ABSTRACT
Wohlfart et al and then Kugelberg and Welander were concerned with patients suffering from proximal limb weakness that resembled muscular dystrophy, but the muscle biopsy and EMG indicated neuronal disease. However, several reports had determined considerable variation according to age of onset, distribution of weakness and rate of progression. Most of familial cases fit an autosomal recessive pattern but dominant and X-linked forms were recorded. The syndrome is recognized by clinical criteria and laboratory abnormalities only that reflect neuronal degeneration. It merges with Werdnig-Hoffmann disease in infant and with amyotrophic lateral sclerosis in adult and with Wohlfart-Kugelberg-Welander syndrome in adolescent. In this report we respresent a case which is probably Wohlfart-Kugelberg-Welander syndrome in Korean sister.
Subject(s)
Adolescent , Adult , Humans , Infant , Age of Onset , Amyotrophic Lateral Sclerosis , Biopsy , Extremities , Muscular Dystrophies , Neurons , Siblings , Spinal Muscular Atrophies of ChildhoodABSTRACT
The authors had studied 55 cases with clinical seizure who were regularly treated with appropriate anticonvulsants over 6 months. In their distribution of age, teens are most freguent and their twenties followed. Males are more affected than female in the ratio of 3 to 2. Seizure may occur at any age; but our study shows that it is commoner in the first to third decades of life, about 72% of all cases, commonest in teens. The figures for 55 cases collected by us are : generalized type, 78% ; partial type, 22% ; 60% of all cases 33 are ldiopathic group, who had no demonstrable underlying neurologic abnormality. The major part of all idiopathic cases had their attacks of seizure before 30 years of age and; those of symptomatic cases are rather late over age of 30, and their figures are; cerebrovascular disorder, 6 cases; brain tumor, 5 cases; cerebral cysticercosis, 4 cases. 51 cases of all patients had evident abnormalities on their EEG. According to both their type of seizure and EEG findings, their drug was determined and, if need be, second drug was added. Freguently used drugs were dilantin, phenobarbital & Valproic acid etc. Mean therapeutic serum levels of anticonvulsants are: dilantin, 8.3 mg/L phenobarbital, 12.4 mg/L; which levels are less than those in U. S. A. In our study, the reponse to anticonvulsants were mostly good in about 2 years.
Subject(s)
Adolescent , Female , Humans , Male , Anticonvulsants , Brain Neoplasms , Cerebrovascular Disorders , Cysticercosis , Electroencephalography , Phenobarbital , Phenytoin , Seizures , Valproic AcidABSTRACT
The characteristics of radionuclide brain scan imaging of intracranial lesion are determined, in part, by the rate and quantity of radioactive material in the region of involvement, which is associated with alterations in the blood brain barrier. Findings, noted on the blood flow studies, often serve to direct attention to forthcoming imaging of specific pathological change on the status studies, e.g, carotid artery occlusion and cerebral infarction. We have seen that the 62 year old woman with left side hemiparesis shows typical "Flip-Flap" pattern in brain scan imaging, thought to be due to carotid artery occlusion.
Subject(s)
Female , Humans , Middle Aged , Blood-Brain Barrier , Brain , Carotid Arteries , Cerebral Infarction , ParesisABSTRACT
Measurements were made of the residual level of chlorinated hydrocarbon compound and mercury compound in the tissues of wild birds and herbs in Korea from November 1974 to August 1975. Aldrin was detected in all of the wild birds analyzed. The residue levels of aldrin varied from 0.353ppm to 16.115ppm. Among the tissues analyzed, the feathers contained the highest concentration of aldrin, but chloridane could not be detected in wild birds. The pesticides detected in wild birds were (alpha+beta)-BHC gamma-BHC, delta-BHC, heptachlor, aldrin, TDE and DDT. Dieldrin was detected only in the stomach of eastern dunlin caught at the Nakdong River basin. Residue levels of mercury were measured in all wild birds analyzed. Among tissues analyzed for mercury compound concentration, here also the feathers showed the highest level. The feathers of the eastern dunlin showed a high content of mercury compound which was 76.665 ppm at the highest level. Herbs used as material for oriental remedies were contaminated by chlorinated hydrocarbons which were (alpha+beta)-BHC, gamma-BHC, dieldrin, DDT, heptachlor, TDE, aldrin and epoxide. The insect materials from Cicadae testa, Bombycis corpus, and Scolopendia were much more contaminated by pesticides than plant materials. Herbs cultivated in arable areas were also found to be more contaminated by pesticides than wild ones. Herbs, on the whole, contained lower levels of chlorinated hydrocarbons than wild birds. The incidence of pesticide residues in natural products and in wild birds, however, should be considered as a global environmental pollution problem. The present investigation could contribute as a baseline study for the monitoring of pesticide pollution, its application and dispersal, and the hazard limit for food and human health.
Subject(s)
Humans , Animals , Birds/metabolism , Hydrocarbons, Chlorinated/analysis , Korea , Mercury/analysis , Pesticide Residues/analysis , Plants/analysisABSTRACT
It has been known that immobilization of patient caused by chronic illness, orthopedic or neurological conditions can be one of etiological factorof urinary stone The authors have recently experienced a case of renal sand filling the pelviwalycea1 system simulating renal staghorn calculus which wasspontaneously flushed out following excessive fluid intake and forced mobilization. This patient had stones in the contralateral kidney and ureter.