A comparative crossover study on the treatment of hemifacial spasm and blepharospasm: preseptal and pretarsal botulinum toxin injection techniques.

Hemifacial spasm (HFS) and benign essential blepharospasm (BEB) are chronic and disabling abnormal craniofacial movements that produce involuntary eyelid twitching and closure. The efficacy and safety of botulinum toxin type A (BoNT-A) injections have been accepted and widely used for the treatment of HFS and BEB. However, different injection sites may influence the effectiveness, doses, and side effects. The aim of this study is to compare the efficacy, patient satisfaction, and complications of low-dose BoNT-A injections between injection at the preseptal (PS) and the pretarsal (PT) portion of the orbicularis oculi muscle. A total of 40 patients, 31 patients with HFS and 9 patients with BEB, participated in this study. Each patient received both PS and PT BoNT-A injections in a crossover design study. Latency to response, duration of improvement, the Jankovic Rating Scale (JRS), self-response scale, patient satisfaction scale, and complications were compared. Low-dose injections of BoNT-A at the PT portion produced a significantly higher response rate in terms of latency to response, duration of improvement, JRS, self-response scale, and patient satisfaction scale than the PS injections. Major side effects including ptosis and droopy eyelid were observed only after the PS injections. These findings confirmed that low-dose injections of BoNT-A at the PT portion provide more efficacy, patient satisfaction, and fewer complications than the PS injections for the treatment of involuntary eyelid twitching and closure in patients with HFS and BEB.

Associations between HLA class I and cytochrome P450 2C9 genetic polymorphisms and phenytoin-related severe cutaneous adverse reactions in a Thai population.

BACKGROUND: Phenytoin is one of the most common causative drugs of several types of severe cutaneous adverse reactions (SCAR) such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reactions with eosinophilia and systemic symptoms (DRESS). Genetic polymorphisms of the human leukocyte antigens (HLA) and cytochromes P450 (CYP) have been proposed as key elements for the susceptibility to phenytoin-related SCAR in certain ethnicities. This study investigated the associations between the genetic polymorphisms of HLA class I and CYP2C9 and phenytoin-related SCAR in a Thai population. MATERIALS AND METHODS: Sixty phenytoin-related SCAR (i.e. 39 SJS/TEN and 21 DRESS) and 92 phenytoin-tolerant patients were enrolled in the study. The genotypes of HLA class I and CYP2C9 were determined. RESULTS: Six HLA alleles including HLA-A*33:03, HLA-B*38:02, HLA-B*51:01, HLA-B*56:02, HLA-B*58:01, and HLA-C*14:02 were significantly associated with phenytoin-related SJS/TEN, whereas only the HLA-B*51:01 was significantly associated with phenytoin-related DRESS. The odds ratios of phenytoin-related SJS/TEN in the patients who carried one of these alleles ranged from 4- to 10-fold. The frequencies of patients who carried the HLA-B*15:02 in the SJS/TEN (12.82%) or the DRESS (9.52%) groups were not significantly different from that of the controls (14.13%). The higher risk of phenytoin-related SJS/TEN was observed in the patients with CYP2C9*3 (odds ratio=4.30, 95% confidence interval=1.41-13.09, P<0.05). CONCLUSION: Neither SJS/TEN nor DRESS caused by phenytoin was significantly associated with the HLA-B*15:02. The CYP2C9*3 variant was significantly associated with phenytoin-related SJS/TEN, but not DRESS. Certain alleles of HLA, particularly HLA-B*56:02, were significantly associated with phenytoin-related SCAR in the study population.

Pyridoxine-induced sensory ataxic neuronopathy and neuropathy: revisited.

