Lec 23 Nutritional and Metabolic Disease of CNS Flashcards by Anna Cushing

What are effects of lead poisoning in children vs adults?

children: behavioral and IQ impairment, acute encephalopathy
adults: abdominal pain, motor neuropathy [wrist drop], microcytic anemia

burton’s line = discoloration of upper gums due to lead deposition

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What is burton’s line?

discoloration of upper gums due to lead deposition

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What is treatment for lead poisoning?

Ca + EDTA

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What are effects of mercury poisoning?

encephalopathy, peripheral neuropathy, seizures, metallic aste

mad hatter syndrome = tremor, red cheeks, irritability

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what is treatment for mercury poisoning?

Ca + EDTA

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What are effects of arsenic poisoning?

encephalopathy, N/V, renal failure, arrhythmias

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What type of poison associated with mad hatter syndrome?

mercury

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What type of poison associated with wrist drop and ab pain?

lead

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How do you test for arsenic?

urine test [instead of blood test]

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What do you get thallium poisoning from?

rat poison

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What are effects of thalium poisioning?

vomitting, diarrhea, paresthesis, confusion

- hair loss with chronic exposure

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What are effects of manganese poisoning?

confusion, tremor [parkinson’s-like], hyperreflexia

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What is manganese poisoning associated with?

arc welding, steel industry

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How do manganese symptoms differ from parkinsons?

in manganese poisoning have hyperreflexia

this is absent in parkinsons

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What is marasmus?

undernutrition = balanced starvation, insufficient breast milk

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What are signs of marasmus? who do you see it in?

occurs in 1st year of life

- causes wasting, mental changes, growth retardation

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What is kwashiorkor?

protein starvation

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what are signs of kwashiorkor? who do you see it in?

occurs in 1-3 yo

- causes encephalopathy, muscle wasting

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What is pickwickian syndrome?

rare complication of extreme obesity

= cardiorespiratory distress

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what is sleep apnea? who is at risk?

at risk = extremely obese

- hypersomnia, oxygen desaturation, arrhythmias, can be fatal

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What are neurologic complications of diabetes?

dementia [due to small vessel disease]
increase stroke risk
ischemic cranial nerve palsies
peripheral neuropathies, blindness
loss of limbs

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Which cranial neuropathy in particular is found associated with diabetes?

painful 3rd nerve palsy that affects extra-ocular muscles but spares the pupil/pupillary reflex

What is effect of vitamin A toxicity?

due to excessive intake – causes high ICP [pseudotumor cerebri], skin changes, alopecia

as a teratogen: causes cleft palate, cardio abrnomalities

What is affect of water intoxication [too much water]?

encephalopathy

What is effect of pyridoxine [B6] intoxication?

peripheral neuropathy

What causes eosinophlic myalgia syndrome?

contaminated tryptophan

What is effect of emetine/ipecac toxicity? who is prone?

- myopathy | - seen in anorexia nervosa/bulimia

What are rum fits?

early onset convulsions within 12-48 hrs of alcohol withdrawal

What is delirium tremens?

later onset convulsions from alcohol withdrawal, confusion, agitation, high mortality

What are effects of brain atrophy related to alcohol use?

have stretching of bridging veins + coagulopathy + more likely to fall over --> more likely to get subdural hematoma

What are effects of fetal alcohol syndrome?

- learning difficulties, mental retardation, behavioral difficulties, growth impairment, cataracts, small head/philtrum/maxilla

What are effects of alcoholic hepatic encephalopathy?

asterixis, confusion, coma due to buildup of ammonium / hepatic failure

What are effects of alcoholic peripheral neuropathy?

length-dependent sensory or sensorimotor neuropathy pain invariable

What is effect of thiamine deficiency?

rapid progressing peripheral neuropathy

What is wernicke's encephalopathy triad?

- ataxia + opthalmoplegia + confusion - usually due to impaired absorption [bariatric surgery] or low thiamine [alcoholism], HIV - typically have nystagmus

What is korsakof's psychosis?

associated with alcoholism | - have demented state -- retrograde/anterograde amnesia, confabulation [make up stores], apathy

What type of myopathy in alcoholism?

cardiomyopathy

What is marchiafava-bignami disease?

associated with alcoholism - demyelination of corpus callosum - causes rapid psychosis, stupor, coma

What is central pontine myelinolysis?

associated with overly virgorous correction of alcoholism, hyponatremia, liver disease, or malnutrition get brain swelling that causes focal myelinolysis of the pons - have severe paralysis, locked in syndrome, difficulty swallowing/speaking

What is alcoholic cerebellar degeneration?

superior cerebellar vermis atrophies --> causes loss of purkinje cells - pts present with truncal ataxia - irreversible, does not respond to treatment

What type of vitamin deficiency should you consider if painful distal neuropathy?

thiamine

What type of lesions lead to wernicke's encephalopathy?

- lesions of thalamic nuclei, mammillary boides, PAG, brainstem nuclei, superior cerebellar vermis

What happens if untreated wernickes?

can lead to korsakoff's psychsosi

What is pathology of korsakoff's?

neuronal loss and gliosis | hemorrhage into mammillary bodies

What are 3 types of manifestations of B6 [pyridoxine] deficiency?

1. neuropathy [due to insufficient NT synthesis] - -> irritability, somnolesence, peripheral neuropathy 2. pellagra [due to low niacin] - -> dermatitis, diarrhea 3. anemia [due to low porphyrin] - -> microcytic anemia

What are pyridoxine dependent seizures?

can be seen in newborn, will not respond to antieplipetic for all children with seizure < 18 mo give B6 if you don't know the cause

What should you give all children < 18 mo with seizures of unknown etiology?

B6 supplement! [pyridoxine]

What is required for vit B12 absorption?

- terminal ileum absorption | - intrinsic factor + Ca needed

What is etiology of B12 deficiency?

- congeital pernicious anemia [lack of intrinsic factor] - inadequate intake or diretary deficiency - inhibition of B12 - vit b12 malabsorption from intestinal disease

What is disease of B12 [cobalamin] deficiency?

- damage to peripheral nerves caused by demyelination and irreversible nerve cell death symptoms: - paresthesias [pin/needles] of hands/feet - loss of position sensation + Romberg but loss of reflexes - progressive spastic + ataxic weakness - optic atrophy, irritability - diffuse/symmetric myelopathy

What are signs of posterolateral column disease?

- due to B12 deficiency | - romberg +, reflexes lost or hypo/hyper; + babinksi, superimposed peripheral neuropathy

What is effect of vit E defiency?

- progressive spinocerebellar syndrome + posterior column degeneration, peripheral neuropathy + hemolytic anemia

Abuse of NO can mimic what disease?

posterolateral column disease

What are signs of NO intoxication?

- same as subacute combined degeneration due to B12 deficiency