Use of the Montreal Cognitive Assessment Thai Version to Discriminate Amnestic Mild Cognitive Impairment from Alzheimer's Disease and Healthy Controls: Machine Learning Results.

BACKGROUND: The Montreal Cognitive Assessment (MoCA) is an effective and applicable screening instrument to confirm the diagnosis of amnestic mild cognitive impairment (aMCI) from patients with Alzheimer's disease (AD) and healthy controls (HCs). OBJECTIVES: This study aimed to determine the reliability and validity of the following: (a) Thai translation of the MoCA (MoCA-Thai) and (b) delineate the key features of aMCI based on the MoCA subdomains. METHODS: This study included 60 HCs, 61 aMCI patients, and 60 AD patients. The MoCA-Thai shows adequate psychometric properties including internal consistency, concurrent validity, test-retest validity, and inter-rater reliability. RESULTS: The MoCA-Thai may be employed as a diagnostic criterion to make the diagnosis of aMCI, whereby aMCI patients are discriminated from HC with an area under the receiver-operating characteristic (AUC-ROC) curve of 0.813 and from AD patients with an AUC-ROC curve of 0.938. The best cutoff scores of the MoCA-Thai to discriminate aMCI from HC is ≤24 and from AD > 16. Neural network analysis showed that (a) aberrations in recall was the most important feature of aMCI versus HC with impairments in language and orientation being the second and third most important features and (b) aberrations in visuospatial skills and executive functions were the most important features of AD versus aMCI and that impairments in recall, language, and orientation but not attention, concentration, and working memory, further discriminated AD from aMCI. CONCLUSIONS: The MoCA-Thai is an appropriate cognitive assessment tool to be used in the Thai population for the diagnosis of aMCI and AD.

Associations between hypoxia parameters in obstructive sleep apnea and cognition, cortical thickness, and white matter integrity in middle-aged and older adults.

OBJECTIVE: This study aimed to investigate the association between each parameter of intermittent hypoxia in obstructive sleep apnea (OSA) and the cognitive profile, cortical thickness, and white matter integrity in middle-aged and older adults. METHODOLOGY: Participants were newly diagnosed with moderate or severe OSA from the King Chulalongkorn Memorial Hospital, Bangkok, Thailand. Respiratory parameters from polysomnography were extracted. Each participant was tested on a battery of neuropsychological tests and underwent an MRI scan of the brain. Cortical thickness analysis and diffusion tensor imaging analysis were performed. Participants were classified as having either severe or mild hypoxia based on parameters of hypoxia, i.e., oxygen desaturation index, lowest oxygen saturation, and the percentage of total sleep time spent below 90% oxygen saturation. RESULTS: Of 17 patients with OSA, there were 8 men (47%). Median age was 57 years and median AHI was 60.6. Comparison of cortical thickness between the severe and the mild group of each hypoxic parameter revealed two clusters of cortical thinning at the right inferior frontal gyrus (p-value = 0.008) and right inferior parietal gyrus (p-value = 0.006) in the severe desaturation group and a cluster of cortical thinning at the superior parietal gyrus (p-value = 0.008) in the high oxygen desaturation index group. There was no difference in cognitive function or white matter integrity between groups. CONCLUSIONS: The magnitude of the degree and frequency of desaturations in OSA are associated with a decrease in cortical thickness at the frontal and parietal regions.

Reversible Cerebral Vasoconstriction Syndrome: A Report on Three Cases.

Reversible cerebral vasoconstriction syndrome (RCVS), a recently recognized syndrome, is defined as an intermittent segmental vasospasm of cerebral arteries accompanied by thunderclap headache. The major complications of RCVS include ischemic or hemorrhagic stroke, which may cause morbidity and mortality. It is important to detect RCVS in clinical practice because misdiagnosis may lead to inappropriate treatment. In Thailand, there are only two reported cases of RCVS, which may reflect an underdiagnosis of this syndrome. To raise awareness of RCVS, we reported a case series of three RCVS cases. Two of the presented cases had interesting precipitating factors, and two cases had an unusual delayed clinical course.

Idiopathic hypertrophic pachymeningitis at King Chulalongkorn Memorial Hospital.

