Creutzfeldt-Jakob disease, unspecified
ICD-10 A81.00 is a billable code used to indicate a diagnosis of creutzfeldt-jakob disease, unspecified.
Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disorder caused by prions, which are misfolded proteins that induce abnormal folding of normal cellular proteins in the brain. This leads to brain damage and the characteristic symptoms of CJD, including rapidly progressive dementia, ataxia, and myoclonus. The disease typically presents in middle to late adulthood, with a median onset age of 68 years. Patients may initially exhibit psychiatric symptoms, such as depression or anxiety, followed by cognitive decline and neurological deficits. The disease progresses rapidly, often leading to severe disability and death within months to a few years after symptom onset. Diagnosis is primarily clinical, supported by MRI findings, EEG changes, and cerebrospinal fluid analysis for 14-3-3 protein. There are no effective treatments, and management focuses on supportive care. CJD is classified into several forms, including sporadic, familial, and acquired (iatrogenic or variant), but this code is used when the specific type is not specified.
Detailed neurological examination findings, imaging results, and laboratory test outcomes.
Patients presenting with rapid cognitive decline, myoclonus, or ataxia.
Ensure all relevant diagnostic criteria are documented to support the diagnosis of CJD.
History of potential exposure to prion diseases, including surgical history and dietary habits.
Patients with a history of exposure to infected tissues or blood products.
Document any relevant travel history or potential zoonotic exposure.
Used to assess for abnormal brain activity in suspected CJD cases.
Document the indication for EEG and findings.
Neurologists should ensure EEG findings correlate with clinical symptoms.
Common symptoms include rapidly progressive dementia, ataxia, myoclonus, and visual disturbances. Patients may also experience personality changes and psychiatric symptoms.
