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[Facial hemiatrophy (Parry-Romberg syndrome)].

N F Poliakova, I V Lebezeva, S A Kisel'

    Zhurnal Nevropatologii I Psikhiatrii Imeni S.S. Korsakova (Moscow, Russia : 1952)
    |January 1, 1991
    PubMed
    Summary

    This study examines a rare facial atrophy syndrome, likely idiopathic, with hypothalamic damage. Findings suggest suprasegmental, not peripheral, autonomic nervous system involvement.

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    Area of Science:

    • Neurology
    • Genetics

    Background:

    • Describes three cases of a rare, poorly understood disease causing progressive hemifacial atrophy.
    • Suggests dysembryogenesis and hypothalamic damage as underlying causes.

    Observation:

    • The disease primarily affects facial tissues, with less muscular injury.
    • In one case, atrophy spread to the hand and body; in two others, it was linked to vegetovascular dystonia.

    Findings:

    • Neurological examination revealed no gross changes in the animal nervous system.
    • Autonomic nervous system evaluation, including sympathetic potentials and pupillary tests, showed no peripheral lesions.

    Implications:

    • The findings suggest the disease involves suprasegmental autonomic structures rather than peripheral ones.
    • Further research into the central autonomic pathways is warranted for this rare syndrome.

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