Neuro Flashcards
Bells Palsy definition, sx, dx, tx
Idiopathic, unilateral CN VII/facial nerve palsy leading to hemifacial weakness & paralysis due to inflammation or compression – LMN disorder
sx
Sudden onset ipsilateral hyperacusis (ear pain) 24-48hrs followed by unilateral facial weakness or paralysis involving the forehead
*unable to life the affected eyebrow
*wrinkled forehead, loss of the nasolabial fold
*drooping of the corner of the mouth
*taste disturbances (anterior 2/3)
*biting the inner cheek
*eye irritation (d/t ↓ lacrimation & inability to fully close)
Bell phenomenon: eye on the affected side moves laterally & superiorly when eye closure is attempted
Weakness/paralysis ONLY affecting the face
dx: exclusion
tx
No treatment required (>85% resolve in 1mo)
Supportive: artificial tears
Prednisone (esp. if started within 72hrs of sxs onset) reduces the time to full recovery and increases the likelihood of complete recuperation
Transient Ischemic Attack definition, sx, dx, tx
Transient episodes of neurologic deficits caused by focal brain, spinal cord, or retinal ischemia w/o acute infarction
3 main types:
- embolic: AFIB, left ventricular thrombus
- lacunar: penetrating small vessels
- large artery: ischemia due to atherosclerosis
sx
Neurologic deficits lasting <24hrs – most last for a few minutes w/ complete resolution in 1hr
Internal carotid artery: amaurosis fugax: transient monocular vision loss “temporary shade down on one eye”
ICA/MCA/ACA: cerebral hemisphere dysfunction – sudden HA, speech changes, confusion
PCA: somatosensory deficit
Vertebrobasilar: brainstem/cerebral sxs – gait & proprioception
PE: carotid bruits may be heard
dx
Neuroimaging + neurovascular imaging + r/o cardioembolic source
*neuroimaging: CT initial, MRI more sensitive
*neurovascular: CT/MR angiography, carotid Doppler
Conventional angiography: definitive (invasive)
Ancillary testing: r/o cardioembolic source (EKG, telemetry, echocardiogram), r/o metabolic or hematologic source (hypoglycemia, CBC)
tx
Place pt in supine position to increase cerebral perfusion, avoid lowering BP unless >220/120
- thrombolytics contraindicated
Noncardiogenic TIA:
- antiplatelet therapy: aspirin, clopidogrel
ABCD2 score 0-3: aspirin alone
ABCD2 score ≥4: aspirin + clopidogrel
- carotid endarterectomy recommended if internal carotid artery stenosis >70%
Cardiogenic TIA (AFIB): oral anticoagulation
Lacunar Infarcts definition, sx, dx, tx
Small vessel disease of the penetrating branches of cerebral arteries in the pons & basal ganglia
sx
5 classic presentations:
Pure Motor (MC): hemiparesis or hemiplegia in the absence of sensory or “cortical” signs (aphasia, agnosia, neglect, apraxia, hemianopsia)
Ataxic Hemiparesis: ipsilateral weakness & clumsiness
Pure Sensory Deficits: numbness, paresthesias of arm, face, leg on one side of the body in the absence of sensory or “cortical” signs
dx
Sensorimotor: weakness & numbness of the face, arm, leg on one side of the body in the absence of “cortical” signs
Dysarthria (Clumsy Hand Syndrome): dysarthria, facial weakness, dysphagia, & slight weakness & clumsiness of one hand in the absence of “cortical” signs
DX: CT scan – small punched-out hypodense areas (lacunar infarcts) usually in central & noncortical areas (e.g., basal ganglia)
TX: Aspirin, control RF (HTN, DM)
