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The Holmes-Adie plus syndrome.

W Mak1, R T Cheung

  • 1Division of Neurology, University Department of Medicine, Queen Mary Hospital, Hong Kong.

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
|August 16, 2000
PubMed
Summary

Holmes-Adie syndrome, characterized by tonic pupil and areflexia, may involve cranial nerves. This case highlights a variant with trigeminal and chorda tympani nerve involvement, suggesting a broader pathophysiological process.

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Area of Science:

  • Neurology
  • Clinical Neuroscience
  • Autonomic Nervous System Disorders

Background:

  • Holmes-Adie syndrome classically presents with a tonic pupil and diminished tendon reflexes.
  • Associated autonomic and peripheral neuropathies are recognized complications.
  • The underlying mechanism of areflexia is hypothesized to involve spinal reflex pathway synaptic dysfunction.

Observation:

  • A variant of Holmes-Adie syndrome was observed in a 41-year-old woman.
  • The patient presented with insidious sensory symptoms affecting the left trigeminal and chorda tympani nerves.
  • Clinical examination revealed generalized tendon areflexia and a left-sided Adie's pupil.

Findings:

  • Electrophysiological studies indicated an absent blink reflex upon stimulation of the left supraorbital and infraorbital nerves.

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  • Imaging studies did not reveal any structural abnormalities.
  • These findings suggest a dysfunction within the brainstem reflex arc.

    • Implications:

    • This case expands the known clinical spectrum of Holmes-Adie syndrome.
    • The findings suggest that the pathophysiological process in Holmes-Adie syndrome may extend beyond peripheral and spinal pathways to affect cranial nerves and brainstem reflexes.
    • Further research is warranted to elucidate the full extent of neurological involvement in this syndrome.