Neurology Flashcards

(268 cards)

1
Q

What is the most common headache type?

A

Tension

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2
Q

What headache is most common to lead to PCP visit?

A

Migraine

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3
Q

What are the primary headache types?

A

Migraine, tension, cluster, new daily persistent

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4
Q

Migraine

A

episodic attacks of severe headaches often associated with nausea, photophobia and/or phonophobia

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5
Q

Phases of Migraines

A

Prodrome-hours-days prior
Aura-w/in hour before headache
Headache
Postdrome-up to 48 hours after

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6
Q

Migraine Prodrome Symptoms

A

fatigue, difficulty concentrating, neck stiffness, photo/phonosensitivity, nausea, blurred vision, yawning, pallor

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7
Q

Migraine Postdrome Symptoms

A

tired, difficulty concentrating, neck stiffness

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8
Q

Common Migraine Criteria

A

At least5 lasting 4-72 hours
@least 2: Unilateral, pulsating, mod-severe pain, aggravated w/ activity
@least 1: N/V, photo and phonophobia

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9
Q

Migraine w/ Aura

A

Classic migraine WITH
@least 1: visual, sensory, speech, motor, brainstem, retinal
@least 3: aura symptom spreads gradually, 2+ occur in succession, each aura symptom lasts 5-60 minutes, at least 1 symptom unilateral and positive, followed by headache w/in 60 minutes

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10
Q

Aura

A

transient focal neurologic reversible symptoms preceding or accompanying the headache

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11
Q

Brainstem Aura (Basilar migraine)

A

No motor or retinal symptoms w/ @least 2: dysarthria, vertigo, tinnitus, hypacusis, diplopia, ataxia, decreased level of consciousness

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12
Q

Retinal Migraine

A

Aura of fully reversible monocular positive/negative visual phenomena confirmed by clinical visual field or patients drawing of monocular field defect
@least 2: aura spreads over 5 minutes, lasts 5-60 minutes and followed by headache w/in 60 minutes

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13
Q

Hemiplegic Migraine

A

Aura has BOTH: reversible motor weakness and reversible visual, sensory, and or speech symptoms

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14
Q

Menstrual Migraine

A

-2 to +3 days of cycle, aura uncommon, related to decline in estrogen

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15
Q

Menstrual Migraine Treatment

A

Preventative: NSAIDs -7 to +6 days, Triptans -2 to +4 days, Magnesium day 15-menses
Extended-cycle hormonal treatment
Abortive: rest, dark, quiet

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16
Q

Chronic Migraine

A

> 15 days/month for 3 months, at least 8 days/month has features of migraine

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17
Q

Migraine Treatment

A

Rest, quiet, dark

NSAIDs, acetaminophen, triptans, nit-emetics, ergotamine

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18
Q

Mild-moderate Migraine treatment

A

NSAIDs first line (ibuprofen, naproxen, ketorolac (injection))

2nd: acetaminophen/tylenol
3rd: Excedrin (ASA/acetaminophen/caffeine)

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19
Q

Mod-Severe Migraine Treatment

A

1st line: Triptans (canc ombrine w/ naproxen for ^ efficacy)

2nd line: Ergots

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20
Q

Triptans

A

MOA: vasoconstrictors, activate serotonin receptors
Avoid in pregnancy (cat C)
Containdicated: coronary/vascular disease, hemiplegic or basilar migraine, hx of stroke/uncontrolled HTN, prinzmetal angina
Side effects: N/V

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21
Q

Ergots

A

MOA: serotonin agonist
Rectal formulations available
Avoid in CVD, CYP3A4 inhibitors
Pregnancy cat X

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22
Q

Alternative Migraine Treatment (last resort)

A

opioids-can cause tolerance, abuse, dependence

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23
Q

Adjunctive Migraine Therapy

A

Antiemetics/dopaminereceptor blockers for N/V: metoclopramide, prochlorperazine, promethazine
Butalbital containing combo analgesics: high risk of overuse/dependence
Hydration

