Dec 5, 2018

An autopsy case of progressive multifocal leukoencephalopathy after rituximab therapy for malignant lymphoma

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Reiji MutoKoichi Ohshima

Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare fatal demyelinating disease of the central nervous system caused by reactivation of the JC virus (JCV), which is named after the initials of the patient from whom the virus was first isolated. JCV is highly prevalent worldwide, infects humans in early childhood, and the infection persists throughout the course of life in latent form. The present paper deals with the second autopsy case report of rituximab-associated PML in Japan. A 63-year-old woman who had undergone chemotherapy for non-Hodgkin lymphoma developed progressive dysarthria and cerebellar ataxia. Head magnetic resonance imaging (MRI) revealed small, scattered, hyperintense areas in the midbrain, pons and thalamus, and the patient was first diagnosed as having cerebral infarction. Follow-up MRI showed tendency toward cerebellar atrophy and multiple system atrophy cerebellar type was suggested, which we concluded must have coincidentally occurred. It was challenging to perform biopsy due to the location of the foci and the patient's condition. Twelve months later she died of aspiration pneumonia caused by the bulbar lesion. At autopsy, the histological examination suggested the presence of demyelinating foci ...Continue Reading

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Mentioned in this Paper

Cerebellar Diseases
Monoclonal antibodies, antineoplastic
Immune System
Multifocal
Lymphoma, Non-Hodgkin
Scattered
Histology Procedure
Leukoencephalopathy, Progressive Multifocal
Virus
Follow-up

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