Purpose: Pseudohypoparathyroidism is a hereditary disorder characterized by symptoms and signs of hypoparathyroidism, typically in association with distinctive skeletal and developmental defects. Hypoparathyroidism is caused by a insufficient end-organ response to PTH (parathyroid hormone). Hypoparathyroidism consists of four types in which the most common form, pseudohypoparathyroidism-Ia, accompany with Albright's hereditary osteodystrophy. We experienced a case of a woman who had been suffering from calcified mass on left foot, diagnosed Albright's hereditary osteodystrophy. Methods: We present a case of a 24-year-old Korean female who visited plastic surgery department with a painful mass on dorsum of the left foot. On the physical exam, bony hard and painful mass, fixed to dermis, was noted. Plain X-ray films demonstrate suspicious calcification on subcutaneous tissue of dorsum of the left foot. The patient was diagnosed pseudohypoparathyroidism 2 years ago at the plastic surgery department. At the visiting time, the laboratory results were within normal range even though the patient actually had a disease. The reason is because the patient has been treated with Vit.D, calcium replacement therapy and thyroid hormone therapy. Moreover, the patient has been treated with anticonvulsant agents due to epilepsy. On the brain computer tomography (CT), calcification was noted on the basal ganglia and dentate nucleus. So we decided the total excision of entire mass from the left foot. Results: We excised main mass with numerous pinhead sized masses which were scattered around the main mass. The $6.0{\times}4.0{\times}0.5\;cm$ sized main mass was bony hard, and its surface was flat and margin was irregular. The permanent biopsy was confirmed that the main mass and all the scattered tiny masses were heterotopic calcification. The patient did not suffer from the pain after the mass excision. The wound has been healed without any problem. Conclusions: Heterotrophic calcification is often accompanied with pseudohypoparathyroidism, but such a huge one is uncommon. We report a case of pseudohypoparathyroidism with heterotrophic calcification developed in dorsum of left foot who was diagnosed by excisional biopsy.
The Journal of Korea Assosiation for Disability and Oral Health
/
v.6
no.1
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pp.15-18
/
2010
Sturge-Weber syndrome is a rare nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation involving the tissue of brain and face. The clinical features are characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement and neurologic involvement such as epilepsy, mental retardation, and contralateral hemiplegia. Oral manifestations include unilateral blood vessel expansion of the oral mucosa, vascular hyperplasia of gingiva, pyogenic granuloma-like massive hemangiomatous proliferation of oral mucosa, macrodontia, ipsilateral macroglossia, blood vessel anomaly of maxilla or mandible and abnormal tooth eruption sequence. This case report is about 11-year-old Sturge-Weber syndrome patient presented port wine nevus on the face, venous malformation on soft plate and buccal mucosa. In this case we performed simple extraction of several deciduous teeth and periodic oral hygiene management. If a patient with Sturge-Weber syndrome has to undergo dental surgery in affected areas of the mouth, great care must be taken to prevent severe hemorrhage.
In order to prove the experimental effectiveness of ChongsimOndamTang by categotizing COTⅠ, COTIⅡ, COTⅢ and COTⅣ according to the volume of COT extract,the anticonvulsion action, the antipyretic action, the analgesic action,the sedative action, and the activity of GABA transaminase, the actuvity concentration of GABA,the activity of GAD in GABAergic system comparing data with control group and observation data show the results as follows.1. The anticonvulsion effect on the convulsion induced by strychine it was significantly effective in COTⅣ and the time to death after the occurrence of the convulsion it was significantly effective in COTⅢ and COTⅣ, and the time to death after the occurrence the convulsion induced by the electrical shock of ECT unit it was significantly effective in all sample groups.2. The hypothemic effect was significantly effective in COT Ⅲ after 1 hour and 2 hour and was significantly effective COTⅣafter 1 hour and 4 hour, and the antipyretic effect on the febrile induced by endotoxin it was significantly effective in COTⅢ every 3 hour and was significantly effective in COTⅣ after 3 hour and 4 hour.3. The analgesic effect was significantly effective in COTⅢ and COTⅣ by decreasing the number of writhing syndrome.4. The sedative effect was decreased significantly all in COTⅢ and COTⅣ after 60 min, 90 min and 120 min.5. The activity of GABA transaminase was decreased significantly in COTⅡ and COTⅢ. 6. The activity concrntration of GABA was increased signifivantly in COTⅡ and COTⅢ. 7. The activity of GAD was increased significantly in COTⅡ and COTⅢ. The results show that ChongsimOndamTang can be an effective cure in mice on the anticonvulsion,the antipyretic , the analgesic,the sedative and the control of the GABAergic system in brain, and it can be used of the epilepsy and convulsive diseases clinically.
