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Open Access Case report

Clinicoradiological changes of brain NK/T cell lymphoma manifesting pure akinesia: a case report

Susumu Ishihara1, Osamu Kano2, Ken Ikeda2*, Reiko Shimokawa3, Kiyokazu Kawabe2 and Yasuo Iwasaki2

Author Affiliations

1 Department of Hematology and Oncology, Toho University Omori Medical Center, Tokyo, Japan

2 Department of Neurology, Toho University Omori Medical Center, Tokyo, Japan

3 Division of Pathology, Saiseikai Yokohamashi Tobu Hospital, Yokohama, Japan

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BMC Neurology 2011, 11:137  doi:10.1186/1471-2377-11-137

Published: 2 November 2011

Abstract

Background

Pure akinesia (PA) is a distinct form of parkinsonism characterized by freezing phenomena. Little is known about brain tumor-associated PA. We highlight the clinicoradiological changes in a patient with PA and central nervous system (CNS) metastases of natural killer/T-cell lymphoma (NKTL).

Case presentation

A 68-year-old man with stage IVB extranodal NKTL developed a gait disturbance. Neurological examination of his gait revealed freezing, start hesitation, short step, forward flexion posture, festination and postural instability. Mild facial hypomimia and micrographia were observed. There was no rigidity or tremor in any of the four extremities. Brain magnetic resonance imaging (MRI) displayed T2-hyperintense lesions in the dorsal brainstem, cerebellum and periventricular white matter. Diffusion-weighted imaging (DWI) and the apparent diffusion coefficient (ADC) revealed hyperintensity in these regions. Cerebrospinal fluid cytology revealed CD56-positive cells on immunohistochemical staining. The patient's neurological deficits did not respond to L-dopa treatment and intrathecal administration of methotrexate (MTX). Two weeks later, he displayed confusion and generalized convulsions. T2-hyperintense lesions spread to the basal ganglia and the infratentorial regions. Gadolinium enhancement was observed in the cerebellum and frontal subcortex. DWI and the ADC revealed diffusion-restricted lesions in the middle cerebellar peduncles, left internal capsules and cerebral white matter. MTX pulse therapy and intrathecal administration of cytosine arabinoside and MTX were performed. Two months later, his ambulatory state was normalized. Brain MRI also revealed marked alleviation of the infratentorial and supratentorial lesions.

Conclusions

The clinicoradiological profile of our patient suggested that dorsal ponto-mesencephalic lesions could contribute to the pathogenesis of PA. Physicians should pay more attention to striking CNS seeding of metastatic NKTL. MTX pulse therapy had an excellent effect in improving serious symptoms and brain lesions in our patient.