A 73-year-old man presented with a three-month history of muscle wasting, cramps and marked weight loss. Examination revealed severe global amyotrophy (Figure), fasciculations, reduced deep tendon reflexes and flaccid quadriparesis. Nerve conduction studies were unremarkable. Needle electromyography showed acute and chronic partial denervation in the cervical, thoracic and lumbosacral segments. A full-body CT scan disclosed a large mass in the anterior mediastinum, which showed a type B2 thymoma.
Findings in subacute motor neuronopathy. Severe muscle wasting in proximal (A) and distal muscle groups (B; black arrow). (C) Rarefaction of interference patterns in submaximal voluntary contraction, and fibrillation and positive sharp wave at rest. (D) Chest CT scan showed a large mass in the anterior mediastinum (white arrow).
Paraneoplastic neuropathies represent an expanding group of immune-mediated neuropathies associated with a known or unidentified neoplasm11. Graus F, Dalmau J. Paraneoplastic neuropathies. Curr Opin Neurol. 2013;26(5):489-95. https://doi.org/10.1097/WCO.0b013e328364c020
https://doi.org/10.1097/WCO.0b013e328364...
. Pure motor neuropathy is represented by subacute motor neuronopathy11. Graus F, Dalmau J. Paraneoplastic neuropathies. Curr Opin Neurol. 2013;26(5):489-95. https://doi.org/10.1097/WCO.0b013e328364c020
https://doi.org/10.1097/WCO.0b013e328364...
, commonly associated with Hodgkin’s and non-Hodgkin’s lymphoma11. Graus F, Dalmau J. Paraneoplastic neuropathies. Curr Opin Neurol. 2013;26(5):489-95. https://doi.org/10.1097/WCO.0b013e328364c020
https://doi.org/10.1097/WCO.0b013e328364...
,22. Stoll DB, Lublin F, Brodovsky H, Laucius JF, Patchefsky A, Cooper H. Association of subacute motor neuronopathy with thymoma. Cancer. 1984;54(4):770-2. https://doi.org/10.1002/1097-0142(1984)54:4<770::AID-CNCR2820540430>3.0.CO;2-E
https://doi.org/10.1002/1097-0142(1984)5...
and, rarely, with thymoma22. Stoll DB, Lublin F, Brodovsky H, Laucius JF, Patchefsky A, Cooper H. Association of subacute motor neuronopathy with thymoma. Cancer. 1984;54(4):770-2. https://doi.org/10.1002/1097-0142(1984)54:4<770::AID-CNCR2820540430>3.0.CO;2-E
https://doi.org/10.1002/1097-0142(1984)5...
.
References
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1Graus F, Dalmau J. Paraneoplastic neuropathies. Curr Opin Neurol. 2013;26(5):489-95. https://doi.org/10.1097/WCO.0b013e328364c020
» https://doi.org/10.1097/WCO.0b013e328364c020 -
2Stoll DB, Lublin F, Brodovsky H, Laucius JF, Patchefsky A, Cooper H. Association of subacute motor neuronopathy with thymoma. Cancer. 1984;54(4):770-2. https://doi.org/10.1002/1097-0142(1984)54:4<770::AID-CNCR2820540430>3.0.CO;2-E
» https://doi.org/10.1002/1097-0142(1984)54:4<770::AID-CNCR2820540430>3.0.CO;2-E
Publication Dates
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Publication in this collection
Jan 2018
History
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Received
22 Feb 2017 -
Accepted
11 Sept 2017