Lec 23 Nutritional and Metabolic Disease of CNS Flashcards
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
What is burton’s line?
discoloration of upper gums due to lead deposition
What is treatment for lead poisoning?
Ca + EDTA
What are effects of mercury poisoning?
- encephalopathy, peripheral neuropathy, seizures, metallic aste
mad hatter syndrome = tremor, red cheeks, irritability
what is treatment for mercury poisoning?
Ca + EDTA
What are effects of arsenic poisoning?
- encephalopathy, N/V, renal failure, arrhythmias
What type of poison associated with mad hatter syndrome?
mercury
What type of poison associated with wrist drop and ab pain?
lead
How do you test for arsenic?
urine test [instead of blood test]
What do you get thallium poisoning from?
rat poison
What are effects of thalium poisioning?
- vomitting, diarrhea, paresthesis, confusion
- hair loss with chronic exposure
What are effects of manganese poisoning?
confusion, tremor [parkinson’s-like], hyperreflexia
What is manganese poisoning associated with?
arc welding, steel industry
How do manganese symptoms differ from parkinsons?
- in manganese poisoning have hyperreflexia
this is absent in parkinsons
What is marasmus?
undernutrition = balanced starvation, insufficient breast milk
What are signs of marasmus? who do you see it in?
occurs in 1st year of life
- causes wasting, mental changes, growth retardation
What is kwashiorkor?
protein starvation
what are signs of kwashiorkor? who do you see it in?
occurs in 1-3 yo
- causes encephalopathy, muscle wasting
What is pickwickian syndrome?
rare complication of extreme obesity
= cardiorespiratory distress
what is sleep apnea? who is at risk?
- at risk = extremely obese
- hypersomnia, oxygen desaturation, arrhythmias, can be fatal
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
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?
- neuropathy [due to insufficient NT synthesis]
- -> irritability, somnolesence, peripheral neuropathy - pellagra [due to low niacin]
- -> dermatitis, diarrhea - 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
