Objective: To report the case of a patient who presented with dystonia  secondary to an inflammatory lesion of the putamen

Background: Behçet’s Disease is a multisystemic vasculitis affecting nearly all organs. Lesions associated with Neuro-Behçet disease are mainly observed in the brainstem, the thalamus and basal ganglia(1).  Dystonia is a hyperkinetic movement disorder manifesting with constant muscle contraction responsible for abnormal, repetitive, contorting movements of the affected body parts. The most frequently encountered lesions responsible for secondary dystonia are tumors, ischemic strokes, angiomas and multiple sclerosis.

Method: Case report

Results: A 29 y.o patient, with a history of vitiligo and neuro-behçet disease treated with monthly IV cyclophosphamide, presented with a progressively worsening hyperkinetic movement disorder 8 months ago. He presented with a progressive onset of symptoms over 2 months with a painful, cramp-like, contortions evoking right hemibody dystonia. At clinical examination, a right hemiparesis rated at 3/5 was found, as well as a spastic hyperreflexia with crossed reflexes and oro-facial apraxia and aphasia. Cranial nerves examination was normal. Brain MRI showed a hyperintense signal in T2 FLAIR sequence on the left putamen and the caudate nucleus, with no contrast enhancement after gadolinium injection in T1 weighted sequences that are in relation with a non-progressive lesion. An EEG was also performed, and identified spike wave like generalized epileptic anomalies. The patient was treated according to the electrophysiological findings with carbamazepine, as it is also indicated in the treatment of dystonia according to the literature(2). He was also treated with oral baclofen therapy. Physical therapy was performed during his hospital stay and intrathecal baclofen pump placement was suggested to the patient.

Conclusion: Movement disorders associated with neuro-behçet disease are unfrequent(3). Lesions of the putamen and the Globus pallidus have been linked with dystonia. Dystonia secondary to parenchymal neurobehçet disease is scarce in the literature.

References: 1. Borhani-Haghighi A, Kardeh B, Banerjee S, Yadollahikhales G, Safari A, Sahraian MA, et al. Neuro-Behcet’s disease: An update on diagnosis, differential diagnoses, and treatment. Multiple Sclerosis and Related Disorders. avr 2020;39:101906.
2. Jankovic J. Medical treatment of dystonia: Dystonia Treatment. Mov Disord. 15 juin 2013;28(7):1001‑12.
3. GÜR ÖZMEN S, HANAĞASI H, GÜRVİT H, EMRE M, AKMAN DEMİR G. Neuro-Behçet’s Disease with Chorea. Noro Psikiyatr Ars. juin 2015;52(2):200‑1.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/hemibody-dystonia-secondary-to-neurobehcet-disease-a-case-report/