Yum, Mi-Sun;Bae, Keun Wook;You, Su Jeong;Ko, Tae Sung
Clinical and Experimental Pediatrics
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v.50
no.4
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pp.386-389
/
2007
Cyclic vomiting syndrome (CVS) is a paroxysmal, recurrent vomiting disorder of unknown pathophysiology and target organ. It has been hypothesized that CVS shares the same mechanism as migraine. We describe here a 5-year-old boy with CVS characterized by episodic vomiting attacks. These recurrent vomiting episodes began at 3 years of age, occurred every month and lasted for 5 days at a time. At the time of admission, no abnormal physical or neurological findings were observed and laboratory findings, including brain MRI and endoscopic examination, revealed nothing specific. The vomiting episodes were self-limited but recurrent and severely interrupted his daily life. When this patient was treated with topiramate, he showed a marked increase of symptom-free periods.
Tuberous sclerosis complex (TSC, MIM#191100) is an autosomal dominant neurocutaneous syndrome caused by mutation or deletion of TSC1 encoding hamartin or TSC2 encoding tuberin and characterized by seizure, mental retardation, and multiple hamartomas or benign tumors in the skin, brain, retina, heart, kidney, and lungs. The TSC2 gene on chromosome 16p13.3 lies adjacent to the PKD1 gene which is responsible for autosomal dominant polycystic kidney disease (MIM#173900). The TSC2/PKD1 contiguous gene syndrome (TSC2/PKD1 CGDS, MIM#600273) is caused by deletion of both TSC2 and PKD1 gene. We recently experienced a 15 month-old boy and a 26 month-old girl with TSC2/PKD1 CGDS confirmed by multiplex ligation-dependent probe amplification (MLPA) analysis. They showed not only typical neurologic manifestations of TSC such as epilepsy, subependymal nodules, and subcortical tubers, but also polycystic kidney disease. The contiguous gene syndrome involving PKD1 and TSC2 should be suspected in children with enlarged polycystic kidneys and TSC. MLPA analysis is a useful method for the genetic confirmation of TSC2/PKD1 CGDS.
Kim, Young Ock;Lee, Sang Won;Yang, Seung Ok;Na, Sae Won;Kim, Su Kang;Chung, Joo Ho
Korean Journal of Medicinal Crop Science
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v.22
no.1
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pp.46-52
/
2014
In traditional Korean and Chinese medicine, safflower (Carthamus tinctorius L.) for the treatment of central nervous system-related symptoms such as tremor, seizure, stroke and epilepsy. We investigated the effects of safflower could influence cerebral ischemia-induced neuronal and cognitive impairments. Administration of safflower for 1 day (200 mg/kg body weight, p.o.) increased the survival of hippocampal CA1 pyramidal neurons after transient global brain ischemia. And neurological functions measured as short term memory. Post-treatment with safflower for 2 times decreased the induction/reduction - induced production of neuronal cell loss from global cerebral ischemia. Safflower markedly decreased neuronal cell death and also caused a decrease in the content of thiobarbituric acid-reacting substances (TBARS) ($55.2{\pm}9.4{\mu}mol\;mg^{-1}$) and significant improvement of activities of glutathione (GSH) ($27.2{\pm}5.0{\mu}mol\;mg^{-1}$) in hippocampus. We conclude that treatment with safflower attenuated learning and memory deficits, and neuronal cell loss induced by global cerebral ischemia. These results suggest that safflower may be a potential candidate for the treatment of vascular dementia.
I reached following conclusion through a bibliographic study about the drug dependence. 1. The drug dependence is the case that taking drugs continually in order to get around discomfort and get mental drug efficacy. that is also the state of poisoning that shows compulsions that using all means to get drugs. the drug dependence is coincident with alcolism in Oriental Medicine. 2 Medicinal matters that causes the drug dependence consist of two field. one is licit drugs, including a tranquilizer, a sleeping pill, anti-anxiety drug, alcohol, caffeine, tobacco, etc. the other is illict drugs, including opium products, psychostimulant, a hallucinogen, aromatic agent(adhesives, LSD, etc.) 3. Drugs that causes dependences has the habit which causing mental dependences and the medicinal poisining which causing physical dependences. 4. A syndrome of abstain from the drug which rides on all kinds of drugs is analogous to depressive psychosis, epilepsy, insanity, depressive syndromes, disorder of internal organs, histery, dizziness, etc. 5. The drug dependence causes visceral dysfunction, that is chiefly inflammatory lesion of brain, heart lung etc. (inflammatory lesions os mainly due to infect.) and injuries liver which removes toxic agents and kidney which is an excretory organ. 6. The treatment of the drug dependence, which needs at first check the medical record and the syndrome, is consist of the expectant treatment and isolating treatment as a rule and sometimes mental therapeutics is going on at the same time. 7. The oriental medical cure of the drug dependence needs more concrete study.