High dose pyridoxine is neurotoxic. Previous case reports were sparse and little is known about the clinical and electrodiagnostic findings. Three patients with pyridoxine-induced sensory ataxic neuropathy were studied and a review of the involved literature was performed. Three patients, aged 80, 83 and 83 years old, presented with sensory ataxia for 3-8 months. Examination showed signs of polyneuropathy and sensory ataxia. Six hundred milligrams of pyridoxine was consumed each day for 3-10 years, in the form of vitamin B1-6-12 combination tablet. Investigations for other causes of neuropathy were unremarkable. Blood levels of vitamin B6 were markedly elevated at 104.6, 81.4 and 66.9 times of upper normal limits. Electrodiagnostic tests showed symmetric axonal sensory polyneuropathy in two patients. Two years after vitamin discontinuation, all patients showed no significant improvement in the neuropathy and gait. In conclusion, consumption of high dose pyridoxine can cause sensory neuronopathy and axonal sensorimotor polyneuropathy, leading to sensory ataxia which may not be reversible.

Rivastigmine patch for treatment of Alzheimer's disease in clinical practice in Thailand.

BACKGROUND: Rivastigmine is a cholinesterase inhibitor for treatment of mild to moderate Alzheimer's disease (AD) and dementia associated with Parkinson's disease. The new patch formulation was recently made available. We assessed the safety, tolerability, and cognitive outcome of rivastigmine patch in treatment of mild to moderate AD in clinical practice in Thailand. METHODS: A multicentre, hospital-based, prospective observational study was conducted in nine hospitals across Thailand. Patients with probable mild to moderate AD who received the rivastigmine patch were enrolled. Data were collected data at baseline, weeks 4-8 and after week16. RESULTS: A total of 116 AD patients were screened, and three were excluded. Of 113 patients, 62.8% were women with a mean age of 73.3 ± 9.2 years; 79.7% were newly diagnosed. One-third of all patients had been using antipsychotic or antidepressant medication. Common comorbidities were hypertension and dyslipidemia. The Thai Mental State Examination score significantly increased from 18.6 to 20.3 (weeks 4-8) and 20.4 (week 16+) (P < 0.001). Scores based on physicians' (Clinical Global Impression) and caregivers' (Patients' Caregiver Global Impression of Change) impressions of improvement suggested minimal improvement. Because of adverse events, seven patients's dosages were reduced 10 cm(2) to 5 cm(2) or from 5 cm(2) to nothing. Itching was the most common adverse symptom. CONCLUSIONS: During the first 16 weeks after initiation of rivastigmine patch therapy, patients with probable mild to moderate AD had statistically significant improvement in cognitive function, but clinically marginal benefit. Rivastigmine was safe and well tolerated.

0.025% capsaicin gel for the treatment of painful diabetic neuropathy: a randomized, double-blind, crossover, placebo-controlled trial.

BACKGROUND: Topical therapy may provide additional benefit in patients with painful diabetic neuropathy (PDN). This study was conducted to study the safety and efficacy of 0.025% capsaicin gel in this condition. METHODS: A 20-week, double-blind, crossover, randomized, single-center study enrolled subjects with PDN. They received 0.025% capsaicin gel or placebo for 8 weeks, with a washout period of 4 weeks between the two treatments. Primary efficacy end point was percent change in visual analog scale (0-100 mm) of pain severity. Secondary outcomes were score change in Neuropathic Pain Scale (NPS), short-form McGill Pain Questionnaires (SF-MPQ), proportion of patients who had pain score reductions of 30% and 50%, and adverse event. RESULTS: Of the 35 subjects screened, 33 were enrolled and 33 completed at least an 8-week treatment period. Intention-to-treat analysis showed no significant improvement in pain with capsaicin gel, compared with placebo with visual analog scale (VAS) score 28.8 mm vs. 34.6 mm (P = 0.53). No significant difference between the groups was found in SF-MPQ (7.4 vs. 7.71, P = 0.95), NPS (29.4 vs. 31.3, P = 0.81), and proportion of patients who had 30% or 50% pain relief. Capsaicin gel was well tolerated with minor skin reaction. CONCLUSIONS: 0.025% capsaicin gel is safe and well tolerated, but does not provide significant pain relief in patients with PDN.