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24
Q

Preventative Migraine treatment

A

Acupuncture, avoid triggers, behavioral modification, headache diary

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25
Botox
FDA approved for chronic migraine | Blocks release of Substance P and CGRP, inhibits peripheral signals to CNS ad blocks central sensitization
26
Tension Headache Criteria
Last 30 minutes-7 days, @least 2: bilateral, pressing/tightening (non-pulsating) band, mild-mod intensity, not aggravated by activity BOTH: no N/V, no more than 1 of photo/phonophobia
27
Tension Headache Treatment
1st line: NSAIDs, acetaminophen, aspirin; canc combine w/ caffein for ^ effect Tricyclic antidepressants (amitriptyline), other antidepressants (mirtazapine, venlafaxine), anticonvulsants (topiramate, gabapentin), tizanidine, lidocaine in trigger points, botox NO: opioids, butalbital, muscle relaxants
28
Tension Headache Non-pharma treatment
Biofeedback, CBT, relaxation techniques, acupuncture, PT
29
Cluster Headaches
Least common primary headache usually <60 minutes up to 8 times/day, pain unilateral near eye, often at night (awakening), "severe, piercing, boring, exploding, penetrating" Associated w/ suicide ideations
30
Forms of Cluster Headaches
Episodic (more common): phases last 2-16 weeks, then cluster free for 6 months Chronic: no cluster free episodes for >1 month
31
Cluster Headache Criteria
Severe unilateral orbital/temporal pain w/ @least 1: conjunctival injection/lacrimation, nasal congestion/rhinorrhea, mitosis/ptosis, eyelid edema, forehead sweating Sense of restlessness/agitation Frequency 1 every other day to 8/day for >half the time
32
Cluster Headache Treatment
1st line: 100% O2 or sumatriptan intranasal lidocaine, ergots, IV dihydroergotamine Glucocorticoids Prophylactic: verapamil (not in heart block or arrhythmias), greater occipital nerve block, surgical options
33
New daily Persistent Headache Criteria
``` Persistent headache (1.5-24 hours/day) present for >3 months with known onset, may be mix of migraine or tension-type, usually bilateral with nausea, photo/phonophobia Treat like tension or migraine ```
34
Headache Red Flags
Abrupt/sudden onset, onset >50, new onset when pregnant/post-partum, worst HA of life, change in HA, weight loss, fever, severe hypertension
35
Pseudotumor Cerebri
Elevated intracranial pressure in overweight women of childbearing age Headache that can be exacerbated with posture changes, relieved w/ NSAIDs Associated with visual obscurations, pulsatile tinnitus, photopsia, back pain, retrobulbar pain, diplopia, visual loss Could be caused by growth hormones, tetracyclines, hypervitaminosis A
36
Pseudotumor Cerebri Diagnosis/treatment
Elevated ICP on LP, normal CSF, MRI w/ venography negative Weight loss, decrease sodium intake, carbonic anhydrase inhibitors (acetazolamide), loop diuretics, serial lumbar punctures, surgery (nerve shunting/fenestration)
37
Rebound Headache
Most commonly due to opioids, butalbital/analgesics, excedrin (ASA/aceta/caffeine) Least likely with NSAIDs May have nausea, weakness/lack of energy, difficulty concentrating, memory problems, irritability Limit acute meds to <10 days/month
38
Rebound Headache Criteria
HA 15+ days/month with pre-existing HA disorder, regular overuse for >3 months (regular intake >10 days/month for most drugs, >15 days/month for simples like NSAIDs, ASA and acetaminophen)
39
Temporal Arteritis
Most common systemic vasculitis, rare <50 Abrupt onset of throbbing continuous HA w/ neck, torso, shoulder and pelvic girdle pain, jaw claudication and fever; association w/ polymyalgia rheumatica BIOPSY for diagnosis
40
Temporal Arteritis Treatment
High dose corticosteroid prednisone 40-60mg w/ biopsy w/in 1 week, 80-100mg for visual sx Should improve w/in 72 hours, then taper w/ ESR/CRP Could be on steroids up to 5 years
41
Trigeminal Neuralgia
MC aberrant loop of artery or vein, compressing trigeminal nerve root More common in MS patients, often mandibular/maxillary Extreme "electric shock-like/shooting" pain to light tough (like wind) last seconds-minutes
42
Trigeminal Neuralgia Treatment
Antidepressants and anti-seizure meds (carbamazepine), narcotics rarely effective Best long term outcome: microvascular decompression
43
Peripheral Neuropathy
Most commonly sensory but cane motor or autonomic Weakness, sensory loss and/or positive sensory symptoms (burning, tingling); bilateral, gradual onset w/ distal involvement
44
Peripheral Neuropathy Causes
Hereditary: Charcot-marie-tooth disease, porphyria Acquired: Lyme disease, endocrine (diabetes), vitamin deficiencies (B12, Thiamine), B6-also in excess), inflammatory, rheumatic, organ failure, toxins
45
Charcot-Marie-Tooth disease
Most common hereditary PN-demyelination thats asymptomatic until late>distal leg weakness, foot deformities, muscle atrophy below knee, reduced/absent reflexes, sensory deficits Treat with PT/OT and braces
46
Poryphoria
Metabolic disorder caused by deficiency in heme biosynthetic pathway Very rare Presents w/ sharp abdominal pain, agitation, hallucinations, seizures>days later extremity pain/weakness (asymmetric, proximal or distal)
47
Diabetes PN
Most common PN in developed countries | Distal symmetric sensory or sensorimotor polyneuropathy most common
48
Diabetic Neuropathy Presentation
Sensory loss-+/-, "stock glove", painless injuries, not along dermatomes Motor symptoms: distal, proximal or weakness Autonomic: involves CV, GI, GI and sweat glands, ataxia, gait instability, syncope
49
Diabetic Neuropathy Treatment
Glucose control, foot care education, podiatry referral | Anti-epileptics, anti-depressants, Na channel blockers, other analgesics, meds for autonomic dysfunction
50
Hypothyroidism Peripheral Neuropathy
Most common manifestation is carpal tunnel syndrom | Correct hypothyroidism
51
B12 Deficient Peripheral Neuropathy
Glossitis, paresthesias, sensory loss (starting w/hand), hyperreflexiaw/ absent achilles, behavior changes Supplement w/ 1000ug IM weekly for one month, then monthly OR 1000ug PO daily
52
Thiamine (B1) Deficient Peripheral Neuropathy
"beri-beri" disease most commonly caused by alcohol abuse Presents w/ mild sensory loss, burning dysphagia in toes/feet, aching/cramping in calves, distal sensory loss in feet/hands Treat with thiamine replacement until proper levels restored
53
Carpal Tunnel Syndrome
compression neuropathy of median nerve-most common in arm | Can be extrinsic (work/recreation related) or intrinsic small space, fluid retention)
54
Carpal Tunnel Presentation
Aching radiating to thenar area w/ numbness, weakness/dropping objects, pain worse w/activity and at night "flick sign", thenar atrophy, weak thumb opposition
55
Carpal Tunnel Diagnosis/Treatment
Tinnels/Phalens tests, nerve conduction testing prior to sx consult (steroid injection or tunnel release) Short term NSAIDs, PT, ergonomic changes, wrist splint esp @ night