Kim, Byung-Kon;Choi, In-Sun;Cho, Jin-Hwa;Jang, Il-Sung;Lee, Maan-Gee;Choi, Byung-Ju
The Korean Journal of Physiology and Pharmacology
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v.10
no.5
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pp.235-242
/
2006
Cortical malformation-associated epileptic seizures are resistant to conventional anticonvulsant drugs. Relatively little research has been conducted on the effects of antiepileptic drugs (AEDs) on seizure activity in a rat model of dysplasia. We have used rats exposed to methylazoxymethanol acetate (MAM) in utero, an animal model featuring nodular heterotopia, to investigate the effects of ethosuximide (ETX) in the dysplastic brain. Pilocarpine was used to induce acute seizure in MAM-exposed and age-matched vehicle-injected control animals. Field potential recordings were used to monitor the amplitude and number of population spikes, and paired pulse inhibition in response to stimulation of the commissural pathway. Pharmaco-resistance was tested by measuring seizure latencies after pilocarpine administration (320 mg/kg, Lp.) with and without pre-treatment with ETX. Pre-treatment with 300 mg of ETX significantly prolonged the latency to the status epilepticus (SE) in both control and MAM-treated groups. Pre-treatment with ETX 100mg and ETX 200 mg had little effect in MAMexposed rats. However, ETX 200 mg prolonged the latency to the SE in control groups. Spontaneous field potential and secondary after-discharges were higher for MAM-treated rat in comparison with control rats injects with ETX. The main findings of this study are that acute seizures initiated in MAM-exposed rats are relatively resistant to standard ETX assessed in vivo. These data suggest that ETX do not prolong seizure latencies in MAM-rats exposed to pilocarpine.
Objectives: This study was intended to review the research trends of treating neuropsychiatric diseases and symptoms with Traditional Chinese Medicine containing Haematitum. Methods: Articles were obtained through the CNKI (China National Knowledge Infrastructure) by searching with 'Haematitum' as the main key word, and supportive words related with neuropsychiatric diseases and symptoms were selected. There were 61 articles related to clinical fields, which were then classified according to study design. Results: The 61 articles were categorized into the following types of study design: 3 randomized controlled trials, 1 quasi-randomized trial, 3 simple-designed clinical trials, and 54 case studies. Decoctions containing Haematitum were used to treat diseases and symptoms such as vertigo, headache, stroke, epilepsy, neurosis, globus hystericus, fishbilepoisoning, insomnia, mania, post-traumatic brain syndrome, and kinesia. All articles reported a good rate of effectiveness. There was no poor responsiveness regarding the effects of Haematitum in 9 studies, but it was not mentioned in the other 52 studies. Decoctions self-prepared by the authors were used in 28 studies. Modified Seonbokdeja-tang, modified Banhabeakchulcheonma-tang, modified Ondam-tang were used in that order of frequency. The daily dosage of Haematitum provided was 0.2~6 g in powder, and 9~60 g in decoction. Conclusions: Decoctions containing Haematitum are used restrictively in the neuropsychiatric clinical scene. While there were no reports of poor responsiveness of the effects of Haematitum, more research is needed to confirm its clinical stability.
The relationship between copper content in scalp hair and mental retardation was investigated. Samples of scalp hair were collected from 297 mentally retarded children who were students in one of two schools providing special educational services, one, consisted of children living in an orphan home, the other, children living with parents. For comparison, 117 scalp hair samples were collected from the children who had got average or above average academic achivement in a regular elementary school. Hair samples were taken from the nape of the neck and the copper content was determined by an atomic absorption spectrophotometer (IL 551). There was no statistically significant difference in scalp copper levels across different age groups except female orphan group, but no trend or correlation between copper conents and age was found. The hair copper contents of the mentally retarded children groups were significantly lower than that of control groups. But there was no dose-response relationship between degree of mental retardation and hair copper level. The hair copper contents of the group accompanied by Down's syndrome and unknown group were significantly lower than that of control group in both sex, and in the case of accompanied by epilepsy or autism, lower than control group in male. Although the results of this study show no evidence that mental retardation has owed to copper deficiency, the possibility of copper deficiciency in their fetal or infant age could not be ruled out. Thus further study is needed to determine whether mental retardation could be attributed to copper deficiency, through the examinations about their living environments, dietary pattern, eating habit and the impact of copper deficiency on brain development.
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