Incidence and clinical characteristics of adverse neurological events and stroke-like syndrome associated with immune stress-related response after COVID-19 vaccination in 2021 from Thailand.

OBJECTIVES: AEFIs (adverse events following immunizations), especially ISRR ( immune stress related response) which can cause stroke-like symptoms may affect the vaccine roll-out campaign to prevent the coronavirus 2019 outbreak. METHODS: This study aimed to describe the incidence and clinical characteristics of neurological AEFIs and stroke-like symptoms associated with ISRR after COVID-19 vaccination. Characteristics of ISRR were compared to minor ischemic stroke patients during the same period of the study. During March to September 2021, we retrospectively collected data of participants aged ≥ 18 years who received COVID-19 vaccine and developed AEFIs from Thammasat university vaccination center (TUVC). Data of neurological AEFIs patients and minor ischemic stroke patients were collected from hospital electronic medical record system. RESULTS: COVID-19 vaccine were administered at TUVC for 245,799 doses. AEFIs were reported in 129,652 instances (52.6%). ChADOx-1 nCoV-19 viral vector vaccine has the most frequent occurrence of AEFIs (58.0%), and neurological AEFIs (12.6%). 83% of neurological AEFI was headache. Most were mild and did not need medical attention. Of 119 patients who received COVID-19 vaccine from anywhere with neurological AEFIs and presented to TUH, ISRR was diagnosed in 107 patients (89.9%) and all patients who has follow-up data (30.8%) showed clinical improvement. In comparison with minor ischemic stroke (116 patients), ISRR patients had significantly less ataxia, facial weakness, weakness of arm/leg and speech disturbances (P < 0.001). CONCLUSION: The incidence of neurological AEFIs after COVID-19 vaccination was higher among recipients of ChAdOx-1 nCoV-19 vaccine (12.6%) than inactivated vaccine (6.2%) and mRNA vaccine (7.5%). However, most neurological AEFIs were ISRR, had mild severity and resolved within 30 days. Stroke-like symptoms occurred less frequently than patients with minor ischemic stroke.

Randomized controlled trial of home rehabilitation for patients with ischemic stroke: impact upon disability and elderly depression.

BACKGROUND: Patients with major stroke are often left with disability and may have depression and dementia during the recovery phase. Rehabilitation programmes have been shown to improve short-term physical outcome, but their long-term effectiveness and impact on dementia and depression are uncertain. METHODS: We performed a 6-month randomized controlled trial of a home rehabilitation programme and compared it with the standard care patients with recent ischemic stroke receive. The intervention group received home-based physical therapy once a month for 6 months, along with educational support, counselling and audiovisual materials. The control group received rehabilitation as prescribed by a physician and educational materials upon discharge from hospital. The primary measurement was a change in Barthel Index. Secondary measurements were the Hospital Anxiety and Depression Scale (HADS) and Thai Mini-Mental State Examination. RESULTS: Of the 68 screened patients, 60 patients were enrolled. At baseline, there was no significant difference in patient characteristics between the two groups. Over 2 years, the mean Barthel Index and Hospital Anxiety and Depression Scale were significantly improved in the intervention group compared to the control group (Barthel Index mean: from 31.7 ± 5.9 to 97.2 ± 2.8 vs from 33.2 ± 4.8 to 76.4 ± 9.4, P < 0.001; Hospital Anxiety and Depression Scale mean: from 16.1 ± 7.6 to 9.1 ± 0.3 vs 16.4 ± 4.9 to 9.1 ± 0.3, P= 0.003). Depression was strongly associated with being dependent on others. However, the Thai Mini-Mental State Examination in both groups did not significantly differ (Thai Mini-Mental State Examination mean: from 24.4 ± 2.0 to 24.6 vs 23.8 ± 1.9 to 24.1 ± 0.3, P= 0.068). There was no significant interaction between baseline characteristics and treatment outcome. CONCLUSIONS: At 2 years follow-up, it was evident that a 6-month home rehabilitation programme after ischemic stroke improved functional outcome and reduced incidence of depression, but not dementia.

