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  • Transmissible spongiform encephalopathies (TSEs)
  • Creutzfeldt-Jakob disease (CJD)
  • Variant Creutzfeldt-Jakob disease (vCJD)
  • Kuru
  • Fatal familial insomnia
  • Gerstmann-Straussler-Scheinker syndrome

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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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  • 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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Description

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  • Progressive neurodegenerative disorder
  • Abnormal pathogenic agent
  • Leads to brain damage
  • Affects the central nervous system
  • Rapidly progressive and fatal
  • Three classifications
    • Sporadic
    • Familial
    • Acquired

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Essentials of Diagnosis

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  • Presence of microscopic vacuolization of the brain tissue, spongy

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General Considerations

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  • Can affect both humans and animals
  • Currently no cure, fatal

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Demographics

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

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Signs and Symptoms

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  • Dementia
  • Convulsions
  • Ataxia
  • Behavior or personality changes

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Functional Implications

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  • 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

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Possible Contributing Causes

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  • Increased risk
    • Immunosuppressed
    • Perinatal to early childhood period
  • Prior meningitis

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Differential Diagnosis

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  • Stroke
  • Subdural empyema
  • Cerebral abscess
  • Cerebral venous thrombosis
  • Septic embolism
  • Meningitis

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Laboratory Tests

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Imaging

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  • CT scan for detailed imaging
  • MRI

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  • Emergency room if encephalitis is suspected
  • Neurologist both during infection and for follow-up
  • Social work and case management

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  • Arousal
  • Attention
  • Behavior
  • Cognition
  • Cranial nerve integrity
  • Gait
  • Sitting and standing balance
  • Muscle strength
  • Postural control
  • Range of motion
  • Deep tendon reflexes
  • Muscle tone
  • Sensation
  • Bed ...

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