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CONDITION/DISORDER SYNONYMS

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  • Creutzfeldt–Jakob disease (CJD)

  • Fatal familial insomnia

  • Gerstmann– Sträussler –Scheinker syndrome

  • Kuru

  • Transmissible spongiform encephalopathies (TSEs)

  • Variant Creutzfeldt–Jakob disease (vCJD)

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ICD-9-CM CODES

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  • 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

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ICD-10-CM CODES

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  • 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

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PREFERRED PRACTICE PATTERNS1

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  • 5A: Primary Prevention/Risk Reduction for Loss of Balance and Falling

  • 5C: Impaired Motor Function and Sensory Integrity Associated with Nonprogressive Disorders of the Central Nervous System—congenital origin or acquired in infancy or childhood

  • 5D: Impaired Motor Function and Sensory Integrity Associated with Nonprogressive Disorders of the Central Nervous System—acquired in adolescence or adulthood

  • 5I: Impaired Arousal, Range of Motion and Motor Control Associated with Coma, Near Coma, or Vegetative State

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PATIENT PRESENTATION

A 52-year-old man was admitted to the hospital following a fall that resulted in a fractured right ankle. The ankle fractured was surgically repaired. The family reports that the patient had become increasingly unstable while walking; he struggles to get out of his favorite chair at home, and needs help with getting dressed. He is also very confused and seems to struggle with remembering things from one moment to the next. These concerns started approximately 3 weeks ago. Before that time, he was completely independent and worked successfully at a job that required him to think and act quickly.

Physical therapy was ordered in the hospital to address ambulation, non-weight bearing on the right lower extremity.

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KEY FEATURES

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Description
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  • Progressive neurodegenerative disorders

  • Abnormal pathogenic agent

  • Leads to brain damage

  • Affects the central nervous system

  • Rapidly progressive and fatal

  • Human prion diseases

    • Kuru

    • Fatal familial insomnia

    • CJD

    • vCJD

    • Gerstmann–Sträussler–Scheinker syndrome

  • Animal prion diseases

    • Bovine spongiform encephalopathy (BSE)

    • Chronic wasting disease (CWD)

    • Scrapie

    • Transmissible mink encephalopathy

    • Feline spongiform encephalopathy

    • Ungulate spongiform encephalopathy

  • Three classifications

    • Sporadic

    • Familial

    • Acquired

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FIGURE 53-1

Appearance of brain with spongiform encephalopathy. (Left) Normal brain. (Right) Brain infected with a prion. Note the spongelike appearance. (Reproduced with permission from Nester EW, Anderson DG, Roberts CE Jr, Nester MT. Microbiology: A Human Perspective. 6th ed. New York, NY: McGraw-Hill; 2008.)

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