• Journal of the Korean Child Neurology Society
• /
• v.26 no.4
• /
• pp.197-204
• /
• 2018

Purpose: West syndrome is a severe form of age-specific epilepsy that typically affects infants younger than 2 years of age with mitochondrial disease. We aimed to examine age-specific characteristics of the syndrome in these patients. Methods: We retrospectively analyzed 54 patients with West syndrome diagnosed with mitochondrial disease between March 2006 and March 2016. We compared treatment strategies and diagnostic and clinical variables between patients with early-onset (<6 months of age) and late-onset (${\geq}6$ months of age) seizures. Results: Seizure was the first symptom in 30 (90.9%) and 13 (65%) patients of the early-onset and late-onset groups, respectively (P=0.046). Delayed development was observed in 3 (9.1%) and 7 (35%) patients of the early-onset and late-onset groups, respectively (P=0.023). Lactate levels were normal in 17 patients (55%) of the early-onset group and 5 (25%) of the late-onset group (P=0.036), while initial brain magnetic resonance imaging (MRI) findings were normal in 23 (67.6%) and 8 (40%) patients of the early-onset and late-onset groups, respectively. Final MRI findings were abnormal in 32 patients (94.1%) of the early-onset group and 18 (90%) of the late-onset group (P=0.036). Although ketogenic diets reduced seizure frequency in both groups, the difference was not significant. Conclusion: There is no significant difference in epilepsy-related variables when patients are divided based on a cut-off age of 6 months. However, differences in the first symptom at onset and MRI findings were observed. Although lactate levels were not of significant diagnostic value in the early-onset group, they may be in the late-onset group.

• The Journal of the Society of Stroke on Korean Medicine
• /
• v.7 no.1
• /
• pp.11-16
• /
• 2006

Objectives : Transcranial Doppler (TCD) has been reported to be established as useful in detecting spasm after subarachnoid hemorrhage and to be probably useful in diagnosing stenosis or occlusion in intracranial arteries. In the detection of intracranial stenosis using TCD there have been reported some kinds of diagnostic criteria. This study was aimed to evaluate the accordance between TCD and magnetic resonance angiography (MRA) in detection of intracranial stenosis and to find out more accurate criteria for intracranial stenosis using TCD. Methods : Seventy-six stroke patients were evaluated by TCD and MRA. TCD criteria for middle cerebral artery (MCA) stenosis were used by 3 methods; ≥ 80cm/sec of mean velocity(Vm), ≥ 140 cm/sec of systolic velocity(Vs), and both. For stenosis of vertebral(VA) and basilar arteries(BA), the TCD criteria followed by 2 methods; ≥ 70 cm/sec of Vm and ≥ 100 cm/sec of Vs. The stenosis of intracranial artery in MRA followed by the interpretation of specialist in the department of radiology. The sensitivity, specificity, positive predictive value, negative predictive value, diagnostic accuracy and kappa agreement were calculated in each criteria of TCD compared with the result of MRA. Results : The sensitivity, specificity, positive predictive value, negative predictive value, diagnostic accuracy and kappa agreement using ≥ 80cm/sec of Vm for MCA stenosis were 55.6%, 81%, 34.5%, 91.0%, 77.1%, and 0.293, respectively. Using 140 cm/sec of Vs, those were 44.4%, 92.0%, 50.5%, 90.2%, 84.7%, 0.380, and using both criteria those were 44.4%, 95.0%, 61.5%, 90.5%, 87.3%, 0.445, respectively. Those using ≥ 70 cm/sec of Vm for VA and BA stenosis were 71.4%, 93.7%, 26.3%, 99.0%, 93.0%, 0.186 and using ≥ 100 cm/sec of Vs those were 71.4%, 97.3%, 45.5%, 99.1%, 96.5%, 0.539, respectively. Conclusion : These results suggested that for the diagnosis of MCA stenosis using TCD we should use the criteria of both ≥ 80cm/sec of Vm and 140 cm/sec of Vs, and for the VA and BA stenosis we adapt the criteria of ≥ 70 cm/sec of Vm.