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2007 ICD-9-CM Volume 1 Diagnosis Codes Home > Infectious And Parasitic Diseases 001-139 > Poliomyelitis And Other Non-Arthropod-Borne Viral Diseases Of Central Nervous System 045-049 > ICD-9-CM Diagnosis 046 Slow virus infection of central nervous system 046 is a non-specific code that cannot be used to specify a diagnosis ICD-9-CM Diagnosis 046.0 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) 046.0 is a specific code that can be used to specify a diagnosis 046.0 contains 2 index entries ICD-9-CM Diagnosis 046.1 Jakob-creutzfeldt disease 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. (From N Engl J Med, 1998 Dec 31;339(27)) 046.1 is a specific code that can be used to specify a diagnosis 046.1 contains 11 index entries ICD-9-CM Diagnosis 046.2 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) 046.2 is a specific code that can be used to specify a diagnosis 046.2 contains 13 index entries ICD-9-CM Diagnosis 046.3 Progressive multifocal leukoencephalopathy An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7) 046.3 is a specific code that can be used to specify a diagnosis 046.3 contains 2 index entries ICD-9-CM Diagnosis 046.8 Other specified slow virus infection of central nervous system 046.8 is a specific code that can be used to specify a diagnosis 046.8 contains 5 index entries ICD-9-CM Diagnosis 046.9 Unspecified slow virus infection of central nervous system 046.9 is a specific code that can be used to specify a diagnosis 046.9 contains 3 index entries