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Midbrain cleft as a cause of chronic internuclear ophthalmoplegia, progressive ataxia, and facial weakness

Omar Ahmad1, Stephen Reddel, Christian J Lueck

  • 1Department of Neurology, The Canberra Hospital and Australian National University Medical School, Canberra, Australia.

Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society
|April 16, 2010
PubMed

Insights

A rare midbrain cleft, a developmental abnormality, can cause progressive ataxia and bilateral internuclear ophthalmoplegia (INO). This condition is often overlooked on MRI, leading to misdiagnosis and highlighting the need for careful radiological assessment.

Area of Science:

  • Neuroscience
  • Neurology
  • Radiology

Background:

  • A developmental anomaly termed "midbrain cleft" or "keyhole aqueduct syndrome" is rarely reported.
  • This condition can be mistaken for multiple sclerosis due to overlapping symptoms like ataxia and ophthalmoplegia.

Purpose of the Study:

  • To highlight the clinical presentation and diagnostic challenges of the midbrain cleft.
  • To emphasize the importance of recognizing this rare condition on neuroimaging.

Main Methods:

  • Case presentation of a 44-year-old man with progressive ataxia and facial weakness.
  • Review of previously reported cases of midbrain cleft.
  • Analysis of brain MRI findings, noting an overlooked midbrain anatomic cleft.

Main Results:

  • The patient presented with progressive ataxia, facial weakness, bilateral adduction deficits, and abducting nystagmus.
  • The overlooked midbrain cleft was identified as the cause of bilateral internuclear ophthalmoplegia (INO).
  • Previously reported cases consistently show bilateral INO, often with progressive ataxia.

Conclusions:

  • Midbrain cleft is a rare developmental abnormality that can lead to debilitating progressive ataxia and INO.
  • Accurate diagnosis requires careful neuroimaging review to avoid misdiagnosis, such as with multiple sclerosis.
  • The etiology of midbrain cleft is uncertain, and no effective treatment is currently known.

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