Background: Duchenne muscular dystrophy (DMD) is the most common hereditary neuromuscular disease. No curative treatment for DMD is known. Prednisone therapy is the first medical treatment that alters the course of DMD. Several studies about the doses and administrations of prednisone or prednisolone had been reported.
Objectives: To review clinical features, laboratory findings, and the result of treatment of DMD. Methods: DMD patients who came to Srinagarind Hospital, Thailand from January, 1995 to January, 2007 were retrospectively analyzed.
Results: Sixty-two patients fulfilled the study criteria. All patients were male (100 %). Mean age at onset was 4.9 years. Family history was found in 10 families (16 %). The most common symptoms were weakness, standing difficulty, and gait abnormality (100, 97, and 93 % respectively). The most common clinical signs were calf hypertrophy, weakness, and Gower sign (100, 100, and 94 % respectively). Serum creatine kinase (CK) was raised in all of the patients with mean serum CK 13,026 IU/L. Fifty patients received prednisolone. Twelve received only supportive treatments. The overall outcomes of prednisolone treatment were better, same, and worse in 37, 51, and 12 % respectively. Mean age at wheel chair was 10.8 years. Three patients with associate diseases; adult respiratory distress syndrome (ARDS), Sturge Weber syndrome, and autism were presented. To the best of our knowledge, this is the first report about DMD concomitant with ARDS and DMD with Sturge-Weber syndrome. DMD with autism, a very rare occurrence, is presented.
Conclusion: Clinical features, laboratory findings, and the outcomes of treatments of 62 DMD patients were presented. Prednisolone treatment had some beneficial effects and had significant side effects. Starting with a low dose, and then increasing to high dose in the no response patient is recommended.
To review the result of the infantile spasms' treatment with sodium valproate followed by nitrazepam or clonazepam.Descriptive retrospective study.Srinagarind Hospital, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.Twenty-four infantile spasms admitted between January 1994 and December 2003 were analyzed. The inclusion criteria were the patients with infantile spasms clinically diagnosed by the pediatric neurologist, having hypsarrhythmic pattern EEG, and receiving sodium valproate with or without nitrazepam or clonazepam. The patients who had an uncertain diagnosis, incomplete medical record, or that were incompletely followed up were excluded. Data were collected on sex, age at onset of seizure, type of infantile spasms, associated type of seizure, predisposing etiological factor, neuroimaging study, and the result of treatment including cessation of spasms, subsequent development of other seizure types, quantitative reduction of spasms, relapse rates of spasms, psychomotor development, and adverse effects of AEDs.The mean age at onset was 177 days. The male-to-female ratio was 1:1.2. There were 13 cryptogenic (54.2%) and 11 symptomatic (45.8%) infantile spasms. The most common predisposing etiological factors in symptomatic cases were hypoxic ischemic encephalopathy (45.5%) and microcephaly (36.4%), respectively. Ten patients received sodium valproate (41.7%), another 10 received sodium valproate with clonazepam (41.7%), and four received sodium valproate with nitrazepam (16.7%). Both, the complete cessation rate and the 50% reduction of spasms rate were 45.8%. The duration to complete cessation was 70 days. The relapse rate was 18.2%. The rate of delayed psychomotor development was 83.3%. The mean duration of follow-up was 49.6 months.The authors propose to use sodium valproate concomitantly with benzodiazepines, especially clonazepam, in situations such as unavailability, intolerability, or adverse effects of ACTH or vigabatrin, or in a patient who does not respond to ACTH or vigabatrin.
A 7-year-old girl presented at a university hospital with ptosis of the left eye. This resolved spontaneously within 4 weeks but then the right eye became similarly affected but responded to prostigmine. Left hypertropia with restriction of the right inferior rectus, mild exophthalmos, non-tender diffuse enlargement of the thyroid, normal thyroid function tests, anti-thyroglobulin, and anti-microsomal antibodies indicated an association of autoimmune thyroiditis and ocular myasthenia. The ptosis was remedied with pyridostigmine and short-course oral prednisolone, but the hypertropia persisted.
