Clinical Features, Disability and Socioeconomic Status of Patients With Muscular Dystrophy in Korea

BACKGROUND: Since 2001, the Ministry of Health and Welfare in Korea has designated muscular dystrophy (MD) to be a rare and intractable disease, and has ensured that patients with this condition obtain support from the National Health Insurance Corporation for their medical expenditure. However, the health-related and socioeconomic status of Korean patients with MD has yet to be established. METHODS: We selected 441 patients with MD who received medical services at 17 neuromuscular centers during 2005. The medical records of selected patients were analyzed, and the subtype of MD was classified by its clinical course and diagnostic tests. A total of 95 patients or their family members participated in this health-related and socioeconomic status survey. RESULTS: Medical record analysis showed similar clinical and diagnostic characteristic data to those published previously in other countries: male predominance, being young at onset, and muscular weakness of the extremities as a predominant symptom in most patients. The diagnostic tests for MD were based on laboratory and electrophysiological studies. The most frequent form of MD among our cohort was Duchenne/Becker muscular dystrophy (42%). Our survey revealed the effect of the patients' profound disability on their activities of daily living. One-half of the patients were dissatisfied with the medical expenditure support service that was made available to them, and most patients suffered from a financial burden. The most important medical services to be developed in the future are expansion of the public health service or development of a rehabilitation hospital. CONCLUSIONS: This is the first multicenter-based epidemiologic study on the health-related and socioeconomic status of patients with MD in Korea. The findings indicate that medical coverage and public health service are currently inadequate and hence should be expanded in the future.

The Brain-MR Studies in the Brain Death Patient: Report of 3 Cases: The Utility of the Anesthetic Mapleson Circuit-F System

Brain-MR studies are sensitive to intracranial ischemia and vascular flow. However, brain MR study for brain death is clinically limited because keeping the ventilation is difficult during study. In our hospital, three 'brain death patients' brain-MR studies were performed under the anesthetic Mapleson's circuit-F system. Three patients' clinical states were not changed after the studies. We confirmed that brain herniation, absent intracranial flow void, no intracranial contrast enhancement, poor gray/white matter differentiation, and prominent nasal enhancement findings. The value of brain-MR study for brain death may be possible.

An Unusual Case of Todd's Paralysis Mimicking Large Cerebral Infarction after Open Heart Surgery / 대한흉부외과학회지

Improved operative, anesthetic, and cardiopulmonary bypass (CPB) techniques have significantly reduced postoperative complications; however, neurologic disorders remain a serious complication after open heart surgery. Possible explanations for neurologic complications are microembolism from CPB, decreased cerebral perfusion pressure due to intraoperative hypotension and unexpected metabolic changes. Amomg these, seizure has low incidence and Todd`s paralysis after open heart surgery is extremely rare. Todd's paralysis is a complication of a seizure due to neuronal exhaustion mimicking large cerebral infarction after open heart surgery.

A Case of Status Epilepticus Characterized by Ictal Hemiplegia

A 39-year-old woman has had several episodes of transient right hemiplegia. On neurological examination during the ictal period she had alert consciousness with aphasia, head and eyeball deviation to the right side, and right hemiplegia. Brain MRI was normal. EEG-video monitoring of ictal period showed continuous ictal discharge in the midline frontocentral area coincided with right hemiplegia. After injection of diazepam, the ictal discharge and right hemiplegia disappeared. We report a case of status epilepticus characterized by ictal hemiplegia.

Combined Oculopalatine and Midbrain Tremor in Upper Limb Caused by a Pontine Hemorrhage

A 67-year-old man, with a history of pontine hemorrhage, was admitted because of ocular tremors. Also, tremors in the right upper limb and palates were detected. Oculopalatine tremors showed synchronous vertical oscillations and the limb showed midbrain tremor. Brain MRI revealed high signal intensities of both inferior olivary nuclei and right superior cerebellar peduncle. Electromyogram showed different tremor frequencies in the oculopalate and limb. Clinical, MRI and electromyographic findings indicate that the origins of oculopalatine and limb tremors are different.