUS:
- located below and ventral to thalamus
- controls homeostasis
brainstem
MIDBRAIN:
controls eye mvmt, relays visual and auditory info
PONS:
REGULATES BREATHING, SERVES AS A RELAY STATION BTW CEREBRAL HEMISPHERE AND MEDULLA
-involved in motor control and sensory input
MEDULLA OBLONGATA:
extension of spinal cord
-regulates vital body fx such as heart rate, breathing, autonomic centers, swallowing and coughing
cerebellum
posterior to medulla
maintains posture and balance, coordinates voluntary mvmt and controls certain head and eye mvmt
basal ganglia disorders
EXTRAPYRAMIDAL SYMPTOMS:
- Dyskinesia
- Dystonia - sustained contraction, twisting abn position
- Myoclonus - invol jerk/twitch
- Tics - suppressible
- Chorea - rapid invol jerky, uncontrolled, purposeless
- Muscle spasms - tonic (sustained rigid) or clonic (repeat, rapid)
- Tremors
PARKINSONISM
- Parkinson Disease
- Dopamine antagonists (haloperidol, olanzapine, risperidone, promethazine, metoclopramide)
- Lewy body disease - loss of dopaminergic neurons, anticholinergic neurons
- Other: head trauma, HIV, carbon mo, mercury poisoning, cns dysfunction
Huntington disease
huntington gene –> neurotoxicity as well as cerebral, putamen and caudate nucleus atrophy
- sx:
- Behavioral: personality, cognitive, intellectual and psych including irritability
- Chorea: rapid, invol or arrhythmic mvmt of face, neck, trunk and limbs initially; worse w/ vol mvmts and stress
- Dementia: most devo dementia before 50y (primarily exec fx)
- gait abnormalities/ataxia, incontinence, facial grimacing
- brisk DTR
-dx:
CT: CEREBRAL AND CAUDATE NUCLEUS ATROPHY
PET: dec glucose metabolism in caudate nucleus and putamen
- tx:
- usually fatal w/in 15-20y after presentation
- CHOREA MANAGEMENT: antidopinergics: typical/atypical antipsychotics, tetrabenazine, benzos
essential familial tremor
-AUTO DOM, onset usually in 60s
- sx:
- INTENTIONAL TREMOR - POSTURAL, BILATERAL ACTION TREMOR OF HANDS, FOREARMS, HEAD, NECK OR VOICE
- mc in upper extremities and head
- WORSE W/ EMO STRESS AND INTENTIONAL MVMT
- SHORTLY RELIEVED W/ ETOH INGESTION
- normal neuro exam otherwise
- tx
- PROPANOLOL MAY HELP SEVERE OR SITUATIONAL
- Primidone (barbiturate) if no relief or instead of propanolol
- Alprazolam (benzo) 3rd line
parkinson disease
IDIOPATHIC DOPAMINE DEPLETION –> failure to inhibit acethylcholine in basal ganglia (Ach is excitatory nt and dopamine is inhibitory)
- onset 45-65 mc
- LEWY BODIES (cytoplasmic inclusions), LOSS OF PIGMENT CELLS SEEN IN SUBSTANTIA NIGRA
- sx:
- RESTING TREMOR MC SIGN (PILL ROLLING) - worse w/ rest and emo stress; less w/ voluntary activity/intentional movement, usually starts w/ one limb for years until generalized
- BRADYKINESIA - slow vol mvmt, dec automatic mvmt
- RIGIDITY - inc resist to passive mvmt (COGWHEEL); festination = increasing speed while walking, norm DTR
- FACE INVOLVEMENT - FIXED FACIAL EXPRESSIONS, widened palpebral fissure; MYERSON’S SIGN: tapping bridge of nose repetitively causes a sustained blink
- POSTURAL INSTABILITY - usually late finding, “pull test”
- dementia in 50% (late finding)
- tx:
- drug tx not used initially if sx mild
- LEVODOPA/CARBIDOPA - MOST EFFECTIVE - levo converted into dopamine (carbidopa reduces amt of levo needed, reducing SE)
- DOPA AGONISTS: BROMOCRIPTINE, PRAMIPEXOLE, ROPINEROLE - stimulate dopamine receptors; sometimes used in young pt to delay use of levodopa