BACKGROUND: Idiopathic Hypertrophic Pachymeningitis (IHP) is a rare chronic inflammatory disorder of the dura. Classic clinical symptoms include headaches and cranial neuropathy. Because of scarce clinical data from Thailand, the present study aimed to determine the clinical features, neuroimaging findings, natural histories, therapeutic options, and outcomes for treatment of IHP in a tertiary care center. MATERIAL AND METHOD: A retrospective study was carried out on all adult IHP patients hospitalized at King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between January 2000 and November 2011. Diagnostic criteria included 1) clinical symptom compatibility with IHP, 2) neuroimaging to reveal enhanced hypertrophic dura compatible with clinical syndrome, and 3) ruled out secondary causes of LHP, using appropriate clinical profiles and investigations including tissue biopsy. RESULTS: Thirty-two patients were enrolled with 21 females and 11 males, mean age of 49.03 +/- 16.12 years. The two most common symptoms were headache (93.8%) and diplopia (43.8%). The most common neurological finding was multiple cranial neuropathies (84.4%). Cranial nerve III was affected in 56.3% of the patients, followed by other cranial nerves including CN VI, IV, V and II. Headache without a neurological deficit was observed in 12.5% of the cases. Focal and diffuse enhanced thickening of the dura were observed in 96.9% and 3.1% of the cases respectively. Focal thickening in the supratentorium included the cavernous sinus, orbital apex, sphenoid wing, and superior orbital fissure. Focal thickening in the infratentorium included the falx cerebelli, the dura at the base of the skull, Meckel's cave, and foramen magnum. CSF examination showed lymphocyte pleocytosis with a slight increase in CSF proteins. Headache subsided in all of the patients after treatment with corticosteroid In relapsing and recurrent patients, a combined treatment of steroids and azathioprine was prescribed. With the combined treatment, clinical complete recovery, relapsing and recurrence were detected in 40%, 40% and 20% of the cases respectively. All relapsing and recurrence were due to rapid tapering of for early discontinuation of the steroids treatment. Only one patient had a spontaneous remission. CONCLUSION: The most common clinical manifestations of IHP were headache and multiple cranial nerve involvement. Almost all of the patients had good initial response to steroid therapy. Relapse or recurrence was usually caused by rapid tapering off or early discontinuation of the steroid treatment. Long-term treatment with combined immunosuppression may be necessary in some cases.

Blepharoclonus: anatomical localization and etiological consideration..

Blepharoclonus refers to myoclonic rhythmic eyelid closure. This is an extremely rare abnormal movement of the eyelids. The symptom has an ill-defned anatomical localization and hypothesized etiologies are diverse. We describe a 42 year-old woman with known poorly controlled hypertension (HTN) who presented with a three-week history ofataxia, dysmetria, and uncontrolled eyelid twitching. The bilateral abnormal eyelid movement occurred during either eyelid closure or opening, and was compatible with blepharoclonus. MRI revealed multiple cerebral infarctions at red nucleus, dentate nucleus, and inferior olives. These foci are within Guillain-Mollaret's triangle. The ataxia and dysmetria gradually improved within three weeks. While the blepharoclonus improved, it persisted after one year offollow-up. Our conclusion was one of HTN leading to a lacunar infarct that manifested partially as blepharoclonus. Due to the neuroimaging findings and clinical course, we propose that blepharoclonus may be a variant ofpalatal myoclonus and may be considered as another lacunar syndrome.

Spontaneous craniocervical arterial dissection: a clinical and vascular neuroimaging study.

