Coexistence of IgLON5-IgG and SOX1-IgG in a Patient with Progressive Brainstem Dysfunction.

PURPOSE: The coexistence of IgLON5-IgG and SOX1-IgG is rare. Previous reports have shown that patients with IgLON5-IgG spectrum disease present with sleep disorders, bulbar involvement, and autonomic abnormality, while SOX1-IgG positive patients present with peripheral nervous system symptoms such as the Lambert-Eaton Myasthenic Syndrome (LEMS). CASE REPORT: We report a patient who presented with progressive ophthalmoplegia, ptosis, oropharyngeal dysphagia, gait instability, and sleep disorders. The paraneoplastic antibody screening tested doublepositive for IgLON5-IgG and SOX1-IgG. However, there was no clinical sign of LEMS in this patient. After extensive cancer screening, only lung nodules with hilar adenopathy were noted. CONCLUSION: The coexistence of IgLON5-IgG with onconeuronal SOX1-IgG would suggest an underlying immune-mediated paraneoplastic process rather than secondary autoimmunity because of neurodegeneration. This is the first IgLON5-IgG case reported in Thailand, with a case of doublepositive IgLON5-IgG and SOX1-IgG as well. Keyword: IgLON5-IgG, SOX1-IgG, Paraneoplastic process, case report.

Serum NT-proBNP level for predicting functional outcomes after acute ischemic stroke.

N-terminus pro-brain natriuretic peptide (NT-proBNP) has been studied and recognized as a biomarker of cardiac thrombogenicity and stroke risk. However, the association between NT-proBNP and functional outcomes following acute ischemic stroke is still debated. This study aimed to investigate whether serum NT-proBNP level is associated with functional outcomes in acute ischemic stroke individuals. This prospective cohort study included patients diagnosed with acute ischemic stroke, and serum NT-proBNP levels were measured within 72 h. At 3 months, all patients were followed up for a modified Rankin Scale (mRS), and logistic regression models were used to evaluate the association of NT-proBNP on the primary outcome, in which a score of 3-6 was classified as an unfavorable functional outcome. Sixty-seven patients were enrolled in the study, and 23 (34.3%) patients were identified with an unfavorable functional outcome. Elevated serum NT-proBNP levels (> 100 pg/mL) were observed in 57 (85.1%) patients, and the Youden index demonstrated a cutpoint estimation of poor outcomes at 476 pg/mL with 74% sensitivity and 63% specificity. Multivariate regression analysis showed an elevation of NT-proBNP above the cutpoint level was an independent predictor for unfavorable functional outcomes, odds ratio 3.77, 95% confidence interval (1.04-13.62), P = 0.04. The present study demonstrated that elevated serum NT-proBNP levels were expected among acute ischemic stroke patients and represented the risk of unfavorable functional outcomes, suggesting that NT-proBNP might be a useful biomarker for predicting prognosis after ischemic stroke.

A Simplified Risk Score to Predict In-Hospital Newly-Diagnosed Atrial Fibrillation in Acute Ischemic Stroke Patients.

Purpose: Atrial fibrillation (AF) is a significant cause of stroke, and newly diagnosed AF (NDAF) is typically detected in the early period of stroke onset. We aimed to identify the factors associated with in-hospital NDAF in acute ischemic stroke patients and developed a simplified clinical prediction model. Methods: Patients with cryptogenic stroke aged 18 years or older who were admitted between January 2017 and December 2021 were recruited. NDAF was determined by inpatient cardiac telemetry. Univariable and multivariable regression analyses were used to evaluate the factors associated with in-hospital NDAF. The predictive model was developed using regression coefficients. Results: The study enrolled 244 eligible participants, of which 52 NDAFs were documented (21.31%), and the median time to detection was two days (1-3.5). After multivariable regression analysis, parameters significantly associated with in-hospital NDAF were elderly (>75 years) (adjusted Odds ratio, 2.99; 95% confident interval, 1.51-5.91; P = 0.002), female sex (2.08; 1.04-4.14; P = 0.04), higher admission national institute of health stroke scale (1.04; 1.00-1.09; P = 0.05), and presence of hyperdense middle cerebral artery sign (2.33; 1.13-4.79; P = 0.02). The area under the receiver operating characteristic curve resulted in 0.74 (95% CI 0.65-0.80), and the cut-point of 2 showed 87% sensitivity and 42% specificity. Conclusion: The validated and simplified risk scores for predicting in-hospital NDAF primarily rely on simplified parameters and high sensitivity. It might be used as a screening tool for in-hospital NDAF in stroke patients who initially presumed cryptogenic stroke.

