Neurology Flashcards
1
Q
Poliomyelitis and spinal muscular atrophy (spinal cord lesion)
A
LMN les*
Anterior horn
2
Q
Multiple sclerosis (spinal cord lesion)
A
Demyelination
Random and asymmetric les*
Esp white matter
3
Q
Amyotrophic lateral sclerosis (spinal cord lesion)
A
UMN + LMN deficits
No sensory, No oculomotor
Fatal
ttt: riluzole (↑survival)
4
Q
Anterior spinal artery occlusion (spinal cord lesion)
A
All spinal cord but spares dorsal columns
5
Q
Tabes dorsalis (spinal cord lesion)
A
Demyelination of dorsal columns + roots
Progr sensory ataxia
Poor coordination
Absence of DTRs and ⊕Romberg
6
Q
Syringomyelia (spinal cord lesion)
A
Anterior white commissure of spinothalamic tract
From trauma/tumor/Chiari (in 35%)
7
Q
Vitamin B12 deficiency (spinal cord lesion)
A
Subacute combined degeneration
Demyelination of dorsal columns, lateral corticospinal tracts, spinocerebellar tracts
8
Q
Facial nerve lesions (UMN vs LMN)
A
-UMN: les* of motor cortex (contralateral paralysis of lower face)
-LMN: ipsilat facial paralysis + inabil to close ipsil eye
Is complic of: AIDS, Lyme, Sarcoidosis, Tumors, Diabetes
Gradual recovery
9
Q
Stroke (RF)
A
#5 mortality in US Disrup bld flow → neurons death → acute focal neuro def Ischemic (80%) or hgic (20%)
Modif RF: CAD, obes, AF, carot steno, hyperchol, smok, HTN, DM, illicit drugs
Nonmodif RF: fam Hx MI/strok, >60yo. male, blac/hisp/asian
10
Q
Stroke (etiologies)
A
- Atheroscl: int/comm carot, basilar, verteb art
- Chron HTN, hyperchol, DM: deep Vx → lacunar infarcts
- Card or Ao emboli
- Others: hypercoag, craniocerv dissect, venous sinus thromb, SCD, vasculitis
11
Q
Stroke in MCA (PE)
A
Contral paresis + sensory loss: Face + arm
Gaze to side of les*
- Nondom hemisph: neglect
- Domin hemisph: aphasia
12
Q
Stroke in ACA (PE)
A
Contral paresis + sensory loss: Leg
Cognit or personal changes
13
Q
Stroke in PCA (PE)
A
Vertigo
Homonymous hemianopsia
4D’s of post stroke: Diplopia; Dizziness; Dysphagia; Dysarthria
14
Q
Lacunar stroke (PE)
A
1 of these:
- Pure motor
- Pure sensory
- Ataxic hemiparesis
- Dysarthria
15
Q
TIA (PE)
A
Sympt dep on location
Neuro def <24h
No findings on MRI
16
Q
Stroke (dg)
A
Emerg head CT w/o contrast (isch <6h not visible)
MRI (early isch)
Immed labs: CBC, PT/PTT, card enz, trop, BUN/creat
Determ underl cause: cardioembol (ECG, echo); thromb (carot US/Doppler, MRA, CTA, angiogr); others
17
Q
Acute ischemic stroke (ttt)
A
-Thrombolytics (tPA) if <3h + No bleed + No CI
SBP<185 and DBP<110 for tPA
-ASA if >3h; switch to Clopidog if already tak ASA
ttt fever + hyperglyc (worse pg)
!monitor for brain swell, ↑ICP, herniation → mannitol + hyperventil
18
Q
Contraindications to tPA therapy
A
Stroke or head trauma ≤ 3mo Anticoag w/ INR >1.7 or ↑PTT MI in ≤ 3mo Prior intracran hge ↓plts <100K SBP >185 or DBP >110 Major surgery ≤ 14d TIA ≤ 6mo GI or urin bleed ≤ 21d Glycemia >400 or <50 Seizures at onset of stroke
19
Q
Preventive and long-term treatment for stroke
A
Prevent complic: aspir pneumo, UTI, DVT
Manage HTN, hyperchol, DM
ASA or clopidogrel
Anticoag: in new AF or hypercoag state, INR 2-3
In prosthetic valve, INR 2.5-3.5
Carotid endarterectomy: if stenosis >60% w/ sympt or >70% w/o sympt; Never if 100%