BACKGROUND: Spontaneous craniocervical arterial dissection (SCAD) is an important cause of stroke in the young and middle-aged population. However clinical data of SCAD is limited in Thailand. This present study aims to describe the clinical profiles of SCAD in a tertiary care center in Thailand. MATERIAL AND METHOD: All SCAD patients admitted to King Chulalongkorn Memorial Hospital between January 1997 and October 2011 were enrolled. All of the patients fulfilled vascular imaging diagnostic criteria for SCAD. Clinical profiles, associated risk factors, vascular neuroimaging patterns, treatments, and outcomes were analyzed by SPSS program version 17. RESULTS: Fifty patients with SCAD were identified (0.5% of total hospitalized ischemic cerebrovascular disease and subarachnoid hemorrhage (SAH) patients). SCAD was found in 1.6% of patients under the age of 45 years. Eighty-six percent of the patients were diagnosed during the last five years of this present study period. Internal carotid artery dissection (ICAD) and vertebral artery dissection (VAD) were detected in 42% and 58% respectively. The mean age was 48.3 +/- 15.3 years. Atherosclerotic risk factors included hypertension (16%), diabetes mellitus (24%), and dyslipidemia (28%). History of previous minor head injury and migraine were encountered in 8% and 4% respectively. Headache was detected in 80% of the cases. Localized headache was observed in 64% of the cases. Diffuse headache due to SAH was detected in 14% of the cases. Neurological syndromes at presentation were ischemic stroke (72%), transient ischemic attack (TIA) (80%), and SAH (16%). Pathognomonic vascular neuroimaging patterns of dissection included wall hematoma (36%), flame-shaped appearance (28%), dissecting pseudoaneurysm (24%), and intimal flap (8%). Other vascular imaging features included dissecting vessel stenosis (58%) and dissecting vessel occlusion (18%). Treatment consisted of anticoagulants (60%), antiplatelets (10%), surgical intervention (22%), and conservative management (8%). Neurological outcomes at discharge with MRS 0-3 were 72%. No recurrent dissection or recurrent cerebrovascular events were observed during the six-month follow-up period In this present study, significant differences between ICAD and VAD in terms of percentage of SAH, severity, and outcomes were observed CONCLUSION: SCAD results in diverse cerebrovascular events such as ischemic stroke, TIA, and SAH in the young and middle-aged population. Advances in vascular neuroimaging play a crucial role in the diagnosis of SCAD. Prompt management is essential for SCAD with a rather favorable outcome.

Cognitive impairments predict the behavioral and psychological symptoms of dementia.

Introduction: The purpose of this study was to (1) validate the Thai version of the Neuropsychiatric Inventory Questionnaire (NPI-Q) as a screening tool for behavioral and psychological symptoms of dementia (BPSD), and (2) examine the relationship between cognitive performance and BPSD in an elderly population with amnestic mild cognitive impairment (aMCI) and dementia of Alzheimer's type (DAT). Methods: One hundred and twenty participants, comprising 80 aMCI and 40 DAT patients, and their respective caregivers were included in the study. Participants completed the NPI-Q and the Neuropsychiatric Inventory (NPI) within 2 weeks of each other and cognitive performance was primarily assessed using the Montreal Cognitive Assessment (MoCA). Results: The Thai NPI-Q had good validity and reliability. Pure exploratory bifactor analysis revealed that a general factor and a single-group factor (with high loadings on delusions, hallucinations, apathy, and appetite) underpinned the NPI-Q domains. Significant negative correlations between the MoCA total score and the general and single-group NPI-Q scores were found in all subjects (aMCI + DAT combined) and DAT alone, but not in aMCI. Cluster analysis allocated subjects with BPSD (10% of aMCI and 50% of DAT participants) into a distinct "DAT + BPSD" class. Conclusion: The NPI-Q is an appropriate instrument for assessing BPSD and the total score is largely predicted by cognitive deficits. It is plausible that aMCI subjects with severe NPI-Q symptoms (10% of our sample) may have a poorer prognosis and constitute a subgroup of aMCI patients who will likely convert into probable dementia.

Association between genetic polymorphisms and sites of cervicocerebral artery atherosclerosis.

Ischemic stroke is a multifactorial disease with strong genetic elements. The purpose of this case-control study was to find relationships between apolipoprotein E (APOE), angiotensin-converting enzyme (ACE), and methylenetetrahydrofolate reductase (MTHFR) genotypes and atherosclerosis of the extracranial internal carotid and intracranial arteries in the Thai population. Patients aged >45 years with significant intracranial stenosis (IC group) or extracranial carotid artery stenosis (EC group) diagnosed by duplex ultrasound and/or computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were studied. The control group comprised volunteers with no history of stroke and no evidence of significant cervicocerebral artery stenosis by ultrasound. Genomic DNA was extracted and genotyped for APOE isoforms, ACE insertion/deletion (I/D) polymorphism, and MTHFR C677T polymorphisms. There were 141 cases (83 in the IC group and 58 in the EC group) and 167 controls. The APOE ε3/ε4 genotype and APOE ε4 allele were significantly associated with extracranial carotid artery stenosis (odds ratio, 2.55; 95% confidence interval, 1.07-6.05 and odds ratio, 2.85; 95% confidence interval, 1.35-5.99, respectively). These associations were not observed in patients with intracranial atherosclerosis. There was no significant association between ACE and MTHFR polymorphisms and stenosis at any site. In a multivariate model, sex, diabetes mellitus, hypertension, ischemic heart disease, and APOE ε4 allele remained predictive of extracranial atherosclerosis. In our Thai population, the ε4 allele in the APOE gene contributes to the genetic susceptibility of extracranial internal carotid atherosclerosis. The low prevalence of extracranial carotid stenosis in this population might result from low frequencies of the APOE ε4 allele.

