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[Alternating hemiplegia. Partial effectiveness of treatment with flunarizine]
M J Alvarez Gómez1, J Narbona García, P Barona Zamora
1Departamento de Pediatría y Neurología, Clínica Universitaria de Navarra, Facultad de Medicina, Pamplona.
Insights
Alternating hemiplegia, a rare neurological disorder, can be managed with Flunarizine. Early diagnosis and treatment with this calcium channel blocker showed a 30% reduction in attacks and halted cognitive decline.
Area of Science:
- Neurology
- Pediatric Neurology
Background:
- Alternating hemiplegia is a rare, severe neurological disorder characterized by recurrent, transient episodes of hemiplegia affecting alternating sides of the body.
- The exact pathophysiology remains unclear, but it is often associated with mutations in the ATP1A3 gene and presents with diverse neurological symptoms.
Observation:
- A case of alternating hemiplegia in a young girl presenting at three months of age with seizure-like episodes, followed by alternating hemiplegic attacks.
- The patient exhibited significant autonomic disturbances, oculomotor abnormalities, acquired mental retardation, and residual focal neurological deficits.
Findings:
- Treatment with Flunarizine, a calcium-entry blocker, resulted in a 30% reduction in the frequency and severity of hemiplegic attacks after one year.
- The progression of acquired mental retardation was halted during the treatment period.
Implications:
- This case highlights the importance of early diagnosis and intervention in alternating hemiplegia.
- Flunarizine demonstrates partial therapeutic efficacy, suggesting a role for calcium channel modulation in managing this condition and its associated cognitive decline.
Abstract:
A case of alternating hemiplegia in a young girl is presented. The partial benefits of treatment with a calcium antagonist Flunarizine in this patient and in those reported in literature are reviewed. The onset of the disease in this girl was at three months of age with several episodes that were diagnosed as seizures; afterwards she presented, besides, repeated attacks of hemiplegia involving both sides of her body in an alternating way each time with daily frequency and hours of days of duration accompanied of bad mood and irritability as well as autonomic disturbances, oculomotor abnormalities, acquired mental retardation and residual focal neurological abnormalities. After one year of treatment with a calcium-entry blocker: Flunarizine, there was a 30% reduction in the attacks frequency as well as in its severity and stop of the progression of mental retardation. So we report the consequence of precocious diagnosis and treatment of this not well known entity whose clinical signs resemble paroxistic vascular anomalies in the brainstem territory.