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Update on Susac's syndrome.

Menachem Gross1, Ron Eliashar

  • 1Department of Otolaryngology/Head and Neck Surgery, The Hebrew University School of Medicine, Hadassah Medical Center, Jerusalem, Israel. drgrossm@hotmail.com

Current Opinion in Neurology
|May 14, 2005
PubMed
Summary

Susac's syndrome, a rare condition causing encephalopathy, vision loss, and hearing loss, is often underdiagnosed. Magnetic resonance imaging (MRI) is crucial for identifying characteristic brain lesions, aiding diagnosis.

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Area of Science:

  • Neurology
  • Ophthalmology
  • Radiology

Background:

  • Susac's syndrome is a rare neurological disorder characterized by a clinical triad: encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss.
  • Recent findings highlight variable clinical presentations, including epileptic seizures and transient visual disturbances.
  • The syndrome is frequently underdiagnosed due to its multisystemic nature and resemblance to other conditions like multiple sclerosis.

Purpose of the Study:

  • To review recent advancements in understanding the clinical course of Susac's syndrome.
  • To explore updated imaging modalities for diagnosing Susac's syndrome.
  • To emphasize the diagnostic utility of magnetic resonance imaging (MRI) in Susac's syndrome.

Main Methods:

  • Review of recent literature on Susac's syndrome.

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  • Analysis of clinical presentations and diagnostic criteria.
  • Evaluation of neuroradiological findings, particularly MRI patterns.

    • Main Results:

    • Magnetic resonance imaging (MRI) reveals distinctive patterns in the brain's white matter, grey matter, and leptomeninges.
    • Characteristic MRI findings include multiple small hyperintense foci on T2-weighted images and contrast enhancement.
    • Callosal lesions, typically involving central fibers, are highly suggestive (probably pathognomonic) of Susac's syndrome.

    Conclusions:

    • Susac's syndrome requires a high index of suspicion, especially in patients with unexplained encephalopathy and visual or hearing disturbances.
    • MRI is the neuroimaging modality of choice for diagnosing Susac's syndrome.
    • Characteristic MRI findings in the corpus callosum, white and grey matter, and leptomeninges are key diagnostic indicators.