20
Q
Subarachnoid hemorrhage (etiologies, PE)
A
Rupt saccul aneurysm (berry), AVM, trauma of Willis
Aneur: abrupt intense pain, then mening irritat* (neck stiff; photoph; N/V; Kernig/Brudzinski)
1/3 have Hx of sentinel bleed (d-wks prior)
If no interv, rapid obstruc hydroceph or seiz → coma/death
21
Q
Subarachnoid hemorrhage (dg, ttt)
A
Dg: immed head CT w/o contrast; if CT⊖ then LP (RBCs, xanthoch, ↑prot, ↑ICP); angiogr when SAH confirmed (see source)
ttt: neurosurg (defin) w/ coiling +/or stenting
Prev rebleed (in 24h): SBP<150
Prev vasospasm + isch stroke (in 4-10d): CCB (nimodip)
↓ICP: immed raise head + hyperventil
ttt hydroceph: lumb drain, serial LPs, VPshunt
22
Q
Conditions ass w/ berry aneurysms
A
Marfan sd Aortic coarctation Kidney ds (ADPCKD) Ehlers-Danlos sd SCD Tobacco Atherosclerosis Fam Hx HTN Hyperlipidemia
23
Q
Intracerebral hemorrhage (RF, PE)
A
Bleed in parenchyma; esp deep regions (basal gg, thalamus, pons, cerebellum)
RF: HTN (#1), tumor, illicit drugs
- Early: focal motor or sensory def, worse as hematoma expands
- Late: ↑ICP (vom, headac, bradycard, ↓alert)
24
Q
Intracerebral hemorrhage (dg, ttt)
A
Dg: immed head CT w/o contrast (hyperdense area, mass eff, edema, herniation)
ttt: neurosurg (defin) w/ coiling +/or stenting Prev rebleed (in 24h): SBP<150 Prev vasospasm + isch stroke (in 4-10d): CCB (nimodip) ↓ICP: immed raise head + hyperventil Monitor herniation (Cushing triad, fixed pupils, loss consci); medical emerg; evacuate bld/CSF + give CS or mannitol
25
Subdural hematoma
Trauma → bridging veins
Bld betw/ dura and arachnoid membr
Elder, alcoholic
Subacute or chronic: headac, mental stat chang, contral hemiparesis, focal neuro def
CT: crescent-shap, concave hyperdensity (acute), isodens (subacute), hypodens (chron)
ttt: neurosurg evacuat* if sympt; may regress spont
26
Epidural hematoma
Severe trauma → lat skull fx → middle meningeal artery
Bld betw/ skull + dura mater
Immed loss of consc then lucid interval
CT: lens-shaped, biconvex hyperdensity
ttt: emerg neurosurg evacuat*
Complic: herniat*, death
27
Cavernous sinus thrombosis (etiology, PE)
By uncontrol inf (central fac skin, orbit, nas sinus)
S.aureus (#1)
Early: headac, edema, vis disturb (nerv involv), fev
Late: ment sta chang, sepsis, coma
28
Cavernous sinus thrombosis (dg, ttt)
Dg: MRI w/ Gado; MR venogr; CT angiogr/venogr
Bld Cx
ttt: aggress + empir broad AB in IV x3-4wks
Penicillinas-resist peni + C3G or C4G
Metronid (anaerob); Vanco (MRSA); +/- antifungal
If no resp in 24h, surg drain
29
Migraine headache (RF)
W>M; familial; start early 20s
Chang in vasc tone + neurotransm (serot, dopam)
Trigg: food, fasting, stress, menses, OCPs, bright light, abNl sleep pattern
30
Migraine headache (PE, dg)
Throbb unilat, pulsat for 4-72h
Ass w/ N/V, photoph, phonoph, aura (visual/scotoma/light)
↑activ, ↓sleep/dark
Dg: Hx + ⊖workup
31
Migraine headache (ttt)
Avoid known triggers
Abortive ttt: NSAIDs but when failure, triptans +/- naproxen
ttt nausea
Prophyl (fqt/sev): anticonv (gabapentin, topiramate), TCAs (amitriptyline), B⊖ (propranolol), CCBs
32
Cluster headache (PE)
M>W; 25yo
Brief excruciat unilat periorbit headac; 0.5-3h
