Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content ++ Transmissible spongiform encephalopathies (TSEs)Creutzfeldt-Jakob disease (CJD)Variant Creutzfeldt-Jakob disease (vCJD)KuruFatal familial insomniaGerstmann-Straussler-Scheinker syndrome ++ 046.0 Kuru046.1 Jakob-Creutzfeldt disease046.11 Variant Creutzfeldt-Jakob disease046.19 Other and unspecified Creutzfeldt-Jakob disease046.2 Subacute sclerosing panencephalitis046.3 Progressive multifocal leukoencephalopathy046.7 Other specified prion diseases of central nervous system046.71 Gerstmann-Sträussler-Scheinker syndrome046.72 Fatal familial insomnia046.79 Other and unspecified prion disease of central nervous system046.8 Other specified slow virus infection of central nervous system046.9 Unspecified slow virus infection of central nervous system ++ A81.00 Creutzfeldt-Jakob disease, unspecifiedA81.01 Variant Creutzfeldt-Jakob diseaseA81.09 Other Creutzfeldt-Jakob diseaseA81.1 Subacute sclerosing panencephalitisA81.2 Progressive multifocal leukoencephalopathyA81.81 KuruA81.82 Gerstmann-Sträussler-Scheinker syndromeA81.83 Fatal familial insomniaA81.89 Other atypical virus infections of central nervous systemA81.9 Atypical virus infection of central nervous system, unspecified ++ 5A: Primary Prevention/Risk Reduction for Loss of Balance and Falling5C: Impaired Motor Function and Sensory Integrity Associated with Nonprogressive Disorders of the Central Nervous System - congenital origin or acquired in infancy or childhood5D: Impaired Motor Function and Sensory Integrity Associated with Nonprogressive Disorders of the Central Nervous System - acquired in adolescence or adulthood5I: Impaired Arousal, Range of Motion and Motor Control Associated with Coma, Near Coma, or Vegetative State +++ Description ++ Progressive neurodegenerative disorderAbnormal pathogenic agentLeads to brain damageAffects the central nervous systemRapidly progressive and fatalThree classificationsSporadicFamilialAcquired +++ Essentials of Diagnosis ++ Presence of microscopic vacuolization of the brain tissue, spongy +++ General Considerations ++ Can affect both humans and animalsCurrently no cure, fatal +++ Demographics ++ Familial type is genetic: defect in the prion protein gene +++ Signs and Symptoms ++ DementiaConvulsionsAtaxiaBehavior or personality changes +++ Functional Implications ++ Inability to perform ADLs independentlyInability to perform functional mobility independentlyInability to perform cognitive tasks appropriate to age and educational levelInability to execute fine and gross motor tasks independently +++ Possible Contributing Causes ++ Increased riskImmunosuppressedPerinatal to early childhood periodPrior meningitis +++ Differential Diagnosis ++ StrokeSubdural empyemaCerebral abscessCerebral venous thrombosisSeptic embolismMeningitis +++ Laboratory Tests ++ Cerebral spinal fluid testingImmunohistochemical analysis of brain tissueElectroencephalogram (EEG) +++ Imaging ++ CT scan for detailed imagingMRI ++ Pulvinar sign on MRIPeriodic sharp waves on electroencephalogram (EEG) ++ Emergency room if encephalitis is suspectedNeurologist both during infection and for follow-upSocial work and case management ++ ArousalAttentionBehaviorCognitionCranial nerve integrityGaitSitting and standing balanceMuscle strengthPostural control... Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth