Paraneoplastic subacute lower motor neuron syndrome associated with solid cancer.
We retrospectively analyzed three patients with pure motor neuronopathy followed for more than four years in our center. The patients presented a rapidly progressive lower motor neuron syndrome (LMNS) over the course of a few weeks leading to a severe functional impairment. The neurological symptoms preceded the diagnosis of a breast adenocarcinoma and a thymoma in the first two patients, one of them with anti-CV2/CRMP5 antibodies. Cancer was not detected in the third patient who had circulating anti-Hu antibodies. A final diagnosis of paraneoplastic syndrome was made after investigations for alternative causes of lower motor neuron syndrome. Early diagnosis, combined treatment of the underlying cancer, and immunomodulatory treatment led to neurological improvement of the disease in two out of the three cases in which the cancer was diagnosed. Cases of subacute LMNS with rapid progression may occur as an expression of a paraneoplastic neurological syndrome. Identification of these syndromes is important, as the treatment of underlying malignancy along with immunomodulatory treatment may result in a favorable long-term outcome of these potentially fatal diseases.
Reference Center for Neuromuscular Diseases and ALS, Hôpital de la Timone, Marseille, France. Electronic address: annie.verschueren@ap-hm.fr.
Annie
A
Verschueren
Reference Center for Neuromuscular Diseases and ALS, Hôpital de la Timone, Marseille, France.
Julien
J
Gallard
Laboratoire d'immunologie, Hôpital de la Conception, Marseille, France.
José
J
Boucraut
French Reference Center on Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292, Université Claude Bernard Lyon 1, F-69372 Lyon, France.
Jerome
J
Honnorat
Reference Center for Neuromuscular Diseases and ALS, Hôpital de la Timone, Marseille, France.
Jean
J
Pouget
Reference Center for Neuromuscular Diseases and ALS, Hôpital de la Timone, Marseille, France.
Shahram
S
Attarian
D000368:Aged; D000369:Aged, 80 and over; D001943:Breast Neoplasms / Q000150:complications*; D005260:Female; D006801:Humans; D056747:Immunomodulation; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D020361:Paraneoplastic Syndromes, Nervous System / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D013953:Thymus Neoplasms / Q000150:complications*
Lower motor neuron syndrome; Motor neuronopathy; Paraneoplastic; Solid cancer; Thymoma