Relationship between poststroke depression and ischemic lesion location.

BACKGROUND: Depression is more frequently detected in stroke patient compared to other medical illness with equal disability. The relationship between poststroke depression and ischemic stroke lesion location is controversial. OBJECTIVE: To determine the relationship between early onset poststroke depression and ischemic stroke lesion location. MATERIAL AND METHOD: A cross-sectional analysis was conducted. In-patients diagnosed with first acute ischemic stroke were enrolled. CT scan and MRI of the brain were performed to confirm the diagnosis of ischemic stroke as well as ischemic stroke subtypes and to determine the ischemic stroke lesion locations. Hamilton Depression Rating Scale was used to assess early onset poststroke depression within two weeks after the onset of stroke. Statistical analysis was conducted to determine the relationship between early onset poststroke depression and ischemic stroke lesion location as well as early poststroke depression and other potential factors. RESULTS: Thirty-nine patients were enrolled. The mean age (+/- SD) is 59.7 (+/- 12.3) years. Male: female ratio was 2:1. Early onset post stroke depression was found in 11 patients (28.2%). Mild depressive, less than major depressive, and major depressive level were found in five patients (12.8%), five patients (12.8%), and one patient (2.6%) respectively. Factors that statistically significantly related to early onset poststroke depression are left sided stroke lesion, female gender and absence of hypertension. CONCLUSION: Left sided stroke lesion, female gender, and absence of hypertension are factors contributing to early onset poststroke depression.

A national registry to determine the distribution and prevalence of Parkinson's disease in Thailand: implications of urbanization and pesticides as risk factors for Parkinson's disease.

BACKGROUND: Parkinson's disease (PD) occurs worldwide but prior to this review of data from the Thailand Parkinson's Disease Registry there had been no nationwide PD registry reported globally. OBJECTIVE: To determine the distribution and prevalence of PD in Thailand and related risk factors in order to more adequately develop and allocate prevention and treatment resources where they are most needed and to ascertain risk factors that are specific to the Thai population. DESIGN: The Thailand Parkinson's Disease Registry is a new resource, and data collection began in March 2008. Data is collected by the Registry from physicians, and a mechanism is also provided for patients to self-report. This data was further analyzed by the capture-recapture methodology (CRM) to assess reporting biases. METHODS: The three main sources of data input to the Registry, i.e. (1) public hospitals, (2) private hospitals and (3) self-registration, require checking for duplicates and also allow estimation of completeness of recording (the degree of underreporting) in this disease registry. There is underreporting because of poor record keeping and administrative procedures in some facilities, and there is an unknown number of persons with PD who are not properly diagnosed because of inadequate facilities and staffing in some areas. Since our data sources should be overlapping in some way, and assuming that the likelihood of being detected in one system is independent of the others, we estimated these data sources' actual coverage and the expected total number of patients utilizing the 'capture-recapture' statistical technique. RESULTS: As of March 2011, the Thailand PD Registry had identified 40,049 PD patients. Employing log-linear modeling, the CRM analysis based on the three data sets estimated underreporting of 20,516 cases. The revised estimated total is thus 60,565 cases, resulting in a crude and age-adjusted prevalence of 95.34 and 424.57 PD cases/100,000 population, respectively. The prevalence of PD was 126.83/100,000 in urban areas and 90.82/100,000 in rural areas (p < 0.001). Preliminary regional comparisons revealed a higher prevalence of PD in residents of the central plain valley of Thailand, an area with a large amount of pesticide use. CONCLUSIONS: The combination of a passive registry and the CRM technique allowed us to derive population prevalence estimates for PD in Thailand. Thai PD prevalence estimates were similar to previous ones published for Asian countries; in addition, they suggested that urbanization and exposure to pesticides may both be risk factors for PD in the Thai population.

