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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
Summary
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.