Disease Information for Kuru

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Clinical Manifestations
Signs & Symptoms
Ataxia
Ataxia, Cerebellar type
Clumsiness/poor coordination
Dysarthria
Paralysis
Slurred speech
Staggering Gait
Tremor
Difficulty Talking
Urine Incontinence
Shivering
Weakness
Blurred Cloudy Vision
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Lethal
Course/Prognosis bad/usually
Course/Progressive
Course/Relentless
Course/Subacute
Demographics & Risk Factors
Travel, Geographic & Climate Related Factors
Residence/travel/New Guinea
Past History
Past History/Ritual brain cannibalism
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Children/all
Population/Female
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Right Shift (Viral) Differential Smear (Lab)
Diagnostic Test Results
Pathology
PATH/Brain Kuru neuron/cortex plaques
PATH/Brain Spongiform degeneration
PATH/Brain/Glial proliferation/ganglion cell lysis
BX/Tonsil Prion/C-J protein test positive
CT Scan
MRI/Head Brain FLAIR Image Abnormal
MRI/Head Brain T2 bright signal Pulvinar
MRI/Head T2 and fluid -attenuated inversion recovery
MRI/Head/Thalamus Lesion
Associated Diseases & Rule outs
Associated Disease & Complications
Paralytic Disorder
Ataxia Disorder
Disease Mechanism & Classification
Specific Agent
AGENT/Endemic disease (ex)
AGENT/Prion transmissable disease (ex)
AGENT/Slow CNS virus disease (ex)
AGENT/Slow virus (ex)
AGENT/Virus (category)
Class
CLASS/Neurologic (category)
Pathophysiology
Pathophysiology/Axon degeneration nerve endings
Pathophysiology/CNS degeneration
Pathophysiology/CNS Prion Protein Abnormality
Pathophysiology/Developmental degeneration CNS
Pathophysiology/Spongy degeneration of neuraxis/process
Process
PROCESS/INCIDENCE/Extremely rare disease
PROCESS/INCIDENCE/Regional specific
PROCESS/Infection/agent specific (category)
Definition

A prion disease found exclusively among the Fore liguistic group natives of the highlands of NEW GUINEA; The illness is primarily restricted to adult females and children of both sexes; It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence; Death occurs within 3-6 months of disease onset; The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued; Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques; (From Adams et al, Principles of Neurology, 6th ed, p773); spongioform degeneration consistant with mad cow disease and jacob-creutzfeld disease; prognosis is poor.

Kuru is a rare progressive degenerative brain disorder that occurs exclusively in members of the Fore linguistic tribal group of the New Guinea highlands; Associated symptoms include progressively impaired coordination of voluntary movements (ataxia) of the trunk, arms, and legs; slurred speech (dysarthria); a "shivering-like" tremor; visual disturbances; and paralysis; Neurodegenerative changes include generalized loss of nerve cells, particularly in the outer region of the brain (cerebral cortex), and the development of characteristic plaques (ie, "kuru plaques"). Transmission of the disease is thought to result from ritualistic handling and ingestion (cannibalism) of brain tissue of deceased relatives; The incidence of kuru has dramatically declined with the cessation of such practices

---------------[NORD 2005]--------------------

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External Links Related to Kuru
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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