Encephalopathic disorders (Smarty PANCE) Questions and
Answers
A group of progressive, invariably fatal, A neurological disorder induced by thiamine,
conditions that affect the brain are caused by the vitamin B1, deficiency. This is the most important
accumulation of misfolded prion proteins giving encephalopathy due to a single vitamin
the brain a "spongy" appearance?- deficiency and presents with the classic triad of
Spongiform encephalopathy ocular findings, cerebellar dysfunction, and
confusion.- Wernicke encephalopathy (WE)
Diagnosis of spongiform encephalopathy?-
Lumbar puncture ↑ levels of 14-3-3 protein. Treatment of Wernicke encephalopathy?-
Definitive diagnosis with brain biopsy This is a medical emergency and requires
infusion of thiamine over a few days to
What is Creutzfeldt-Jakob disease (CJD)?- normalized thiamine levels
CJD is classified as a transmissible
spongiform encephalopathy (TSE) along with Occurs in individuals with chronic liver disease
other prion diseases that occur in humans and such as cirrhosis or hepatitis. Early symptoms
animals. include forgetfulness, confusion, and breath with
a sweet or musty odor. Advanced symptoms
How does bovine spongiform encephalopathy include shaking of the hands or arms (asterixis),
(mad cow disease) relate to humans?- disorientation, slurred speech, and coma?-
Bovine spongiform encephalopathy (BSE), Hepatic encephalopathy
commonly known as mad cow disease, is an
incurable and invariably fatal neurodegenerative Treatment of hepatic encephalopathy?-
disease of cattle. Symptoms include abnormal Treatment includes removing toxic
behavior, trouble walking, and weight loss. Later substances from the intestine. Lactulose (to
in the course of the disease the cow becomes increase NH4 generation) and rifaximin
unable to function normally. The time between (decrease intraluminal NH3 production)
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
1/1
Answers
A group of progressive, invariably fatal, A neurological disorder induced by thiamine,
conditions that affect the brain are caused by the vitamin B1, deficiency. This is the most important
accumulation of misfolded prion proteins giving encephalopathy due to a single vitamin
the brain a "spongy" appearance?- deficiency and presents with the classic triad of
Spongiform encephalopathy ocular findings, cerebellar dysfunction, and
confusion.- Wernicke encephalopathy (WE)
Diagnosis of spongiform encephalopathy?-
Lumbar puncture ↑ levels of 14-3-3 protein. Treatment of Wernicke encephalopathy?-
Definitive diagnosis with brain biopsy This is a medical emergency and requires
infusion of thiamine over a few days to
What is Creutzfeldt-Jakob disease (CJD)?- normalized thiamine levels
CJD is classified as a transmissible
spongiform encephalopathy (TSE) along with Occurs in individuals with chronic liver disease
other prion diseases that occur in humans and such as cirrhosis or hepatitis. Early symptoms
animals. include forgetfulness, confusion, and breath with
a sweet or musty odor. Advanced symptoms
How does bovine spongiform encephalopathy include shaking of the hands or arms (asterixis),
(mad cow disease) relate to humans?- disorientation, slurred speech, and coma?-
Bovine spongiform encephalopathy (BSE), Hepatic encephalopathy
commonly known as mad cow disease, is an
incurable and invariably fatal neurodegenerative Treatment of hepatic encephalopathy?-
disease of cattle. Symptoms include abnormal Treatment includes removing toxic
behavior, trouble walking, and weight loss. Later substances from the intestine. Lactulose (to
in the course of the disease the cow becomes increase NH4 generation) and rifaximin
unable to function normally. The time between (decrease intraluminal NH3 production)
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
1/1