Attacks in clusters (same part of head, same time of day (sleep), same season of year)
Ass w/ ipsil lacrimat*, conjunc inject*, Horner sd, nas congest
33
Cluster headache (dg, ttt)
Dg: Hx; No imaging if suspect
If 1st episode, do workup (MRI, carot US) + r/o brain les*, carot dissect, cav sin inf
ttt: acute (high-flow O2 or sumatriptan inject*)
Prophyl: verapamil (#1), lithium, valpr ac, topiram
34
Tension-type headache
Tight bandlike pain, ↑fatig/stress; at end of day
Dg: ≥2 of these: bilat, pressing qlty, mild-mod, not ↑by activ
R/o giant cell arteritis in >50yo w/ new headac by ESR, even if no other sympt/sign
ttt: relax, massage, hot bath, avoid exacerb fact
NSAIDs or acetamino (#1); triptans
35
Secondary headaches (red flags)
- Fever, rash (meningitis, other inf)
- Jaw claudic (tempor arteritis)
- ↓weight (neopl, inflamm, inf)
- Photoph, N/V, neck stiff (aneurys SAH, meningitis)
- Neuro sequelae (ment stat chang, dizzy, atax, visu dist, papilled, pupil abNl, focal def)
- Sudden severe headac; nocturnal headac; morning vomit; >50yo; Hx of head trauma
36
Secondary headaches (dg, ttt)
Dg: when ⊕ red flags
If SAH suspect, head CT w/o contrast
CBC (r/o inf); if tempor arteritis suspect (ESR)
ttt: underl cause; analgesics
37
Pseudotumor cerebri (RF)
Idiopathic intracranial HTN
Sympt suggest brain tumor + CSF pressure but Nl imag
RF: obes, tetracycl, GH, excess vitA
38
Seizure disorders (etiologies by age)
- Infants: perinat injur; inf; metab; congen
- 2-10yo: idiop; inf; traum; fev
- Ado: idiop; traum; drug withdraw; AVM
- 18-35yo: traum; alcoh; brain tumor
- >35yo: traum; stroke; metab; alcoh; brain tumor
39
Seizure disorders (etiologies)
-Idiop: recurr, unprovoked; genet/environm/...
- Acquir:
* Struct brain les* (tumor, stroke, AVM hge): focal seiz
*Non-neuro cause (hypoGlyc/Na/Ca, hepatic enceph, hyperOsm/urem/therm, drug overdose/withdrawal, eclamps, HTN encephalop, trauma, cereb hypoperf)
40
Partial seizure disorders
```
≥1 discrete regions
Motor/sensory/autonom/psychic
Ass w/ aura; postict focal neuro def (<24h)
-Simple
-Complex (esp tempor lobe; unconsc)
```
41
Generalized seizure disorders
Both cerebr hemispheres; unconsc
-Tonic-clonic: idiop, transfo of simple/complex partial seiz
Incontin, tongue bit, cyanotic, postictal confu/drowsy
-Childhood absence: brief (5-10sec) hundreds/day
Trigg: hyperventilat*
No postictal; stops before adult
42
Seizure disorders (dg)
EEG (partial=focal; absence=3/sec spike; tonic-clonic=10Hz+slow waves)
R/o systemic causes (CBC, e-, glu, LFTs, renal, ESR, toxicol)
If focal, CT/MRI w/ contrast (r/o brain les*)
43
Seizure disorders (ttt)
Secure airway
If 2*, ttt underl cause
-Partial or tonic-clonic: levetiracetam, pheytoin, carbamazepine, valproic acid, phenobarbital (#1 child)
If 1 ineff, try another. If ineff, try multiple
-Absence: ethosuximide (#1), valproic acid (#2)
-Intractable tempor: ant tempor lobectomy
!!! if >5min
44
Status epilepticus (etiologies)
!!!Emerg; seiz >5min or ≥2 seiz w/o return to baseline
```
Anticonvuls withdraw/noncomply
Anoxic brain injury
Alcoh/sedative withdraw
Drug intoxic
Metab disturb
Head trauma
Infection
```
45
Status epilepticus (dg)
ttt+dg started together!!! Morta 10-20%