Impact of behavioral and psychological symptoms of Alzheimer's disease on caregiver outcomes.

This study was to determine the prevalence of behavioral and psychological symptoms of dementia (BPSD) and its association with dementia severity and to explore the association between specific BPSD and caregiver stress, burden, and depression. A cross-sectional study involving the interviewing of the primary caregivers of patients with Alzheimer's disease (AD) was conducted. Multivariable analysis was used to analyze the associations between specific symptoms of BPSD and caregiver outcomes. A total of 102 AD patients (age 79.4 ± 7.9 years, 70.6% female) and their caregivers were included. Nearly 46% had moderate-to-severe AD. Nearly all patients (99.0%) had at least one BPSD. Apathy was among the most common symptoms (74.5%), and hallucination was the only symptom associated with severity of AD (p = 0.017). After adjustment, agitation was associated with Patient Health Questionnaire-9 (PHQ-9) and Zarit Burden Interview (ZBI-22) (p = 0.021 and 0.007, respectively); sleep disorders were associated with only PHQ-9 (p = 0.049). In conclusion, the BPSD, especially agitation and sleep disorders, can give rise to difficulties for both patients and their caregivers. The prevalence of BPSD is high (99.0%), and the symptoms can start early. Routine screening of BPSD in all AD patients is advocated.

Outcomes of asymptomatic recombinant tissue plasminogen activator associated intracranial hemorrhage.

BACKGROUND: Intracranial hemorrhage (ICH) is the most devastating complication of recombinant tissue plasminogen activator (rtPA) treatment in acute ischemic stroke patients. Data on rtPA-associated asymptomatic ICH (aICH) are limited. OBJECTIVES: To determine the incidence, risk factors, characteristics, management, and clinical outcome of rtPA-associated aICH. METHODS: The data were retrieved from the Chiang Mai University Hospital Stroke Registry between 1995 and 2019. Consecutive ischemic stroke patients were included if they were 18 or older and received rtPA. Study outcomes were the incidence and characteristics of aICH, management, 90-day modified Rankin scale (mRS), National Institute of Health Stroke Scale (NIHSS), Barthel index, and all-cause mortality. RESULTS: Of 725 rtPA treated patients, 166 (16.0%, 95% confidence interval [CI] 13.4-18.9) had aICH, 50 (6.9%, 95% CI 5.2-9.0) had symptomatic ICH (sICH). Patients with aICH had more hemorrhagic infarctions (HI) compared to sICH (81.9% vs 2.0%, P-value < 0.001). Fresh Frozen Plasma and cryoprecipitate were the most common blood products used to reverse the anticoagulant effect in sICH. Craniotomy was performed in 1% and 60% of patients who had aICH and sICH. At 90 days, patients who had aICH had poorer clinical outcomes (mRS, NIHSS and Barthel index) as compared to those without ICH. Compared to non-ICH patients, aICH patients were associated with increased risk of 90-day mortality, the hazard ratio (HR), 3.7, 95% CI 1.6-8.9. CONCLUSIONS: The rtPA-associated aICH increased the risk of morbidity and mortality outcomes. Further treatment consensus, guideline generation, or clinical trials focusing on the treatment of rtPA-associated aICH may be required.

Cerebral venous sinus thrombosis in immune thrombocytopenia patients treated with thrombopoietin receptor agonist: Case reports and literature review.