HLA-B*1502 strongly predicts carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in Thai patients with neuropathic pain.

BACKGROUND: Carbamazepine (CBZ) is one of the standard pharmacological treatments for neuropathic pain. However, its serious adverse drug reactions include Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Recently, HLA-B*1502 allele was implicated as a genetic marker of CBZ-induced SJS/TEN in some Asian epilepsy populations. METHODS: This is a case control study to describe the clinical characteristics of SJS/TEN in Thai patients with neuropathic pain who were treated with CBZ, and to determine the association of HLA-B*1502 in these patients, comparing with those who exposed to CBZ for at least 6 months without any cutaneous reactions. RESULTS: Thirty-four SJS/TEN patients and 40 control patients were included in this study. Mean age of SJS/TEN patients was 47 years. SJS/TEN was developed in 10.8 ± 1.4 days after initiation of CBZ. HLA-B*1502 allele was found in 32 of 34 SJS/TEN patients (94.1%) but it was found only in 7 of 40 control patients (17.5%). The association was very strong with an odds ratio of 75.4. Sensitivity and specificity of this HLA-B*1502 genotype test were 94.1% and 82.5%, respectively, while the positive predictive value and negative predictive value were 1.43% and 99.98%, respectively. Positive and negative likelihood ratios were 5.37 and 0.07, respectively. CONCLUSIONS: HLA-B*1502 is a strong genetic marker for CBZ-induced SJS/TEN in Thai patients with neuropathic pain. The screening for this marker should be performed prior to initiation of CBZ treatment to assess the risk of this serious side effect.

The differences in clinical characteristics and natural history between essential tremor and essential tremor plus.

The diverse clinical manifestation of essential tremor (ET) has led to the question whether the different phenotypes may affect the clinical outcome and progression. This study aimed to estimate the clinical characteristics and natural history of ET and ET-plus. A total of 221 patients with ET were included, 117 (52.9%) reclassified as ET and 104 (47.1%) as ET-plus. Patients with ET-plus were significantly older in age at onset (P < 0.001); had a higher frequency of cranial tremors (P < 0.001), neurological comorbidities (P < 0.001) and psychiatric comorbidities (P = 0.025); more tremor progression (P < 0.001); and poorer response to medical treatment (P < 0.001) compared to ET patients. Regression analysis revealed that late-onset tremor (OR 11.02, 95% CI 2.79-43.53), neurological comorbidities (OR 3.38, 95% CI 1.56-7.31), psychiatric comorbidities (OR 4.29, 95% CI 1.48-12.44), cranial tremors (OR 2.10, 95% CI 1.02-4.30), and poor response to medical treatment (OR 3.67, 95% CI 1.87-7.19) were associated with ET-plus diagnosis. ET and ET-plus differ in the age of onset, tremor distribution, comorbidities, treatment response rate, and progression. Identifying the ET phenotypes may increase the clinical value in therapeutic strategies and clinical research in the future.

Current concepts in the management of diabetic polyneuropathy.