56
Ulnar Neuropathy "Cubital Tunnel Syndrome" Presentation
Ulnar nerve compression/trauma Parasthesias, tingling, numbness in last 2 fingers, pain @ elbow/forearm w/ weakness Atrophy and weakness of pinky/ring fingers
57
Ulnar Neuropathy Treatment
Avoid aggravationg factors, elbow pads | Surgery-after 6-12 weeks of no improvement, progressive palsy/paralysis, long-standing lesion (clawing)
58
Radial Neuropathy
Transient compressive injury (from crutches) presenting with wrist drop, finger weakness (thumb side) Usually spontaneously recovers in 6-8 weeks but can use cock-up wrist/finger splints to avoid further compression
59
Lateral Cutaneous Femoral Neuropathy
Parasthesias, numbness, pain in lateral thigh increased with standing or walking-normal strength and reflexes Resolves spontaneously but treat w/ weight loss, lido patch, NSAIDs, neuropathy meds, avoid tight belts
60
Peroneal Neuropathy
Presents w/ foot drop, sensory loss, sudden onset, no pain | Mange w/ weight loss, ankle brace, knee pad, avoid leg crossing-resolves spontaneously
61
Bells Palsy
``` Facial nerve (CN7) neuropathy, lower motor neuron lesion affection all branches >weakness/paralysis Diabetes and pregnancy are risk factors ```
62
Bells Palsy presentation
Sudden onset peaked in 3 days Unilateral facial muscle paralysis-forhead, facial creases, mouth drooping, eyelid sagging; tearing and loss of corneal reflex Decreased tasted, numbness, difficulty eating/speaking, face feels stiff
63
Bells Palsy Diagnosis
H&P | Can use electrodiagnostic testing, high res CT, serology testing, audiometry
64
Bells Palsy Treatment
Prednisone w/in 3 days of symptom onset Valacyclovir for severe palsy or HZV presentation Eye protection, acupuncture, PT
65
Complex Regional Pain Syndrome
Triad of burning pain, autonomic dysfunction and trophic changes preceded by sx or trauma Disorder of extremities characterized by autonomic and vasomotor instability
66
CRPS Presentation
Findings localized to arm or leg but not a single nerve-most common in hand Pain (burning/aching) aggravated by stress or environmental changes, color/temp changes, changes in skin and nails, limited ROM, edema, weakness, tremor, spasm
67
CRPS Diagnosis
Starts 4-6 weeks after limb trauma but no longer explained by the trauma, goes beyond region involved in trauma Bone changes Budapest Consensus criteria
68
Budapest Consensus Criteria
Continuing pain disproportional to inciting event | >1 symptom in 3 categories and more than 1 sign in 2 categories: sensory, vasomotor, sudomotor//edema, motor/trophic
69
CRPS Treatment
NSAIDs for mild, Red for severe with edema Tricyclic antidepressants, anticonvulsants, SNRIs, lido, calcitonin, tramadol, neuromodulation, PT/OT Prevent with early mobilization after injury/surgery
70
Tourette Syndrome
Neurodevelopmental disorder manifested by motor and phonic tics, childhood onset Often accompanied by ADHD/OCD
71
Tourette Manifestations
Motor: simple (blinking, shrugging, jerking), complex (gait, kicks, jumping, scratching), echopraxia-mimicking gestures, copropraxia (obscene gestures/flipping off) Verbal: Simple (sniffing, coughing, grunting), coprolalia (obscene words), Echolalia (repetition of words), Palilalia (repeating phrases/babbling) Ritualistic Behavior
72
Tourettes Diagnostics
Brain MRI if abnormal neurons exam EEG if possible seizures Criteria: both multiple motor and 1+ phonic tics, many times a day most days for more than a year, prior to 18 years old
73
Tourette Treatment
``` Treat comorbids first Comprehensive behavioral intervention for tics (CIBT) First line: Clonidine or guanfacine Favored: Tetrabenazine Severe tics: Haloperidol Botox for focal motor tics ```
74
Mallampati Scoring
Risk of Sleep Apnea Class 1: tonsils, uvula, soft palate fully visible Class 2: Hard/soft palate, upper portion of uvula and tonsils visible Class 3: soft and hard palate and base of uvula visible Class 4: only hard palate visible
75
Polysomnogram
Measure sleep stages, respiratory effort and airflow, oxygen saturation, limb movement, heart rhythm, etc Uses EEG, EOG (electrooculogram) and EMG (surface electromyogram) all in one
76
Home sleep test
Tests for obstructive sleep apnea
77
Multiple Sleep Latency Test (MSLT)
helpful for narcolepsy-measures propensity to sleep during day after restful night of sleep
78
Wakefulness Test
Measues ability to sustain wakefulness during daytime; evaluates efficacy of therapy, narcolepsy and sleep apnea
79
Epworth Sleepiness Scale (ESS)
Score of >10 is significant sleepiness
80
Insomnia Criteria
Symptoms 3x/week, problem with sleep maintenance or initiation w/ adequate opportunity and circumstance to sleep and daytime consequences Short term <3 months or related to stressor Chronic >3 months
81
Insomnia Diagnosis and Treatment
Diagnose w/ sleep history, meds, conditions, etc Treat w/ education of sleep hygiene and stimulus control counseling; relaxation/CBT/sleep restriction therapy Meds: Benzos, nonbenzos (zaleplan, zolpidem), melatonin agonsists (ramelteon), tricyclics (doxepin) and suvorexant (orexin antagonist)
82
Obstructive Sleep Apnea Diagnosis
Caused by repetitive collapse of upper airway during sleep 15+ events per hour Can diagnose if: excessive sleepiness/fatigue/insomnia, waking up gasping/choking, habitual snoring, HTN, mood disorder, cognitive disfunction, CAD, stroke, HF, DM, afib
83
Sleep Apnea Presentation
Excessive daytime sleepiness, snoring, choking or gasping during sleep Obesity, crowded/narrow OP airway, elevated BP, signs of pulmonary HTN or cor pulmonale, mallampati scoring
84
Sleep Apnea Diagnosis/Treatment
Polysomnography Treatment: weight loss/exercise, sleep positioning, avoid alcohol/benzos; CPAP (20-40% pts don't use it or only 4 hours/night), upper airway sx, hypoglossal nerve stimulation
85
Narcolepsy
Loss of hypothalamic neurons that produce orexin neuropeptides; difficulty sustaining wakefulness, poor regulation of REM, disturbed nocturnal sleep Type 1 w/ cataplexy Type 2 w/out cataplexy
86
Cataplexy
Sudden muscle weakness without loss of consciousness, usually triggered by strong emotions
87
Hypnagogic Hallucinations
dream-like hallucinations at sleep onset or upon awakening (hypnopompic)
88
Narcolepsy Presentation
Excessive/severe daytime sleepiness but feel rested upon wakening, ESS>15, "sleep attacks" Fragmented sleep, other sleep disorders, obesity, psychiatric comorbidities
89
Narcolepsy Diagnosis
Polysomnogram to rule out other cause | MSLT-sleep latency <8 minutes and REM episodes in at least 2 of the naps
90
Narcolepsy Treatment
Adequate sleep with 1-2 20min naps, screen for depression, anxiety, CV, avoid meds (Benzes, opioids, antipsychotics, alcohol) 1st line: modafinil (wake promoting) Methylphenidate or amphetamines-CNS stimulants Sodium Oxybate for cataplexy (CNS depressant) or antidepressants
