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Thomas P. Baumann, MD; Nina Hurwitz, MD; Eva Karamitopolou-Diamantis, MD; Alfonse Probst, MD; Richard Herrmann, MD; Andreas J. Steck, MD

Arch Neurol. 2000;57:374-377.

Objective  To describe a patient with unusually good outcome of a rare, high-grade lymphoma that often involves the nervous system.

Design  Case report.

Setting  University hospital.

Case  A 70-year-old pharmacist first presented with meningoencephalitislike symptoms and 6 months later with acute confusional state followed by complex partial status epilepticus. Diagnosis of intravascular lymphomatosis was made using detection and biopsy of a bilateral adrenal tumor.

Main Outcome and Results  Polychemotherapy consisting of CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone) led to complete remission. The patient's survival time currently exceeds 2 years.

Conclusions  The possibility of intravascular lymphomatosis should be considered in adult patients with unclear meningoencephalitic syndrome, acute confusional state, dementia, or other unexplained neurologic conditions with signs of a systemic disease. In intravascular lymphomatosis, as in other high-grade non-Hodgkin lymphomas, CHOP polychemotherapy should be the standard treatment.

From the Departments of Neurology (Drs Baumann and Steck), Pathology (Drs Hurwitz, Karamitopolou-Diamantis, and Probst), and Oncology (Dr Herrmann), University Hospital, Basel, Switzerland.

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