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Hiroyuki Ishiura, MD; Masato Morikawa, MD; Masashi Hamada, MD; Takuro Watanabe, MD, PhD; Shinichi Kako, MD; Shigeru Chiba, MD, PhD; Toru Motokura, MD, PhD; Akira Hangaishi, MD, PhD; Junji Shibahara, MD, PhD; Masaaki Akahane, MD, PhD; Jun Goto, MD, PhD; Shin Kwak, MD, PhD; Mineo Kurokawa, MD, PhD; Shoji Tsuji, MD, PhD

Arch Neurol. 2008;65(5):662-665.

Objective  To report the successful treatment of a patient with lymphomatoid granulomatosis (LYG), a rare Epstein-Barr virus–positive lymphoproliferative disorder, using rituximab (anti-CD20 monoclonal antibody). The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established. Because central nervous system (CNS) involvement of LYG has been known to show poor prognosis, the establishment of an effective treatment for CNS LYG with mild adverse effects is desired.

Design  Case report.

Setting  University hospital.

Patient  A 48-year-old Japanese man presenting with slowly progressive spastic paraparesis diagnosed as LYG involving the CNS and lungs.

Interventions  The patient was treated with rituximab (375 mg/m², once weekly for 1 month) alone.

Main Outcome Measure  Improvement of the lesions on imaging.

Results  The neurological signs resolved and the lesions in the CNS and lungs were mostly diminished after the rituximab monotherapy without any adverse effects. The patient stayed in remission for 18 months.

Conclusion  Rituximab monotherapy was effective in treating the patient; hence, rituximab should be considered as the initial treatment against LYG involving the CNS.

Author Affiliations: Departments of Neurology (Drs Ishiura, Hamada, Goto, Kwak, and Tsuji), Hematology and Oncology (Drs Morikawa, Watanabe, Kako, Chiba, Motokura, Hangaishi, and Kurokawa), Pathology (Dr Shibahara), and Radiology (Dr Akahane), Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

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