Diabetic sensorimotor polyneuropathy (DSPN) is encountered in approximately one-third of people with diabetes. This, in turn, might markedly impoverish their quality of life, mainly owing to neuropathic pain and foot ulcerations. Painful DSPN might be as frequent as 25% in diabetes patients. Symptoms as a result of DSPN typically comprise pain, paresthesia and numbness in the distal lower limbs. Asymptomatic DSPN might reach 50% among patients with this condition. Unfortunately, DSPN is still not adequately diagnosed and treated. Its management has three priorities: (i) lifestyle improvement, near-normoglycemia and multifactorial cardiovascular risk intervention; (ii) pathogenesis-oriented pharmacotherapy; and (iii) symptomatic alleviation of pain. Intensive diabetes therapy showed evidence for favorable effects on the incidence and deterioration of DSPN in type 1 diabetes, but not type 2 diabetes. Among pathogenesis-oriented treatments, α-lipoic acid, actovegin, benfotiamine and epalrestat are currently authorized to treat DSPN in several countries. Symptomatic therapy uses analgesics, notably antidepressants, opioids and anticonvulsants, reducing pain by ≥50% in approximately 50% of individuals, but might be limited, particularly by central nervous system-related adverse events. Local treatment with the capsaicin 8% patch might offer an alternative. In addition to pain relief, therapy should improve sleep, mobility and quality of life. In conclusion, multimodal treatment of DSPN should consider the individual risk profile, pathogenetic treatment and pain management using pharmacotherapy (combinations, if required), as well as non-pharmacological options.

Association between HLA-B*1502 and carbamazepine-induced severe cutaneous adverse drug reactions in a Thai population.

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 x 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.

Factors correlating quality of life in patients with myasthenia gravis.

We studied factors associated with quality of life (QOL) among myasthenia gravis (MG) patients in two university hospitals in Thailand: Thammasat University (TU) and Khon Kaen University (KKU). Consecutive MG patients from an outpatient neurology clinic of both sites were enrolled and their clinical variables and QOL by the Short-Form 36 questionnaire were assessed. There were 31 and 40 subjects enrolled at TU and KKU, respectively. The mean values of the SF-36 score in seven dimensions were higher at the TU site. The significant factors between both sites were mean age, and numbers of participants with myasthenic symptoms and steroid treatment. The frequency of MG symptoms was the only factor associated with the SF-36 score (correlation coefficient -0.66, p value < 0.01). In conclusion, the frequency of MG symptoms might be the main factor that lowers QOL in MG patients in both physical and mental aspects.

Quality of life of myasthenia gravis patients.

OBJECTIVE: To investigate the impact of myasthenia gravis (MG) on the quality of life (QOL) of MG patients. MATERIAL AND METHOD: QOL was assessed with SF-36 questionnaire. RESULTS: Thirty-one patients participating in the present study, 74.2% female, with an average age of 44.9 years old. From the SF-36 questionnaire, emotional well being had the lowest score while other components were in average or high range. Ocular and mild generalized MG had better physical functioning than moderate generalized MG With treatment, those who had no or minimal symptoms had better QOL in both physical and mental aspects. Immunosuppressant was not associated with poor QOL. CONCLUSION: QOL of Thai MG patients is better than Westerners. However, MG still has significant impact to both physical and mental aspects. More emphasis is needed for mental aspects. The degree of disease control is very important in QOL and the use of immunosuppressant does not impair QOL.

Cost-effectiveness analysis of home rehabilitation programs for Thai stroke patients.

BACKGROUND: The individual home rehabilitation program for ischemic stroke patients was conducted in a Thai healthcare setting. The program demonstrated that it was statistically significantly more effective than the conventional method. However for policy makers to adopt this program, the question of cost-effectiveness must be answered. OBJECTIVE: To compare the costs and effects of a home rehabilitation program versus conventional hospital care for ischemic stroke patients in Thailand. MATERIAL AND METHOD: Cost-effectiveness analysis was conducted alongside a clinical trial. An open-label randomized control trial was conducted to explore the efficacy of a home rehabilitation program for acute stroke care for three months after hospital discharge. The Barthel Index and Modified Rankin Scale were used to evaluate the outcome measures. Success was defined as improvement by at least one level of the outcome scales. An incremental cost-effectiveness ratio, including sensitivity analysis, was presented. RESULTS: Fifty-eight patients were included in the study. Patients were randomly assigned to the study and control groups (28 and 30, respectively). The cost and number of successful cases in the study group were higher than those of the control group. The incremental cost-effectiveness ratio (ICER) was lowest--13,644 Thai Baht (THB)--regarding the Modified Rankin Scale measurement. For patients achieving mild disability and no disability based on the Barthel Index, the ICERs were 14,212 THB and 24,364 THB, respectively. Sensitivity analyses regarding variations in number of patients and cost of home visits demonstrated more cost-effectiveness than the base case. CONCLUSION: Providing a home rehabilitation program with higher cost resulted in a greater number of patients avoiding disability than via conventional hospital care. The hospital had to pay approximately 24,000 THB for each additional disability-avoided patient when switching from conventional hospital care to a home rehabilitation program. This was assumed to be cost-effective when compared to per capita gross domestic product.

