Movement disorders in patients with alternating hemiplegia: "Soft" and "stiff" at the same time.
(2020) Neurology — Vol. 94, n° 13, p. e1378-e1385 (2020)
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- (en) OBJECTIVE: To assess nonparoxysmal movement disorders in ATP1A3 mutation-positive patients with alternating hemiplegia of childhood (AHC). METHODS: Twenty-eight patients underwent neurologic examination with particular focus on movement phenomenology by a specialist in movement disorders. Video recordings were reviewed by another movement disorders specialist and data were correlated with patients' characteristics. RESULTS: Ten patients were diagnosed with chorea, 16 with dystonia (nonparoxysmal), 4 with myoclonus, and 2 with ataxia. Nine patients had more than one movement disorder and 8 patients had none. The degree of movement disorder was moderate to severe in 12/28 patients. At inclusion, dystonic patients (n = 16) were older (p = 0.007) than nondystonic patients. Moreover, patients (n = 18) with dystonia or chorea, or both, had earlier disease onset (p = 0.042) and more severe neurologic impairment (p = 0.012), but this did not correlate with genotype. All patients presented with hypotonia, which was characterized as moderate or severe in 16/28. Patients with dystonia or chorea (n = 18) had more pronounced hypotonia (p = 0.011). Bradykinesia (n = 16) was associated with an early age at assessment (p < 0.01). Significant dysarthria was diagnosed in 11/25 cases. A history of acute neurologic deterioration and further regression of motor function, typically after a stressful event, was reported in 7 patients. CONCLUSIONS: Despite the relatively limited number of patients and the cross-sectional nature of the study, this detailed categorization of movement disorders in patients with AHC offers valuable insight into their precise characterization. Further longitudinal studies on this topic are needed.
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Panagiotakaki, E., Doummar, D., Nogue, E., Nagot, N., Lesca, G., Riant, F., Nicole, S., Delaygue, C., Barthez, M. A., Nassogne, M.-C., Dusser, A., Vallée, L., Billette, T., Bourgeois, M., Ioos, C., Gitiaux, C., Laroche, C., Milh, M., Portes, V. D., et al. (2020). Movement disorders in patients with alternating hemiplegia: “Soft” and “stiff” at the same time. Neurology, 94(13), e1378-e1385. https://doi.org/10.1212/WNL.0000000000009175 (Original work published 2020)