- ANTICHOLINERGICS: TRIHEXYPHENIDYL, BENZTROPINE - blocks excitatory cholinergic effects
- Amantadine - inc pre-synaptic dopa release
- MAO-B Inhibitors - inc dopa
tourette syndrome
-onset usually childhood (2-5y), mc boys
+/- assoc w/ obsessions-compulsions
- sx:
- MOTOR TICS of face, head, neck
- VERBAL OR PHONETIC TICS: grunts, throat clearing, obscene words (coprolalia), repeating others (echolalia)
- self-mutliating tics: hair pull, nail bite, bite lips
- tx:
- HABIT REVERSAL THERAPY - 50% REVERSE BY 18
- Dapamine-blocking agents: HALOPERIDOL, RISPERIDONE
- Alpha-adrenergics: CLONIDINE, GUANFACINE, clonazepam as adjunct
amyotrophic lateral sclerosis (ALS)
-necrosis of BOTH upper and lower motor neurons –> PROGRESSIVE MOTOR DEGENERATION
- sx:
- muscle weakness, LOSS OF ABILITY TO INITIATE AND CONTROL MOTOR MVMT
- MIXED UPPER AND LOWER MOTOR NEURON SIGNS
- Upper: spasticity, stiffness, hyperreflexia
- Lower: progressive bilateral fasciculations, muscle atrophy, hyporeflexia, muscle weakness
- bulbar sx: dysphagia, dysarthria, speech problems, eventual respiratory dysfunction
- SENSATION, URINARY SPHINCTER AND VOLUNTARY EYE MOVEMENTS ARE SPARED
- tx:
- RILUZOLE reduces progression for up to 6 mo
- usually fatal w/in 3-5 years of onset
cerebral palsy
-CNS disorder assoc w/ muscle tone and postural abnormalities due to brain injury during perinatal or prenatal period
- sx:
- SPASTICITY HALLMARK - varying degrees of motor deficits, often assoc w/ intellectual/learning disabilities and devo abnormalities
- HYPERREFLEXIA, limb-length discrepancies, congenital defects
- tx:
- multidisciplinary approach
- spasticity –> Baclofen, Diazepam, antiepileptics for seizures
restless legs syndrome (Willis-Eckbom disease)
-sleep-related mvmt disorder; usually primary but may occur secondary to cns Fe deficiency, pregnancy, peripheral neuropathy
- dx:
- uncomfortable or unpleasant sensation (itching, burning, paresthesias, etc) in the leg that creates an urge to move the legs
- worse at night, tends to occur w/ prolonged periods of rest or inactivity
- improves w/ movement
- tx:
- DOPAMINE AGONIST TX OF CHOICE: PRAMIPEXOLE, ROPINIROLE
- Alpha-2-Delta Calcium channel ligands: GABA, PREGAB
- Benzos
- Opioids in disease resistant to above
- FE SUPPLEMENT REC IN PT W/ SERUM FERRITIN LOWER THAN 75mcg/L bc of assoc w/ Fe deficiency in cns
guillain barre syndrome
- acute/subacute acquired inflammatory DEMYELINATING POLYRADICULOPATHY of PERIPHERAL NERVES
- INC INCIDENCE W/ CAMPYLOBACKER JEJUNI MC or other antecedents RESPIRATORY OR GI INFX (CMV, EBV, HIV, mycoplasma, immunizations, postsurgical)
-Patho: immune-med demyelination and axonal degeneration slows nerve impulses –> symmetric weakness and paresthesias; post infx immune response cross-reacts w/ peripheral nerve components
- sx:
- ASCENDING WEAKNESS AND PARESTHESIAS (usually symmetric)
- DEC DTR (lower motor neuron), may involve muscles of respiration or bulbar muscles (swallowing), CNVII palsy
- AUTONOMIC DYSFUNCTION: tachy, hypoTN/hyperTN, BREATHING DIFFICULTIES
- dx:
- CSF: HIGH PROTEIN W/ NORMAL WBC (high protein after 1-3 wks of sx)
- Electrophysicoligc studies: dec motor nerve conduction
- tx:
- PLASMAPHERESIS best if done early - removes harmful auto-ab, equally effective as IVIG
- IVIG: suppress harmful inflam/auto-ab and induces remyelination; most recover w/in months
- mech vent if respiratory failure
- PREDNISONE CONTRAINDICATED
- PROGNOSIS: 60% FULL RECOVERY IN 1 YR, 10-20% left w/ permanent disability
