Variant Creutzfeldt-Jakob disease
ICD-10 A81.01 is a billable code used to indicate a diagnosis of variant creutzfeldt-jakob disease.
Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal neurodegenerative disorder caused by prion infection, primarily associated with the consumption of beef products contaminated with the agent responsible for bovine spongiform encephalopathy (BSE), commonly known as mad cow disease. The disease is characterized by progressive neurological decline, including psychiatric symptoms, cognitive impairment, and motor dysfunction. Patients may present with behavioral changes, memory loss, and ataxia, which can rapidly progress to severe dementia and ultimately lead to death, typically within a year of symptom onset. Diagnosis is often challenging and relies on clinical evaluation, MRI findings, and, in some cases, brain biopsy. The disease is distinct from classic Creutzfeldt-Jakob disease (CJD) and is associated with specific epidemiological factors, including exposure to infected cattle. Vaccination status is not directly relevant to vCJD, as it is not a viral infection and does not have a vaccine. However, understanding the patient's dietary history and potential exposure to BSE is crucial for accurate diagnosis and management.
Comprehensive neurological examination findings, imaging results, and patient history related to dietary exposure.
Patients presenting with rapid cognitive decline, atypical psychiatric symptoms, or unexplained neurological deficits.
Ensure thorough documentation of symptom progression and any relevant family history of prion diseases.
Detailed exposure history, including travel and dietary habits, and any relevant laboratory findings.
Patients with suspected prion disease following exposure to BSE or presenting with atypical neurological symptoms.
Consideration of other infectious agents that may mimic symptoms of vCJD.
Used to evaluate for prion disease when vCJD is suspected.
MRI reports must be included in the patient's medical record.
Neurologists should ensure that imaging findings correlate with clinical symptoms.
vCJD is primarily caused by the consumption of beef products contaminated with the prion responsible for BSE.
Diagnosis is based on clinical evaluation, MRI findings, and sometimes brain biopsy, along with a detailed patient history.
