Atypical virus infections of central nervous system (A81)
Clinical Information
Kuru - A prion disease found exclusively among the Fore linguistic group natives of the highlands of NEW GUINEA. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773)
New Guinea - Originally an island of the Malay Archipelago, the second largest island in the world. It divided, West New Guinea becoming part of Indonesia and East New Guinea becoming Papua New Guinea.
Subacute Sclerosing Panencephalitis - A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8)
Instructional Notations
Includes
This note appears immediately under a three character code title to further define, or give examples of, the content of the category.
- diseases of the central nervous system caused by prions
Use Additional Code
The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.
- code, if applicable, to identify:
- dementia with anxiety F02.84 F02.A4 F02.B4 F02.C4
- dementia with behavioral disturbance F02.81 F02.A1 F02.B1 F02.C1
- dementia with mood disturbance F02.83 F02.A3 F02.B3 F02.C3
- dementia with psychotic disturbance F02.82 F02.A2 F02.B2 F02.C2
- dementia without behavioral disturbance F02.80 F02.A0 F02.B0 F02.C0
- mild neurocognitive disorder due to known physiological condition F06.7
Certain infectious and parasitic diseases (A00–B99)
Viral and prion infections of the central nervous system (A80-A89)
A81 Atypical virus infections of central nervous system
A81.0 Creutzfeldt-Jakob disease
- A81.00 Creutzfeldt-Jakob disease, unspecified
- A81.01 Variant Creutzfeldt-Jakob disease
- A81.09 Other Creutzfeldt-Jakob disease
- A81.1 Subacute sclerosing panencephalitis
- A81.2 Progressive multifocal leukoencephalopathy
A81.8 Other atypical virus infections of central nervous system
- A81.81 Kuru
- A81.82 Gerstmann-Straussler-Scheinker syndrome
- A81.83 Fatal familial insomnia
- A81.89 Other atypical virus infections of central nervous system
- A81.9 Atypical virus infection of central nervous system, unspecified
Atypical virus infections of central nervous system (A81)