Application of magnetic resonance spectroscopy in patients with alternating hemiplegia of childhood: findings on metabolic dysfunctions.

OBJECTIVE

Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental syndrome of uncertain etiology. Although the use of magnetic resonance spectroscopy (MRS) for the study of neurologic diseases has grown rapidly over the past decade, its use for AHC patients is quite new. This study was aimed at investigating changes of brain metabolites in patients with alternating hemiplegia of childhood (AHC) during the hemiplegic ictal phases and interictal phases by proton magnetic resonance spectroscopy ((1)H-MRS).

METHODS

(1)H-MRS was used in AHC patients during the hemiplegic ictal phases and interictal phases to evaluate functional activity in certain brain regions. A total of 10 unmedicated, healthy volunteers served as controls.

RESULTS

N-acetylaspartate (NAA)/Creatine(Cr) ratio of the frontal lobes, basal ganglia, and temporal lobes in contralateral hemiplegic hemisphere of AHC patients during the ictal phases was significantly lower than that in AHC patients during interictal phases and control subjects. Significantly increased choline-containing compounds (Cho)/Cr were obtained in corresponding regions.

CONCLUSIONS

These findings suggest neuronal metabolic dysfunctions in frontal lobes, temporal lobes and basal ganglia in AHC patients during ictal phases that perhaps are involved in the pathogenesis of AHC.