Introduction and importance: Cerebral venous sinus thrombosis is an uncommon adverse event in immune thrombocytopenia (ITP) patients treated with thrombopoietin receptor agonists (TPO-RAs). Case presentation: We reported two cases of cerebral venous sinus thrombosis after eltrombopag administration. The first case is a 29-year-old ITP woman who recently initiated eltrombopag one month before admission. She presented with progressive headache, visual disturbance, and nausea for six days with unremarkable physical examination except for bilateral optic disc edema. She was treated with enoxaparin and switched to edoxaban when discharged. The second case is a 75-year-old man with a history of vaccine-induced ITP. He was initially treated with dexamethasone and eltrombopag. One month later, he developed acute cerebral venous thrombosis with hemorrhagic infarction in the bilateral frontal lobes. Even though he was treated with intravenous heparin, his status was not improved. He received the best supportive care. Discussion: The pathophysiology of TPO-RAs-associated cerebral venous sinus thrombosis remained unclear but might associate with platelet activation. Most cases of cerebral venous sinus thrombosis occur within two months, thus closed platelet monitoring is important. Conclusion: Careful use and closed monitoring might prevent this event. Indications of initiation and tapering must be considered before TPO-RAs administration. Off-label use may enhance TPO-RA side effects.

Implications of the Presence of Hyperdense Middle Cerebral Artery Sign in Determining the Subtypes of Stroke Etiology.

BACKGROUND: Identifying stroke subtypes is crucial in choosing appropriate treatment, predicting outcomes, and managing recurrent stroke prevention. OBJECTIVES: To study the association of hyperdense middle cerebral artery sign (HMCAS) on noncontrast computed tomography (NCCT) brain and subtypes of stroke etiology. METHODS: This is a retrospective hypothesis testing study. Patients aged 18 or over who had middle cerebral artery occlusion symptoms with HMCAS with verification on brain NCCT and received intravenous thrombolysis between January 2016 and June 2019 were enrolled. The demographic data, clinical outcomes, stroke subtypes, and characteristics of HMCAS were collected from medical records. RESULTS: Ninety-nine out of 299 enrolled patients presented with HMCAS. The most common stroke subtype was cardioembolism (59%). Of the baseline characteristics, hypertension was more common in cases of large-artery atherosclerosis (LAA) (86.4%), and atrial fibrillation (AF) was the highest in cardioembolism (44.8%). HMCAS disappearance in cardioembolism was lowest compared to LAA and others (63% vs. 91% vs. 94.7%, respectively). The univariable analysis found that HMCAS disappearance is significantly associated with all stroke subtypes (Odds ratio, 95% confidence interval 10.58, 1.31-85.43; P = 0.027 for other and 5.88, 1.24-27.85; P = 0.026 for LAA). Multinomial logistic regression found that body weight and hypertension were associated with the LAA subtype. AF and intracranial hemorrhage (ICH) were associated with cardioembolism. CONCLUSION: The most likely diagnosis from the presence of HMCAS is cardioembolism, but the definite stroke etiologic subtype can not be identified. Combining the patient risk factors, including body weight, hypertension, and AF, with HMCAS and its characteristics will predict stroke subtypes more accurately.

Randomised, controlled trial of erenumab for the prevention of episodic migraine in patients from Asia, the Middle East, and Latin America: The EMPOwER study.

