Prion diseases

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

• 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

• 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

• 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

Description

• 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

Demographics

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

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

Laboratory Tests

• Cerebral spinal fluid testing
• Immunohistochemical analysis of brain tissue
• Electroencephalogram (EEG)

Imaging

• CT scan for detailed imaging
• MRI

• Pulvinar sign on MRI
• Periodic sharp waves on electroencephalogram (EEG)

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

• Arousal
• Attention
• Behavior
• Cognition
• Cranial nerve integrity
• Gait
• Sitting and standing balance
• Muscle strength
• Postural control
...