Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Autonomic neuropathy as the first presentation of T-cell malignant lymphoma -a case report-

Soutarou Taguchi, M.D.1)2), Tomohiko Nakamura, M.D., Ph.D.1), Takako Yamada, M.D., Ph.D.1), Hiromu Takamido, M.D., Ph.D.1), Manabu Doyu, M.D., Ph.D.2) and Akira Takahashi, M.D., Ph.D.1)

1)Department of Neurology, Tokai Central Hospital
2)Department of Neurology, Aichi Medical University

A 61-year-old man noted flu-like symptoms. Not long afterwards, he felt constipation, nausea, and blackout when standing or sitting. His blood pressure was 110/70 mmHg in the supine position. On sitting blood pressure dropped to 73/34 mmHg. Heart rate increased from 65 to 78 beats per minutes. He did not have fever, edema, or skin rash. The remainder of the general medical examination was normal. A neurological examination revealed normal higher mental, and sensori-motor functions. The blood test revealed leukocytosis 7,320/μl, LD 1,426 IU/l, IL-2R 921 U/ml, and CRP 11.5 mg/dl. A whole body CT scan and cranial MR imaging showed no significant change. Thoracic spine MR imaging revealed multiple T1 low signal small foci in part of the vertebral body suggesting bone metastasis of the tumor. The heart/mediastinum ratio of 123I-meta-iodobenzylguanidine scintigraphy early imaging was 2.42. The nerve conduction study and electrocardiogram coefficient of variation of R-R intervals showed no abnormalities. Two months after the onset of symptoms, he was found to have glove-and-stocking-form muscle weakness and sensory impairment. The nerve conduction study performed four months after the onset revealed a decreased conduction velocity and conduction block suggesting demyelinated nerve. His neurological manifestations progressed subacutely, despite high-dose intravenous immunoglobulin therapy. Five months after the onset, a histopathological diagnosis of T-cell malignant lymphoma was made on a skin biopsy specimen from the facial rash. To summarise, the present case was a rare example of paraneoplastic autonomic neuropathy as the initial clinical feature in association with T-cell malignant lymphoma.
Full Text of this Article in Japanese PDF (638K)

(CLINICA NEUROL, 55: 29|32, 2015)
key words: paraneoplastic neurological syndrome, malignant lymphoma, autonomic failure, autonomic neuropathy, sensori-motor neuropathy

(Received: 27-Mar-14)