Primary muscle diseases in Thammasat University Hospital: muscle biopsy study of 12 cases.

We reviewed retrospectively 12 muscle biopsies of patients who were clinically diagnosed with a primary muscle diseases from the clinical data base of Thammasat University Hospital from January 2005 to January 2007. Most patients were male and had median age of 30.5 years (range 14 to 56). The most common clinical presentation was proximal muscle weakness. Nine of eleven patients had elevated CK concentrations ranging from 338 to 1023 IU/L. Clinicopathological correlation revealed specific diagnoses in nine patients. Suspected cases of mitochondrial neurogastrointestinal encephalopathy (MNGIE), myofibrillar myopathy (MFM) and distal myopathy with rimmed vacuoles (DMRV) were confirmed by molecular genetic studies examining thymidine phosphorylase, GNE, ZASP myotilin, desmin, abeta-crystalline and filamin C genes. Specific histopathological findings on muscle biopsy help to select cases for advance molecular testing.

Myofibrillar myopathy with limb-girdle phenotype in a Thai patient.

Myofibrillar myopathy (MFM) encompasses a genetically and clinically heterogeneous group of inherited or sporadic skeletal muscle disorders characterized pathologically by the presence of myofibrillar dissolution associated with accumulation of myofibrillar degradation products and ectopic expression of multiple proteins especially Z-disk related proteins. Patients with MFM initially present with muscle weakness and commonly developed cardiomypathy in the advanced stage. To date, mutations of genes encoding Z-disk proteins or proteins maintaining myofibrillar integrity including ZASP, MYOT, DES, FLNC and CRYAB underlie MFM. The authors herein report a 29-year-old Thai woman with a clinical diagnosis of autosomal dominant limb-girdle muscular dystrophy (LGMD1) who has one affected grandmother. The patient was subsequently found to have MFM based on her myopathological findings. Analyses of all MFM-genes known to date revealed no mutations. The current case emphasizes the importance of muscle biopsy in LGMD1 patients and a wide range of phenotypic variations among patients with MFM. The causative genes underlying the majority of MFM remain uncovered. Close monitoring of the cardiac function is crucial to prevent mortality among these patients.

0.075% capsaicin lotion for the treatment of painful diabetic neuropathy: A randomized, double-blind, crossover, placebo-controlled trial.

OBJECTIVE: A randomized, double-blinded, crossover, placebo controlled trial was conducted to evaluate the efficacy and safety of 0.075% capsaicin lotion for treating painful diabetic neuropathy (PDN). PATIENTS AND METHODS: PDN subjects were randomized to receive 0.075% capsaicin/placebo for 8 weeks, then crossing over to the other treatment after a 4-weeks washout period. Primary endpoint was the change in visual analog scale score of pain severity. Secondary outcomes were score changes in Neuropathic Pain Scale, short-form McGill Pain Questionnaires, and proportions of patients with pain score reductions of 30% and 50%, and adverse events. RESULTS: A total of 42 subjects were enrolled, 27 completed at least an 8-week treatment period. Intention-to-treat analysis showed no significant improvement in pain control with capsaicin lotion compared with placebo for all pain measures and proportion of patients who had 30% or 50% pain relief, respectively. Per protocol analysis were consistent. Capsaicin lotion was well tolerated but local skin reactions were common. CONCLUSION: In patients with PDN, the efficacy of 0.075% capsaicin lotion was similar to placebo but was well tolerated. More work is needed to assess different capsaicin formulations.

