A Case of Familial Moyamoya Disease: A Case in Brother and Sister / 대한소아신경학회지

Moyamoya disease is a progressive and occlusive disorder of the cerebral vasculature with particular involvements of the circle of Willis and the arteries that feed it. It occurs commonly in Japan and Korea, but less frequently in the Western countries. The etiology of moyamoya disease is still unclear, but frequent familial occurrence suggests that some genetic factors may be important in its etiology. Approximately 7-10% of moyamoya disease are familial. We experienced 2 siblings with moyamoya disease, and report the case with a review of previously published cases of moyamoya disease within a family.

Clinical Analysis of Spinal Cord Tumor

OBJECTIVE: This study was undertaken to evaluate operative results and prognosis according to preoperative clinical status and histopathological finding of spinal cord tumor. METHODS: We analyzed of clinical feature, tumor location, histopathologic finding, operative results and prognosis in 55 patients with spinal cord tumor during last 10 years. RESULTS: 1) The incidence of spinal cord tumors varies with the age of affected patients who are 2 to 75 years of age. Peak incidences were in the 5th and 7th decade of life, and the ratio of male to female was 1.2:1. 2) The most common histopathologic type was neurinoma(41.9%). 3) The tumors were located most frequently in the thoracic area(22 cases, 40.0%) and in the intradural extramedullary space(30 cases, 54.5%). 4) The most common initial clinical feature was pain in 20 cases(36.4%). For neurologic status on admission, 30 cases(54.5%) showed motor disturbance. 5) In radiologic studies, there were abnormal finding in 21 cases from plain X-rays among 37 cases. The entire 20 cases in when myelography was done showed subarachnoid blockade, either complete or incomplete. The magnetic resonance imaging, regard as the most accurate diagnostic method, revealed the exact location of the tumor and the relationship of the tumor with the adjacent anatomical structure. 6) The total removal was possible in 36 cases(65.5%), subtotal removal in 17 cases(30.9%) and biopsy in 2 cases (3.6%). Nineteen cases(90.5%) among 21 cases with preoperative radiculopathy group showed recovery or improvement, where as only 11 cases(36.7%) among 30 cases with preoperative motor weakness group showed recovery or improvement, with statistically significant difference(p<0.01). Pathologically, 26 cases(83.9%) among 31 cases of neurinoma and meningioma showed postoperative recovery or improved, but only 1 case(6.3%) among 16 cases of metastatic tumor, astrocytoma and ependymoma recovered. Postoperative complication noted in 5 cases(9.1%), and were noted postoperative hematoma, pneumonia, pulmonary edema and spinal cord infarction. CONCLUSION: Preoperative neurologic status and histopathologic finding are considered important factors of Postoperative outcome in patients with spinal cord tumor.

Prognosis and Complications of Depressed Skull Fracture

Complications such as infection, seizure, or intracranial hemorrhage are relatively common after depressed skull fractures. However, indications, methods, and efficacy of the surgical treatment are controversial. This study includes 113 patients with depressed skull fractures who were treated at Soonchunhyang University Chonan Hospital between 1989 and 1992. Methods of treatment, complications, and the prognosis were retrospectively collected and the prognostic factors were analysed. Depressed skull fracture was common below the age of 20 years(38%). Male to female ration was 5.3:1. Traffic injury was the most common of depressed skull fracture(68.1%). Glasgow coma score(GCS) on admission was 13-15 in 52%, 9-12 in 18.6%, and closed in 32%. Intracranial injury was accompanied in 43%. The frontal area was the most common site of depression(53%), and the parietal region was the next(28%). In 65% of open fractures and 28% of closed ones, the lesions were surgically corrected. Cranitomy with primary reconstruction was the most common operative procedure. The mean hospital stay was 26 days in surgically treated patients, and it was 17 days in conservatively managed patients. Prophylactic anticonvulsants were administered in 43%. Seizures occured in 6.2%, and infections were encountered in 3.5%. The outcome was favorable(good recovery and moderate disability) in 80% and the mortality rate was 16%. The outcome was better when the GCS on admission was high(P<0.005), the pupillary response was normal(P<0.005), and the lesion was treated by surgery(P<0.005). Seizures were more common when the GCS was low(P<0.005). Although infections were developed in surgically treated patients only, this difference lacked a statistical significance.