91
Shift Work Disorder Presentation
Fragmented sleep, difficulty falling/staying asleep, poor sleep quality, reduced sleep duration
92
Shift Work Disorder Management
Improve daytime sleep, regular sleep schedule during off-work periods too, sleep hygiene, CBT Meds: short acting hypnotics, exogenous melatonin, caffeine,wake promoting agents, provigil
93
Parasomnias
More common in kids NREM: confusional arousal, sleep walking/terrors/eating REM: sleep paralysis, nightmare disorder, abberations
94
NREM Parasomnias
Usually occur during N3 stage in first 3rd of major sleep period Criteria: recurrent episodes of incomplete wakening, absent responsiveness, limited cognition/dream report, partial or complete amnesia for event Diagnose w/ polysomnogram and history
95
Sleep Terrors
Sudden arousal with sitting up, intense fear, piercing scream, intense autonomic activation (tachycardia, tachypnea, diaphoresis, flushing, mydriasis) Fightened, confused, inconsolable, no recollection of event, calmly returns to sleep after several minutes
96
Sleep Walking
Slow, quiet movement with eyes open, terminate spontaneously, can be agitated or aggressive when aroused Activities: making/eating food, cleaning, rearranging, driving, inappropriate behavior
97
Sleep Related Eating Disorder
Variant of sleep walking; involuntary eating w/ diminished LOC during arousal from sleep, not linked to daytime eating disorders
98
NREM Parasomnia Management
Avoid sleep deprivation, alcohol, meds Maintain consistent sleep schedule, safety intervention but allowed to move freely/not woken up Anticipatory awakening before event if its regular
99
Pathways to CNS infections
Invasion of bloodstream Retrograde neuronal pathway Direct contiguous spread
100
Lumbar Puncture
Opening pressure gives us the intracranial pressure, normal is clear and colorless with water-like viscosity Tests cell count w/ diff, glucose, protein, culture, gram stain, viral PCR Done below the level of spinal cord (L3-L5)
101
Contraindications to Lumbar Puncture
Cushing's triad, decreased LOC, cal neurologic symptoms, papiledema, severe coagulopathy, skin infection/spinal abscess at LP site, mass lesion IF PRESENT DO CT FIRST-if normal can do LP
102
Cushing's Triad
ALL 3: respiratory depression, bradycardia, hypertension
103
CSF Analysis
Abnormal may be cloudy, purulent or pigment-tinged Cloudiness begins with WBC>200 or RBC>400 or protein>150 Visibly bloody has RBC>6,000 (can be hemorrhagic-will clear byte 3, subarachnoid hemorrhage, intracerebral hemorrhage or cerebral infarct) Elevated WBC indicates infection, vasculitis, leukemia infiltration or traumatic tap
104
Encephalitis
Acute inflammation brain parenchyma causing abnormalities of brain function 70% viral, 20% bacterial Can be primary (neuronal involvement, virus present) or post-infectious (virus no longer present, demyelination occurs, possible autoimmune, no neuronal involvement)
105
Encephalitis risk factors
Outdoors (forestry workers, campers, hunters), travel to endemic areas, compromised immunity, lack of vaccines
106
Viral Encephalitis Pathogens
Herpes, arthropod-borne (west-nile, st Louis, la cross, Colorado tick fever), rabies, HIV, enteroviruses (coxsackie, polio), measles, flu, mumps, adenovirus
107
Bacterial Encephalitis Pathogens
Borrellia burgdorferi (lyme), M. Tuberculosis, treponema pallidum (syphilis), lots of others
108
Noninfectious Encephalitis causes
Paraneoplastic syndrome, drug toxicity, autoimmune (SLE, sarcoid), radiation, metabolic disorders
109
Encephalitis Presentation
Fever, altered mental status, seizures, focal neurologic symptoms (motor/sensory, paralysis, nerve palsies, exaggerated reflexes, speed disorder) No signs of meningeal irritation
110
Encephalitis Diagnostics
MRI (but start with CT w/out contrast if sick to rule out mass lesion) LP/CSF analysis (lymphocytes, ^WBC, pressure, protein, cloudy, normal glucose) CBC, serum culture Brain biopsy is gold standard
111
Encephalitis Treatment
Stabilize: ET tube, ventilator, circulatory support, electrolytes Empiric antiviral (Acyclovir 10mg/kg IV Q8H) DVT and ulcer prophylaxis Elevated ICP: elevate bed 30-45*, avid jugular compression, corticosteroids and mannitol
112
Meningitis
Inflammatory disease of leptomeninges
113
Meningitis Risk Factors
Extremes of age, birth history/maternal infection, immunocompromised, vaccine status, exposure risk, injection drug use
114
Meningitis Presentation
Headache, N/V, fever, neck stiffness, photophobia, drowsiness, seizures Bacterial until proven otherwise
115
Viral Meningitis
MC is enterovirus, can be HSV, HIV, WNV present w/ normal symptoms +URI symptoms, rash or diarrhea Normal to mild elevations on CSF
116
Bacterial Meningitis
Rapid progression In neonates from birth canal or through placenta, in adults from nearby infection or penetrating injury Cerebral edema, increased ICP, neurologic damage, death
117
Bacterial Meningitis Pathogens
Neonates (<28 days): Group B strep (S. agalactiae) Babies, children, adults: S pneumoniae Teens, young adults: Neisseria meningitides
118
Bacterial Meningitis Presentation
Rapid or slow onset, fever, meningismus, photophobia, severe headache, N/V, back pain, altered mental status, widened pulse pressure, brudzinski and kerning signs In babies: "floppy", rash (petechiae)
119
Bacterial Meningitis Diagnosis
Blood cultures, CBC, LP/CT (don't wait for this before antibiotics though!) CSF: ^ICP, ^^WBC, ^^^protein, no RBC, low glucose, neutrophils present
120
Bacterial Meningitis Treatment
Antibiotics ASAP Empiric: Ceftriaxone/cefotaxime + vanco+ampicillin (if>50) Need high doses to cross BBB Supportive care: fluids, elevate bed, dexamethasone (reduce risk of hearing loss), +/- induced hypothermia
121
Bacterial Meningitis Prognosis
Emergency-100% death if untreated, can still be long term probs w/ treatment (hearing loss, intellectual impairment, seizures) Mortality increases with age and risk factors
122
Bacterial Meningitis Prevention
Cipro for close contact (prolonged contact >8 hours or direct exposure to oral secretions) Antibiotics (cillins) during labor-tested @ 35-37 weeks Vaccines
123
Encephalitis vs Meningitis
Difficult to distinguish, will usually be treated for both (antibiotics plus acyclovir)
124
Fungal Meningitis
Rare, consider in immunocompromised NO person-person spread cryptococcus, visto, blastomyces, cocci
125
Aseptic Meningitis
Clinical and lab evidence of meningeal inflammation w/out signs of bacterial infection Can be caused by meds (bacterium, NSAIDs, IVIG, chemo, immunosuppressives), malignancy, SLE, head injury, brain surgery
126
Multiple Sclerosis
Autoimmune disorder of CNS associated with destruction of myelin and nerve fibers Unknown cause, can be genetic, environment and immune system factors Most frequent cause of permanent disability in young adults other than trauma
127
Types of MS
