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Condition/Disorder Synonyms

  • Transmissible spongiform encephalopathies (TSEs)

  • Creutzfeldt-Jakob disease (CJD)

  • Variant Creutzfeldt-Jakob disease (vCJD)

  • Kuru

  • Fatal familial insomnia

  • Gerstmann-Straussler-Scheinker syndrome

ICD-9-CM Codes

  • 046.0 Kuru

  • 046.1 Jakob-Creutzfeldt disease

  • 046.11 Variant Creutzfeldt-Jakob disease

  • 046.19 Other and unspecified Creutzfeldt-Jakob disease

  • 046.2 Subacute sclerosing panencephalitis

  • 046.3 Progressive multifocal leukoencephalopathy

  • 046.7 Other specified prion diseases of central nervous system

  • 046.71 Gerstmann-Sträussler-Scheinker syndrome

  • 046.72 Fatal familial insomnia

  • 046.79 Other and unspecified prion disease of central nervous system

  • 046.8 Other specified slow virus infection of central nervous system

  • 046.9 Unspecified slow virus infection of central nervous system

ICD-10-CM Codes

  • 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

  • A81.81 Kuru

  • A81.82 Gerstmann-Sträussler-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

Preferred Practice Patterns1

Key Features


  • Progressive neurodegenerative disorder

  • Abnormal pathogenic agent

  • Leads to brain damage

  • Affects the central nervous system

  • Rapidly progressive and fatal

  • Three classifications

    • Sporadic

    • Familial

    • Acquired

Essentials of Diagnosis

  • Presence of microscopic vacuolization of the brain tissue, spongy

General Considerations

  • Can affect both humans and animals

  • Currently no cure, fatal


  • Familial type is genetic: defect in the prion protein gene

Clinical Findings

Signs and Symptoms

  • Dementia

  • Convulsions

  • Ataxia

  • Behavior or personality changes

Functional Implications

  • Inability to perform ADLs independently

  • Inability to perform functional mobility independently

  • Inability to perform cognitive tasks appropriate to age and educational level

  • Inability to execute fine and gross motor tasks independently

Possible Contributing Causes

  • Increased risk

    • Immunosuppressed

    • Perinatal to early childhood period

  • Prior meningitis

Differential Diagnosis

  • Stroke

  • Subdural empyema

  • Cerebral abscess

  • Cerebral venous thrombosis

  • Septic embolism

  • Meningitis

Means of Confirmation or Diagnosis

Laboratory Tests

  • Cerebral spinal fluid testing

  • Immunohistochemical analysis of brain tissue

  • Electroencephalogram (EEG)


  • CT scan for detailed imaging

  • MRI

Findings and Interpretation

  • Pulvinar sign on MRI

  • Periodic sharp ...

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