OBJECTIVE: EMPOwER, a double-blind, randomised, phase 3 study, evaluated the efficacy and safety of erenumab in adults with episodic migraine from Asia, the Middle East, and Latin America. METHODS: Randomised patients (N = 900) received monthly subcutaneous injections of placebo, erenumab 70 mg, or 140 mg (3:3:2) for 3 months. Primary endpoint was change from baseline in monthly migraine days at Month 3. Other endpoints included achievement of ≥50%, ≥75%, and 100% reduction in monthly migraine days, change in monthly acute migraine-specific medication treatment days, patient-reported outcomes, and safety assessment. RESULTS: At baseline, mean (standard deviation) age was 37.5 (9.9) years, 81.9% were women, and monthly migraine days was 8.2 (2.8). At Month 3, change from baseline in monthly migraine days (primary endpoint) was -3.1, -4.2, and -4.8 days for placebo, erenumab 70 mg, and erenumab 140 mg, respectively, with a statistically significant difference for erenumab versus placebo (P = 0.002 [70 mg], P < 0.001 [140 mg]). Both erenumab doses were also significantly superior to placebo on all secondary endpoints, including the proportion of patients achieving ≥50% reduction from baseline in monthly migraine days, change from baseline in monthly acute migraine-specific medication treatment days and change from baseline in the Headache Impact Test-6™ scores. The safety profile of erenumab was comparable with placebo; no new safety signals were observed. CONCLUSIONS: This study of erenumab in patients with episodic migraine from Asia, the Middle East, and Latin America met all primary and secondary endpoints. A consistent numerical benefit was observed with erenumab 140 mg versus erenumab 70 mg across all efficacy endpoints. These findings extend evidence of erenumab's efficacy and safety to patients under-represented in previous trials.ClinicalTrials.gov identifier: NCT03333109.

Trigger of a migraine headache among Thai adolescents smartphone users: a time series study.

The study aims to investigate the effect of modern technology in the trigger of migraine headaches in high school students by using generalized estimating equation analysis. The prospective time series study was conducted in one hundred and forty-five smartphones using high school students in Chiang Mai Province who each completed a headache diary giving a total of 12,969 data entries. The smartphone output power was measured and recorded by a smartphone application. Smartphone use, sleep quality, anxiety, and depression also were assessed. Results revealed that the prevalence of repeated headache be 13.4% and migraine 16.9%. The migraine had the strongest association with handfree device use and internet use, followed by age and anxiety. Furthermore, the strongest effect of smartphone output power in triggering a migraine was found at ≤ 1.79×10-5 and 1.80-1.99×10-5 mW ranges. Meanwhile, Lag_6 of daily smartphone output power exposure produced the migraine effect in a reverse dose-response manner. The smartphone electromagnetic radiation was a primary migraine trigger. The study results led to the recommendation to avoid triggers by use of smartphone talking with hand-free devices to prevent a recurrent migraine.

Perceived stress and depressive symptoms not neuropsychiatric symptoms predict caregiver burden in Alzheimer's disease: a cross-sectional study.

BACKGROUND: Caregiver burden affects the caregiver's health and is related to the quality of care received by patients. This study aimed to determine the extent to which caregivers feel burdened when caring for patients with Alzheimer's Disease (AD) and to investigate the predictors for caregiving burden. METHODS: A cross-sectional study was conducted. One hundred two caregivers of patients with AD at Maharaj Nakorn Chiang Mai Hospital, a tertiary care hospital, were recruited. Assessment tools included the perceived stress scale (stress), PHQ-9 (depressive symptoms), Zarit Burden Interview-12 (burden), Clinical Dementia Rating (disease severity), Neuropsychiatric Inventory Questionnaires (neuropsychiatric symptoms), and Barthel Activities Daily Living Index (dependency). The mediation analysis model was used to determine any associations. RESULTS: A higher level of severity of neuropsychiatric symptoms (r = 0.37, p < 0.01), higher level of perceived stress (r = 0.57, p < 0.01), and higher level of depressive symptoms (r = 0.54, p < 0.01) were related to a higher level of caregiver burden. The direct effect of neuropsychiatric symptoms on caregiver burden was fully mediated by perceived stress and depressive symptoms (r = 0.13, p = 0.177), rendering an increase of 46% of variance in caregiver burden by this parallel mediation model. The significant indirect effect of neuropsychiatric symptoms by these two mediators was (r = 0.21, p = 0.001). CONCLUSION: Caregiver burden is associated with patients' neuropsychiatric symptoms indirectly through the caregiver's depressive symptoms and perception of stress. Early detection and provision of appropriate interventions and skills to manage stress and depression could be useful in reducing and preventing caregiver burden.