Clinically isolated syndrome (first attack) Relapsing-remitting (RRMS) Secondary Progressive (SPMS/transitional form) Primary progressive (PPMS)
128
Relapsing Remitting MS
unpredictable attacks that may or may not leave permanent deficits followed by periods of remission Most common form
129
Secondary Progressive MS
Initial RRMS followed by gradual worsening w/ or w/out occasional relapses, remissions and plateaus 10-20 years after disease onset
130
Primary Progressive MS
Steady increase in disability w/out attacks | Poor/short prognosis
131
MS Presentation
Internuclear ophthalmoplegia , optic neuritis (painful monocular vision loss w/ scatoma), Lhermittes sign (electric shock down spine from neck flexion), diplopia, bladder/bowel dysfunction, Uhthoff phenomena (her sensitivity), fatigue, nystagmus
132
Internuclear ophthalmoplegia
Abnormal horizontal ocular movement w/ lost or played adduction and horizontal nystagmus of abducting eye
133
MS Physical exam
Psychomotor slowing, asymmetric reflexes, spasticity, weakness
134
MS Diagnosis
Brain/spinal cord MRI / gadolinium CSF-oligoclonal bands present ONLY in MS MUST HAVE dissemination in space and time (plaques) McDonald Criteria
135
McDonald Criteria
Uses MRI+/- CSF, dissemination in space, dissemination in time
136
MS Treatment
High doses of methylprednisolone for attacks Disease modifying therapy for RRMS Treatment for SPMS or PPMS vary
137
Disease Modifying therapy in MS
Decrease relapse rate, slow accumulation of brain lesions on MRI Interferon*, alemtuzumab, dimethyl fumarate, fingolimod; available as infusion, injections and orally
138
Myasthenia Gravis
Fluctuating degree and variable combination of weakness in ocular, bulbar, limb and respiratory muscles due to antibodies against muscle receptor for acetylcholine "fatiguable weakness" Most common disorder or neuromuscular transmission
139
Myasthenia Gravis Presentation
Occular symptoms initial/MC presentation (ptosis, diplopia, burred vision), weakness of bulbar muscles (2nd most common; drooling, fatiguable chewing, facial weakness, dysarthria, dysphagia), rare limb weakness-asymmetric/proximal Thymoma in 10-15%, hyperplasia in 60-70%
140
Types of MG
Ocular-weakness limited to eyelids and extra ocular muscles; Ab present in 50% Generalized- weakness affects ocular, bulbar, limb and respiratory muscles; Ab present in 90%
141
MG Diagnosis
H&P, ice pack test for ptosis, repetitive nerve stimulation, single-fiber electromyography, serologic testing (AChR-Ab and MuSK-Ab) Chest CT to rule out thymoma/hyperplasia
142
MG Treatment
Symptomatic- anticholinesterase agents FIRST LINE (pyridostigmine, prolongs effect of ACh) Immunosuppresive- glucocorticoids (azathioprine or mycophenolate) Rapid/short acting- plasmapheresis and IVIG Surgical- thymectomy (can still have symptoms after)
143
Myasthenia Crisis
Worsening of myasthenia weakness requiring intubation or ventilation>respiratory failure Life threatening, precipitated by infection/truma/drugs Plasmapheresis speeds recovery Slurred speech, dyspnea, decreased forceful coughing, weak voice
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Plasmapheresis
Blood pumped out of body through machine to remove antibodies, then back into body Similar to dialysis
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Lambert-Eaton Myasthenia Syndrome (LEMS)
Immune system mistakenly attacks own tissues | Antibodies against the nerve where acetylcholine is released
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LEMS Presentation
Depressed/absent reflexes, autonomic dysfunction (dry mouth, slow pupil response, impotence), postexerise facilitation (recovery of reflexes with VIGOROUS muscle activation like sprinting) but proximal legs fatigue with non-vigorous action
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LEMS Diagnosis
H&P Serology (VGCC Ab) Repetitive nerve stimulation-increased amplitude CT chest/abdomen/pelvis bc of risk of malignancy
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LEMS Treatment
Amifampridine (enhances calcium entry), guanidine, pyridostigmine Prednisone and azathioprine together or alone for immunosuppression IVIG (temporary improvement)
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Guillain-Barre Syndrome
Immune mediated polynephropathy; antibodies attack axons and/or myelin Acute, paralyzing illness provoked by preceding infection by 2-4 weeks (Campylobacter jejuni)
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Guillain Barre Types
MC: acute inflammatory demyelinating polyneuropathy (AIDP)-parasthesias in hands/feet Acute motor axonal (AMAN) Acute motor/sensory axonal (AMSAN) Miller Fisher Syndrome-only one with eye component
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Guillain Barre Presentation
Symmetric ascending muscle weakness and diminished reflexes w/ no visible atrophy Radicular or spinal pain, sensory symptoms (paresthesia in extremities) Can present independently or together-often gait disturbance Can have CN involvement, respiratory failure, autonomic disturbances (unstable HR/BP, hyponatremia, urinary retention) NO fever, meningeal signs, leukocytosis, papilledema,
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Guillain Barre Diagnosis
LP/CSF: ^^protein w/ normal WBC Nerv conduction tests Serology (GQ1b Ab in Miller Fisher) Spine MRI (enhanced intrathecal nerve roots/cauda equina)
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Guillain Barre Treatment
Supportive Care-hospitalization for respiratory/CV function, DVT prophylaxis Plasmapheresis, IVIG No corticosteroids Usually spontaneous recovery in 3-4 weeks
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Essential Tremor
Most common action tremor in adults, can be familial
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Essential Tremor Presentation
Classically affects hands and arms, bilateral, slightly asymmetric Most pronounced with movement, absent when relaxed May also involve head, voice and legs, can be relieved w/ alcohol Exacerbated by anxiety/stress, illness, meds, NOT caffeine, worsens over time
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Essential Tremor Diagnosis
Clinical Isolated tremor of bilateral arms for at lest 3 years +/- head, voice or leg tremor with normal neurons exam Can use DaTscan to rule out Parkinsons
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Essential Tremor Treatment
1st line: propranolol or primidone, often combined after 1 year Can use Benzes or alcohol for exacerbations Deep brain stimulator for severe
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Parkinsons Presentation
``` Rest tremor (pill rolling), rigidity (cogwheel or lead pipe), bradykinesia (most common) and postural instability (pull test) Cognitive dysfunction/dementia w/ Lewy bodies, sleep disturbance, autonomic (BP) dysfunction, psychosis/hallucinations ```
