Anti-CRMP5 paraneoplastic chorea: selective external capsule MRI involvement resolving after tumour resection
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LETTER TO THE EDITOR
Anti-CRMP5 paraneoplastic chorea: selective external capsule MRI involvement resolving after tumour resection Giovanna De Michele 1
&
Enrica Pisano 1 & Anna De Rosa 1 & Sirio Cocozza 2
Received: 21 July 2020 / Accepted: 9 October 2020 # Fondazione Società Italiana di Neurologia 2020
Dear Editor, Paraneoplastic chorea is a rare immune-mediated hyperkinetic disorder, caused by the remote effect of a neoplasm on the central nervous system [1]. The most commonly detected autoantibodies are anti-CRMP5 (collapsin response mediator protein 5), which are most often associated with small cell lung cancer and thymoma [2]. Treatment of the underlying neoplasm can improve the neurological syndrome. Brain MRI in paraneoplastic chorea can be normal or can reveal basal ganglia abnormalities, usually characterized by nonenhancing T2-weighted hyperintensities or less commonly by isolated T1-weighted hyperintensities [3]. Here, we report a case of anti-CRMP5 paraneoplastic chorea in which MRI revealed selective external capsule T2hyperintensities, without involvement of deep grey matter. Interestingly, after tumour surgery, both clinical and neuroradiological findings showed improvement. The patient was a 70-year-old woman who presented with a subacute onset of involuntary movements. Two months before, she was diagnosed with stage IIB lung cancer of the right lower lobe, being scheduled for surgery and chemotherapy. Her medical history was positive for hypertension and smoking, while she had never been exposed to dopamine receptor blocking agents. There was no relevant family history. On examination, she was slightly unsteady in walking and had mild generalized choreic movements, predominantly affecting her mouth and lower limbs. Routine blood tests, including thyroid hormones and antiphospholipid antibodies, were normal. Brain MRI demonstrated the presence of a mild * Giovanna De Michele [email protected] 1
Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University, Via Sergio Pansini 5, 80131 Naples, Italy
2
Department of Advanced Biomedical Sciences, Federico II University, Naples, Italy
bilateral WM T2-weighted hyperintensity at the level of the external capsule, with relative sparing of the internal capsule, extending to the corona radiata and hippocampal cortices, besides minor signs of pallidal mineralization and few hyperintense punctate foci in the cerebral white matter (WM), consistent with cerebral small vessel disease (Fig. 1). This signal change was not associated to significant mass effect, diffusion restriction, or signs of hemosiderin depositions. No cortical or deep grey matter (GM) atrophy was found. Serum onconeural antibody panel testing showed high positivity (+++) of antiCV2/CRMP5 IgG, confirming the clinical suspicion of paraneoplastic chorea. The patient was treated with tetrabenazine (up to 100 mg/ die), resulting in choreic movements decrease. One month later, she underwent right inferior lobectomy, and pathological examination revealed
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