DSM-IV

In DSM-IV, this disorder is called Dementia Due to Creutzfeldt-Jakob Disease

For more information, see Dementia Due to Other General Medical Conditions

The essential feature of Dementia Due to Creutzfeldt-Jakob Disease is the presence of a dementia that is judged to be the direct pathophysiological consequence of Creutzfeldt-Jakob disease. Jacob-Creutzfeldt disease is one of the subacute spongiform encephalopathies, a group of central nervous system diseases caused by transmissible agents known as "slow viruses" or prions. Typically, individuals with Creutzfeldt-Jakob disease manifest the clinical triad of dementia, involuntary movements (particularly myoclonus), and periodic EEG activity. However, up to 25% of individuals with the disorder may have atypical presentations, and the disease can be confirmed only by biopsy or at autopsy with the demonstration of spongiform neuropathological changes. Creutzfeldt-Jakon disease may develop at any age in adults, but most typically when they are between ages 40 and 60 years. From 5% to 15% of cases may have a familial component. Prodromal symptoms of Creutzfeldt-Jakob disease may include fatigue, anxiety, or problems with appetite, sleeping, or concentration and may be followed after several weeks by incoordination, altered vision, or abnormal gait or other movements that may be myoclonic, choreoathetoid, or ballistic, along with a rapidly progressive dementia. The disease typically progresses very rapidly over several months, although more rarely it can progress over years and appear similar in its course to other dementias. There are no distinctive findings cerebrospinal fluid analysis, and nonspecific atrophy may be apparent on neuroimaging. In most individuals, the EEG typically reveals periodic sharp, often triphasic and synchronous discharges at a rate of 0.5-2 Hz at some point during the course of the disorder. The transmissible agent thought to be responsible for Creutzfeldt-Jakon disease is resistant to boiling, formalin, alcohol, and ultraviolet radiation, but it can be inactivated by pressured autoclaving or by bleach. Transmission by corneal transplantation and human growth factor injection has been documented, and anecdotal cases of transmission to health care workers have been reported. Therefore, when neurosurgery, brain biopsy, or brain autopsy is undertaken, universal precautions should be taken with both tissue and equipment that comes in contact with tissue.

DSM-5

For more information, see Major and Mild Neurocognitive Disorders

Diagnostic Criteria

A. The criteria are met for major or mild neurocognitive disorder.

B. There is insidious onset, and rapid progression of impairment is common.

C. There are motor features of prion disease, such as myoclonus or ataxia, or biomarker evidence.

D. The neurocognitive disorder is not attributable to another medical condition and is not better explained by another mental disorder.

Differential Diagnosis

Other major neurocognitive disorders

Major NCD due to prion disease may appear similar in its course to other NCDs, but prion diseases are typically distinguished by their rapid progression and prominent cerebellar and motor symptoms.

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