Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

Abstract

Chorea and other movement disorders are rarely described as paraneoplastic. The aim of this study was to describe 13 patients with paraneoplastic chorea and dystonia collected by the members of the paraneoplastic neurological syndrome (PNS) EuroNetwork and to review 29 cases from the literature. We analyzed neurological symptoms, severity of the neurological syndrome, delay in neurological diagnosis, associated cancer, oncological and neurological treatments received, and outcome. Eleven (1.2%) out of 913 patients with PNS were identified in the EuroNetwork register. Two more patients not included in the register were added. The overall population consisted of 13 patients with a median age of 75 years (range 49-82 years). In most patients, the movement disorder was classical choreoathetosis with symmetric involvement of the trunk, neck, and limbs. A minority of patients presented unilateral chorea, dystonia, and orobuccal dyskinesia. Associated symptoms, as polyneuropathy, encephalitis, psychiatric disturbances, or visual defects, were often present. The movement disorder usually had a subacute course. The most frequently associated cancer was small cell lung cancer (SCLC). Lymphoma, bowel, or kidney cancers were also reported. CV2/CRMP5 was the most frequently associated antibody, followed by Hu. Hyperintense lesions of the basal ganglia on T2-weighted images were seldom observed. Response to cancer therapy was observed in a minority of patients, but survival was short (17 months). As in other neurological diseases, movement disorders should also be suspected as paraneoplastic when they develop subacutely in older patients (usually over 50) and often in the presence of other ancillary neurological symptoms.

Autoimmune chorea in adults

“…The cancers we encountered most frequently were small-cell carcinoma and various adenocarcinomas. Other reported cancer associations include renal cell carcinoma 17,18 and acute lymphoblastic leukemia. 19 Our findings in patients with paraneoplastic chorea accord with the findings described by a European collaborative group in 13 patients, with respect to male predominance, late age at onset, subacuity, early generalization and severity, substantial weight loss, and poor treatment responses.…”

“…19 Our findings in patients with paraneoplastic chorea accord with the findings described by a European collaborative group in 13 patients, with respect to male predominance, late age at onset, subacuity, early generalization and severity, substantial weight loss, and poor treatment responses. 17 We determined additionally that several of these characteristics pertain to paraneoplastic cases more than to idiopathic cases of chorea. We speculate that the greater weight loss in paraneoplastic cases reflects caloric expenditure from severe hyperkinetic movements, 20 cancer-related cytokine effects, 21 or both.…”

Paraneoplastic neurological syndromes in Hodgkin and non-Hodgkin lymphomas

“…However, 56 well-documented cases have been reported, 4 of them associated with NHL and 1 with HL. 57,58 The chorea was generalized and associated with peripheral neuropathy or encephalopathy in 2 patients. Brain MRI may demonstrate bilateral hyperintensity of caudate nuclei and putamen, as can be seen in paraneoplastic chorea associated with other tumors, mainly SCLC.…”

“…Neurological improvement may occur after immunotherapy or treatment of the lymphoma but a complete remission is rarely achieved. 57 …”

Paraneoplastic Manifestations of Lymphoproliferative Neoplasms

“…In the PNS EuroNetwork experience, only five cases of PC (four due to NHL and one due to HL) secondary to lymphomas have been reported. 53 PC is associated with CV2/Collapsin response mediator protein 5 (CRMP5) antibodies regardless of the tumor association. Finding these antibodies, when present, is a very helpful diagnostic clue, 54 as up to 50% of the patients may not have any onconeural antibodies.…”