The Clinical Manifestations and Prognosis of Acute Disseminated Encephalomyelitis / 대한소아신경학회지

PURPOSE: Acute disseminated encephalomyelitis is an acute demyelinating autoimmune inflammatory disease of the central nervous system, which develops after infection or vaccination. The diagnosis is dependent on its clinical features and the brain MRI finding. Severely damaging white matter, acute disseminated encephalomyelitis which can lead to marked neurologic dysfunctions, may be fatal, and produce a permanent residual static disability or it may fully recover. We performed this study to investigate the clinical manifestations and prognosis of acute disseminated encephalomyelitis. METHODS: Ten cases (5 males and 5 females) of acute disseminated encephalomyelitis, who were diagnosed in the Department of Pediatrics at Yonsei University from January 1990 to December 1998, were reviewed retrospectively. RESULTS: The age at onset of symptoms was between 2 years 8 months and 13 years 8 months, and the time to the onset of symptoms was between 1 day and 7 days. The proceding events were nonspecific upper respiratory infections in 6 cases, and the other 4 cases had no proceeding events. The initial symptoms were altered consciousness, hemiparesis, seizure, fever, headache, and vomiting. The findings of cerebrospinal fluid were normal in 7 cases and the increase of protein and pleocytosis in 3 cases. Brain MRI showed multifocal high signal intensity lesions on T2-weighted image mainly in cerebral white matter, basal ganglia, and less frequently in the brain stem and cortex. Follow-up brain MRI was taken in 7 cases, and in all 7 cases brain MRI demonstrated partial to complete resolution of the lesions. In 8 cases, EEG was performed, and all 8 cases showed generalized or focal slow waves. All patients were treated with IV globulin and dexamethasone. The durations of the treatment were 4 or 5 days for IV globulin, and 8 days to 20 days for dexamethasone. The treatment was started within the first to third day of their admission, and the symptoms were improved within the second to the thirtieth day after the treatment started. All patients were followed-up for more than 2 months and up to 5 years 8 months. Among 10 cases, 9 of them were fully recovered, and only 1 case had left hemiparesis. CONCLUSION: Acute disseminated encephalomyelitis shows various clinical manifestations depending on its involvement with brain lesions, such as altered mental state, hemiparesis, and seizure. And IV globulin and dexamethasone were very effective in treating acute disseminated encephalomyelitis. In this study, the prognosis of acute disseminated encephalomyelitis did not correlate to its initial symptoms, CSF findings, EEG findings, MRI findings, or to the duration of its treatment. In most cases, the symptoms were fully reversible; however, in some cases, there remained permanent residual neurologic consequences.

Clinical Study on Carbon Monoxide Intoxication in Children

Carbon monoxide intoxication has long been one of the most serious public health problems in Korea. This is mainly due to the wide use of anthracite coal briquettes as domestic fuel for cooking and under-the floor heating. One hundred and seven cases of CO intoxicated children hospitalized at Yonsei Medical center from January 1970 to December 1986 have been investigated clinically. The sex ratio was 1.3:1 (male 60 cases, female 47 cases) with the peak incidence occuring in patients between 12 and 14 years of age (28%). The most common symptoms were vomiting convulsions and headache; and the most frequent signs were altered mental state, increased deep tendon reflex and a positive Babinski sign. The outcome of patients was as follows: 4 cases (3.7%) expired, 77 cases (72.0%) recovered without neurologic sequelae and 26 cases (24.3%) survived with neurologic sequelae. The neurologic sequelae included persistent convulsions (7 cases), cortical blindness (3 cases), peripheral neuropathy (2 cases) and delayed neurologic sequelae (11 cases). Neurologic sequelae occurred most frequently in comatose patients (45.5%) and least often in mentally alert patients (6.1%), more frequently m patients exposed to CO gas for more than 8 hours than in those exposed for less than 8 hours, and in patients who did not receive hyperbaric oxyen therapy(29.4%) than in those who did(19.6%). Delayed neurologic sequelae were mental retardation (72.7%), epilepsy (36.4%), mutism (18.2%) etc. The lucid interval in 11 cases of delayed neurologic sequelae ranged from 2 to 20 days. The results of this study suggest that every patient exposed to CO gas should receive prompt and efficient oxygenation including hyperbaric oxygen therapy and that expeditious reduction of cerebral edema maybe of value. The importance of providing follow-up facilities in anticipation of a relapse of the delayed neurologic sequelae has been established.

A Case of Acardi's Syndrome

No abstract available.

A Clinical Observation of Congenital Diaphragmatic Hernia

Diaphragnatic hernia is one of the surgical emergencies of the childhood because of the life-threatening cardiorespiratory embarrassment frequently associated with it. This congenital condition is characterized by varying degrees of protrusion of the abdominal viscera into the thoracic cavity through an abnormal opening in the diaphragm this opening results from a defect in the complex embryologic developement of the diaphragm between the thoracic and abdominal cavities. We expenienced 16 cases of congenital diaphragmatic hernia which were diagnosed at Severance Hospital from May 1964 to April 1977, and obtained the following results. 1) In 16 cases, male were ten and female were six. They were eleven cases of bochdalek hernia, two cases of diaphragmntic eventration, one case of hiatal and two undetermined. 2) Onset of symptoms were within 24 hours in 7 cases and in the rest cases symptoms were occured up to 24 months. 3) Most common symptoms were dyspnea and cynosis. 4) Most common signs were decreased breathing sound on affected lung, PMI shifting, scaphoid abdomen and subcostal retraction. 5) Combined anomalies were band adhesion of intestinal loop, hypoplasia of left lung etc. 6) Small intestine were more frequently herniated as well as large intestine. 7) mortality rate between abdominal procedure and thoracic procedure was equal. 8) Cases whose symptoms were occured within 24 hours were 7, 5 of these 7 were expired, Expired cases were associated with marked respiratory difficulties and pulmonary hypoplasia.