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The clinical efficacy of lidocaine for convulsive status epilepticus in 53 convulsive episodes was examined in 37 children (17 males, 20 females). Mean age of patients receiving lidocaine was 3 years 7 months (SD 3y 5mo). Lidocaine administration achieved control of status epilepticus in 19 of 53 convulsive episodes (35.8%). Seizures ceased within 5 minutes of lidocaine administration in all 19 patients who were responsive to the drug. Regarding aetiology of status epilepticus and types of seizures, there was no statistical difference in effectiveness. Mild decrease of oxygen saturation, monitored by pulse oximetry, was observed in one patient, which improved by oxygenation using a mask. Lidocaine is a useful anticonvulsive agent; however, the response rate to lidocaine appears to be quite low, as less than half of the seizures were effectively controlled by lidocaine. Favourable properties of the drug include prompt responses, less alteration of consciousness, and fewer adverse effects, including less respiratory depression.

We report on a girl with early onset Huntington disease (HD). Her initial symptoms at 2 years of age included oral motor dysfunction and gait disturbance. Magnetic resonance imaging of the head revealed severe atrophy of both the vermis and the cerebellar cortex in addition to the common findings of basal ganglia including the caudate nuclei, putamen, and globus pallidus. Molecular analysis showed 160 CAG repeats in the HD gene. This mutation was inherited from her mother who was also affected, with a HD CAG expansion of 60 repeats. Cerebellar symptoms should be considered as a manifestation of early onset HD.

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Summary:Purpose: To elucidate the relation between alterations of regional cerebral blood flow (rCBF) by adrenocorticotropic hormone (ACTH) therapy and developmental outcomes of cryptogenic West syndrome.Methods: Quantitative measurement of rCBF, with autoradiography method using N-isopropyl-( 123 I) p-iodoamphetamine single photon emission computed tomography before and after ACTH therapy, was performed on 17 infants with cryptogenic West syndrome. Regions of interest for rCBF were placed bilaterally in the cerebellum, the thalamus, the caudate nucleus, and the frontal, temporal, and occipital cortices. RCBFs and the alteration ratios calculated from rCBFs before and after ACTH therapy were compared between two groups: the normal and delayed groups, which were divided by developmental outcome at 2 years old.Results: RCBFs before the therapy were not different statistically between the normal and delayed groups, and between those groups and the control group also. RCBFs after ACTH therapy of the occipital, thalamic and cerebellar regions were different between the normal and delayed groups (p < 0.05). Alteration ratios were different between the normal and delayed groups, in all of the regions but the frontal region (p < 0.05).Conclusions: This study showed the differences of rCBF response by ACTH therapy between the normal and delayed groups of cryptogenic West syndrome. The difference of rCBF alteration might be associated with maturation of the cerebrovascular system, or influence of corticotropin-releasing hormone regarding the brain-adrenal-axis.

SUMMARYPurpose: To elucidate the abnormality of interictal regional cerebral blood flow (rCBF) of West syndrome at the onset. Methods: Quantitative measurement of rCBF with an autoradiography method using N-isopropyl-( 123 I) p-iodoamphetamine single photon emission computed tomography (SPECT) was performed on 14 infants with cryptogenic West syndrome. Regions of interest (ROIs) for rCBF were placed automatically using an automated ROI analysis software (three-dimensional stereotactic ROI template), and were grouped into 12 segments: callosomarginal, precentral, central, parietal, angular, temporal, posterior cerebral, pericallosal, lenticular nucleus, thalamus, hippocampus, and cerebellum. We compared rCBF between the patients and seven age-matched infants with cryptogenic focal epilepsy as a control group. The patients were divided into two groups according to the duration from onset to SPECT, to compare rCBF. Results: Quantitative analysis revealed cerebral hypoperfusion in cryptogenic West syndrome with normal SPECT images under visual inspection. In bilateral central, posterior cerebral, pericallosal, lenticular nucleus, and hippocampus, and in the left parietal, temporal, and cerebellum, and in the right angular and thalamus segments there were statistical differences (p < 0.05). Compared with the duration from onset to SPECT, there were no significant differences of rCBF in all segments. Discussion: Broad cerebral hypoperfusion with posterior predominance involving the hippocampus and lenticular nucleus implies that even cryptogenic West syndrome has a widespread cerebral dysfunction at least transiently, which would correspond to clinical manifestations of hypsarrhythmia and epileptic spasms. Hippocampal hypoperfusion suggests the dysfunction of hippocampal circuitry in the brain adrenal axis, and may contribute to subsequent cognitive impairment of cryptogenic West syndrome.