A prospective study in one year cumulative incidence of depression after ischemic stroke and Parkinson's disease: a preliminary study.

BACKGROUND AND AIMS: 1) To measure the one year cumulative incidence of depression after ischemic stroke event and 2) to compare its incidence with that of Parkinson's disease (PD) in an outpatient neurology department. MATERIALS AND METHODS: Stroke patients were recruited after their first diagnosis and PD patients were recruited during the same recruitment period. Main measures included: 1) disability (Barthel Index and Modified Rankin Scale), 2) cognitive function (Thai Mental State Examination and 3) depression (Clinical Interview Schedule-Revised). The patients were assessed at 1, 2, 3, 6 and 12 months. RESULTS: Seventy-seven stroke patients with hemispheric infarction and 59 PD patients were recruited. The baseline characteristics of the two groups were comparable except that stroke patients were 4 years younger. The cumulative one year incidence of depression was 12% after stroke and 5.1% in PD with no significant difference. Cox regression analysis showed that the risk for depression among stroke cohort was almost three times higher, although not statistically significant, than that among PD cohort (hazard ratio 2.92). In stroke, depression mainly occurred within 3 months after the event but in PD, depression developed randomly throughout the follow up period. CONCLUSION: The one year cumulative incidence of post-stroke depression in the Thai population is much lower than in the Caucasian population. However, its adjusted cumulative incidence was much higher to that of PD. The findings are in line with previous studies that stroke significantly contributes to the pathogenesis of depression.

A novel ECGF1 mutation in a Thai patient with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE).

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive, multisystem disorder, which is clinically defined by ptosis, ophthalmoparesis, gastrointestinal dysmotility, cachexia, peripheral neuropathy, and leukoencephalopathy. MNGIE is caused by mutations in the nuclear gene, endothelial cell growth factor 1 (ECGF1), encoding thymidine phosphorylase (TP). ECGF1 mutations cause severe loss of TP activity, abnormal accumulations of thymidine and deoxyuridine in plasma, and alterations of mitochondrial DNA. Here, we report the first Thai patient with MNGIE confirmed genetically by the identification of a homozygous novel ECGF1 gene mutation, c.100insC, which causes a frameshift and premature truncation of TP protein.

Natural killer cell malignancy associated with Epstein-Barr virus and hemophagocytic syndrome.

Natural killer cell malignancy is a rare and aggressive lymphoid neoplasm encompassing extra-nodal NK/T-cell lymphoma, nasal-type (ENKLN) and aggressive NK-cell lymphoma/leukemia (ANKL). A case of cutaneous ENKLN and a case of ANKL in Thai patients are reported Both patients developed hemophagocytic syndrome and shortly succumbed to death. The cells in cutaneous ENKLN are small to medium in size with minimal cytoplasm, round nuclei, irregular nuclear membrane, andfine chromatin with inconspicuous nucleoli. While that of ANKL are medium to large-sized mononuclear cells with moderate cytoplasm. Their nuclei are elongated to embryo-like with irregularly thickened nuclear membrane, fine chromatin, and small to occasional prominent nucleolus. Ancillary techniques studied on paraffin embedded tissues of both cases demonstrated that the neoplastic cells exhibit cytoplasmic CD3+, CD56+ and cytotoxic granules + by immunohistochemistry, absence of T cell receptor gene rearrangement by PCR, and presence of Epstein-Barr virus mRNA (EBER) transcripts by in situ hybridization. The authors reviewed the literature on natural killer cell neoplasm and compared the clinical characteristics, natural history, and association of Epstein-Barr virus infection with hemophagocytic syndrome.