Lactic acidosis associated with severe neuromuscular weakness and stavudine therapy.

Nucleoside analogue reverse-transcriptase inhibitors (NRTIs) especially stavudine, used for the treatment of HIV infection have been rarely associated with lactic acidosis syndrome (LAS) and severe neuromuscular weakness mimicking Guillain Barre syndrome. A 36-year-old man presented with a one-week history of nausea, vomiting, epigastric pain, dyspnea associated with progressive muscle weakness and numbness in glove and stocking pattern. He had symptomatic HIV infection, diagnosed 2 years before the admission and was treated with GPOvir (lamivudine, stavudine and nevirapine). Physical examination revealed afebrile dyspnic drowsy man with crepitation in both lungs and hepatomegaly. Neurological examination showed areflexic symmetrical weakness of both extremities and decreased pin-prick sensation in glove and stocking pattern as well as loss of vibration and touch sensation in both hands and feet. He developed cardiopulmonary arrest and was intubated. Investigations revealed severe lactic acidosis (lactic acid = 21.1 mg/dl). Electrophysiological studies revealed severe sensorimotor axonopathy predominantly involved the lower extremities. Stavudine was discontinued. Severe LAS dramatically improved and polyneuropathy gradually recovered with symptomatic as well as supportive interventions. Monitoring of LAS and neuromuscular weakness is advocated in HIV patient who receive stavudine therapy. Immediate discontinuation of the medication after detection of these complications may prevent this fatal complications.

Neurofibromatosis type I associated multiple sclerosis.

Neurofibromatosis (NF) type I is a common autosomal dominant disease that principally affects the skin and peripheral nervous system. Neurofibromatosis type I associated multiple sclerosis is a very rare condition. A 28-year old NF1 man developed progressive spastic-ataxic gait, left side dysmetria, right internuclear ophthalmoplegia, spastic dysarthria. MRI of the brain depicted Dawson finger appearance demyelination of the corpus callosum and other multifoci demyelinating lesions typical for MS. CSF revealed high CSF protein with negative oligoclonal band. Visual evoked potential showed prolonged P100 latency, abnormal waveform and temporal dispersion bilaterally. The syndrome partially responded and stabilized with corticosteroid. Six months later progression of the syndrome characterized by paraparesis, bilateral cerebellar hemispheric syndrome and bilateral internuclear ophthalmoplegia occurred. Repeated MRI revealed more extensive white matter lesions extended into centrum semiovale. The progressive syndrome did not respond to corticosteroid. Primary progressive multiple sclerosis was diagnosed. Only thirteen cases with NF1 and multiple sclerosis have been described in the literature. The association has been hypothesized to be related to mutations in the neurofibromin protein or oligodendrocyte-myelin glycoprotein (OMgp) gene.

Statin-associated myasthenic weakness.

BACKGROUND: Statin-associated myasthenic weakness is uncommonly recognized. Since 2002, there have been 14 cases described in literatures. However, the underlying mechanism is still unknown. CASE REPORT: In 2007, a 50-year-old woman with generalized, limb predominated, myasthenia gravis (MG), whose MG status has been "minimal manifestation" for several years, developed moderately severe fluctuating bulbar weakness a few weeks after starting simvastatin 20 mg/day. Simvastatin was discontinued and dosage of cholinesterase inhibitor was temporarily increased. The symptoms resolved and she was back to her previous status in one month. In 2008, two weeks after re-challenge with simvastatin 10 mg/day, bulbar weakness re-occurred Antibody to acetylcholine receptors was measured 4.25 nmole/L. Serum creatine phosphokinase was normal. Electrophysiologic tests showed evidences of postsynaptic neuromuscular junction disorder without evidence of myopathy. The symptoms were again resolved after discontinuation of statin and temporarily increased dosage of cholinesterase inhibitor. She was back to previous status in two months. Hypercholesterolemia was then controlled with ezetimibe without any worsening in MG status. CONCLUSION: Because of the wide use of statins in clinical practice, physicians should be aware of this potential adverse effect. The incidence ofstatin-associated myasthenic weakness should be clearly investigated Challenge with other brands of statin or with reduced dosage is not beneficial in these patients. Non-pharmacological treatment and non-statin medication may be considered

Etiology of Dementia in Thai Patients.

