PANCE Blueprint Neurology (7%)

Encephalopathic disorders

Encephalopathic disorders
Spongiform encephalopathy Group of progressive, invariably fatal, conditions that affect the brain are caused by the accumulation of misfolded prion proteins giving the brain a "spongy" appearance

  • Transmitted by infected meat, blood transfusions, corneal transplants, and contaminated surgical instruments
  • Symptoms may not show for decades then progress rapidly and may cause death
  • Ataxia, poor memory, behavioral changes, dementia, myoclonus
  • Diagnosed on MRI
  • Lumbar puncture ↑ levels of 14-3-3 protein
  • Definitive diagnosis with brain biopsy
  • Currently no treatments, genetic counseling for familial form, restrict blood transfusions

Creutzfeldt-Jakob Disease (CJD) which has four main forms

  • Familial CJD - Mutation in PRNP Gene
  • Variant CJD - Eating meat of cows with prions in muscle tissue (mad cows disease)
  • Iatrogenic CJD - caused by medical procedures
  • Sporadic CJD - no clear cause

KURU - caused by cannibalism of infected flesh

Fatal Familial Insomnia - mutation in PRNP gene, prion proteins build up in thalamus and affect sleep, patients develop insomnia, exaggerated startle response

Hashimoto encephalopathy (HE) Hashimoto encephalopathy (HE) is a syndrome of acute or subacute encephalopathy that is associated with elevated anti-thyroid antibody titers

  • The presence of elevated antithyroid antibody titres and the exclusion of other causes of encephalopathy support the diagnosis of HE
  • Treated with corticosteroids
Wernicke encephalopathy (WE) and Wernicke-Korsakoff syndrome Complication of thiamine (vitamin B1) deficiency

The term refers to two different syndromes, each representing a different stage of the disease:

  • Wernicke encephalopathy (WE) is an acute syndrome requiring emergent treatment to prevent death and neurologic morbidity
    • Ophthalmoplegia  - weakness or paralysis of eye muscles
    • Ataxia - unsteady gait
    • Changes in mental state - confusion, apathy, difficulty concentrating
    • Coma and death
  • Korsakoff syndrome (KS) refers to a chronic neurologic condition that usually occurs as a consequence of WE
    • Severe memory impairment
    • Confabulation - creates stories to fill gaps in memory

Most often associated with chronic alcoholism

  • WE occurs also in the setting of poor nutrition caused by malabsorption, poor dietary intake (anorexia), increased metabolic requirement (eg, during systemic illnesses), or increased loss of the water-soluble vitamin thiamine (eg, in renal dialysis)

Diagnose - thiamine levels and LFTs,  confirmed by MRI - degeneration of mammillary bodies

TX: medical emergency, infusion of thiamine over a few days to normalized thiamine levels

  • Given with glucose ** Must normalize thiamine levels first or will cause metabolic acidosis due to increased lactic acid

Hepatic encephalopathy Occurs in individuals with chronic liver disease such as cirrhosis or hepatitis

  • Toxins that are produced by the digestive breakdown of proteins, such as ammonia and normally cleared in the liver accumulate in the blood eventually traveling to and damaging the brain
  • Triggers include infection and dehydration.
  • Early symptoms include forgetfulness, confusion, and breath with a sweet or musty odor
  • Advanced symptoms include shaking of the hands or arms (asterixis), disorientation, and slurred speech, and coma

Treatment includes removing toxic substances from the intestine

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