Ischemic Strokes definition, sx, dx, tx
Acute onset of neurological deficits due to death of brain tissue from ischemia
MC type of stroke
Causes:
*thrombotic: MC (2/3)
*embolic: (1/3) commonly come from heart, aortic arch, or large cerebral arteries – sources: AFIB, valvular disease, patent foramen ovale
Risk Factors: HTN most significant & modifiable RF
- dyslipidemia, DM, AFIB, smoking
- nonmodifiable: male, ↑ age, ethnicity, family hx
sx
Carotid/Ophthalmic – amaurosis fugax (monocular blind)
MCA – aphasia, neglect, hemiparesis, gaze preference, homonymous hemianopsia
ACA – leg paresis, hemiplegia, urinary incontinence
PCA – homonymous hemianopsia
Basilary Artery – coma, cranial nerve palsies, apnea, drop attack, vertigo
dx
CT head w/o contrast – best initial to r/o hemorrhagic stroke; may be normal in first 6-24hrs
Ancillary testing:
- neurovascular: CT/MR angiography
- carotid Doppler U/S
- EKG
- echocardiography
- cardiac monitoring
tx
Immediate management:
- within 3hrs of sxs onset: alteplase (thrombolytic) if no contraindications (BP >185/110, recent/bleeding d/o, recent trauma)
- >3-4.5hrs: aspirin
- BP control IF ≥185/110
Long-term management:
- antiplatelet therapy: aspirin, clopidogrel, dipyridamole
- anticoagulation ONLY if cardioembolic (AFIB)
- statin (regardless of LDL level)
Epidural Hematoma (Hemorrhage) definition, MC location, sx, dx, tx
Location: arterial bleed MC between skull & dura
Mechanism: MC after temporal bone fracture 🡪 middle meningeal arterial disruption
sx
Brief LOC 🡪 lucid interval 🡪 coma
HA, N/V, focal neuro sxs, rhinorrhea (CSF fluid)
CN III palsy if tentorial herniation
dx
CT: convex (lens-shaped) bleed
*does NOT cross suture lines
tx
+/- herniate if not evacuated early
Observation if small
If ↑ ICP: mannitol, hyperventilation, head elevation, +/- shunt
Subdural Hematoma (Hemorrhage) definition, MC location, sx, dx, tx
Location: venous bleed MC
- between dura & arachnoid d/t tearing of cortical bridging veins
MC in elderly
Mechanism: MC blunt trauma (“contre-coup”), venous bleed
sx
Varies, may have focal neuro sxs
Chronic:
*insidious onset of HA
*cognitive impairment
*somnolence
*occasional seizures
CT 🡪 HYPOdense
dx
CT: concave (crescent-shaped) bleed
*bleeding CAN cross suture lines
tx
Hematoma evacuation vs. supportive
Evacuation if massive or ≥5mm midline shift
Intracerebral Hemorrhage definition, sx, dx, tx
*bleeding within the brain parenchyma
*may compress the brain, ventricles, & sulci
sx
Neurologic sxs usually increase within min-hrs
*HA, N/V
*syncope
*focal neuro sxs (hemiplegia, hemiparesis, seizures)
*altered mental status
PE:
*may have focal motor & sensory defects
dx: CT w/o contrast
tx
Supportive: gradual BP reduction
Prevention of increased intracranial pressure
*raising head of the bed 30 degrees
*limiting IV fluids
*BP management
*analgesia, sedation
Reduction of intracranial pressure: IV mannitol
Subarachnoid Hemorrhage definition, sx, dx, tx
Bleeding between the arachnoid membranes & the pia mater
Etiologies:
*MC due to a ruptured berry aneurysm at the anterior communicating artery
*AVM, stroke, trauma
Risk Factors:
*smoking, HTN
*PCKD, atherosclerotic disease, ETOH, Ehlers-Danlos syndrome, Marfan syndrome, family hx
sx
*sudden, intense thunderclap HA (unilateral, occipital area)
“worse HA of my life”
*N/V, meningeal sxs (photophobia, neck stiffness, fever)
