The treatment and management of alternating hemiplegia of childhood

Abstract

Alternating hemiplegia of childhood has many factors that make it difficult to manage. These include its rarity of about one case per million, the variability of the manifestations, with seven characteristic features, and the potential for disabilities and acute, often severe, episodes in a disease that is of uncertain cause and for which treatment evidence is sparse. An integrated multidisciplinary team and emergency availability are key medical requirements, as well as an educational setting that understands the variations in performance that occur. The mainstays of treatment have been flunarizine, antiepilepsy drugs for the 50% of patients with epilepsy, attempts to avoid trigger situations, and the rapid encouragement of sleep when attacks begin. The diagnostic and management predicament of child, parent, and paediatrician in complex rare disorders are well illustrated by this condition.

Hemiplejía alternante de la infancia: presentación de un caso y revisión de la bibliografía

“…La hemiplejía alternante tiene una incidencia de 1/1 000 000, 1 aunque, dados el poco conocimiento de esta enfermedad y la gran variabilidad de presentaciones clínicas posibles, podría ser motivo de una subestimación.…”

“…Su incidencia se estima en 1/1 000 000 y su etiología permanece desconocida. 1 La ausencia de biomarcadores específicos torna el diagnóstico muy difícil, que reside exclusivamente en el reconocimiento clínico evolutivo tras haberse descartado otras probables patologías neurológicas.…”

Child Neurology: Alternating hemiplegia of childhood

“…It has a reported incidence of 1 in 1,000,000 children but this may be an underestimate due to misdiagnosis and variability in the clinical presentation. 2 Since this disease occurs so infrequently and is variable in presentation, knowledge of the clinical characteristics, response to treatment, and prognosis is based on a few small cohort studies. 3,4 Clinical characteristics.…”

“…The most commonly used medication is flunarizine, a calcium channel blocker, in a dose of 5 to 20 mg daily. 2,4,6 This has been reported to decrease the frequency and severity of attacks, but not to stop them completely. Other proposed treatments have included beta blockers, an- ticonvulsants, and other medications such as methysergide, amantadine, aripiprazole, and haloperidol, but these are not frequently used.…”

Alternating Hemiplegia of Childhood: Report of Two Cases

“…1 The clinical features of AHC were first described more than three decades ago. Children with AHC often have a delay in diagnosis or are misdiagnosed.…”