Migraine is a common headache syndrome in adult populations. Prophylaxis is necessary to improve the quality of life but some patients with migraine have contraindication or suffer from side effects of medication, and therefore, establishing non-medical, neuromodulatory approaches is necessary. Past evidence had shown that consecutive motor cortex (M1) stimulation with anodal transcranial direct current stimulation (tDCS) was effective to relieve central pain.To determine whether 20 consecutive days of the left M1 can be an effective prophylactic treatment for migraine.Forty-two episodic migraine patients who had never received any prophylactic treatment, failed prophylactic treatment, or discontinued treatment due to adverse events were recruited in the present study. Patients were randomized to receive either active tDCS or sham tDCS 1mA, 20 m for 20 consecutive days and followed up for 12 weeks. Differences between and within groups were determined using repeated measures ANOVA. The level of significance was set at p < 0.05.Thirty-seven patients participated in the final analyses (active: n = 20, sham: n = 17). Between-groups comparison of attack frequency, pain intensity, and abortive medications used were performed at 4, 8, and 12 weeks after treatment. The results showed statistically significant reduction in attack frequency and abortive medications at week 4 and 8 after treatment. The pain intensity was statistically significant reduced at week 4, 8, and 12. All patients tolerated the tDCS well without any serious adverse events.The present study suggests that anodal M1 tDCS may be a safe and useful clinical tool in migraine prophylaxis. The mechanism of action of anodal tDCS on neuromodulation in migraine patients needs further investigation.
To review the clinical manifestations and neuroimaging features of patients with Sturge-Weber syndrome (SWS) treated at Srinagarind Hospital over a 12-year period.A retrospective study of sixteen patients with SWS (9 males and 7 females) was conducted. The medical records, photographs, and neuroimaging studies were reviewedAll patients had port-wine stain (PWS) involving the eyelid. Bilateral cutaneous lesions were revealed in four patients (25%). Glaucoma was the main ocular disease being diagnosed in 11 eyes of nine patients (56.25%); four eyes were finally blind. The cyclodestructive procedure and/or surgical treatment was required in four eyes. Other ocular abnormalities were refractive error dilated episcleral vessels, corneal abnormalities, tortuous retinal vessels, choroidal hemangioma, amblyopia, and strabismus. Twelve patients (75%) had neurological impairment including seizure, hemiparesis, headache, and delayed development. However the most common neurological manifestation was epilepsy (75%), which could be controlled with antiepileptic drugs. Neurological imaging was performed in the majority of cases (14 patients). Intracranial abnormalities were demonstrated in 11 patients (78.57%). These included cerebral atrophy (81.82%), cerebral calcification (54.55%), leptomeningeal angioma (27.27%), and enlarged choroidal plexus (27.27%). The ocular complication and intracranial abnormalities were usually ipsilateral to the PWS. One patient with unilateral PWS, however had bilateral intracranial lesion.Port-wine stains, glaucoma, and seizure were the most common clinical features of Sturge-Weber syndrome detected in the present study. Complete ophthalmic and neurological evaluation should be performed at the time ofdiagnosis. Multidisciplinary team management as well as lifelong follow-up is needed.
Summary Carbamazepine (CBZ) has been reported as the most common culprit drug for Stevens‐Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in several Asian countries including Thailand. A strong association between HLA‐B*1502 and CBZ‐induced SJS/TEN has been reported in Han Chinese but not in Caucasian and Japanese populations. A case–control study was conducted to determine whether HLA‐B*1502 is a valid pharmacogenetic test for SJS/TEN caused by CBZ in a Thai population. Among 42 CBZ‐induced patients with SJS/TEN, 37 (88.10%) patients carried the HLA‐B*1502 while only 5 (11.90%) of the CBZ‐tolerant controls had this allele. The risk of CBZ‐induced SJS/TEN was significantly higher in the patients with HLA‐B*1502, with an odds ratio (OR) of 54.76 [95% confidence interval (CI) 14.62–205.13, p = 2.89 × 10 −12 ]. The sensitivity and specificity of HLA‐B*1502 for prediction of CBZ‐induced SJS/TEN were 88.10%. By assuming a 0.27% as a prevalence rate of CBZ‐induced SJS/TEN in a Thai population, the positive predictive value (PPV) and negative predictive value (NPV) of the HLA‐B*1502 were 1.92% and 99.96%. Results from this study suggest that HLA‐B*1502 may be a useful pharmacogenetic test for screening Thai individuals who may be at risk for CBZ‐induced SJS and TEN.
Abstract Aim To compare the efficacy of 0, 5, and 20 sessions of transcranial direct current stimulation (tDCS) for reducing symptoms of autism spectrum disorder (ASD). Method Thirty‐six male children with ASD (mean age 2 years 3 months, SD 4 months, age range 1 years 6 months–2 years 11 months) were balanced and stratified by age, sex, and baseline severity of ASD, to: (1) a control group that received 20 sessions of sham tDCS; (2) a 5‐session tDCS group (5‐tDCS) that received 5 sessions of active tDCS followed by 15 sessions of sham tDCS; and (3) a 20‐session tDCS group (20‐tDCS) that received 20 sessions of active tDCS. All groups participated in the special school activity of Khon Kaen Special Education Center, Thailand. The primary outcome was autism severity as measured by the Childhood Autism Severity Scale. Results The 5‐tDCS and 20‐tDCS groups evidenced greater reductions in autism severity than the control group at days 5 and 14, and months 6 and 12. There were no significant differences in the outcome between the 5‐ and 20‐tDCS groups at any time point. Within‐group analysis showed clinically meaningful improvements starting at month 6 for the participants in the control group, and clinically meaningful improvements starting on day 5 in both active tDCS groups, all of which were maintained to month 12. Interpretation The 5‐ and 20‐session tDCS seems to reduce autism severity faster than sham tDCS. These effects maintained at least for 1 year. What this paper adds Twenty sessions of transcranial direct current stimulation (tDCS) were not superior to five sessions. Sham tDCS with a special school activity can reduce autism severity starting at 6 months after treatment. The benefits observed for 5 and 20 sessions of tDCS last for at least 12 months.
หลกการและวตถประสงค : ไมเกรนเปนกลมอาการปวดศรษะเปนๆหายๆทมความชกทวโลกประมาณรอยละ 3–12 ในเพศชาย และ 6–29 ในเพศหญง การปองกนเปนสงจำเปนในการเพมคณภาพชวต แตผปวยไมเกรนบางรายมขอหามหรอมอาการขางเคยงจากยาปองกนไมเกรน ดงนนการปองกนโดยวธไมใชยาจงเปนสงจำเปน ในการศกษาทผานมาแสดงใหเหนวาการกระตนดวยไฟฟากระแสตรงผานกะโหลกบรเวณเปลอกสมองสวนมอเตอรดานซายสามารถลดอาการปวดเรอรงในอาการปวดจากระบบประสาทได ดงนนการศกษานจงมวตถประสงคเพอทจะประเมนวาการกระตนดวยไฟฟากระแสตรงผานกะโหลกบรเวณเปลอกสมองสวนมอเตอรตอเนองกน 20 วน จะสามารถปองกนอาการปวดในไมเกรนไดหรอไม วธการศกษา : ผปวยไมเกรนทมและไมมออรา (aura) จำนวน 13 รายตามนยามของสมาคมปวดศรษะนานาชาต ถกคดเลอกตามเกณฑคดเขาคอ 1) อายระหวาง 18-65 ป 2) ไดรบการวนจฉยโดยแพทยมาแลวอยางตำ 1 ป 3) มอาการเกดขนมากกวาหรอเทากบ 3 ครงใน 4 สปดาห โดยอยางนอย 3 เดอนทแลว ผปวยจะตองมอาการเกดขนดวย 4) ไมเคยไดรบยาปองกน หรอเคยไดรบยาปองกนแตลมเหลวอยางตำ 3 เดอนกอนจะมาเขาการศกษา 5) ยอมรบทจะไมรบการรกษาดวยวธปองกนอนทงแบบการใชยาและไมใชยาตลอดการวจย 6) ยอมรบการตดตามผลการรกษาเปนเวลา 3 เดอน ผปวยจะไดรบการกระตนดวยไฟฟากระแสตรงอยางออนขนาด 1 มลลแอมแปร เปนเวลา 20 นาททกวนตอเนองกน 20 วน และไดรบการตดตามผลการรกษาเปนเวลา 12 สปดาห โดยตดตามทกๆ 4 สปดาห ความแตกตางระหวางกอนและหลงการรกษาใชสถต repeated measures ANOVA. ผลการศกษา : ผปวยไมเกรนทสามารถเขารวมโครงการศกษาจนสนสดโครงการมจำนวน 10 ราย ผลการศกษาแสดงการลดลงอยางมนยสำคญของความถของการเกดอาการ ในสปดาหท 4 หลงรกษา(0.86, 95%CI: 0.84 to 1.01, p =0.02) และสปดาหท 8 หลงรกษา (0.68, 95%CI: 0.62 to 0.84, p =0.03) ในขณะทไมมการลดลงอยางมนยสำคญทางสถตในสปดาหท 12 หลงรกษา (-0.25, 95%CI: -0.32 to 0.18, p =0.41) ผปวยทกรายทนตอการกระตนดวยไฟฟากระแสตรงไดเปนอยางดโดยไมมอาการไมพงประสงคทรายแรง สรป : ผลการศกษานำรองนแสดงใหเหนวาการกระตนดวยไฟฟากระแสตรงอยางออนบรเวณเปลอกสมองสวนมอเตอรอาจจะเปนประโยชนและปลอดภยในการปองกนอาการปวดในไมเกรนโดยกลไกการเพมการยบยงและปรบเปลยนการรบความรสกเจบปวดในสมอง แตอยางไรกดควรมการศกษาเพมเตม โดยการสมและมกลมควบคมเปรยบเทยบ คำสำคญ : ไมเกรน, การปองกนไมเกรน, การกระตนสมองแบบไมรกราน , การกระตนดวยไฟฟากระแสตรงผานกะโหลก Background and Objective: Migraine is a common episodic headache syndrome with estimated prevalence ranging 3–12% in men and 6–29% in women. Prophylaxis is necessary to improve the quality of life but some patients with migraine have contraindication or suffer from side effects of medications, and therefore, establishing non-medical, neuromodulatory approaches is necessary. Past evidence has shown that stimulation of motor cortex (M1) with anodal transcranial direct current stimulation (tDCS) is effective to relieve central pain. This study aims to determine whether 20 consecutive days of the left M1 can be an effective prophylactic treatment for migraine. Method: Thirteen migraine patients with/without aura were identified according to the International Headache Society. Inclusion criteria were 1) ages between 18 and 65 years; 2) diagnosed by a physician and present for at least one year before enrollment; 3) three or more migraine episodes per 4 weeks during at least the previous 3 months; 4) had never received any prophylactic treatment, failure of the previous prophylactic treatment, or discontinuation of treatment due to adverse events for at least 3 months prior to the start of the stimulation; 5) agreement not to take any concurrent prophylactic treatment for headaches both by pharmacological and non-pharmacological treatments; and 6) agreement to be available for a follow-up period at least 3 months. Patients received 1mA, 20 m anodal tDCS for 20 consecutive days and followed up for 12 weeks. The differences between before and after study were determined using repeated measures ANOVA. Results: Only 10 patients could participate up to the final analyses. The results showed statistically significant reduction in the attack frequency