*LOC
PE:
*meningeal signs: nuchal rigidity, + Brudzinski, + Kernig
*CN III palsy – fixed, dilated, “blown” pupil
*Terson Syndrome: retinal hemorrhages
dx
CT scan w/o contrast
LP 🡪 performed if CT (-)
*xanthochromia
4-vessel angiography
*usually performed after confirmed SAH to identify source of bleeding & other aneurysms
sx
Supportive: bed red, stool softeners, lower intracranial pressure
*Nimodipine reduces cerebral vasospasms, improving neurologic outcomes
Cerebral Aneurysm definition, sx, dx, tx
Weak bulging spot of cerebral artery – MC anterior communicating artery in Circle of Willis
Size:
*Small: <15mm
*Large: 15-25mm
*Giant: 25-50mm
*Supergiant: >50mm
Shape:
*Saccular (“Berry”): MC type (80-90%) – MCC of SAH
*Fusiform: dilation of entire circumference of vessel
*Traumatic: caused by closed head injury
*Mycotic: infected emboli
*Ruptured AVM
sx
Usually asymptomatic unless ruptured 🡪 sudden, severe HA, N/V, seizures, AMS, increased BP
dx
Found incidentally or when a pt presents w/ SAH
Non-contrast head CT for investigational
LP: elevated opening pressure
*bloody fluid – xanthochromia, RBC
Cerebral angiography – gold standard
tx
Surgical clipping
Endovascular coiling within first 24hrs
Restore respiration
Trigeminal neuralgia definition, sx, dx, tx
MC overall cause of primary HA – mean age of onset ~30yrs
Risk Factors: mental stress, sleep deprivation, eye strain
sx
HA: paroxysmal, brief, episodic, stabbing, lancinating or shock-like pain in the 2nd/3rd division of the trigeminal nerve, lasting sec-mins
*worse w/ touch, chewing, brushing teeth, drafts of wind, & movements (often unilateral)
Pain starts near mouth & shoots to eye, ear, & nostril on the ipsilateral side & often occurs many times throughout the day
PE: usually normal but light palpation of “trigger zones” may trigger attack
dx
Clinical dx in the absence of hx or physical findings suggestive of a serious underlying cause
tx
Carbamazepine first line; oxcarbazepine
Gabapentin, baclofen, lamotrigine
Surgery
Migraine HA definition, sx, dx, tx
MC in women; family hx (80%)
Types:
Migraine w/o aura (MC)
Migraine w/ aura
sx
Usually lateralized, pulsatile (throbbing) HA often associated w/ N/V, photophobia, phonophobia; 4-72hrs duration, mod-severe intensity
*worsened w/ routine physical activity, stress, lack or excessive sleep, ETOH, specific foods (chocolate, red wine), hormonal (OCPs, menstruation), dehydration
Auras: focal neurologic sxs that usually last <60min; accompany or follow the HA within 60min
*visual (MC)
*auditory
*somatosensory
*loss of function (aphasia, hearing)
dx: clinical
tx
Symptomatic (abortive) management
- NSAIDs, acetaminophen, aspirin first line if mild; some meds have caffeine to improve sxs
- IV fluids, placing pt in dark/quiet room
- triptans or ergotamines if mod-severe or no response to analgesics
- antiemetics (metoclopramide, prochlorperazine)
Prophylactic (preventative)
- anti-HTN: BBs (propranolol), CCBs
- TCAs, antidepressants, anticonvulsants (valproate, topiramate), NSAIDs
Cluster HA definition, sx, dx, tx
Predominantly young & middle-aged males (10x MC than women)
Associated w/ multiple frequent HA w/ high intensity & brief duration
sx
Triggers: worse at night, ETOH, stress, specific foods
