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Malignant hyperthermia


Inhalational anesthetics: halothane, isoflurane, sevoflurane, and desflurane

depolarizing muscle relaxants: succinylcholine

Note: it is safe to use thiopental, etomidate, and propofol

Presentation: masseter spasm often earliest indicator, then tachypnea, tachycardia, increasing end-tidal carbon dioxide levels and acidosis then hyperthermia and cyanosis, rigidity, rhabdomyolysis

Note: can pre-screen with halothane-caffeine contracture test in vitro

Treatment: stop offending agent, supportive measures



Neuroleptic-induced heat stroke


More in elderly and young / impaired sweating / risk increased by concomitant use of anticholinergics (Cogentin) / can produce seizures

Treatment: supportive, rapid cooling, fluids



Acute lethal catatonia


Presentation: posturing, waxy flexibility (cerea flexibilitas), hyperactivity preceded by behavioral changes in weeks leading up to motor deficits, which then may progress to hyperthermia, akinesia, and rigidity and death from respiratory and circulatory failure

Treatment: electroconvulsive therapy (ECT)


Serotonin syndrome

Causes: SSRI, amphetamines, cocaine, dextromethorphan, meperidine, TCA, tramadol, MAO

Presentation: mental status changes, neuromuscular (tremor, rigidity, seizures + shivering, ataxia, hyperreflexia, myoclonus, ankle clonus), autonomic dysfunction (flushing, labile, HTN, fever, though not as high as NMS, salivation, tachycardia), gastrointestinal dysfunction (nausea, vomiting, diarrhea)

Complications: CV collapse, lactic acidosis, multiorgan failure, coma, death are all rare but possible outcomes

Labs: WBC, CK, LFT variably elevated

Course: recovery in 1-7 days after drug stopped / usually no sequelae

Treatment: can give SSRI antagonists, cyproheptadine, chlorpromazine

Central anticholinergic syndrome


Presentation: altered mental status +/- hyperthermia, decreased sweating, mydriasis, dry mouth, and urinary retention

Treatment: physostigmine (and supportive measures)


CNS injury


  • CVA

  • intracranial hemorrhage

  • venous sinus thrombosis

  • CNS vasculitis


Cerebrovascular Accident (CVA, stroke)
Carotid/vertebral artery disease (10-15%)

Cardiac emboli (25-30%)

Intracardiac thrombus or mass

MI (anterior wall, septum, akinetic segment), cardiomyopathy, arrhythmias (Afib), cardiac myxoma



Valvular

rheumatic heart disease, bacterial endocarditis, non-bacterial endocarditis

(Libman-Sacks, carcinoma), mitral valve prolapse, prosthetic valve

Intracranial (40-50%)

Vasculitides

Primary CNS vasculitis

systemic vasculitis (PAN, allergic angiitis), CTD (RA, scleroderma, Sjögren’s), Wegener’s, Behçet’s, GCA, Takayasu’s, lymphomatoid granulomatosis

Hypersensitivity vasculitis (serum sickness, drug-induced, cutaneous)

Infectious vasculitis (lyme, meningitis, Tb, AIDS, opthalmic zoster, HBV)

Sub-arachnoid hemorrhage or vasospasm

Hematologic

Hemoglobinopathies (sickle cell, HbSC)

Hyperviscosity syndromes (polycythemia, thrombocytosis, leukocytosis,

macroglobulinemia, multiple myeloma)

Hypercoagulable states (carcinoma, pregnancy, puerperium, protein C/S

deficiency, antiphospholipid antibodies)



Drug-related: street drugs (cocaine, amphetamines, lysergic acid, PCP, meth,

heroin, pentazocine), alcohol, OCPs



Other: lipohyalinosis, fibromuscular dysplasia, arterial dissection, homocystinuria,

migraine, moyamoya, other embolic (cholesterol, fat, bone, air)


Types of Stroke (see specific syndromes)
MCA: contralateral hemiparesis (hemiplegia), hemianesthesia, homonymous hemianopsia

dominant  aphasia,

nondominant  apraxia, neglect

Lacunar (often MCA territory, internal capsule): pure motor or sensory stroke, dysarthria-clumsy hand syndrome, ataxic hemiparesis

ACA: leg paresis

PCA: homonymous hemianopsia

Basilar: coma, apnea, cranial nerve palsies
TIA: any of above < 24 hrs

Note: only bilateral hemispheric and basilar (RAS) strokes cause loss of consciousness!


Aphasia

Suggests left hemisphere lesion (most people, even left-handed have language on left)

Wernicke’s – from left inferior MCA – fluid but meaningless speech / hemiparesis mild or absent

Broca’s – from left superior MCA – impaired speech / hemiparesis, hemisensory loss is common


Ddx: brain tumor, hematoma (all kinds), abscess, endocarditis, MS, metabolic (ex. hypoglycemia), neurosyphilis

Work-Up:

CT without contrast (faster to get and good for r/o herniation, bleed)

MRI

CBC, glucose, coagulation, lipid, ESR, VDRL



EKG and echo

Vascular: carotid ultrasound, MRA or CT angiography / transcranial doppler or MRA

Hypercoagulable work-up if relevant

Treatment:


  • Thrombolysis (tPA) < 3 hrs (see contraindications); does no higher than 0.9mg/kg over 60 mins / RF for ICH older age, female, black, h/o CVA, HTN > 140/100

  • Consider anti-platelets if no hemorrhage

    • ASA has been shown to have slight reduction in mortality and risk of recurrence (plavix may be more effective but slightly higher cost and GI bleed risk)

  • Consider heparin if no hemorrhage

    • no studies (as of 2001) show mortality increase with heparin for acute CVA (some neurologists use it in specific circumstances)

  • maintain cerebral perfusion (so-called ischemia penumbra): do not overtreat HTN (SBP goal should be 160 to ?)

