Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Dramatic but temporary improvements in a case of CNS intravascular malignant lymphomatosis

Masahiko Aoyama, M.D.1), Tomoko Aoki, M.D.1), Yutaka Matsuura, M.D.1), Takahiko Yamanoi, M.D.1), Akiko Watanabe, M.D.1), Naoshi Saitou, M.D.1), Mari Honma, M.D.1), Toshihito Ishikawa, M.D.2), Namio Kodama, M.D.2) and Teiji Yamamoto, M.D.1)

1)Department of Neurology, Fukushima Medical University School of Medicine
2)Department of Neurosurgery, Fukushima Medical University School of Medicine

A 54-year-old man with a past history of gastric malignant lymphoma treated by the total gastrectomy and the chemotherapy, developed bilateral sudden deafness one year later. Two years after the gastrectomy he became abruptly paraplegic with sensory impairments of the lower extremities and neurogenic bladder. Serum LDH and soluble IL-2 receptor were high in titers (552 U/l and 1,090U/l, normal range 145-519). Although the imaging studies of the spinal cord were negative, the myelopathic symptoms resolved dramatically after a course of pulse dose methylprednisolone therapy. However, he soon developed an abnormal behavior and mental deterioration in 3 weeks. The MRIs of the brain revealed abnormal signals compatible with multiple cerebral infarctions.
As intravascular malignant lymphomatosis (IML) was suspected because of the laboratory and MRI findings, biopsies of the skin, the bone marrow, the muscle and the lymph node were carried out, without evidence of lymphoma. The brain biopsy ultimately confirmed the presence of IML.
The patient remarkably responded to biweekly CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) therapy in terms of regaining the mental alertness and improved hearing. However, the CHOP therapy was prematurely interrupted prior to completion because of infective arthritis. The relapse soon ensued, and he died 6 months after admission.
This case was of interest because a solid gastric lymphoma appears to have transformed into the form of intravascular lymphomatosis without mass formations or leukemic changes. Although the neurological symptoms in association with IML are thought to be the results of ischemic events, this case illustrates a remarkable reversibility of the symptoms. This implies that the cerebral symptoms are not necessarily the results of typical ischemic infarction, but due to relative ischemia because of chiefly capillary-venous occlusion by lymphoma cells. The majority of the symptoms is thus attributable to the functional impairment. Therefore, the therapeutic intervention may dramatically improve the symptoms due to IML.

(CLINICA NEUROL, 43: 6－11, 2003)
key words: intravascular malignant lymphomatosis (IML), deafness, brain biopsy, CHOP therapy

(Received: 18-Jul-02)