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Character changes from idiopathic cranial pachymeningoencephalitis

Takuya Matsushita1, Hiroyuki Murai, Masakazu Kawajiri

  • 1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, 812-8582, Japan.

Journal of the Neurological Sciences
|March 1, 2006
PubMed

Insights

This study details a rare case of recurrent cranial pachymeningoencephalitis presenting with personality changes. Steroid treatment resolved the frontal lobe dysfunction and associated MRI lesions.

Area of Science:

  • Neurology
  • Neuroinflammation
  • Cranial Pachymeningoencephalitis

Background:

  • Idiopathic cranial pachymeningoencephalitis is a rare condition affecting the dura mater.
  • This case highlights a unique presentation involving significant personality and behavioral changes.

Observation:

  • A 66-year-old male presented with orbital pain and marked character changes, including irritability and obsessive focus on minor details.
  • Neurological examination revealed a history of transient abducent nerve palsy.
  • Brain MRI demonstrated dural thickening with enhancement and bilateral frontal white matter lesions.

Findings:

  • Cerebral biopsy showed microglial proliferation and mild lymphocytic perivascular infiltration, ruling out infection.
  • Treatment with methylprednisolone pulse therapy and oral prednisolone led to normalization of behavior.
  • Follow-up MRI confirmed the resolution of frontal lobe lesions.

Implications:

  • This case suggests that cranial pachymeningoencephalitis can manifest with psychiatric symptoms due to frontal lobe involvement.
  • Early diagnosis and immunosuppressive therapy may reverse neurological and behavioral deficits.
  • Further research is warranted to understand the neuroinflammatory mechanisms underlying such presentations.

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