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Parkinsons Diagnosis
Gold standard: neuropathologic exam Can do MRI to rule out other dx Improved bradykinesia/rigidity with dopaminergic drugs
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Parkinsons Treatment
MAOI type B (rasagiline, safinamide), dopamine agonists (prmiprexole, ropinerole, bromocriptine), Carbidopa/levodopa @nd line: COMT inhibitors (entacapone, tolcapone) Deep brain stimulator, continuous carbs-levo infusion (LCIG), continuous subQ apomorphine infusion (CSAI)
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Huntingtons Disease
Autosomal dominant disorder with mid-life onset, high cause of suicide Caused by >36 repeated of mutated HTT/HD gene (CAG)
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Huntingon Presentation
Chorea (defining symptom), psychiatric illness (paranoia, delusions, hallucinations, depression, irritability), dementia, weight loss/cachexia
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Juvenile Huntington Disease
Presents before 20 years | Myoclonus, seizures, behavioral problems and Parkinsonism are more common, but chorea is absent
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Huntington Diagnosis
Clinical features, family history, confirmatory genetic testing for CAG repeat in HTT gene MRI may show caudate atrophy
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Huntington Treatment
None; therapy focused on symptom management and supportive care w/ multidisciplinary team and palliative care Dopamine depleting agents or neuroleptics for chorea SSRIs for depression, ST for dysphagia, NSAIDs for pain
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Muscular Dystrophy
X-linked recessive defect that impairs normal muscle function Most common forms are Duchenne (DMD-2/3 from mother 1/3 spontaneous; loss of functional expression, low dystrophin levels) and Becker (BMD-mostly all from mother; reduced functional expression with normal dystrophin and altered protein), some carriers may show mild symptoms
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Muscular Dystrophy Presentation (DMD)
Growth delay in first year, cognitive impairment, Gowers sign (uses hands to get up), toe walking, calf hypertrophy, impaired respiratory function, scoliosis, dilated cardiomyopathy, incontinence Elevated AST/ALT
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Muscular Dystrophy Presentation (BMD)
Growth delay and cognitive impairment uncommon Live longer, less muscular and respiratory involvement Cardiac involvement is prominent feature Elevated AST/ALT
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Muscular Dystrophy Diagnosis
Serum creatine kinase levels (50-100x normal in DMD, 5x in BMD) Genetic testing for Xp21 gene mutation Muscle biopsy (absence of dystrophin in DMD, diminished quality/quantity in BMD)
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Muscular Dystrophy Treatment
``` Daily prednisone (deflazacort is alternative) PT, sx for scoliosi/muscle contracture, ambulatory and ventilatory support, cardiac meds ```
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Amyotrophic Lateral Sclerosis (Lou Gehrigs)
Mixed upper/lower motor neuron disease UMN: slowness, hyperreflexia, spasticity LMN: weak, atrophy, fasciculation Most common motor neuron disease, commonly in sporadic form
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ALS Presentation
Asymmetric limb weakness is most common (split hand syndrome), bulbar symptoms are second most common (dysarthria, dysphagia), spastic gait, tongue fasciculations, jaw clenching, may have cognitive and autonomic symptoms Neuromuscular respiratory failure is most common cause of death
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ALS Diagnosis
El Escorial Criteria, EMG looking for degeneration or fasciculations, MRI of brain,, C and T spine Really just ruling out other things
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El Escorial Criteria (ALS)
Evidence of LMN degeneration, evidence of UMN degeneration, progressive spread of symptoms or signs
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ALS Treatment
Riluzole-only impact on survival | Edaravone slows deterioration
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Restless Leg Syndrome
Unpleasant or uncomfortable urge tome legs during periods of inactivity, esp evenings Primarily white women with uremia and low iron
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RLS Presentation
Sensation of crawling, tingling, restless, cramping, pulling, painful, electric worsens with age, antihistamines, dopamine agonists and antidepressants
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RLS Diagnosis
Iron and BUN levels, review meds | Criteria: urge to move w/ uncomfortable sensation, worsened during inactivity, received by movement, worse in evening
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RLS Treatment
Dopaminergic agents (pramipexole, ropinerole) Alpha-2-delta Ca channel ligands (gabapentin, pregabalin) Benzos Opioids (only if refractory) Iron replacement
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Glascow Coma Scale
scale to assess mentation 15 is best score <8 is comatose 3 is unresponsive/death
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Altered Mental Status
Subjective difficulty thinking clearly to abnormal thought and states of depressed consciousness Can be medical, neurologic or psychiatric in origin
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Assessing Mental Status
Level of consciousness (response to stimuli; alert, clouded, confused, lethargic, obtunded, stupor, coma) Orientation to envronment
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Delirium
Reduced ability to focus, reduced awareness, develops over short periods of time (hours-days) and fluctuates throughout the day; additional disturbance in cognition (memory, disorientation, language), not due to neurocognitive disorder or coma Evidence that disturbance is direct consequence of another medical condition, intoxication/withdrawal, or toxin exposure
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Causes of delirium
Infection is #1 (UTI, pneumonia), medication, catheters, restraints, withdrawal, anemia, uncontrolled pain, electrolyte abnormalities, hypothyroidism
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Delirium Management
Prevention, stop causative drugs Treat reversible contributors Maintain behavioral control (sitter, avoid restraints) Small doses of haloperidol if necessary-NO BENZOS prevent complications
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Altered Mental Status Presentation
Confusion, agitation, combative, disoriented, delusions, hallucinations, sedation, personality chane
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Altered Mental Status Causes