Hx, PE, CBC, e-, glu, ABG, LFTs, BUN/creat, ESR
Antiepilep level, toxicol screen
Continuous EEG
Stat CT if intracran pathol suspect then +/- LP
46
Status epilepticus (ttt)
!!! Airway, Breathing, Circulation
ttt cause
Thiamine then glucose + naloxone
-IV benzo (loraz/diaz) in 0-5min
-2nd dose benzo at 5-10min
-If seiz still at 20min: fosphenytoin, valproate Na, phenobarb, levetirac, continuous midazolam
-If refract, continuous antiepilep w/ bolus + intubation
47
Benign paroxysmal positional vertigo
Recurr periph vertigo; otolith
Transient, episodic vertigo (< 1min) + nystagm (to affected side) trigg by chang head posit*
Dg: Dix-Hallpike maneuver
If >1min, gait disturb, vomit: think central les*
ttt: Epley maneuver; resol in wks-mos; !recurr
!!! CI antivertigo meds
48
Acute peripheral vestibulopathy (labyrinthitis or vestibular neuritis)
Acute vertigo, haed mot* intoler, gait unsteadiness
+ N/V, nystagm
- Labyr: auditory or aural sympt (tinnit, ear fullness, hear loss); #dg lateral pontine/cerebellar stroke (AICA)
- Vestib neuritis: No auditory or aural sympt; #dg lateral medullary/cerebellar stroke (PICA)
49
Acute peripheral vestibulopathy (dg, ttt)
```
Dg: of exclusion (r/o cerebell stroke)
AbNl vestibulo-ocular reflex
Horizont nystagm (1 direct*, opposite to les*)
No vertic eye misalignment
If high risk/atypical → MRI w/ diffus*
```
ttt: acute CS (in <72h) + antivertigo (meclizine); resolv in wks-mos
50
Meniere disease (PE, dg)
Recurr sev vertigo + auditory sympt (hear loss, tinnit, ear fulln) for hours-days; N/V
Progr losing low-fqcy hearing in years on affected side
Dg: clinic; 2 episodes of ≥20min w/ remission betw/ + hearing loss (documented) + tinnit/fullness
All 3 criteria needed after r/o other causes
51
Meniere ds (ttt)
Acute: meclizine or benzo; antiemetics
Chronic: limit salt intake, diuretics
Sev unilat: intratympanic inject* of gentamicin (↓fqcy and severity)
52
Syncope (definition)
Loss of cs 2/2 sudden drop in cerebral perfus*
Cardiac or noncardiac
Presyncope (ass w/ noncard): imminent loss of cs w/o fainting
Trigg: orthostat, long stand, see bld, Valsalva
53
Syncope (etiologies)
```
-Noncardiac: prodrom sympt (N, diaphor) then loss of cs + M. tone; < 30sec; recover in secs
Orthost hypoTN (dehydr, autonom neuropathy)
Vasovagal (↑vagal tone; by prolong stand, emot*, pain)
```
-Cardiac: No prodrom/postdrom sympt; Hx of arrhyth, valv ds, struct heart ds
LVOTO (Ao sten, hypertroph obstruc CM): sync w/ exert*
Arrhythmia (CAD, MI, CM, WPW sd, low EF)
Tors de pointes (by K+/Mg+ abNl, any medic that ↑QT)
54
Syncope (dg, ttt)
Dg: unless clear vasovag in young ptt w/o card Hx/RF; all ptts should have Holter monito + ECG + card enz
+/- echocardio, tilt-test, neuroimag
!seizure (EEG) if jerk, prolong confus*, tongue bite
ttt: underl cause + avoid trigg
55
Myasthenia gravis (PE)
Ab anti-postsynapt ACh recept
Young W; ass w/ thymoma, thyrotoxicosis, other AI ds
Ptosis/double vis*; dysarth/dysphag; prox muscle weakn
Sympt worse by end of day
Myasth crisis: lethal complic (resp)
56
Myasthenia gravis (dg, ttt)
Dg: ACh recept Ab; Edrophonium (Tensilon; anticholinesterase); confirm w/ EMG or ↓response to repetitive nerv stimul; Chest CT (thymoma)