HA: severe, unilateral periorbital or temporal pain (sharp, lancinating); bouts last <2hrs w/ spontaneous remission – bouts occur several times a day; may have 1-2 cluster periods a year (each lasting wks-mos)
PE: ipsilateral findings – Horner’s syndrome (ptosis, miosis, anhidrosis), nasal congestion, rhinorrhea, conjunctivitis, lacrimation
dx: clinical
tx
Acute:
- 100% oxygen first line
- antimigraine meds help during attack: SQ sumatriptan or ergotamines
Prophylaxis: verapamil first line
- corticosteroids, ergotamines, valproic acid, lithium
Idiopathic Intracranial HTN (Pseudotumor Cerebri) definition, sx, dx, tx
Idiopathic increased intracranial (CSF) pressure w/ no clear cause evident on neuroimaging (CT/MRI)
Pseudotumor Cerebri: mimics a brain tumor w/ N/V, visual disturbances
Risk Factors:
- obese women of childbearing age
- meds: steroid withdrawal, GH, thyroid replacement, OCPs, long-term tetracycline use, vitamin A toxicity
- venous sinus thrombosis
s/sxs of increased ICP:
*HA: pulsatile, worse w/ straining or changes in posture
*retrobulbar pain that may be worse w/ eye movements
*N/V, tinnitus
*visual changes – may lead to blindness if not treated
Ocular Exam:
*fundoscopy: papilledema (usually bilateral, symmetric)
*may have visual field loss
*may have diplopia due to a cranial nerve V1 (abducens) palsy
dx
CT scan: performed prior to LP to r/o intracranial mass
LP: ↑ CSF pressure (≥250mmH2O) + otherwise normal CSF
MRI w/ MR venography
tx
Acetazolamide first line (decreases CSF production) & weight loss recommended
- furosemide may be adjunct
Short course of systemic steroids may be indicated if acute visual loss as a temporizing measure prior to surgical intervention
Repeat LP reduces intracranial pressure
Refractory: ventriculoperitoneal shunt or optic nerve sheath fenestration
Triptans MOA
MOA: serotonin (5HT-1b/d) agonists causes vasoconstriction & block pain pathways in the brainstem
Indications: mod-severe migraines or no response to analgesics in mild dz; can be combined w/ analgesics
Ergotamines MOA
MOA: serotonin (5HT-1b/d) agonists cause vasoconstriction & block pain pathways in the brainstem
Indications: reserved use d/t ADRs and contraindications
Antiemetics: Metoclopramide,
Chlorpromazine MOA
MOA: dopamine receptor antagonists; may also help reduce HA pain intensity
Indications: N/V in pts w/ migraine
Coma definition, sx, dx, tx
Definition: deep state of prolonged unconsciousness in which a person cannot be awakened
Causes of altered level of consciousness: AEIOU TIPS
Alcohol or AAA
Electrolytes, endocrine
Insulin
Opiates
Uremia
Trauma, temperature, or toxemia
Infections – sepsis, meningitis
Psychogenic or pulmonary embolism
Space occupying lesions, strokes, shock, seizure
sx
*fails to respond normally to painful stimuli, light, or sound
*lacks a normal wake-sleep cycle
*does not initiate voluntary actions
dx
*vital signs, blood glucose level
*hx & neurologic exam
*CBC, electrolytes, calcium, magnesium, phosphorus
*liver & kidney function tests
*urine toxicology screen
*serum ammonia
*ABGs
*blood cultures
*EKG, CXR, CT, MRI, LP
tx
Directed at underlying cause
Consider reversible causes:
*hypoglycemia 🡪 dextrose
*opiate OD 🡪 naloxone (Narcan)
*Wernicke’s (thiamine deficiency) 🡪 thiamine
Complex Regional Pain Syndrome definition, sx, dx, tx
Autonomic dysfunction following bone or soft tissue injuries
FOLLOWS TRAUMA!!!!
sx
*sensory: pain, hyperalgesia
*motor/trophic changes
*edema or sweating changes
*vasomotor: temperature & skin color asymmetry w/ autonomic dysfunction
dx
Clinical – at least 1 sxs in 3/4 categories
tx
*NSAIDs, PT/OT, anesthetic blocks, oral steroids
*TCAs, transcutaneous electric nerve stimulation
Concussion definition, sx, dx, tx
Mild traumatic brain injury leading to alteration in mental status, w/ or w/o LOC
May result after blunt force or an acceleration/deceleration head injury
sx
HA, dizziness, psychosocial sxs, cognitive impairment
Confusion: confused or blank expression, blunted affect
Amnesia: pretraumatic (retrograde) or posttraumatic (anterograde)
Visual disturbances: blurred or double vision
Delayed response/emotional changes: emotional instability
Signs of ↑ ICP: persistent vomiting, worsening HA, increasing disorientation, changing levels of consciousness
dx
CT w/o contrast
MRI: if prolonged sxs >7-14d or w/ worsening of sxs not explained by concussion
CT angiography if vascular injury suspected
tx
Cognitive & physical rest
Resume strenuous activities after resolution of sxs & recovery of memory as well as cognitive functions
Delirium definition, sx, dx, tx
Acute, abrupt, transient confused state due to an identifiable cause (e.g., medications, infections, electrolyte abnormalities, CNS injury, uremia, organ failure, illicit drug intoxication or withdrawal, etc.)
High Risk: post-op, esp. if heart disease or DM
MC presentation of AMS in the inpatient setting
MCC: alcohol abuse (delirium tremens)
*thyroid storm
Underlying organic cause: UTI, pneumonia, metabolic changes, CVA, MI, TBI, medications (anticholinergics, BDZs, opioids)
sx
Rapid onset associated w/ fluctuating mental status changes & marked deficit in short-term memory
*Acute & rapid deterioration in mental status (hrs-days)
*Fluctuating level of awareness
*Disorientation
Visual hallucinations (MC)
Fall precautions – pts w/ delirium are 6x more likely to fall
dx
Mental status exam (MMSE)
Labs:
*chemistry
*B12/folate
Febrile + delirious 🡪 LP (cerebral edema)
tx
Usually associated w/ full recovery within 1wk in most cases
Treat the cause (almost always reversible)
Supportive care:
*sedation
Encephalitis definition, sx, dx, tx
Infection of the brain parenchyma
Etiologies:
*HSV1 MCC
*VZV, EBV, measles, mumps, rubella, HIV
sx
Meningeal sxs:
*HA, neck stiffness
*photosensitivity
*fever, chills, N/V
*seizures
*AMS, changes in personality, speech, & movement
PE: focal neurologic deficits
*hemiparesis
*sensory deficits
*cranial nerve palsies
dx
FIRST 🡪 CT scan
THEN 🡪 LP
*normal glucose, increased lymphocytes
MRI: temporal involvement characteristic of HSV
tx
*IV acyclovir
Giant Cell Arteritis “Temporal Arteritis” definition, sx, dx, tx
*large & medium vessel granulomatous vasculitis of the extracranial branches of the carotid artery (temporal artery, occipital artery, ophthalmic artery, posterior ciliary artery)
sx
*HA, jaw claudication w/ mastication, visual changes
*scalp tenderness
*fever, fatigue, weight loss, night sweats, malaise
dx
*increased ESR/CRP
*temporal bx
tx
*high-dose corticosteroids to prevent blindness
*low dose aspirin to decrease risk of CVA & vision loss
Alzheimer’s Disease definition, sx, dx, tx
MC type of dementia
Risk Factors: ↑ age, genetics, family hx
PATHO: unknown – 3 hypotheses
Amyloid hypothesis: extracellular amyloid-beta protein deposition (senile plaques) in the brain are neurotoxic
Cholinergic hypothesis: acetylcholine deficiency leads to memory, language, & visuospatial changes
sx
Shorter term memory loss – often first symptom
Progresses to long term memory loss & cognitive deficits – disorientation, behavioral & personality changes, language difficulties, loss of motor skills, etc.
