Encephalopathic disorders (Smarty PANCE)

A 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?

Spongiform encephalopathy

Diagnosis of spongiform encephalopathy?

Lumbar puncture ↑ levels of 14-3-3 protein. Definitive diagnosis with brain biopsy

What is Creutzfeldt-Jakob disease (CJD)?

CJD is classified as a transmissible spongiform encephalopathy (TSE) along with other prion diseases that occur in humans and animals.

How does bovine spongiform encephalopathy (mad cow disease) relate to humans?

Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is an incurable and invariably fatal neurodegenerative disease of cattle. Symptoms include abnormal behavior, trouble walking, and weight loss. Later in the course of the disease the cow becomes unable to function normally. The time between infection and onset of symptoms is generally four to five years. Time from onset of symptoms to death is generally weeks to months. Spread to humans is believed to result in variant Creutzfeldt-Jakob disease (vCJD)

Syndrome of acute or subacute encephalopathy that is associated with elevated anti-thyroid antibody titers?

Hashimoto encephalopathy (HE)

Diagnosis of Hashimoto encephalopathy?

The presence of elevated antithyroid antibody titers and the exclusion of other causes of encephalopathy support the diagnosis of HE

Treatment of Hashimoto encephalopathy?

Treated with corticosteroids

A neurological disorder induced by thiamine, vitamin B1, deficiency. This is the most important encephalopathy due to a single vitamin deficiency and presents with the classic triad of ocular findings, cerebellar dysfunction, and confusion.

Wernicke encephalopathy (WE)

Treatment of Wernicke encephalopathy?

This is a medical emergency and requires infusion of thiamine over a few days to normalized thiamine levels

Occurs in individuals with chronic liver disease such as cirrhosis or hepatitis. Early symptoms include forgetfulness, confusion, and breath with a sweet or musty odor. Advanced symptoms include shaking of the hands or arms (asterixis), disorientation, slurred speech, and coma?

Hepatic encephalopathy

Treatment of hepatic encephalopathy?

Treatment includes removing toxic substances from the intestine. Lactulose (to increase NH4 generation) and rifaximin (decrease intraluminal NH3 production)