ttt: anticholinesterase (pyridostigmine); prednisone/immsuppressants; plasmapher; IVIG
Resect* of thymoma; avoid aminoglyc/fluoroq/B⊖
57
Lambert-Eaton myasthenic syndrome (PE)
Paraneopl AI ds (small cell lung carcin)
Ab anti-presynap Ca channels Ab
Prox muscl weakn; ↓DTR
!!! Extraocular, resp, bulbar muscl spared
Weakn improve w/ activ
58
Lambert-Eaton myasthenic syndrome (dg, ttt)
Dg: repetit nerv stimulat* w/ incremental resp
Presynap Ca chann autoAb
ttt: lung cancer ttt; 3-4diaminopyridine or guanidine; pyridostigmine; CS or azathioprine
59
Multiple sclerosis (PE)
T-cell mediated AI ds; esp W; 20-40yo
Relaps-remitt; 1* progress; 2* progress; progress relapse
Multi neuro sympt (separate time/space; not single les*)
Worse w/ hot shower
Charcot's triad: scanning speech, INO, nystagmus
Attacks ~/1.5y + last 2-8wks
60
Multiple sclerosis (dg, ttt)
Dg: clinic; MRI (multip, asymm, periventric white matter les* esp corpus callosum); LP (↑IgG)
ttt: acute (high-dose IV CS; if refract, plasma exchange)
Immunomodulat (↓relaps); Interferon-B1a + Int-B1b + copolymer-1
Sympt ttt: baclofen (spastic), cholinerg (ur retent*), antichol (ur incont), carbamaz/amitript (painful paresth), antidepr
61
Guillain-Barre syndrome (PE)
Acute, rapid progress demyelin AI ds; periph nerves
Ass w/ campylob jejuni, viral inf, influ vacc
Almost complete recovery ≤1y
Progr symm ascend paralysis, areflexia
Sev: paralys trunk, diaphr, cranial N
Autonom or sensory N may be affect (paresth, autonom dysregul)
62
Guillain-Barre syndrome (dg, ttt)
Dg: N conduct* study (diffuse demyel; ↓cond velocity)
CSF: prot >55mg/dL, albuminocytologic dissociat*
ttt: fqt respir monito (ICU?); plasmapher + IVIG (#1); No CS
Aggress phys rehab
63
Amyotrophic lateral sclerosis (PE)
Chron progress degenerative ds
Loss of upp + low motor neurons
In 5y, almost always resp failure + death
M>W; 40-80yo
Asymm, slow progr weakn (mo to ys); arm/leg/diaph/low cranial N; ↓weight
Spare: sensat*, eye mvts, sphinct tone
64
Amyotrophic lateral sclerosis (dg, ttt)
Dg: clinic; EMG/nerv conduct* (widespr denerv + spontan AP); exclude other demyelin motor neurop
CT/MRI cervical (exclud struct les*)
Bulbar involv: tongue (CN XII) or orophar M. (CN IX/X) suggest path above foramen magnum (# cervic spondylosis)
ttt: support ttt + ptt educat*; Riluzole may delay progr (↓glutamate)
65
Alzheimer disease (RF, PE)
RF: age; W; fam Hx; Down sd; low educat*
Neurofibrill tangles, neuritic plaques, amyloid
Amnesia (new info) → visuospatial def, language def, cognit↓; + depress* + agitat*
MMSE <24 (# Nl aging MMSE >24)
Mild cognit impairm: 10y before
66
Alzheimer disease (dg, ttt)
Dg: of exclus* (clinic + progr cogn↓ w/o motor impairm; autopsy (defin dg); MRI/CT (atrophy esp tempor/pariet + r/o other dementias); Nl CSF; neuropsy test (r/o depress)
R/o hypothyr, vitB12 def, neurosyph
ttt: prevent progr (cholinest inh; donepezil) in mild-mod, (NMDA recep antag; memantine) in mod-sev
Support (ptt + fam); ttt depress*/agit/sleep dso/halluc/delus*
67
Vascular dementia
Stepwise ↓ in cogn fct*
Ass w/ other sympt of stroke (senso/motor def)
RF: age, HTN, DM, embol, Hx of stroke
