Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Oral cyclophosphamide therapy for multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis

Masayoshi Tada, M.D.1), Osamu Onodera, M.D.2), Kenju Hara, M.D.1), Keiko Tanaka, M.D.1), Hitoshi Takahashi, M.D.3), Shoji Tsuji, M.D.4) and Masatoyo Nishizawa, M.D.1)

1)Deparment of Neurology, Brain research institute, Niigata University,
2)Department of Molecular Neuroscience, Resource Branch for Brain Disease Research
3)Department of Pathology
4)Department of Neurology, Graduate School of Medical Sciences, University of Tokyo

A 54-year-old man was admitted to our hospital because of a headache, dry cough, low grade fever and hearing loss sustained for 6 months. Physical and neurological examinations revealed bilateral conjunctival hyperemia, fine crackles in the lower lungs, cutaneous scars, horizontal gaze evoked nystagmus, bilateral moderate sensorineural deafness and mild hyperreflexia. Hypertrophic intracranial pachymeningitis (HIP) accompanied by episcleritis, pulmonary fibrosis, subcutaneous fibrosis of the trunk and upper limbs, bilateral chronic otitis media and sinusitis of the paranasal cavities were observed. Histopathological investigation of biopsied tissues from the dura matter, lung, skin and nasal mucosa showed marked fibrosis with lymphocyte and plasma cell infiltrations. The diagnosis of multifocal fibrosclerosis (MF) was made; this is a rare syndrome of unknown etiology characterized by fibrosis involving multiple organ systems. Although steroid pulse therapy and cyclophosphamide (CP) pulse therapy was not effective in his illnesses, the combination therapy of corticosteroid and oral CP was dramatically effective. We concluded that HIP can be a manifestation of MF, and additional oral CP should be considered as a treatment for steroid-resistant MF with HIP.

(CLINICA NEUROL, 46: 128－133, 2006)
key words: multifocal fibrosclerosis, hypertrophic pachymeningitis, oral cyclophosphamide, prednisolone

(Received: 7-Mar-05)