A 60 year old man with progressive dementia of recent onset presents with intermittent irregular jerky movemet EEG shows periodic sharp biphasic waves. The most likely diagnosis is
Correct Answer: Creutzfeldt Jakob disease
Description: Creutzfeldt-Jakob disease (CJD), also known as classic Creutzfeldt-Jakob disease, is a fatal degenerative brain disorder.] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. This is accompanied by physical problems such as speech impairment, balance and coordination dysfunction (ataxia), changes in gait, and rigid posture. In most people with CJD, these symptoms are accompanied by involuntary movements and the appearance of an atypical, diagnostic electroencephalogram tracing. The duration of the disease varies greatly, but sporadic (non-inherited) CJD can be fatal within months or even weeks.Most victims die six months after initial symptoms appear, often of pneumonia due to impaired coughing reflexes. About 15% of people with CJD survive for two or more years. The symptoms of CJD are caused by the progressive death of the brain's nerve cells, which is associated with the build-up of abnormal prion protein molecules forming amyloids. When brain tissue from a person with CJD is examined under a microscope, many tiny holes can be seen where whole areas of nerve cells have died. The word "spongiform" in "transmissible spongiform encephalopathies" refers to the sponge-like appearance of the brain tissue Ref Harrison20th edition pg 2234
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