Hereditary progressive dystonia develope in the first decade of ?life and is characterized by marked diurnal fluctuation of symptoms(dystonic postures and movements), i.e.symptoms aggrevated towards evening and alleviated after sleep. These symptoms and signs responded dramatically to levodopa. With the high incidence of familial cases this disorder is considers to be hereditary, but inheritance is not clear yet. Segawa regarded this disorder as a dystonia different from Parkinson's disease and other types of dystonic movement disorders, thereafter it has been reported under name of hereditary progressive dystonia with marked diurnal fluctuation.
Dystonia* ; Incidence ; Levodopa ; Movement Disorders ; Parkinson Disease ; Posture ; Wills

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Pure sensory stroke (PSS), first described by Fisher in 1965, is a clinical condition characterized by numbness and paresthesia of the face, arm and trunk on one side, in absence of other neurologic deficit. PSS could arise anywhere along the sensory system from the cerebral cortex to the medulla. The authors experienced 5 patients with PSS: one patient had a hemorrhage on the thalamocortical pathway including the internal capsule and the corona radiata. Two another had thalarnic lesions. The fourth had a pontine hemorrhage with perioral onionpeel distributed face sensor- involvement. And the last had a pontine lacune involving crossed trigeminothalamic tract and lateral spinothalamic tract.
Arm ; Cerebral Cortex ; Hemorrhage ; Humans ; Hypesthesia ; Internal Capsule ; Neurologic Manifestations ; Paresthesia ; Spinothalamic Tracts ; Stroke*

BACKGROUND: Congenital heart disease may be complicated by pulmonary hypertension. We assessed whether inhaled nitric oxide would produce selective pulmonary vasodilation in pediatric patients with congenital heart disease and pulmonary hypertension. METHODS: Inhaled low dose (10 20 ppm) nitric oxide was administrated in patients who were at risk of pulmonary hypertension after operations for congenital heart disease. To identify the nitric oxide effects, we evaluated hemodynamic and ABGA data before (T0) and after (T1) inhaled nitric oxide and just before (T2) decreasing concentration of inhaled nitric oxide. RESULTS: Inhaled nitric oxide decreased pulmonary arterial pressure and increased PaO2/FiO2 without decreasing systemic arterial pressure. CONCLUSIONS: Inhaled nitric oxide selectively decreased pulmonary arterial pressure in patients with congenital heart disease complicated by pulmonary artery hypertension.
Arterial Pressure ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Nitric Oxide* ; Pulmonary Artery ; Vasodilation

BACKGROUND: Inhaled nitric oxide (NO) therapy causes selective pulmonary vasodilation in patients with pulmonary hypertension. However, attempts to discontinue inhaled NO may be complicated by abrupt life-threatening rebound pulmonary hypertension (RPH). The purpose of this study was to determine the risk factors to develop RPH and to present the adequate weaning methods. METHODS: We studied 19 consecutive children who were treated with inhaled NO because of pulmonary hypertension after surgery for congenital heart disease. We compared the dose of NO at the time of start and withdrawal, the duration of weaning and treatment, hemodynamic data, and blood gas analysis before inhaled nitric oxide withdrawal, between patients without (group I, n = 13) and with RPH (group II, n = 6). RESULTS: Compared with group I, group II patients were older in age (1204 1688 versus 546 1654 days, P < 0.05), had a lower NO concentration just before withdrawal (3 +/- 1.6 versus 5 +/- 2.6 ppm, P <0.05), a shorter duration of NO weaning period (4 +/- 3.3 versus 15 +/- 13.4 hours, P < 0.05) and received NO therapy for a shorter duration (26 +/- 11.6 versus 57 +/- 46.0 hours, P < 0.05). CONCLUSIONS: We recommend a progressive withdrawal of inhaled nitric oxide to avoid life-threatening RPH observed in the sudden discontinuation.
Blood Gas Analysis ; Child ; Heart Defects, Congenital* ; Hemodynamics ; Humans ; Hypertension, Pulmonary* ; Nitric Oxide* ; Risk Factors ; Vasodilation ; Weaning

A 57-year-old woman developed an inflammatory carcinoma in her left breast next to the spot of a silicone injection that had been used for augmentation some 20 years previously. She underwent a modified radical mastectomy with an axillary lymph node dissection. There have been relatively few cases reported in the literature that have noted breast cancer associated with silicone injections. During physical examinations, such breasts are frequently firm, tender, painful, and inflamed. Far more important, in silicone-injected breasts, carcinomas become more difficult to detect by physical examination, mam mography, and other known methods. Although no causal relationship between silicone and breast carci nomas is implied, a heightened awareness of the possible coexistence of silicone mastopathy and a breast carcinoma is necessary.
Breast Neoplasms* ; Breast* ; Female ; Humans ; Lymph Node Excision ; Mastectomy, Modified Radical ; Middle Aged ; Noma ; Physical Examination ; Silicones*

A 57-year-old woman developed an inflammatory carcinoma in her left breast next to the spot of a silicone injection that had been used for augmentation some 20 years previously. She underwent a modified radical mastectomy with an axillary lymph node dissection. There have been relatively few cases reported in the literature that have noted breast cancer associated with silicone injections. During physical examinations, such breasts are frequently firm, tender, painful, and inflamed. Far more important, in silicone-injected breasts, carcinomas become more difficult to detect by physical examination, mam mography, and other known methods. Although no causal relationship between silicone and breast carci nomas is implied, a heightened awareness of the possible coexistence of silicone mastopathy and a breast carcinoma is necessary.
Breast Neoplasms* ; Breast* ; Female ; Humans ; Lymph Node Excision ; Mastectomy, Modified Radical ; Middle Aged ; Noma ; Physical Examination ; Silicones*

Central pontine myelinolysis (CPM) by complicating rapid correction of severe hyponatremia has been widely reported. Additionally, CPM was occasionally reported among patients with post-liver transplantation, burns, chronic renal failure with dialysis, or other diseases associated with or not associated with other electrolyte changes or hyperosmolarity. However, there have been a few reports of CPM occurring in diabetic patients without documented electrolyte changes. This report is, to the best of our knowledge, the first report of CPM in type 2 diabetic patients without electrolyte changes in Korea. A 40-year-old man with type 2 diabetes mellitus with abruptly developed dysarthria and ataxia was admitted to our facility. He suffered from poor glucose control and multiple diabetic complications. Brain magnetic resonance imaging (MRI) revealed a well-defined bilateral symmetric hyperintense lesion in the central portion of the pons on T2- and diffusion-weighted images, which was consistent with CPM. After the patient's blood glucose and blood pressure normalized, his dysarthria and ataxia improved. Six months after discharge, follow-up MRI showed a persistent, but greatly reduced symmetric lesion in the central pons. It is certainly possible for CPM to be overlooked clinically in diabetic patients, but more cases could be diagnosed if careful attention was paid to this syndrome.
Adult ; Ataxia ; Blood Glucose ; Blood Pressure ; Brain ; Burns ; Diabetes Complications ; Diabetes Mellitus, Type 2 ; Dialysis ; Dysarthria ; Follow-Up Studies ; Glucose ; Humans ; Hyponatremia ; Kidney Failure, Chronic ; Korea ; Magnetic Resonance Imaging ; Myelinolysis, Central Pontine ; Pons ; Transplants