at week 4 (0.86, 95%CI: 0.84 to 1.01, p =0.02) and week 8 (0.68, 95%CI: 0.62 to 0.84, p =0.03) while there was no statistically significant reduction in the attack frequency at week 12 (-0.25, 95%CI: -0.32 to 0.18, p =0.41). All patients could tolerate the tDCS well without any serious adverse events. Conclusion : Our pilot study suggests that anodal motor cortex tDCS may be a safe and useful clinical tool in migraine prophylaxis. Increased cortical inhibition and modulated pain perception through corticothalamic loop may underlie these effects. However, further study with a randomized controlled trial is suggested. Key words : Migraine, Migraine prophylaxis, Noninvasive brain stimulation, Transcranial direct current stimulation
Background and objective : Transcranial direct current stimulation (tDCS) was a neurophysiologic technique using weak electrical currents (1-2 mA) to modulate the activity of neurons in the brain. It was discovered in the 1,800, and then reintroduced by the reasonably well-controlled experiments 12 years ago. They suggested that electrodes placed on the head can produce noticeable neurological changes depended on the current direction. This study aims to review a basic technique of the instrument , mechanism of action, and application in clinical researches of tDCS. Material and Method: The tDCS studies were thoroughly reviewed in MEDLINE database using the key words “Transcranial direct current stimulation, tDCS, noninvasive brain stimulation, neurophysiologic technique ” from 1998 to 2010. Some information was derived from transcranial stimulation textbook. Results: The basic technique of the instrument , mechanism of action, application in clinical researches such as stroke, pain syndrome, and craving; safety, side effect, and precaution of tDCS are described. Conclusion: tDCS study is rapidly increasing and accepted as a noninvasive technique. It’s easy to use and safe. And for the date, the outcomes of tDCS in clinical researches are preferable with very little side effects. Key words: Transcranial direct current stimulation, tDCS, noninvasive brain stimulation, neurophysiologic technique.
Needle aspiration, followed by excision should it recur, is the standard method of treating galactocele. Villagers in Northeast Thailand traditionally treat galactocele by probing the obstructed duct with double strands of pleated human hair. The aim of the study was to mimic this method in order to scientifically assess its effectiveness.Sixteen patients were consecutively enrolled between 1995 and 2001. They elected either standard needle aspiration (Group A) or treatment by 6-0 nylon probing (Group B). The results were compared using the Fisher's exact and Mann-Whitney tests at p-value < 0.05.The two groups were similar regarding the children's age, first para, mass size, and duration of symptoms, but patients in the aspiration group were considerably younger than the nylon probing group. Both methods reduced the symptoms completely. Pain from treatment was reported by all patients in the aspiration method while there were none in the nylon probing method (p < 0.001). The aspiration method took 14.8 minutes less time than the nylon probing method (p < 0.001). Recurrence was found in 2 out of 5 patients in the aspiration method, whereas there was none in the 11 patients with the nylon probing method (difference = 40%; 95% CI: -3% to 83%; p = 0.083).The new treatment of galactocele by nylon probing took longer than aspiration but removed the protein plug that caused obstruction of the duct without pain and had a tendency to reduce the recurrence rate.
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