2021 ICD-10-CM Codes for Atypical virus infections of central nervous system (A81)

Clinical Terms for Atypical virus infections of central nervous system (A81)

Central Nervous System Viral Diseases-. Viral infections of the brain, spinal cord, meninges, or perimeningeal spaces.

Creutzfeldt-Jakob Syndrome-. A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS.

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.

Gerstmann-Straussler-Scheinker Disease-. An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration.

Instructional Notations

Includes 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 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 to identify:
  • dementia with behavioral disturbance F02.81
  • dementia without behavioral disturbance F02.80