Alternating hemiplegia of childhood(AHC) is a rare but clinically distinct syndrome characterized by onset before 18 months of age, frequent attacks of alternating paralysis, nystagmus transient ocular palsies, other autonomic dysfunction, and the development of cognitive impairment and a choreoathetotic movement disorder. The case is a 13 month-old boy who has presented repeated episodes of alternating hemiplegia from the age of 6 months at a frequency of several times per month, which was lasting for two or three days. Ths diagnosis was based on clinical features. Frequently such hemiplegic attacks were accompanied by irratability. He had mental retardation, neurodevelopmental delay, and seizure attacks. EEG, brain MRI, brain MR angiography, and Tc-99m HMPAO-brain single photon emission computed tomography(SPECT) failed to reveal any significant abnormal finding during the hemiplegic attacks. We tried calcium-entry block and flunarizine to relieve the hemiplegic attacks, but the frequency and severity of the hemiplegic attacks were not decreased by flunarizine therapy.
Angiography ; Brain ; Diagnosis ; Electroencephalography ; Flunarizine ; Hemiplegia* ; Humans ; Infant ; Intellectual Disability ; Magnetic Resonance Imaging ; Male ; Movement Disorders ; Paralysis ; Seizures

Familial hemiplegic migraine(FHM) is an autosomal dominant subtype of migraine with aura, characterized by the occurrence of hemiplegia during the aura. Two subforms of FHM families exist; pure FHM in 80% and FHM families with cerebellar symptoms in 20%. Half of the known FHM families show genetic linkage to chromosome 19p13, and in these families FHM is caused by missense mutations in a neuronal P/Q type calcium channel alpha-1 subunit gene(CACNA1A gene). Linkages to 1q31 and 1q21-23 have also been established. Other families are linked neither to chromosome 19 nor 1. Clinical variabilities are partially associated with the various types of CACNA1A gene mutations. FHM is distinguished from more frequent migraine types by a clear, dominant inheritance pattern and the relative absense of other headache types. Further investigation of FHM will help to clarify the genetics of more common migraine. We describe a male patient with FHM with a brief review of the literature.
Calcium Channels ; Chromosomes, Human, Pair 19 ; Epilepsy ; Genetic Linkage ; Genetics ; Headache ; Hemiplegia ; Humans ; Inheritance Patterns ; Male ; Migraine Disorders ; Migraine with Aura* ; Migraine without Aura ; Mutation, Missense ; Neurons

PURPOSE: This study was performed to find out the role of routine lumbar puncture in children presented with their first seizure with Fever. METHODS: This study included 220 children, over a 5 year period, from April 1999 to March 2003, who visited or were admitted at Dae Dong Hospital with their first febrile convulsion. Lumbar puncture was performed in all children. We analyzed their age, family history, type of seizure, duration of seizure, cause of fever, and the results of lumbar puncture. RESULTS: In the sex distribution, males(58.6%) outnumbered females(41.4%) and the ratio was 1.2:1. 81.7% of the patients had febrile convulsion from 6 months- to 3 years of age(P<0.05). 30.5% also had family history of febrile convulsion. The types of seizure were generalized tonic-clonic(72.7%), generalized tonic(17.7%), and generalized clonic(6.4 %). For the duration of seizure, 90.4% of the patients were estimated less than 15 minutes(P<0.05). The causes of fever were pharyngotonsilitis(40.5%), gastroenteritis(19.1 %), pneumonia or bronchitis(13.2%), meningitis(11%), otitis media, urinary tract infection, and exanthem subitum. However, 9.1% of the patients were diagnosed meningitis, with 18 of 20 patients under 3 years of age. CONCLUSION: In the first seizure with fever, lumbar puncture is a useful method for meningitis, especially under 3 years of age.
Child* ; Exanthema ; Fever* ; Humans ; Meningitis ; Otitis Media ; Pneumonia ; Seizures* ; Seizures, Febrile ; Sex Distribution ; Spinal Puncture* ; Urinary Tract Infections

Three-dimensional solid model has not been widely used in surgical prediction of orthognathic surgery because freque from occlusal restorations or prosthesis limited the usefulness of simulated surgery involving occlusion. We prepared three-dimensional(3D) solid model from CT data and integrated the 3D solid model with dental cast using a face-bow transfer technique combined with skeletal reference measurement and confirmation with cephalometric radiographs. With this simple and easy method, it was possible to predict bony interference between the proximal and distal segment of the mandible so that we can prevent condylar displacement after sagittal split ramus osteotomy of the mandible with prominent asymmetry. The method error was within 2mm and it seemed to be useful in preoperative planning for maxillofacial surgery with maxillo-mandibular occlusal change
Mandible* ; Orthognathic Surgery* ; Osteotomy, Sagittal Split Ramus ; Prostheses and Implants ; Surgery, Oral

RATIONALE: Studies on ictal SPECT as a method of presurgical evaluation in neocortical epilepsy have been rare and inadequate. We evaluated the accuracy in the localization of epileptic foci with ictal SPECT in neocortical epilepsy and tried to find out the determining factors of its accuracy. METHODS: We performed ictal SPECT 66 times with 99m-Tc-HMPAO in 56 patients, who received video-EEG monitoring and had electroclinical features of neocortical epilepsy. Invasive monitoring was performed in 28 patients & epileptic surgery was done in 31. Their results were also used to help determine the location of epileptic foci. In 54 of 66 scans data about injection time and temporal relationship of HMPAO injection to the onset and secondary generalization of seizures were available. RESULTS: Frontal lobe epilepsy(FLE) was most common(35%) followed by lateral temporal lobe epilepsy(28%), occipital lobe epilepsy(11%), parietal lobe epilepsy(9%), and etc. Ictal SPECT demonstrated unilateral hyperperfusion in 53 of 66 scans (80.3 %) which were concordant with electroclinical lateralization in 48 of 53 (90.6 %). Localized hyperperfusion was evident in 42 of 66 scans (63.6 %) which were concordant with electroclinical localization in 33 of 42(78.6 %). Correctly localized hyperperfusion or correctly-lateralized but diffuse hyperperfusion were detected in 37 of 54 scans in which data about injection time were available. The mean injection time was 32.1 seconds. In 16 scans ictal SPECT showed no hyperperfusion or falsely localized/lateralized hyperperfusion. Their mean injection time was 41.6 seconds. The difference of injection time between two groups was not significant(p-value=0.07), but there was a tendency that the mean injection time of the former group was earlier than that of the latter group. We had chances to perform ictal SPECT repeatedly in 8 patients under the similar conditions. The fact that earlier injection improved the accuracy of ictal SPECT in 6 patients supports the importance of early injection. In 48 studies with ictal injection, only 11 scans gave no or incorrect information of the localization or lateralization of epileptic foci. But, in 6 studies with postictal injection, 5 scans was not helpful. The difference between two groups was statistically significant(p<0.05). The presence of focal lesion, the location of epileptic foci, or secondary generalization did not influence the accuracy of ictal SPECT. CONCLUSIONS: Our data suggest that ictal SPECT in neocortical epilepsy is useful in localization of epileptic foci. Whether injected ictally or postictally and the injection time from seizure onset seems to influence the accuracy of ictal SPECT in neocortical epilepsy.
Epilepsy ; Frontal Lobe ; Generalization (Psychology) ; Humans ; Occipital Lobe ; Parietal Lobe ; Seizures ; Technetium Tc 99m Exametazime ; Temporal Lobe ; Tomography, Emission-Computed, Single-Photon*

A 43-year-old woman presented with numerous cutaneous neurofibromas, limited to the left anterior chest(T2-3) and the right lower back(L1-2). These had been present for 10 years. Neither cafe-au-lait spot, intertriginous freckle, nor Lisch nodule was found. The family history was negative for neurofibromatosis. Biopsy specimens showed circumscribed, nonencapsulated neurofibromas. The present case was a rare form of bilateral segmental neurofibromatosis in that while most of the reported cases involved the same dermatome bilaterally, she had bilaterally different dermatomal neurofibromas.
Adult ; Biopsy ; Cafe-au-Lait Spots ; Female ; Humans ; Melanosis ; Neurofibroma ; Neurofibromatoses*

Korea is rapidly aging society and psychiatric disorders of the elderly increasing Korea is rapidly becoming an aging society and psychiatric disorders of the elderly are increasing significantly in importance. Interviewing the elderly requires special skills because of their physical weakness and cognitive impairments such as difficulty concentrating. There are various diagnostic tools for them, for more accurate diagnosis. Representative scales include the Mini-Mental Status Examination for dementia, the Geriatric Depression Scale for depression, and the Confusional Assessment Method for delirium. Differential diagnosis of psychiatric disorders from cerebral and physical disorders is important.
Aged ; Aging ; Delirium ; Dementia ; Depression ; Diagnosis, Differential ; Geriatric Psychiatry ; Humans ; Korea ; Weights and Measures

No abstract available.
Saphenous Vein*

The hallmark of exfoliation syndrome is the appearance of white granular deposits and dandruff-like flakes on the lens,iris and angle. The incidence of glaucoma is high,and due to zonular weakening, complications are likely to occur in cataract surgery. A histopathological study with electron microscopy was performed on the iris and conjunctival tissue in order to clarify the production process of exfoliation material in the eyes with exfoliation syndrome. The materials were obtained from 13 eyes with exfoliation syndrome during surgery for glaucoma or cataract. The clumps of microfibrils were observed in the extracellular matrix, and the exfoliation materials were closely related with microfibrils.It was found that the basic unit of the exfoliation materials was microfibril. The cellular degenerations were observed in the basement membrane of capillary endothelium, especially the cells near the iris vessels. The exfoliation materials were observed around the degenerated cells; the mature or immature exfoliation materials around the iris melanocyte which show the various degrees of degeneration. The more degeneration and melting of the cells, the larger amount of exfoliation materials. This study suggests that the exfoliation materials in the eyes with exfoliation syndrome are derived from the abnormal microfibril, abnormal basement membrane, and degenerated cell itself.
Basement Membrane ; Cataract ; Endothelium, Vascular ; Exfoliation Syndrome* ; Extracellular Matrix ; Freezing ; Glaucoma ; Incidence ; Iris ; Melanocytes ; Microfibrils ; Microscopy, Electron

Objective: To examine the effects of Tribulus terrestris L. (T. terrestris) extract on the modulation of calcium channels to evaluate its use in topical agents for treatment of atopic dermatitis. Methods: The 70% methanol extract of T. terrestris was prepared. Human HEK293T cells with over-expressed calcium release-activated calcium channel protein 1 (Orai1), transient receptor potential vanilloid 1, or transient receptor potential vanilloid 3 (TRPV3) were treated with T. terrestris extract. Modulation of ion channels was measured using a conventional whole-cell patch-clamp technique. Results: T. terrestris extract (100 mg/mL) significantly inhibited Orai1 activity in Orai1-stromal interaction molecule 1 co-overexpressed HEK293T cells. In addition, T. terrestris extract significantly increased the TRPV3 activity compared with 2-Aminoethyl diphe-nylborinate (100 mmol/L), which induces the full activation of TRPV3. Conclusions: Our results suggest that T. terrestris extract may have a therapeutic po-tential for recovery of abnormal skin barrier pathologies in atopic dermatitis through modulating the activities of calcium ion channels, Orai1 and TRPV3. This is the first study to report the modulatory effect of a medicinal plant on the function of ion channels in skin barrier.