Dg: dementia + ≥2 of these (focal neuro sign; abrupt sympt; imaging not indic unless other susp)
ttt + prevent: same as stroke
68
Frontotemporal dementia (Pick disease)
Rare, progress
Disinhib + signif chang in behav/personal (early)
Speech disturb, inattent, compulsiv, extrapyr signs
Dg: clinic + MRI/CT (atrophy of frontal + tempor)
ttt: symptom
69
Normal pressure hydrocephalus
Impair reabs of CSF; potentially tttable
Triad: dementia, gait apraxia, urin incont
No signs of ↑ICP (papilled, headac)
Dg: clinic; CT/MRI (ventric enlarg+++); LP (Nl CSF press)
ttt: LP or continuous lumbar drainag xd; VP shunt
70
Creutzfeldt-Jakob disease (PE)
1 of the transmiss spongiform encephalop
Neur degen, astrocytic prolif
AbNl protease-resist prion prot accum
Subacute dementia + ataxia, myoclonic jerks + rapid progr (wks-mos)
71
Creutzfeldt-Jakob disease (dg, #dg, ttt)
```
Dg: clinic; ↑CSF 14-3-3 and tau prot; brain biopsy/autopsy (defin); MRI or EEG help
#dg: limbic encephalitis, Hashimoto encephalop, toxic encephalop
```
No ttt; death ≤1y
!!! transmission
72
Lewy body dementia
Progr cognit chang, visual-spatial prob, execut dysfct*
Parkinson-like mvt abNl (bradykin, rigid, tremor, shuffl gait)
Psychiatric: halluc, delus*, depress, anxiety
Dg: clinic; imaging + neuropsy eval (r/o other)
Brain biopsy/autopsy (defin; alpha-synuclein prot accum = Lewy bodies)
ttt: sympt
73
Huntington disease
AD; CAG triplet repeats; anticipation
30-50yo; chorea; alter behav; dementia; ↓weight; depres
Dg: clinic; confirm w/ genetics; CT/MRI (cerebr atroph, caudate/putamen)
ttt: No cure; reserpine/tetrabenazine (↓mvt); atyp antipsych (↓psychosis w/o extrapy side eff); genet counsel (kids)
74
Parkinson disease (PE)
Idiop; >50yo; dopamin deplet* in subst nigra
-Parkinsonism: by antipsych; muti subcortic infarcts; toxins; trauma
Tetrad: rest tremor (4-6Hz; ↓w/ mvt); rigidity (cogwheel); bradykin + gait abNl (w/o arm swing); postural instab
Other: masked facies; ↓memory; micrographia
75
Parkinson disease (ttt)
- Amantadine: early ttt; short-term (akines; rigid; tremor)
- Levodopa/carbidopa: Levo cross BBB; Carbi block periph conv of Levo
- Dopamine agon: ropinirole/pramipexole/bromocriptine (early)
- Selegiline (MAO-B inh): neuroprotect; ↓need for levodopa
- COMT inh: entecapone/tolcapone w/ levodopa
- Anticholin: trihexyphenidyl/benztropine; early for tremor
- If all insuff: surg pallidotomy or deep brain stimulators
76
Intracranial neoplasms
- 1* (30%)
- Meta (70%): lung/brst/kidn/GI/skin; at gray-white junct*; rapid, invasiv, necrosis
Mass eff, cereb edema, ↑ICP, seiz, focal progr def
Dg: CT/MRI; biopsy (histo)
ttt: surg, RT, chemoT; manag ICP; antiepilep
77
Common 1* neoplasms in adults
- Glioblastoma (Gr. IV astrocytoma): malignant; poor pg
- Meningioma: benign
- Vestibular schwannoma: benign
78
Common 1* neoplasms in children
- Pilocytic astrocytoma: benign; GFAP⊕
- Medulloblastoma: highly malign; neuroectodermal
- Craniopharyngioma: benign; calcificat*
79
Neurofibromatosis (NF1 vs NF2)
-NF1: ≥2 of these: 6 café au lait spots, 2 neurofibromas, freckling in axill/inguin, optic glioma, 2 lisch nodules, bone abNl, 1st* relative w/ NF1
-NF2: bilat vestib schwannomas OR
