A Case of Moyamoya Disease with Neurofibromatosis Type I / 소아과

Neurofibromatosis type I is an autosomal dominant disorder with varied manifestations in bone, soft tissue, the nervous system and skin. This is characterized by cafe-au-lait spots, neurofibromas, Lisch nodules, optic glioma, bony displasia, and intertriginous freckling. One of the more serious aspect of the disease relates to the arterial involvement. Vascular changes in neurofibromatosis may occur in any arterial tree from the proximal aorta to the small arteries but these changes are most common in the renal arteries, aorta, celiac arteries and mesenteric arteries. Of the many complications observed in neurofibromatosis type I, cerebrovascular lesions may be the least appreciated. About 40 cases of neurofibromatosis type I associated with occlusive cerebrovascular disorders have been reported in the literature, but MRI and angiographic findings typical of moyamoya disease are rarely described. We experienced a case of moyamoya disease associated with neurofibromatosis type I in a 3-year-old girl who of complained gait disturbance and paraparesis and showed findings typical of moyamoya disease on MRI and carotid angiogram.

The Incidence and Risk Factors of Posttraumatic Seizure in Children / 소아과

PURPOSE: The goal of our study was to identify the incidence and clinical, neurophysiological and neuroradiological variables with predictive value for posttraumatic seizure(PTS). METHODS: The medical records of 625 children with head traumas under 15-year-old who were admitted to the Wonju Christian Hospital, from January, 1993 to January, 2002 were retrospectively reviewed. Among them, 472 patients were included in this study. The PTS patients were divided into early PTS, in whom seizure occurred within one week after head trauma and late PTS, in whom seizure occurred beyond the first week after head trauma. The injuries were classified into mild(Glasgow Coma Scale, GCS 13 to 15 or no brain CT abnormality and a brief hospital stay), moderate(GCS, 9 to 12, or a GCS above 12 and longer than 48-hour hospital stay, or brain CT abnormalites) and severe(GCS, below 9). The variables such as age, sex, duration of unconsciousness, GCS, brain CT scan finding, initial neurologic finding and anticonvulsant therapy were analyzed for risk factors of PTS. RESULTS: Early PTS was developed in 41(8.7%) patients, 35(77.8%) patients among them had a seizure within 24 hours after head trauma. Late PTS was developed in 17(3.6%) patients. The frequency and duration of PTS were not correlated with the latency of PTS. And there was correlation between the frequency and duration of PTS. The 82.9% of early PTS and the 76.5% of late PTS were generalized tonic-clonic seizure. There was a significant difference in the incidence of PTS by severity of head trauma. The incidence of PTS after mild head trauma(5.8%) was lower than after severe head trauma(29.9%). The risk factors of early PTS were unilateral hemorrhage, neurologic finding(hemiparesis and coma), GCS(under 12 score), and diffuse contusion. And the late PTS were the same as early PTS, except for diffuse contusion, and age factor(under 2 years was also significant). CONCLUSION: The incidence and risk factors of PTS were correlated with severity of head trauma.