myasthenia gravis
AUTOIMMUNE peripheral nerve disorder; HLA-DR3
MC YOUNG WOMEN
75% have THYMIC ABNORMALITY (HYPERPLASIA OR THYMOMA) or other immune disorders
-Patho: inefficient skeletal muscle neuromusclar transmission d/t AUTOAIMMUNE AB AGAINST ACETYLCHOLINE (NICOTINIC) POSTSYNAPTIC RECPTOR at neuromuscular jx (dec ACh receptors)
-sx:
OCULAR: usually 1st presenting
-extraocular involvement –> DIPLOPIA, PTOSIS/eyelid weakness (more prominent w/ upward gaze) weakness worse w/ repeated use, pupils spared
GENERALIZED: WORSE MUSCLE WEAKNESS W/ REPEATED USE THROUGHOUT DAY (relieved w/ rest)
-normal sensation and DTR
-BULBAR WEAKNESS (oropharyngeal) w/ prolonged chewing, dysphagia
-RESPIRATORY MUSCLE WEAKNESS may lead to RESPIRATORY FAILURE = MYASTHENIC CRISIS**
-dx:
+ ACETYLCHOLINE RECEPTOR ANTIBODIES
+ MuSK (muscle-specific tyrosine kinase) AB
-EDROPHONIUM (TENSILON) TEST: rapid response to short-acting IV Edrophonium
-CT scan or MRI of chest may show THYMOMA or THYMUS GLAND ABNORMAL
-ICE PACK TEST –> eyelid 10 min –> improve ptosis
- tx:
- ACETYLCHOLINESTERASE INHIBITORS: PYRIDOSTIGMINE OR NEOSTIGMINE 1ST LINE –> inc ACh (dec breakdown), SE: CHOLINERGIC CRISIS (weak, n/v, sweat, salivation, diarrhea, miosis, brady, resp failure)
- IMMUNOSUPPRESSION: PLASMAPHERESIS OR IVIG for myasthenic crisis
- Chronic: CORTICO
- THYMECTOMY if thymoma
- AVOID FLUOROQUINOLONES AND AMINOGLYCOSIDES
CRISIS:
- IF FLACCID PARALYSIS IMPROVES W/ TENSILON –> MYASTHENIC CRISIS
- IF FLACCID PARALYSIS WORSENS W/ TENSILON –> CHOLINERGIC CRISIS
- ddx:
- MYASTHENIC SYNDROME (LAMBERT-EATON) MC ASSOC W/ SMALL CELL LUNG CA –> WEAKNESS IMPROVES W/ USE
multiple sclerosis
AUTOIMMUNE, INFLAMMATORY DEMYELINATING DISEASE of IDIOPATHIC ORIGIN
-MC WOMEN AND YOUNG ADULTS 20-40Y
- RELAPSE-REMITTING MC episodic exacerbations
- Progressive: decline w/out acute exacerbations
- Secondary progressive: relapse-remitting that becomes progressive
- sx:
- Sensory Deficits: pain, fatigue, numbness, paresthesias in limbs, TRIGEMINAL NEURALGIA, UHTHOFF’S PHENOMENON (worsening of sx w/ heat), LHERMITTE’S SIGN
- OPTIC NEURITIS (RETROBULBAR) unilateral eye pain worse w/ movement, diplopia, central scotomas, VL
- MARCUS-GUNN PUPIL: swinging flashlight from unaffected to affected –> pupils appear to dilate
- MOTOR: UPPER –> SPASTICITY AND + BABINSKI
- spinal cord sx: bladder, bowel or sexual dysfunction
- CHARCOT’S NEUROLOGIC TRIAD: nystagmus, staccato speech, intentional tremor
-dx:
-mainly clinical - at least 2 discrete episodes of exacer
-MRI W/ GADOLINIUM - TEST OF CHOICE TO CONFIRM
WHITE MATTER PLAQUES (hyperdensities) hallmark
-LUMBAR PUNCTURE: INC IgG (OLIGOCLONAL)
- tx:
- ACUTE: IV CORTICO 1ST LINE, plasmapheresis
- RELAPSE/REMIT/PROGRESSIVE: B-INTERFERON or GLATIRAMER ACETATE decrease #/severity of relapse
- amantadine is helpful for fatigue, baclofen and diazepam for spasticity
bell palsy
IDIOPATHIC, UNILATERAL CN 7/FACIAL NERVE PALSY d/t inflammation or compression
- lower motor neuron lesion, MC RIGHT SIDE
- STRONG ASSOC W/ HSV REACTIVATION
-RF: Dm, pregnancy (esp 3rd tri), post URI, dental nerve block
- sx:
- sudden onset of IPSILATERAL HYPERACUSIS (EAR PAIN) 24-48h –> UNILATERAL FACIAL PARALYSIS, UNABLE TO LIFT AFFECTED EYEBROW
- TASTE DISTURBANCE ANTERIOR 2/3
- ONLY AFFECTS FACE
-dx: of exclusion
-tx:
-most resolve w/in 1 month w/out tx
-PREDNISONE (ESP W/IN 72H)
-ARTIFICIAL TEARS
+/- Acyclovir
- DDx:
- upper motor lesions (cva)
- IF UPPER FACE OK (wrinkle forehead) – NOT BELLS PALSY
headaches - general
PRIMARY (90%): MIGRAINE, TENSION, CLUSTER OR REBOUND - IDIOPATHIC
SECONDARY (4%): meningitis, subarachnoid hemorrhage, intracranial htn, acute glaucoma (suspect if abrupt or progression of severity)
tension headache
mc overall type
-ave age onset 30y, thought to be d/t mental stress
- sx:
- BILATERAL, TIGHT, BAND-LIKE, constant daily
- worse w/ stress, fatigue, noise or glare
- not usually pulsatile
- NO N/V, FOCAL NEURO SX (no photophobia)
- tx:
- treat like migraines; 1st line: NSAIDs, asa, tylenol, anti-migraine meds
- TCAs (amitriptyline) in severe/recurrent, prophylaxis, BB
migraine headache
- mc women, mc cause of morning ha, family hx 80%
- 2 TYPES: W/OUT AURA (COMMON) AND W/ AURA
- thought to be caused by vasodilation of blood vessels innervated by the trigeminal nerve
- sx:
- LATERALIZED, PULSATILE/THROBBING HA, ASSOC W/ N/V, PHOTOPHOBIA usually 4-72h duration, +/-bilat
- WORSE W/ PHYSICAL ACTIVITY, stress, lack/excessive sleep, etoh, foods (choc, red wine), OCPs, menstruation
- AURAS: VISUAL CHANGE MC - light flashes (photopsia), zig-zag light (teichopsia), scotomas, aphasia, weak, numb
-tx:
Symptomatic:
-TRIPTANS OR ERGOTAMINES (serotonin 5HT-1 agonists –> vasoconstrict) c/i CAD, PVD, uncontrolled HTN, hepatic, renal dz, pregnancy
-DOPAMINE BLOCKERS (metoclopramide, promethazine, prochlorperazine) as ANTIEMETICS, GIVE W/ BENADRYL TO PREVENT EPS
-iv fluids, dark room
-mild: NSAIDs/tylenol 1st line
Prophylactic:
-ANTI-HTN (BB, CCB), tca, anticonvulsants (valproate, topiramate)
trigeminal neuralgia
compression of trigeminal nerve root (by superior cerebellar artery or vein) 90%, idiopathic 10%
-mc middle-aged women
- sx:
- BRIEF, EPISODIC, STAB/LANCINATING PAIN in 2nd/3rd division of TRIGEMINAL NERVE (cn 5) lasts sec-min
- WORSE W/ TOUCH, EATING, DRAFTS OF WIND, MOVEMENTS (OFTEN UNILATERAL)
- starts near mouth and shoots to eye, ear and nostril on ipsilateral side
- tx:
- CARBAMAZEPINE (TEGRETOL) 1ST LINE, oxcarbazepine
- GABAPENTIN
- sx decompression in severe cases
*suspect MS in younger pt w/ TGN
cluster headache
-young/middle-aged males mc
- sx:
- SEVERE, UNILATERAL PERIORBITAL/TEMPORAL PAIN (SHARP, LANCINATING)
- LASTS <2 HR w/ spontaneous remission
- several times/day over 6-8 wks
- WORSE AT NIGHT, ETOH, STRESS OR FOODS
- IPSILATERAL HORNER’S SYNDROME (ptosis, miosis, anhydrosis), HASAL CONGESTION/RHINORRHEA, CONJUNCTIVITIS AND LACRIMATION
- tx:
- 100% O2 FIRST LINE
- ANTI MIGRAINE MEDS - SQ SUMATRIPTAN or ergotamines
- Prophylaxis: VERAPAMIL 1ST LINE, cortico, ergotamines, valproic acid, lithium
idiopathic intracranial htn (pseudotumor cerebri)
IDIOPATHIC INC INTRACRANIAL (CSF) PRESSURE and no other cause of intracranial htn evident on ct/mri
-mc in obese women of childbearing age, cortico w/drawal, growth hormone, thyroid replacement, ocp
- sx:
- HEADACHE (worse w/ strain), retrobulbar pain, n/v, tinnitus, VISUAL CHANGE (can lead to blindness)
-dx:
-PAPILLEDEMA
+/- CN6 (abducens) palsy = lateral rectus muscle weakness –> limits of abduction
-CT SCAN: 1ST TO R/O MASS –> LP = INC CSF PRESSURE
- tx:
- ACETAZOLAMIDE (reduces csf pressure/production) +/- furosemide, +/- short course steroids
- optic nerve sheath fenestration of CSF shunting allows evacuation of csf
CSF Findings
MS = high IgG (oligoclonal bands)
GB = high protein, normal WBC
Bacterial = high protein w/ inc WBC (PMNs), dec glucose