  • watch for signs of progression/herniation


Long-term

Treat underlying cause (carotid stenosis, Afib, etc)


Carotid Stenosis

NASCET  in patients with TIA/CVA and ipsilateral carotid artery stenosis > 70%, a carotid endarterectomy reduced stroke rate from 26% to 9% over 2 years

ACAS  11% to 5% over 5 years if > 60% stenosis
TIA (Transient Ischemic Attacks)

4 to 20% will have stroke within next 90 days / 50% within 48 hrs / ABCD score used to help risk stratify / Age > 60 = 1, BP > 140/90 = 1, (unilateral weakness = 2; speech only = 1), (duration > 60 mins = 2; 10-59 mins = 1), diabetes = 1



Specific Stroke Syndromes
Lateral medullary syndrome of Wallenberg - vertebral artery or PICA

Clinical: loss of pain and temperature to ipsilateral face (descending spinal tract and nucleus of 5th CN) and contralateral body (spinothalamic tract), ipsilateral ataxia (cerebellar peduncle), ipsilateral Horner’s (descending sympathetic fibers), ipsilateral weakness of the palate and vocal cords (nuclei of 9th and 10th CN) / may present with nausea, vomiting, vertigo from involvement of vestibular system


Mediobasal mesencephalic syndrome of Weber

infarction of one cerebral peduncle / ipsilateral 3rd nerve palsy and contralateral hemiplegia [pic]


Millard-Gubler syndromebasilar artery

damage to pons (left corticospinal tract proximal to decussation in medulla)



  • 6th and 7th CN affected  impaired lateral gaze, diplopia, facial weakness (all ipsilateral) [pic]

  • contralateral hemiplegia


mid basilar artery occlusion

causes same thing only 5th CN ipsilateral and contralateral hemiplegia


Hypertension
Lenticulostriate arteries  basal ganglia, pons, cerebellum, lacunes (small focal areas, lacunar infarcts)


  • Clinical scenarios: motor hemiplegia, pure sensory stroke, ataxic-hemiparesis syndromes, “clumsy hand” dysarthria (base of pons or internal capsule)


Charcot-Bouchard aneurysm

aneurysms of lenticulostriate arteries


Binswanger’s disease

arteriolosclerotic encephalopathy


Multi-infarct dementia

second leading cause of dementia / step-wise progression (hemiparesis, extensor plantar response, pseudobulbar palsy) / hypoglycemia, vasculitis, infection, compression


Venous and dural sinus thromboses

infectious origin, hemorrhagic, often fatal


Malformation

berry aneurysms (80% single), AVMs (most common congenital, supratentorial)



Perinatal cerebrovascular disease
Germinal matrix hemorrhage

post-hemorrhagic hydrocephalus, sub-ependymal cysts / small blue cell vessels, blood in ventricles


Periventricular leukomalacia

white matter infarct


multicystic encephalomalacia

ischemic lesions of grey matter

pontosubicular karyorhexis (hyperoxemia)
CNS infection
Meningitis (see other)
Brain Abscess

Immunocompetent (from oral spread): peptostreptococcus > fusobacterium, bacteroides

Immunocompetent: zygomyces, staphylococcus (possibly from bacteremia)

glucose normal / increased risk with R-L shunt from congenital cardiac defect (blood skips

alveolar macrophages in lungs)

Evolution of brain abscess: early cerebritis (3 to 5 d), late cerebritis (5 to 14 d), early

encapsulization (14 d, late encapsulization (weeks to months)


  • early  normal or hypodense lesions on noncontrast CT / ill-defined enhancing lesions on contrast CT

  • late  ring-enhancing on contrast CT

Drugs that penetrate CSF:
Empyema

may lead to superior saggital sinus thrombosis


Congenital

rubella, CMV


Vasculitis

mucor, aspergillus


Amoebiasis

N. fowleri, Acanthamoeba, Leptomyxid amoebae



CNS malformations


  • lissencephaly (agyria)

  • agenesis of corpus callosum (batwing X-ray)


Arnold-Chiari

small posterior fossa / herniation of vermis into foramen magnum, hydrocephalus, lumbar myelomeningocoele
syringomyelia soft cavitation of central canal, loss of pain/temperature bilaterally in upper extremities (light touch preserved)
Dandy-Walker

enlarged posterior fossa / absent cerebellar vermis, cyst protruding from 4th ventricle

CNS trauma - accidents 4th most common COD in all ages
fractures diastatic, comminuted, ring
contusions plaque jaune
CSF leak (from basilar skull fracture)

CT  air-fluid levels, opacification of paranasal sinuses, intracranial air

Radioisotope cisternography or HRCT with water-soluble contrast (metrizamide

cisternography) are the gold standards


Epidural hematoma

middle meningeal, skull fracture, lucid interval, progressive hemiparesis/obtundation and ‘blown pupil’ from herniation

CT: lens-shaped convex hyperdensity

Treatment: emergent neurosurgical evacuation


Subdural hematoma

Causes: bridging veins, head trauma, blood dyscrasias, elderly, alcoholics, child abuse (shaken baby)

Presentation: headache, change in mental status, contralateral hemiparesis or other focal

CT: crescent-shaped concave hyperdensity



Treatment: neurosurgical evacuation if symptomatic / avoid anticoagulation


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