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Cerebellar leukoencephalopathy: most likely histiocytosis-related

M S van der Knaap1, W F M Arts, J Y Garbern

  • 1Department of Child Neurology, VU University Medical Center, Amsterdam, The Netherlands. ms.vanderknaap@vumc.nl

Neurology
|October 22, 2008
PubMed

Insights

Cerebellar white matter abnormalities in patients may indicate a paraneoplastic syndrome linked to histiocytosis. Monitoring these patients for histiocytosis is crucial for early diagnosis and management.

Area of Science:

  • Neurology
  • Oncology
  • Radiology

Background:

  • Histiocytosis (Langerhans and non-Langerhans cell types) can present with cerebellar white matter abnormalities, often paraneoplastic.
  • This neurodegenerative syndrome involves ataxia, spasticity, cognitive decline, and hormonal dysfunction, detectable via MRI.

Purpose of the Study:

  • To investigate if 13 patients with similar MRI abnormalities share the neurodegenerative syndrome associated with histiocytosis.

Main Methods:

  • Review of clinical and laboratory data for 13 patients.
  • Evaluation of brain MRIs; spinal cord MRI in seven patients.

Main Results:

  • Patients exhibited cerebellar/pyramidal dysfunction, behavioral issues, and cognitive decline.
  • MRI revealed cerebellar, brainstem, basal ganglia, and cerebral white matter abnormalities; three had spinal cord lesions.
  • Two patients had histiocytic infiltrates in brain lesions, and three showed hormonal dysfunction.

Conclusions:

  • The clinical and MRI findings strongly suggest a shared paraneoplastic syndrome with histiocytosis.
  • While a genetic disorder cannot be entirely excluded, histiocytic lesions support the paraneoplastic etiology.
  • Patients with cerebellar white matter abnormalities require monitoring for histiocytosis.
Abstract

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