Viral = normal glucose, increased WBCs (lymph)
Fungal / TB = decreased glucose, increased WBC (lymph)
Idiopathic Intracranial HTN = increased CSF pressure
Subarachnoid Hemorrhage = xanthochromia, blood in CSF
*IF GLUCOSE NORMAL, VIRAL MENINGITIS MOST LIKELY, IF PMNS PREDOMINANT, BACTERIAL MOST LIKELY
normal pressure hydrocephalus
- dilation of cerebral ventricles w/ normal opening pressures on lumbar puncture
- unknown, thought to be d/t impaired CSF absorption after injury (SAH, tumors, infx, inflam, trauma)
-sx:
TRIAD:
-GAIT DISTURBANCES - wide base, shuffling gait
-DEMENTIA - impaired exec fx, psychomotor depression
-URINARY INCONTINENCE - urgency early in dz
-other: weakness, lethargy, malaise, rigidity, hyperreflexia and spasticity
- dx:
- MRI/CT: ENLARGED VENTRICLES in absence or out or proportion to sulcal dilation (MRI pref)
- LP: CSF usuall normal; lumbar tap test = remove up to 50 cc leads to improvement 30-60 min after
- tx:
- VENTRICULOPERITONEAL SHUNT TX OF CHOICE
concussion
-mild traumatic brain injury –> alteration of mental status w/ or w/out loss of consciousness
- sx:
- CONFUSION, blank expression, blunted affect
- AMNESIA: pre-traumatic (retrograde) or post-traumatic (antegrade)
- headache, dizzy, visual disturbances: blurry/double
- delated responses and emo changes
- signs of inc intracranial pressure: persistent vomiting, worsening headache, inc disorientation, changing levels of consciousness
- dx:
- CT TEST OF CHOICE
- MRI if prolonged sx >7-14d or w/ worsening of sx not explained by concussion
- tx:
- COGNITIVE AND PHYSICAL REST
- pt may resume activity after resolution of sx and recovery of memory/cog fx
delirium
ACUTE, ABRUPT, TRANSIENT CONFUSED STATE d/t an IDENTIFIABLE CAUSE (MEDS, INFX)
- rapid onset assoc w/ fluctuating mental status changes and marked deficit in short-term memory
- recovery w/in 1 week most cases
dementia
PROGRESSIVE, CHRONIC INTELLECTUAL DETERIORATION
- MEMORY LOSS and loss of impulse control, motor and cognitive fx
- includes language dysfx, disorientation, inability to perform complex motor activity and inappropriate social interactions
- RF: age (esp >60) and vascular disease
- Includes: alzheimer’s, vascular, frontotemporal, diffuse lewy body, creutzfeldt-jakob
Alzheimer disease
MC TYPE OF DEMENTIA
- AMYLOID DEPOSITION (SENILE PLAQUES)
- NEUROFIBRILLARY TANGLES (TAU PROTEIN)
- CHOLINGERGIC DEFICIENCY –> memory, language, visuospatial changes, normal reflexes
- sx:
- short-term memory loss (1st sx) progresses to long term, disorientation, behavioral and personality changes
- dx:
- CT SCAN - CEREBRAL CORTEX ATROPHY
- tx:
- AcH-ESTERASE INHIBITORS: DONEPEZIL, TACRINE, RIVASTIGMINE, GLANTAMINE (reverse cholinergic deficiency and sx relief - doesn’t slow)
- NMDA ANTAGONIST: MEMANTINE (blocks nmda receptor, slow Ca influx and nerve damage) –> reduce glutamate excitotoxicity
vascular dementia
2nd MC TYPE
- BRAIN DZ D/T CHRONIC ISCHEMIA AND MULTIPLE INFARCTIONS (LACUNAR INFARCTS)
- HTN MOST IMPORTANT RISK FACTOR
- sx:
- Cortical: forgetfulness, confusion, amnesia, exec difficulties, speech abn
- Subcortical: motor deficits, gait abnormalities, urinary difficulties, personality changes
frontotemporal dementia (Pick’s disease)
-rare type
-LOCALIZED BRAIN DEGENERATION OF FRONTOTEMPORAL LOBES
-MARKED PERSONALITY CHANGES (PRESERVED VISUOSPATIAL)
-apathy, disinhibition, +palmomental and palmar grasp reflexes, no amnesia
+PICK BODIES
