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Lhermitte - Duclos disease with syrinx: case report and literature review

Khursheed Nayil1, Maqbool Wani, Altaf Ramzan

  • 1SKIMS, Department of Neurosurgery, Kashmir, India.

Turkish Neurosurgery
|December 24, 2011
PubMed

Insights

Lhermitte-Duclos disease, a rare cerebellar hamartoma, can present with cervical syringomyelia in young patients. Surgical decompression offers an excellent prognosis for this condition.

Area of Science:

  • Neurology
  • Neurosurgery
  • Pediatrics

Background:

  • Lhermitte-Duclos disease is a rare cerebellar hamartomatous malformation.
  • Typically presents in adults with symptoms of increased intracranial pressure and cerebellar dysfunction.
  • Often associated with other neurological conditions.

Observation:

  • An 18-year-old female presented with headache and gait ataxia.
  • Imaging revealed Lhermitte-Duclos disease.
  • Concurrent cervical cord syrinx was identified.

Findings:

  • This case represents the fifth reported instance of Lhermitte-Duclos disease with syringomyelia in the pediatric population.
  • The co-occurrence of these conditions is rare.
  • The patient's presentation included typical symptoms of both pathologies.

Implications:

  • Surgical decompression of the cerebellar lesion is the standard treatment.
  • Lhermitte-Duclos disease has an excellent prognosis with no risk of malignant transformation.
  • Early diagnosis and intervention are crucial for managing associated syringomyelia.
Abstract

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