Hypoglycemia, hypercalcemiasepsis, hypertensive encephalopathy Drugs, electrolytes, metabolism, emotions, neuro/nutrition, trauma (CT), temperature (hyper/hypothermia), infection, alcohol
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Wernicke's Encephalopathy
Thiamine deficiency causing medical emergency Ophthalmoplegia, ataxia and confusion Treat with thiamine and multivitamins (add Benzos if associated w. alcohol)
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Altered Mental Status Management
``` Address ABCs, Start interventions early, review meds, treat underlying cause (alcohol withdrawal with Benzos) SNOT cocktail (sugar, naloxone/narcan, oxygen, thiamine) ```
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Dementia
Progressive intellectual decline, slow insidious onset, not reversible 4 types: alzheimers (most common), vascular, Lewy bodies, frontotemporal
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Alzheimers
Most common form of dementia Diagnosis by autopsy or brain biopsy Treated only symptomatically
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Dementia with Lewy Bodies
Progressive dementia caused by microscopic deposits that damage brain cells over time present in plaques (deposits of beta-amyloid) or tangles (twisted fibers of tau protein)
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Vascular Dementia
Impaired blood flow to brain (often after stroke) impairment more sudden than alzheimers HTN, hyperlipidemia and smoking are risk factors
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Dementia Presentation
Establish time of onset, short-term memory loss, inattention, difficulty planning/organizing, agitations, aggression, depression, inappropriate laughing/crying (frontal)
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Dementia Diagnosis
Neuropsychological exam, Brain imaging-MRI, PET scan Serum B12, TSH ApoE gene testing to rule out Alzheimers
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Dementia Treatment
Aerobic exercise, mental stimulation (puzzles) Memantine for Lewy bodies Cholinesterase inhibitors for Alzheimers (donepezil for mild-mod, memantine for mod-severe) SSRIs, Trazodone for insomnia Avoid paroxetine bc of anticholinergic effect
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Creutzfield-Jakob disease
Most common cause of rapidly progressive dementia | PRNP gene
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Concussion
Complex pathophysiological process affecting brain induced by traumatic biomechanics forces AKA mild traumatic brain injury Rapid onset of neurologic dysfunction with spontaneous recovery
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Concussion Presentation
+/- loss of consciousness, confusion, memory loss, visual disturbance (diplopia, light sensitivity), vertigo/impaired balance, headache
200
Post-concussive Symptoms
Last more than a week, up to months | Chronic HA, short term memory difficulty, fatigue, difficulty sleeping, personality change, sensitive to light/noise
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Concussion Diagnosis
Clinical eval, Head CT (if GCS <15 hours from injury, sign of skull fracture, >65years, bleeding diathesis/anticoag use), sports protocols
202
Concussion Treatment
Rest and acetaminophen, return to play after symptoms are gone and no meds required
203
Which tumor is most common in children?
Low-grade astrocytoma (Wills tumor)
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Which tumor is most common in adults?
Glioblastoma
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Clinical manifestation of CNS Tumor
Headache, focal seizures, cognitive dysfunction, increased ICP (N/V, papilledema), focal signs/symptoms (weakness, sensory loss aphasia, visual spacial dysfunction)
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CNS Tumor Eval
Physical and Neuro exam Brain MRI WITH contrast Screen for systemic malignancy Lumbar punture
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CNS Tumor Diagnosis
Tissue sample for histopathologic and molecular study | Functional MRI can be used, or frozen section
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Astrocytoma
Glial cells of brain and spinal cord; star shaped cells that help with maintenance, repair and transporting nutrients Most common primary intra-axial brain tumor
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Grade 1/2 Astrocytoma
Most commonly present with seizures, more common in kids/young adults Usually benign but can become malignant Low degree of cellularity w/ preservation of normal brain tissue
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Grade 1/2 Astrocytoma Treatment
Surgical resection curative depending on location, radiation, chemo Slow growing so best approach is to FOLLOW Can treat symptoms with antiepileptic drugs
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Grade 3/4 Astrocytoma
Malignant gliomas 3-anaplastic, slower growing w/ slower onset of symptoms, mean diagnosis @30, prognosis 3-5 years 4- glioblastoma, mean diagnosis @64, prognosis 11-15 months
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Glioblastoma
Most common primary brain tumor and most deadly, can spread through CSF but rarely systemically
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Glioblastoma Treatment
Surgery not curative (bc of tentacles), can do resection or lobectomy Radiation, chemo (temozolomide)
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Meningioma
Slow growing, extra-axial being tumor arising from arachnoid matter Considered non-malignant, usually non-infiltrating, symptoms from pressure Such slow onset seems like normal signs of aging
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Meningioma Diagnosis
MRI w/ contrast, surgical biopsy
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Meningioma Treatment
Complete surgical resection Can observe w/ few symptoms, little swelling, no negative effect on life, older patients Radiation but no chemo
217
Stroke
Sudden focal neurological deficit or acute neurological impairments caused by interruption of blood flow to a specific region of the brain
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Ischemic Stroke
Blood clot causing interruption of blood flow | Commonly cardioembolic, atherosclerosis or lacunar infarction
219
Hemorrhagic Stroke
Weakened vessel leaking blood causing reduced blood flow | 50% mortality, 80% with permanent disability
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Risk Factors of Stroke
Hypertension, Afib, obesity, cardiac disease, smoking, diabetes Age, gender, fam history, ethnicity, previous stroke
221
What ethnicities have higher risk of stroke?
Black-double whites | Mexicans-higher, less likely to know symptoms
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Cincinnati Pre-hospital Stroke Scale