INTRODUCTION: Molecular imaging has been developed and validated in Thai patients, comprising a portion of patients in the dementia registry. This should provide a more accurate diagnosis of the etiology of dementia, which was the focus of this study. METHODS: This was a multicenter dementia study. The baseline characteristics, main presenting symptoms, and results of investigations and cognitive tests of the patients were electronically collected in the registry. Functional imaging and/or molecular imaging were performed in patients with an equivocal diagnosis of the causes of dementia, especially in atypical dementia or young onset dementia (YOD). RESULTS: There were 454 patients in the study. The mean age of the patients was 78 years, with 60% female. Functional imaging and/or molecular imaging were performed in 57 patients (57/454 patients, 13%). The most common cause of dementia was Alzheimer's disease (AD; 50%), followed by vascular dementia (VAD; 24%), dementia with Lewy bodies (6%), Parkinson's disease dementia (6%), frontotemporal dementia (FTD; 2.6%), progressive supranuclear palsy (2%), multiple system atrophy (0.8%), and corticobasal syndrome (0.4%). YOD accounted for 17% (77/454 patients), with a mean age of 58 years. The causes of YOD were early onset amnestic AD (44%), VAD (16%), behavioral variant FTD (8%), posterior cortical atrophy (6.5%), and logopenic variant primary progressive aphasia (5.2%). CONCLUSION: AD was the most common cause of dementia in Thai patients and the distribution of other types of dementia and main presenting symptoms were similar to previous reports in Western patients; however, the proportion of YOD was higher.

Prevalence and risk factors of Parkinson's disease in retired Thai traditional boxers.

Boxing is often believed to be a frequent cause for parkinsonism caused by chronic repetitive head injury, with Muhammad Ali frequently cited as an example. The purpose of this study is to determine the prevalence of Parkinson's disease (PD) in retired Thai traditional boxers. Two standardized screening questionnaires were sent to all registered Thai traditional boxers. Subjects who screened positive for parkinsonism were invited for clinical examinations by two independent neurologists. Among 704 boxers (70%) who completed the questionnaires, 8 boxers (1.14%) had parkinsonism: 5 with PD, 1 with progressive supranuclear palsy and 2 with vascular parkinsonism. Boxers with PD were found to have an older mean age than those without PD (P = 0.003). The analysis of probable risk factors disclosed an association between the number of professional bouts (>100 times) and PD (P = 0.01). The crude prevalence of PD in Thai boxers was 0.71% (95% CI: 0.09-1.33), with a significant increase with age. The prevalence rate of PD in those aged 50 and above was 0.17% (95% CI: 0.15-0.20), age-adjusted to the USA 1970 census, which is comparable to that of the general populations. The analysis determined that the number of professional bouts is a risk factor among these boxers, supporting the notion that repetitive head trauma may pose an additional risk to certain individuals who are already susceptible to PD.

Guillain-Barre syndrome: a clinical study in King Chulalongkorn Memorial Hospital.

BACKGROUND: Guillain-Barre Syndrome (GBS) is an acute,fatal, but treatable polyradiculopathy. Clinical data concerning this entity is scarce in Thailand The purpose of the present study was to describe clinical profiles and management of GBS as well as to determine prognostic factors in GBS. MATERIAL AND METHOD: Clinical data of GBS in King Chulalongkorn Memorial Hospital during 2002-2007 were searched by using in-patients hospital database. Asbury and Cornblath's criteria were applied for the diagnosis of GBS. Clinical data, electrophysiological data, management, and clinical prognostic factors were collected and analyzed by SPSS version 16. RESULTS: Fifty-five patients with GBS were recruited, 26 wire male and 29 were female. Mean age was 43 +/- 17 years. History of antecedent infection included: respiratory tract 29%, gastrointestinal tract 7%, ear 2%, and non-specific infection 14%. Initial presentations were limb weakness 87%, limb numbness 78%, bulbar weakness 31%, and facial weakness 18%. Electrodiagnostic study revealed demyelinating process in 54% and axonopathy in 46%. Twenty-nine patients received intravenous immunoglobulin while 13 patients underwent plasmapheresis. Clinical outcomes were satisfactory in most of the patients and only two patients died from sepsis and pneumonia. On discharge, the status of the patients were Hughes grade 1-4 in 73%, 14%, 5%, and 4% respectively. Bulbar paresis as the presenting symptom was the only clinical prognostic factor that significantly determined airway compromised and subsequently respiratory failure. CONCLUSION: Clinical features of GBS in the present series were characterized by generalized muscle weakness with mild numbness in combination with facial and bulbar paresis in nearly half of patients. Respiratory failure was encountered in 9% of cases. Clinical outcomes were satisfactory in most of the patients with or without specific treatment. The most significant predictor for adverse clinical course was the bulbar paresis as a presenting symptom and patients who presented with less disability score had a better recovery.

