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Isolating Central Nervous System Tissues and Associated Meninges for the Downstream Analysis of Immune cells
Published on: May 19, 2020
Erdheim-Chester disease with isolated craniocerebral involvement
Rajendra Singh Jain1, Raghavendra Bakki Sannegowda, Rahul Jain
1Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India. drrsjain@yahoo.com,
BMJ Case Reports
|April 18, 2013
Summary
Erdheim-Chester disease (ECD), a rare histiocytosis, can manifest as intracranial masses causing seizures. This case showed remarkable lesion resolution with antiepileptic therapy alone.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis.
- Intracranial involvement in ECD is uncommon, typically presenting with diabetes insipidus or cerebellar-brainstem signs.
Observation:
- A 40-year-old male presented with recurrent generalized seizures.
- Brain MRI revealed multiple enhancing intracranial masses in the frontal, temporal, and parietal regions.
- Biopsy confirmed a rare case of ECD.
Findings:
- The patient's seizures were managed with antiepileptic therapy.
- Significant resolution of intracranial lesions was observed on repeat MRI.
- Osteolytic skull lesions also resolved, with no other systemic involvement noted over 12 years.
Implications:
- This case highlights an unusual presentation of ECD with intracranial masses causing seizures.
- Antiepileptic therapy alone led to significant lesion regression, suggesting a potential therapeutic avenue.
- Long-term follow-up confirmed sustained remission without further systemic spread.
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