Symptomatic paroxysmal kinesigenic choreoathetosis as primary manifestation of multiple sclerosis
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Paroxysmal kinesigenic choreoathetosis (PKC) is a rare movement disorder characterized by episodes of uni- or bilateral choreoathetosis precipitated by sudden or rapid movements, startle or hyperventilation. It can be categorized as primary, e.g. idiopathic or hereditary, and secondary PKC . Amongst the more frequent causes of the latter is multiple sclerosis (MS) . Here we describe two patients presenting with symptomatic PKC as a clinically isolated syndrome.
Patient 1, a 34-year old woman, presented with a two-week history of episodes of right-sided numbness, tingling and spasms of her hand and leg occurring 2–15 times a day for 60 s per episode. These episodes were triggered by sudden movements, e.g. turning her head or moving in bed. Patient 2, a 27-year old woman, experienced multiple (>50) daily episodes of painful spasms of her right arm and leg as well as “pressed” speech for a period of about 1 min triggered by repetitive movements or hyperventilation. In...
KeywordsMultiple Sclerosis Dystonia Neurofibromatosis Muscle Soreness Spinal Cord Lesion
Dr. Zittel is supported by the Wegener Stiftung and has received research support by Merz Pharmaceuticals. Dr. Gerloff was supported by advisory boards of Boehringer Ingelheim and Silk Road Medical, and by grants from the DFG (Ge 844/2-1, Ge 844/4-1), the Bundesministerium für Bildung und Forschung (BMBF competence net stroke, BMBF Multicare), and UKE intramural grants. Dr. Münchau is supported by the Deutsche Forschungsgemeinschaft (MU1692/2-2). He received research support and honoraria for lectures by Pharm Allergan, Ipsen, Merz Pharmaceuticals. He is supported by the Dystonia Medical Research Foundation (USA), the Tourette Syndrome Association (Germany) and N.E.MO., charity supporting the research of paediatric movement disorders.
Conflict of interest
Segment 1 (Patient 1) When running on the spot, the patient experiences bradykinesia of right hand and arm after 30 s with associated dystonia and slight chorea. She describes a tingling sensation of right arm and leg. A similar but more intense right sided dystonia and chorea can be elicited by 90 s of hyperventilation and subsides 20 s later. During the attacks symptoms showed a crescendo-decrescendo dynamic. Segment 2 (Patient 2) While running on the spot for 30 s, generalized predominantly right-sided chorea, appendicular and axial dystonia develop accompanied by a “cramp-like” sensation and “pressed” dystonic dysarthria. Symptoms resolved after 60 s (MPG 35100 kb)
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