Neuromyelitis optica with hypothalamic involvement: a case report.

Current diagnostic criteria of neuromyelitis optica (NMO) includes presence of acute optic neuritis (ON) and myelitis with at least two of the three supportive criteria, which consist of spinal cord magnetic resonance image (MRI) lesion extending over 3 vertebral segments, brain MRI lesion, which does not meet the diagnostic criteria for multiple sclerosis, and NMO-IgG seropositive status A 34-year-old woman presented with two episodes of acute demyelinating processes in the central nervous system within three years. Firstly, she presented with a 2-week history of neck pain, oscillopsia, vertigo, and weakness. MRI of the brain revealed a high signal change at cervicomedullary junction. She responded to a short course of high-dose corticosteroid. One year after the first presentation, she developed bilateral optic neuritis. High dose corticosteroid therapy was prescribed for this attack After the second episode, she received long-term azathioprine. Two weeks before admission, she developed hypersomnia and confabulation. General physical examination was unremarkable. Neurological examination revealed visual acuity (VA) of 20/200 in both eyes. Optic fundi were normal. MRI of the brain demonstrated hypersignal intensity lesions at the hypothalamus, tuber cinereum, medial aspect of thalami, dorsal midbrain, and occipital periventricular white matter in T2 weighted and FLAIR images. Cerebrospinal fluid (CSF) analysis revealed a white blood cell count of 33 cells/mm3 (100% lymphocytes), protein of 34 mg/dL, CSF sugar of 55 mg/dL, and blood sugar of 100 mg/dL. Oligoclonal band was negative. Two weeks after admission, she developed quadriparesis, pain, and proprioceptive sensory loss below the 6th thoracic level. She also had urinary retention and constipation. MRI of the whole spinal cord showed multilevel hypersignal intensity lesions on T2 weighted and FLAIR images involving medulla, cervicomedullary junction and all segments of the spinal cord. She was diagnosed as NMO. Hypothalamic and brainstem involvement demonstrated in this patient were uncommon but rather pathognomonic for NMO. The authors proposed that the involvement of hypothalamus and brainstem be included in the criteria for diagnosis ofNMO.

Thai-version Migraine Disability Assessment (MIDAS) questionnaire: concurrent validity, test-retest reliability, internal consistency, and factors predictive for migraine-related disability.

Background: A Thai-version of the Migraine Specific Quality of Life Questionnaire (MSQ 2.1) is available, but a qualified questionnaire used specifically for disability assessment was not available. The most relevant practical disability assessment tested during this study was the Migraine Disability Assessment (MIDAS) Questionnaire. Objectives: To test the concurrent validity, test-retest reliability, and internal consistency of a Thai-version MIDAS questionnaire, and factors to predict disability in people with migraine. Methods: We conducted the present prospective study at a tertiary care teaching hospital in Bangkok. The original English MIDAS Questionnaire was translated into Thai with back-translation into English and the language equivalence was assessed. The Thai-version MIDAS Questionnaire was tested for concurrent validity, test-retest reliability, and internal consistency, and factors including duration of migraine history, migraine characteristics, and comorbidity were assessed for the ability to predict migraine-related disability of migraineurs. Results: Of the 58 participants, 31 were eligible to be included. The validity of the Thai-version questionnaire between the MIDAS total score and the mean headache severity (question B), the mean pain duration per attack, and the mean pain numerical rating scale (NRS) score were moderately correlated with a Spearman correlation coefficient range 0.42-0.58. The test-retest reliability of MIDAS grade had a weighted κ of 0.66, and for individual questions of the MIDAS total score, questions A and B assessed by intraclass correlation coefficients ranged 0.89-0.98. The internal consistency had a Cronbach α of 0.98. The mean pain NRS score in the past 3 months was an independent predictive factor for migraine-related disability. Conclusion: The Thai-version MIDAS Questionnaire has moderate concurrent validity, acceptable internal consistency, and excellent test-retest reliability. It would be helpful to assess clinical outcomes. Future study with a standardized translation process for the Thai-version questionnaire and a larger sample size is warranted to confirm internal consistency and determine all probable predictive factors for migraine-related disability.

