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Autonomic dysfunction in subacute spongiform encephalopathy.

R K Khurana, J H Garcia

    Archives of Neurology
    |February 1, 1981
    PubMed
    Summary
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    Subacute spongiform encephalopathy (SSE) causes significant autonomic nervous system dysfunction, unlike other dementias. Postmortem analysis revealed nerve damage, explaining these autonomic deficits in SSE patients.

    Area of Science:

    • Neurology
    • Autonomic Neuroscience

    Background:

    • Investigating autonomic nervous system (ANS) function is crucial for understanding neurodegenerative diseases.
    • Subacute spongiform encephalopathy (SSE) is a rare, rapidly progressive prion disease affecting the brain.

    Observation:

    • Two patients with SSE exhibited distinct autonomic dysfunctions.
    • Autonomic functions were assessed using pupillary responses, cold face test, atropine administration, and cold pressor test.
    • Control group included four patients with presenile dementia (Huntington's disease and Alzheimer's disease).

    Findings:

    • SSE patients showed diminished lacrimation, abnormal pupillary responses, and reduced heart rate response to stimuli.
    • Anhidrosis and lack of blood pressure increase during the cold pressor test were observed in SSE patients.

    Related Experiment Videos

  • Autonomic functions remained normal in the control group with other forms of dementia.

    • Implications:

    • Autonomic dysfunction is a key clinical feature distinguishing SSE from other dementias like Alzheimer's and Huntington's disease.
    • Morphological changes in sympathetic ganglia and the vagus nerve in postmortem studies correlate with observed autonomic deficits.
    • These findings highlight the importance of assessing ANS function in diagnosing and understanding SSE.