dx
Clinical dx (no specific test)
Workup to r/o other causes: MRI, CBC, renal & liver tests, VRDL/RPR to r/o syphilis, B12, thyroid function studies
MRI preferred neuroimaging test – cortex atrophy (medial temporal lobe atrophy), reduced hippocampal volume, white matter lesions
Histologic findings: amyloid-beta protein deposition (senile plaques)
- APP normally degraded by alpha-cleavage; beta cleavage of APP results in amyloid-beta accumulation
- neurofibrillary tangles = intracellular aggregations of tau protein (an insoluble cytoskeletal microtubule element)
tx
Acetylcholinesterase inhibitors: improves memory function & sxs relief
*donepezil
*tacrine
*rivastigmine
*galantamine
NMDA antagonist: memantine – can be adjunctive or used as monotherapy in moderate to severe disease *neuroprotective
- MOA: blocks NMDA receptor, slowing calcium influx & nerve damage
- excitotoxicity causes cell death, NMDA antagonists reduce glutamate excitotoxicity; may be adjunctive
Vascular Dementia definition, sx, dx, tx
Brain disease due to chronic ischemia & multiple infarctions (e.g., lacunar infarcts)
Risk Factors: HTN (most important), DM, hx of CVA, AFIB
sx
Sudden decline in functions w/ a stepwise progression of sxs (random infarct then decline 🡪 stable then another infarct 🡪 decline, etc.)
Cortical manifestations: depends on area affected
*medial frontal: executive dysfunction, apathy, abulia
*left parietal: apraxia, aphasia, or agnosia
*right parietal: hemineglect, confusion, visuospatial abnormalities
Subcortical manifestations: focal motor deficits, gait abnormalities, urinary difficulties, personality changes
dx
Clinical
Workup similar to Alzheimer’s – r/o other causes: B12 & folate, RPR, CBC, etc.
MRI: white matter lesions, cortical or subcortical infarcts
CT: may show lacunar infarcts
tx: Strict BP control
Frontotemporal Dementia (Pick’s Disease) definition, sx, dx, tx
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
Diffuse Lewy Body Disease definition, sx, dx, tx
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
Essential Familial Tremor definition, sx, dx, tx
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
Guillain Barre Syndrome definition, sx, dx, tx
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
Huntington Disease definition, sx, dx, tx
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
Intracranial Tumors
~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
Viral Meningitis definition, sx, dx, tx
*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
Bacterial Meningitis definition, sx, dx, tx
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)
TB Meningitis definition, sx, dx, tx
*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
Fungal Meningitis definition, sx, dx, tx
**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
Multiple Sclerosis definition, sx, dx, tx
*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
Myasthenia Gravis definition, sx, dx, tx
*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
Parkinson Disease definition, sx, dx, tx
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
Levodopa MOA
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
Dopamine Agonists MOA
- bromocriptine
- pramipexole
- ropinirole
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
Anticholinergics MOA
- trihexyphenidyl
- benztropine
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
Amantadine MOA
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
Seizure Disorders different types
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
- w/ Retained Awareness (Simple)
*no alteration in consciousness - w/ Loss of Awareness (Complex)
*altered consciousness, automatisms (lip-smacking)
*postictal state: confusion & loss of memory
Type II: Generalized Seizures
- Absence (Petit Mal)
*brief lapse of consciousness, staring episodes w/ pauses
*eyelid twitching, lip smacking; NO POSTICTAL - Atonic (Drop Attacks)
*sudden loss of muscle tone - Tonic
*extreme rigidity 🡪 LOC - Clonic
*repetitive rhythmic jerking; often associated w/ postictal state - Myoclonic *no LOC
*sudden, brief, sporadic involuntary twitching - 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
Status Epilepticus definition, sx, dx, tx
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)
Seizure dx
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
Types of syncopes
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