Facial droop, arm drift, abnormal speech (if all 3 present 100% sensitivity 88% specificity)
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Signs of Stroke
One-sided weakness/numbness.tingling, incoordination, sudden change in speech/language, acute confusion, loss of vision (esp one eye), double vision, sudden severe headache
224
NIHSS Scale
Test to measure level of impairment caused by a stroke, and to determine whether its severe enough to warrant use of tPA (0-42; higher is more severe, 15-20 is mod-severe)
225
Thrombotic Stroke
Atherosclerosis is the most common vascular obstruction leading to thrombosis
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Embolic Stroke
Left sided cardiac chambers and artery to artery stroke are most common sources of emboli Smaller than thrombi=otic, many become hemorrhagic
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Acute Ischemic Stroke vs Transient Ischemic Attack
AIS: >24 hours, typically >1 hour, permanent damage TIA: <24 hours, typically <1 hour, no permanent damage
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Transient Ischemic Attack
Transient episode of neurological dysfunction caused by focal brain, spinal cord or retinal ischemia without acute infarction High risk of stroke later in life
229
Risk of Stroke after TIA
10% in first 90 days after TIA | 1/4 to 1/2 that occur within the first 3 months are usually within the first 2 days
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ABCD2 Score
Determines whether a TIA patient needs to be hospitalized | Age, BP>140/90, symptoms, duration >/<60 minutes, diabetes
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Diagnostic Testing for TIA
Vessel imaging, cardiac eval, lab testing, MRI w/ DWI | Class 2: ECG, echo
232
Penumbra
Zone of reversible ischemia around core of irreversible infarction, salvageable in first few hours after ischemic stroke onset Damaged by hypo perfusion, hyperglycemia, fever, seizure
233
Stroke/BP Relationship
Arterial occlusion increases BP, high BP is not a cause of stroke so we don't want to lower it-worsens outcome of penumbra and can cause hemorrhage (red infarcts) If BP>200 you can lower it, want it below 185 but >120
234
Tissue Plasminogen Activator
Must be given within 3-4.5 hours from last known normal before stroke but LOTS of contraindications
235
Predictors of hemorrhagic transformation
size of infarction, afib, NIHSS, hyperglycemia, thrombocytopenia
236
Arteriovenous Malformations
Directly divert blood from artery to vein, may bypass brain tissue ad cause chronic ischemia, can increase risk of rupture
237
Hemorrhagic Stroke Symptoms
Diastolic BP>110, headache, vomiting, coma, neck stiffness, seizures
238
Common CNS Herniations
Subfalcine-common, HA contralateral leg weakness Transtentorial-oculomotor paresis (dilated pupil, EOMs), contralateral hemiparesis Tonsillar-obtundation/comatose
239
Brain herniation
Life threatening, causes by increased intracranial pressure | Cushing reflex: HTN, bradycardia, abnormal respirations
240
Treatment of Cerebral Aneurysm
Endovascular (coil embolization-nickel and titanium), surgical (clip)
241
Subarachnoid Hemorrhage Presentation
Sudden increase in ICP, associated with valsalva
242
Cause of subarachnoid hemorrhage/Diagnosis
aneurysm in circle of willis, familial causes | Diagnose w/ CT without contrast, LP-xanthochromia (golden yellow blood in CSF)
243
Treatment of Subarachnoid Hemorrhage
Decrease ICP with stool softeners, cough suppressant, anxiolytics, analgesics, antiemetics Treat/monitor vasospasm
244
Subdural Hematoma
Blood in skull outside of brain, crescent shaped, CT to distinguish
245
Epidural Hematoma
round hematoma
246
Stroke Management Supportive Care
Prevent complications: aspiration, DVT, UTI, constipation (use docusate), decubitus ulcers, UGI blee (use PPI/H2B, fever)
247
Post Stroke Depression
Often resolves in one year | SSRIs, but if patient takes warfare use escitalopram, citalopram or sertraline
248
Secondary Stroke Prevention Procedures
``` Carotid endarterectomy (CEA) if 70-99% stenosis Carotid angioplasty/stent only in high risk (re-stenosis, radiation induced stenosis, high risk for CEA, contralateral carotid occlusion ```
249
Carotid Revascularization
Men, older pts, recent cerebral ischemia and ulcerated plaque benefit from CEA the most CEA has lower risk of periprocedural stroke/death, less risk ofrestenosis but stunting has lower risk of cranial nerve injury, MI
250
Stroke Management Meds
Every pt gets a statin antithrombotic agent based on cause AVOID estrogen, sympathomimetics, NSAIDs, PPI
251
Epilepsy
2 or more unprovoked seizures >24 hours apart, 1 seizure w/ risk of recurrent seizures, tendency to unprovoked seizures not caused by any unknown medical condition Most common <1 or >65
252
Epilepsy Treatment
Most people respond to first drug but 30-40% are unresponsive to meds (tried at least 2 meds) Start low go slow, only one drug at a time (try not to do combos)
253
What is the most common cause of death in epilepsy?
Sudden unexpected death
254
Seizure
Sudden urge of abnormal electrical discharges from complex chemical changes in brain cells
255
Temporal Lobe Epilepsy
Most common focal epilepsy, good outcome with surgery | Chewing, licking/smacking lips
256
Frontal Lobe Epilepsy
Often mistaken for psychiatric conditions, less favorable outcome, only some good for surgery Bicycle kicking
257
Juvenile Myoclonic Epilepsy
lifelong, genetic basis, choice of drugs is critical
258
Lennox-Gastaut Syndrome
drug resistant seizures, progressive cognitive/behavioral decline
259
Epilepsy Diagnostics
Labs (tox screen, low glucose, low sodium, rule out infection), EEG (evaluate risk of future seizure), Imaging (MRI w/ cuts through temporal lobe Gold standard is video EEG
260
Common Causes of epilepsy
Genetics, structural (stroke, head trauma, tumor), metabolic (glucose, sodium), infection (encephalitis, abscess), most are unknown
261
Comorbidities of epilepsy
Mood disorders (depression/anxiety, short term memory loss)
262
When to treat with ONE seizure?
Abnormal EEG, significant brain abnormality on imaging, nocturnal seizures, older age, social factors (driving, job)
263
What is the worst thing to do for seizures?
Sleep deprivation, need at least 8 hours
264
What epilepsy drugs are not okay in pregnancy?
Valprioc acid, phenobarbital, topiramate
265
What epilepsy drugs can be used in pregnancy?
Lamotrigine and levetiracetam?
266
When can you stop epilepsy meds?
Seizure free for 2+ years with normal neurons exam and EEG | "cured" if off drugs for 5 years and seizure free for 10
267
Status Epilepticus
Continuous seizures for >5 minutes without return to baseline EMERGENT must get to hospital or rescue med (diazepam) biggest risk is hypoxia
268
Status epilepticus Treatment
Benzos in first 30 mins, IV AED 30-120 mins, general anesthesia >120 mins