Autopsy‐proven case of paraneoplastic lower motor neuron disease with sensorimotor neuropathy due to Waldenström's macroglobulinemia

We report a case of a male patient with a 19‐year history of monoclonal and later polyclonal gammopathy who subsequently developed tetraparesis, bulbar palsy, and respiratory failure. Autopsy findings showed degeneration of the hypoglossal nuclei, prominent neuronal loss and atrophy in the anterior...

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Bibliographic Details
Published in:Neuropathology 2018-10, Vol.38 (5), p.568-573
Main Authors: Suzuki, Yasuhiro, Aizawa, Hitoshi, Sakashita, Kento, Kishi, Hideaki, Nomura, Kenta, Yoshida, Kosuke, Aburakawa, Yoko, Kuroda, Kenji, Murakami, Chisato, Kakinoki, Yasutaka, Kimura, Takashi
Format: Article
Language:English
Subjects:
anti‐CD20 antibody
Astrocytes
Atrophy
Autopsy
Case reports
Degeneration
Dorsal roots
Gammopathy
Gliosis
Humans
Inclusion bodies
Inflammation
Lymphocytes B
Macroglobulinemia
Male
Middle Aged
Motor Neuron Disease - etiology
Motor Neuron Disease - pathology
Motor neuron diseases
Motor neurone disease
Neuropathy
Paralysis
paraneoplastic lower motor neuron disease
Paraneoplastic Syndromes, Nervous System - etiology
Paraneoplastic Syndromes, Nervous System - pathology
Peripheral Nervous System Diseases - etiology
Peripheral Nervous System Diseases - pathology
Plasma cells
Sensorimotor system
Spinal cord
TDP‐43
Ventral roots
Waldenstrom Macroglobulinemia - complications
Waldenstrom Macroglobulinemia - pathology
Waldenström's macroglobulinemia
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Summary:We report a case of a male patient with a 19‐year history of monoclonal and later polyclonal gammopathy who subsequently developed tetraparesis, bulbar palsy, and respiratory failure. Autopsy findings showed degeneration of the hypoglossal nuclei, prominent neuronal loss and atrophy in the anterior horn of the whole spinal cord despite the presence of mild astrocytosis, degeneration of the gracilis on one side, and infiltration of inflammatory cells, which included B cells and plasma cells in the anterior and posterior roots of the lumbar spinal cord, iliopsoas muscle, and perivascular area of the cervical cord. On immunostaining, cytoplasmic inclusions of phosphorylated transactivation response DNA‐binding protein of 43 kDa were observed in the motor neurons and astrocytes of the hypoglossal nuclei and whole spinal cord. The final diagnosis was paraneoplastic lower motor neuron disease with sensorimotor neuropathy due to Waldenström's macroglobulinemia.
ISSN:0919-6544
1440-1789
DOI:10.1111/neup.12506