Toxic And Metabolic Disorders Of The Nervous System (Ostrow)

Front 1

Lead

Back 1

-Binds sulfhydryl, affects porphyrin metab. and heme synth.--> anemia
-Acute intox: marked brain edema, seizures, coma, death from herniation
-Chronic intox: headache, abdominal, anorexia, peripheral neuropathy (segmental; motor>sensory, hands>feet).
-Children: chronic intox leads to neurobehavioral and retardation

Front 2

Mercury

Back 2

-"Quicksilver and slow death"
-Hatter's shake: personality change (mad), tremors, ataxia and visual loss: Destruction of granule cells in cerebellar cortex and neurons in the calcarine cortex.
-Thimerosol used to be a preservative in vaccines

Front 3

Phosphorus and organophosphorus compounds

Back 3

-Rat poison, insecticides and match heads
-Potent inhibitors of acetylcholinesterase
-Acute: headache, vomiting, ab cramps (duh), bronchospasm (duh), and miosis (duh)--> can be reversed by atropine
-Delayed neurotoxic effects, appearing 2-5 weeks after exposure, include a symmetric peripheral neuropathy characterized by a “dying back” pattern in which the distal ends of long motor fibers are affected first

Front 4

Carbon Monoxide Tox

Back 4

-Hypoxia from alter O2 carrying capacity of Hb
-Selective localization of injury:
-Globus pallidus with bilateral necrosis
-Layer 3 & 5 of cerebral cortex
-Sommer's sector of hippocampus
-Purkinje cells of cerebellum

Front 5

Wernicke's Encephalopathy (from B1 def.)

Back 5

-Due to B1 (thiamine deficiency)
-Malnourished chronic alcoholics
-*Confusion with ocular palsies and ataxia
-*Small hemorrhages in periventricular areas, mammillary bodies and PAG--> memory issues
-Capillaries are increased and are also leaky

Front 6

B12 deficiency

Back 6

-Potentially irreversible, CNS and particular spinal cord damage
-Myelin vacuolization and axonal degradation in ascending sensory and decending CSTs below mid-thoracic level
-*Subacute Combined Degeneration: bilateral numbness, tingling, ataxia and spastic
weakness in the lower extremities

Front 7

Central pontine myelinolysis

Back 7

-Alcoholic and malnourished patients
-Confusion/delirium followed by rapid evolving limb weakness--> quadriplegia
-Other pontine signs: conjugate gaze probs., dysarthria/phagia,"locked-in syndrome"
-*Major lesion is of pons with demyelination: sparking of axons and neurons, many macs seen
-Thought to be caused by rapid correction of hyponatremia--> edema causes "grid" compression in basis pontis
-Maybe hypoxia involved

Front 8

Alcoholic cerebellar degeneration

Back 8

-Uncertain etiology, more common in men
-Loss of neurons in anterior vermis--> patients have truncal ataxia and wide-based gait

Front 9

Wilson's Disease

Back 9

-Auto recessive: ATP7B Chr 13--> encodes copper-transporting ATPase
-Impairs the incorporation of copper into ceruloplasmin -Inhibits ceruloplasmin secretion into the blood. -Non-ceruloplasmin-bound copper enters the circulation and deposits in other tissues.
-*Copper deposits in the liver (cirrhosis), putamen and globus pallidus *(“hepatolenticular degeneration”).
-Brain lesions are grossly cavitary and brown, with severe loss of neurons and reactive gliosis.
-*Alzheimer type 2 astrocytes
-Kayser-Fleischer rings in the cornea

Front 10

Arsenic

Back 10

-NOT ON STUDY GUIDE
-Encephalopathy
-Peripheral neuropathy (sensory>motor): burning sensations in lower extremities: distal axonal loss with secondary loss of myelin sheaths
-Petechial hemorrhages in brain WM

Front 11

Manganese

Back 11

-NOT ON STUDY GUIDE
-In miners/Mn workers
-Inhibits acetylcholinesterase (inc. ACh) and adenosine deaminase
-Stimulates MAO (dec. dopamine)
-Develop extrapyramidal PS-like symptoms from above
-Some respond to L-dopa

Front 12

Alcohol Withdrawal Symptoms

Back 12

-NOT ON STUDY GUIDE
-Tremulous shakes
-Hallucinosis- disordered perception
-Seizures- "rum fits"
-*Delirium tremens: a situation of profound confusion, vivid hallucinations, delusions and increased autonomic activity

Front 13

Marchiafava-Bignami disease

Back 13

-NOT ON STUDY GUIDE
-Unknown alcoholic etiology, more common in men
-Degen. of corpus callosum and other commissures
-Non-specific: mental/motor, confusion, *primitive reflexes
-*Often improves during hospitalization

Front 14

Fetal Alcohol Syndrome (FAS)

Back 14

-NOT ON STUDY GUIDE
-Most severe form: FASD is a spectrum
-Small size for gest. age
-Short palpebral fissure, flattened philtrum
-Poor coordination, hyperactive, developmental disabilities-->daily/judgement/reasoning problems
-Sleep and sucking problems in infancy

Front 15

Hepatic encephalopathy

Back 15

-NOT ON STUDY GUIDE
-Assoc. with liver failure/cirrhosis/other hepatic disease
-Caused by neurotoxins: *ammonia which the liver normally deals with
-*Impaired mental status and asterixis (flapping hand tremor)
-Path: brain grossly normal, Alz 2 astrocyte cells in cerebral cortex and basal ganglia--> misshapen nuclei with clear centers and marginated chromatin. Not pink like normal (reversible)
-Similar to uremia caused by renal failure