Predicting Prognosis using Neurotrauma Motor Index in Spinal Cord Injury

We present a series of 97 patients with spinal cord injury. Those patients were treated at Soonchunhyan University Chonan Hospital during six-year-period(January 1986-December 1992), and followed up for at least 3 months. They were divide into four groups ; complete-surgical, complete-conservative, partial-surgical, and partial-conservative. Male to female ratio was 3.6:1, and the peak age was the fifth decade. Passenger's traffic accident(46%) was the most common cause of injury and fall/slip(39%) followed next. Level of spinal injury was cervical in 51%, thoracic in 29%, lumbar in 13%, and others in 7%. Type of injury was fracture-dislocation in 31%, dislocation in 19%, compression fracture in 19%, burst fracture in 18%, cord injury without bony lesion in 7%, and others in 7%. Cord injury was complete in 36 patients(37%) and partial in 61 patients(63%). Surgery was performed in 20 patients with complete cord injury and 34 patients with partial injury. Majority of operations were performed for spinal stability, and posterior instrumentation was the most commonly used operative method. The mean neurotrauma motor index on admission was 44.1 in the complete-surgical group, 39.5 in complete-conservative group, 70.1 in partial-surgical group, and 63.2 in partial-conservative group. It improved to 52.5, 50.3, 90.4, 80.3 at 3 months, and 53.4, 51.7, 91.8, 82.0 at final examination(mean follow-up period was 182.2 days), respectively. The mean values of the final index were higher in the surgically treated groups than the conservatively treated groups, but these differences were not statistically significant(student t-test p>0.1) The mean recovery rate was 14.2% in the complete-surgical group, 17.7% in complete-conservative group, 66.1% in partial-surgical group, and 46.1% in partial-conservative group at 3 months. It improved to 16.6%, 19.3%, 70.0%, and 48.7% at final examination, respectively. The mean recovery rates were higher in the partially injured groups than the completely injured groups(student t-test, p<0.01), regardless the methods of treatment. The final neurotrauma motor index was significantly(regression analysis, p<0.01) related to the initial index except the partial-surgical group. Neurotrauma motor index was a useful method for assessing the injury severity, comparing the recovery rate and predicting prognosis.

Plasma Aldosterone Levels in Acute Stage of Cerebral Stroke

We measured plasma aldosterone levels to determine whether or not the values obtained reflect the severity of cerebral stroke. All 36 patients with cerebral stroke studied were diagnosed with CT & MRI, and they were classified into 3 groups according to the Glasgow coma scale(GCS)-mild(GCS 14-15), moderate(GCS 9-13) and severe(GCS<8) group, and control of 10 patients, who were scheduled for elective lumbar laminectomy. Blood samples were taken on admission, the 3rd, and 7th hospital day. Aldosterone was measured by radioimmunoassay(normal values : below 150pg/ml). The results obtained were as follows ; 1) Aldosterone levels in control group(n=10) were 128pg/ml(SD+/-21) on admission, 109pg/ml(SD+/-4) on the 3rd day and 102pg/ml(SD+/-18) on the 7th day after admission. 2) Aldosterone levels in mild group(n=12) were 137.0pg/ml(SD+/-137.4) on admission, 107.5pg/ml(SD+/-89.9) on the 3rd day and 64.4pg/ml(SD+/-65.4) on the 7th day after admission. 3) Aldosterone levels in moderate group(n=6) were 195.1pg/ml(SD+/-159.1) on admission, 153.1pg/ml(SD+/-116.3) on the 3rd day and 85.7pg/ml(SD+/-53.0) on the 7th day after admission. 4) Aldosterone levels in severe group(n=18) were 230.3pg/ml(SD+/-107.3) on admission, 219.7pg/ml(SD+/-135.8) on the 3rd day and 267pg/ml(SD+/-233) on the 7th day after admission. The results of this study indicate that aldosterone levels on admission, the 3rd, and 7th day were significantly higher in severe group than in control group and aldosterone may be useful as biochemical markers in the acute stage of cerebral stroke, especially in severe group.

A Case of Huge Calcified Chronic Subdural Hematoma in the Elderly

After von Rokitansky's description in 1984 of a calcified chronic subdural hematoma, the number of such lesions has reached so far a total of 108. The majority of patients reported have been children and young adults. On review of the literature, elderly patients reported over the age of sixty five years are only 5, so far as we know. A 68-year-old Korean male with a calcified chronic subdural hematoma is reported. He was admitted to our department because of a status epilepticus. During Korean war (1950), abut 37 years before admission, he had been suffered from a blunt head injury, but mistreated then, and afterwards th had several episodes of convulsion. Neurological examination on admission revealed only a drowsy consciousness and mild papilledema on the left. Plain skull X-ray films and brain CT scan demonstrated a left sided biconvex type of intracranial calcification. A left frentoparietal craniotomy was performed. After opening the dura mater, the thickened outer membrane, the subdural hematoma and the thin bone like plate were removed thoroughly. Content of the subdural hematoma was brown muddy substance. In the eight months following the operation, the generalized seizure did not appear. Postoperative CT scan demonstrated complete re-expansion of the brain parenchyme.