Influenza immunization does not predominantly alter levels of phenytoin, and cytochrome P-450 enzymes in epileptic patients receiving phenytoin monotherapy.

OBJECTIVE: The study aims to test the effect of influenza vaccination on phenytoin, CYP2C9, and IFNγ levels in epileptic patients receiving phenytoin monotherapy METHODS: Thirty-one epileptic patients receiving stable-dose phenytoin monotherapy were enrolled onto the study. Serum concentrations of phenytoin, CYP2C9, and IFNγ were compared before and after influenza immunization. The participants were given 0.5 mL of quadrivalent influenza vaccine types A and B subvirion. Blood samples were drawn at baseline, and days 3, 7, 14 post-immunization. The outcomes were levels of phenytoin, CYP2C9, IFNγ, and the incidence of adverse events. RESULTS: No significant changes in serum phenytoin, IFNγ, and CYP2C9 levels between baseline and days 3, 7, and 14 after immunization were found. The mean levels of phenytoin, IFNγ, and CYP2C9, respectively, were 11.94 ± 7.43, 1.14 ± 0.98, and 47.69 ± 37.53 pg/mL (baseline); 12.15 ± 6.57, 2.13 ± 3.41, and 49.44 ± 39.83 pg/mL (day 3); 12.19 ± 7.69, 1.15 ± 0.94, and 49.48 ± 33.83 pg/mL (day 7); 12.79 ± 7.94, 2.15 ± 3.11, and 53.65 ± 40.91 pg/mL (day 14). The incidence of vaccine-related adverse events, which were generally mild and resolved without clinical consequences, was 58.1 %. No seizure or changes in seizure frequency were observed during the study. One patient experienced dizziness and ataxia which were symptoms attributed to phenytoin toxicity (34.57 µg/mL) by day 14. CONCLUSIONS: Influenza vaccine has no significant effect on the serum phenytoin and CYP2C9 levels in epileptic patients receiving chronic phenytoin monotherapy. The administration of influenza vaccine to epileptic patients receiving phenytoin monotherapy appears to be safe. Therefore, it is not necessary to routinely measure the serum phenytoin level after influenza immunization.

Serum Uric Acid, Serum Uric Acid to Serum Creatinine Ratio and Serum Bilirubin in Patients With Parkinson's Disease: A Case-Control Study.

BACKGROUND: Studies have shown that a low serum uric acid (SUA) level associates with Parkinson's disease (PD), but many of them did not exclude patients with impaired renal function. Studies on the association between serum bilirubin level and PD also are limited. This study determined the association between SUA level, SUA/serum creatinine (SCr) ratio and serum bilirubin levels in PD patients with normal renal and liver functions. METHODS: The PD patients from a neurological clinic, and the controls from the club for the elderly, were recruited into this study. The PD stage and motor and non-motor function were determined by the Hoehn-Yahr (H&Y) scale and unified Parkinson's disease rating scale (UPDRS), respectively. RESULTS: Sixty-one PD patients and 135 controls participated. The SUA/SCr ratio, but not SUA, was significantly lower in the PD patients than in the controls (4.12 ± 0.90 vs. 4.59 ± 1.04, P = 0.003). Serum total bilirubin (TB) and indirect bilirubin (IDB) were significantly higher in the PD patients (7.92 ± 3.67 µmol/L vs. 6.59 ± 2.78 µmol/L, P = 0.003 and 4.52 ± 2.48 µmol/L vs. 3.26 ± 1.82 µmol/L, P < 0.001), respectively. Serum TB and IDB, but not SUA or SUA/SCr ratio, were associated negatively with PD stages (P = 0.010 and P = 0.014, respectively). There was no association between TB, IDB, SUA or SUA/SCr ratio and PD disease duration or motor subtypes. No significant correlation was found between SUA or SUA/SCr ratio, serum TB and IDB. CONCLUSION: The SUA/SCr ratio is more sensitive than SUA in determining their association with PD. The high serum TB and IDB levels in PD patients compared with the controls suggest that serum bilirubin might play a role in the pathogenesis of PD. However, the lack of association between SUA or the SUA/SCr ratio and serum TB or IDB suggests that these two biomarkers play a different role in the etiopathogenesis of PD.

