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• CLINICA NEUROL, 64: 85−92, 2024
• Vol.64 No.2 contents

Review

Central nervous system disorders secondary to histiocytoses: neurodegeneration with potential for improvement

Akira Morimoto, M.D., Ph.D. ^1）, Kenichi Sakamoto, M.D., Ph.D. ^2）, Ko Kudo, M.D. ^3） and Yoko Shioda, M.D., Ph.D. ^4）

¹⁾Division of Pediatrics, Showa Innan General Hospital
²⁾Department of Pediatrics, Shiga University of Medical Science
³⁾Department of Pediatrics, Hirosaki University School of Medicine
⁴⁾Children's Cancer Center, National Center for Child Health and Development

Histiocytoses, including Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), are inflammatory myeloid tumors in which monocyte lineage cells aggregate in various organs, causing tissue damage. Most of these tumors harbor oncogenic mutations in mitogen-activated protein kinase (MAPK) pathway genes, typified by BRAF^V600E. Some patients with LCH develop bilateral symmetrical cerebellar lesions and brain atrophy several years after diagnosis when the initial symptoms disappear, leading to cerebellar ataxia and higher cerebral dysfunction. A similar neurological disorder has also been reported in ECD. This neurological disorder can be improved with MAPK inhibitors. When patients with this neurological disorder are identified among neurodegeneration of unknown etiology or histiocytosis patients and treated early with MAPK inhibitors, the disorder can be reversible.
Full Text of this Article in Japanese PDF (2144K)

(CLINICA NEUROL, 64: 85−92, 2024)
key words: Langerhans cell histiocytosis, Erdheim-Chester disease, BRAF^V600E mutation, mitogen-activated protein kinase inhibitor, neurodegeneration

(Received: 30-Jun-23)