Efficacy and economic evaluation of delivery of care with tele-continuous EEG in critically ill patients: a multicentre, randomised controlled trial (Tele-cRCT) study protocol.

INTRODUCTION: Some critically ill patients are confirmed by continuous electroencephalography (cEEG) monitoring that non-convulsive seizure (NCS) and/or non-convulsive status epilepticus (NCSE) are causes of their depressed level of consciousness. Shortage of epilepsy specialists, especially in developing countries, is a major limiting factor in implementing cEEG in general practice. Delivery of care with tele-continous EEG (tele-cEEG) may be a potential solution as this allows specialists from a central facility to remotely assist local neurologists from distant areas in interpreting EEG findings and suggest proper treatment. No tele-cEEG programme has been implemented to help improve quality of care. Therefore, this study is conducted to assess the efficacy and cost utility of implementing tele-cEEG in critical care. METHODS AND ANALYSIS: The Tele-cRCT study is a 3-year prospective, randomised, controlled, parallel, multicentre, superiority trial comparing delivery of care through 'Tele-cEEG' intervention with 'Tele-routine EEG (Tele-rEEG)' in patients with clinical suspicion of NCS/NCSE. A group of EEG specialists and a tele-EEG system were set up to remotely interpret EEG findings in six regional government hospitals across Thailand. The primary outcomes are functional neurological outcome (modified Rankin Scale, mRS), mortality rate and incidence of seizures. The secondary outcomes are cost utility, length of stay, emergency visit/readmission, impact on changing medical decisions and health professionals' perceptions about tele-cEEG implementation. Functional outcome (mRS) will be assessed at 3 and 7 days after recruitment, and again at time of hospital discharge, and at 90 days, 6 months, 9 months and 1 year. Costs and health-related quality of life will be assessed using the Thai version of the EuroQol-five dimensions-five levels (EQ-5D-5L) at hospital discharge, and at 90 days, 6 months, 9 months and 1 year. ETHICS AND DISSEMINATION: This study has been approved by the ethics committees of the Faculty of Medicine, Chulalongkorn University, and of Ramathibodi Hospital, Mahidol University, and registered on Thai Clinical Trials Registry. The results will be disseminated in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: TCTR20181022002; preresults.

Miller-Fisher syndrome at King Chulalongkorn Memorial Hospital.

BACKGROUND: Miller-Fisher syndrome (MFS), a variant of Guillain-Barré syndrome (GBS) is a self-limiting demyelinating disease of the peripheral nervous system. Clinical spectrum, natural history, prognosis, and pathogenesis of MFS are not fully documented. OBJECTIVE: Probe the clinical features of MFS in a tertiary center in Thailand and compare its pattern with other Asian countries. MATERIAL AND METHOD: The clinical recordings were searched from databases at King Chulalongkorn Memorial Hospital (KCMH) between 2002 and 2007. Keywords were "Miller-Fisher syndrome" and "Guillain-Barré syndrome". Cases with MFS were recruited The data regarding clinical features, course, treatments, and investigations were reviewed. Comparisons with other large Asian series were demonstrated. RESULTS: Six patients (male:female; 3:3) with MFS were analyzed The incident rate is 7.7% that of GBS. The median age was 54.3 years (range 28 to 73 years). MFS frequently started with diplopia followed by ophthalmoplegia and ataxia. Other clinical symptoms included limb dysesthesia and weakness, dysphagia, dysarthria, and diffuse headache. Spontaneous recovery occurred in three patients while two patients received plasmapheresis and one received intravenous immunoglobulin (IVIG). Six months after neurological deficits, all patients were almost free of symptoms and had returned to their normal activities. CONCLUSION: The incidence, clinical features, and prognosis of MFS in KCMH were comparable with the previous studies in other Asian countries. High percentage of limb dysesthesia and optic neuropathy were detected in the present series. Headache was also common among Thai MFS. Immuno-pathogenesis of MFS is well documented but immunomodulatory therapy should be considered only in some cases.