Unilat vest schw or 2 of (neurofibromas, meningiomas, gliomas, schwannoma) AND 1st* relative w/ NF2
+/- seiz, skin nodules, café au lait spots
80
Neurofibromatosis (dg, ttt)
Dg: MRI w/ Gado (brain, brainstem, spine); dermato + ophthalmo exams; fam Hx; auditory test
ttt: sympt, No cure
Surg (vestib schw, optic gliom, meningioma)
81
Tuberous sclerosis (PE, dg)
```
Infantile spasm/seiz, ash-leaf hypopigm, mental disab
Sebaceous adenomas (nose/cheeks), shagreen patch (lumbosacral), cardiac rhabdomyom, renal cysts, angiolipom, carcinomas
```
Dg: clinic; MRI brain (tumors), Echocardio, MRI abdo (tumors), EEG (seiz)
82
Tuberous sclerosis (ttt)
Symptomatic, cosmetic
ttt seiz
ttt spasms (ACTH or vigabatrin)
Surg if ↑ICP, seiz, develop delay
83
Broca aphasia
Ds of spoken and/or written language
Intact comprehension
Left post infer frontal cortex; left sup MCA stroke
W/ impaired repetit*, awarness/frustrat*, arm/face hemiparesis, hemisens loss, apraxia (oral muscl)
84
Wernicke aphasia
Ds of language comprehens*
Intact yet nonsensical product*
Left post sup tempor lobe; left inf/post MCA stroke
W/ impaired repetit*, neologism, word substitut*, lack of awarness
85
Coma (etiologies)
Unconsciousness + ↓/no response to stimuli
Dysfct* both cereb hemisph or brainstem
Diffuse hypox/isch encephalop; diffuse axonal injury; brain herniat*; widesprd inf; massive brainstem hge/infarct; central pontine myelinolysis; e- disturb; exogen toxins; general seiz; endocrin/metabol dysfct*
86
Coma (dg)
Dg: comlete Hx (+meds); neuro/ophth/motor/sens/autonom
exams; labs; toxicology screen; Cx (bld/CSF)
CT w/o contr to r/o hge/structure les* + before LP
MRI to r/o ischem/structure les*
R/o catatonia, convers* unresp, locke-in sd, vegetat state
87
Coma (ttt)
Stabilize ptt (ABC)
Reverse the reversible (Dextrose, Oxygen, Naloxone, Thiamine)
Underlying cause + complic
Prevent further damage
D/c artific life support if 2 Drs declare brain death
88
Locked-in syndrome
Wakeful, alert, retained cognit*
Causes: central pontine myelinolysis, brainstem stroke, advanced ALS
Voluntary motor: eyes, eyelids
Respiratory drive ⊕
89
Persistent vegetative state
Wakeful w/o awareness
Eyes open/close; sleep/wake cycles
Causes: diffuse cortical injury; hypoxic ischemic injury
No voluntary mvt
Respiratory drive ⊕
90
Coma vs Brain death
Both: unconscious, eyes closed, No sleep-wake cycles, No voluntary mvt
Same causes: diffuse hypoxic encephalop, widesprd inf, e- disturbances, toxins
Respiratory drive ⊕ in coma but ⊖ in brain death
91
Wernicke encephalopathy vs Korsakoff dementia
WE: triad of encephalop, ophthalmopl, ataxia
By alcoh; hyperemes; starvat*; dialysis; AIDS; ↑by high glucose admin
ttt: revers w/ vitB1 admin
KD: triad + anterog/retrograde amnesia, horiz nystagm, confabulat*
Same causes + if WE ttt too late or inadeq
ttt: irreversible
92
Vitamin B12 deficiency
Periph subacute combined neuropathy
Progress, symm paresth, spasticity, paraplegia, bowel/bladder dysfct; dementia
↑methylmalonic acid; ↑homocysteine
Ptts w/ pernicious An; vegetarians; gastric/ileal resect*/ds; malnutrit*
ttt: vitB12
93
Vitamin B9 deficiency
Irritab; personality chang w/o neuro sympt
In alcoholics
Reversible if early ttt (folate admin)
