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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 > Slow Virus Infection Of Central Nervous System 046.* > 2007 ICD-9-CM Diagnosis 046.1 Jakob-creutzfeldt disease This code may be outdated. View the most recent version of ICD-9-CM 046.1 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 View the ICD-9-CM Volume 1 046.* hierarchy 046.1 also known as: Subacute spongiform encephalopathy Index entries containing 046.1: Creutzfeldt-Jakob disease (syndrome) (new variant) 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10] Disease, diseased - see also Syndrome Creutzfeldt-Jakob (new variant) 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10] Jakob-Creutzfeldt (new variant) 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10] Encephalopathy (acute) 348.30 spongioform, subacute (viral) 046.1 subacute spongioform 046.1 viral, spongioform 046.1 Jakob-Creutzfeldt disease (syndrome) (new variant) 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10] Pseudosclerosis (brain) Jakob's 046.1 spastic 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10] Syndrome - see also Disease Creutzfeldt-Jakob (new variant) 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10] Jakob-Creutzfeldt (new variant) 046.1 with dementia with behavioral disturbance 046.1 [294.11] without behavioral disturbance 046.1 [294.10]