The development and validation of the Cluster Headache Quality of life scale (CHQ).

BACKGROUND: Cluster headache (CH) is a rare, excruciating and highly disabling primary headache disorder. Using non cluster headache specific measures, previous studies have shown that CH has a significant negative impact on patients' quality of life (QoL), but a CH-specific QoL scale is currently unavailable. Thus, the objective of this study was to develop and validate a CH-specific QoL scale. METHODS: Based on a literature review, semi-structured patient interviews and expert panel consultation, we produced a 54-item questionnaire, which was pre-tested in a sample of CH patients and subsequently reduced to 47 items. The revised scale was then administered to CH sufferers attending a tertiary headache clinic and those registered with a patient group. A total of 406 completed questionnaires were received. To assess test-retest reliability, a subsample (N = 56) completed the scale on a second occasion, two weeks after the first. Standard statistical methods were used to analyse the data for validity and reliability. RESULTS: Item reduction and exploratory factor analysis led to 28-items, grouped into four subscales labelled "restriction of activities of daily living", "impact on mood and interpersonal relationships", "pain and anxiety", and "lack of vitality". The final CH-specific QoL scale, the CHQ, demonstrated satisfactory internal consistency (Cronbach's alpha > 0.9) and test-retest reliability (intraclass correlation coefficient > 0.8), with good internal construct validity between subscales (range 0.52-0.75) and convergent validity with other QoL measures. CONCLUSIONS: We have developed and validated the first patient-reported outcome measure of QoL specifically for CH sufferers, which may be used to monitor QoL in clinical care and research.

Quality of life in primary headache disorders: A review.

BACKGROUND: Health-related quality of life (HRQoL) is emerging as an important element of clinical research in primary headache disorders, allowing a measure of the impact of headache on patients' well-being and daily life. A better understanding of this may contribute to improved resource allocations and treatment approaches. OBJECTIVE: The objective of this study is to review available data on HRQoL in primary headache disorders and identify any influencing factors. METHODS: Database searches including MEDLINE, PsycINFO and EMBASE were performed. Studies that investigated HRQoL in patients with primary headache disorders were included and reviewed. Trials that evaluated the efficacy of medications or interventions were excluded. RESULTS: A total of 80 articles were included in the review. Both physical and emotional/mental aspects of HRQoL were impaired across headache subtypes, although the extent varied depending on headache type. A number of factors influencing HRQoL were also identified. CONCLUSION: This narrative review suggests that headache, particularly in its chronic form, has a great impact on HRQoL. Clinical practice should not solely focus on pain alleviation but rather adopt routine assessment of HRQoL. Furthermore, identification and management of associated psychological comorbidities, which can significantly influence HRQoL in headache sufferers, are essential for optimal clinical management.

Clinical analysis of adult-onset spinocerebellar ataxias in Thailand.

BACKGROUND: Non-ataxic symptoms of spinocerebellar ataxias (SCAs) vary widely and often overlap with various types of SCAs. Duration and severity of the disease and genetic background may play a role in such phenotypic diversity. We conducted the study in order to study clinical characteristics of common SCAs in Thailand and the factors that may influence their phenotypes. METHODS: 131 (49.43%) out of 265 Thai ataxia families with cerebellar degeneration had positive tests for SCA1, SCA2, Machado-Joseph disease (MJD) or SCA6. The study evaluated 83 available families including SCA1 (21 patients), SCA2 (15), MJD (39) and SCA6 (8). Comparisons of frequency of each non-ataxic sign among different SCA subtypes were analysed. Multivariate logistic regression analyses were undertaken to analyze parameters in association with disease severity and size of CAG repeat. RESULTS: Mean ages at onset were not different among patients with different SCAs (40.31 ± 11.33 years, mean ± SD). Surprisingly, SCA6 patients often had age at onset and phenotypes indistinguishable from SCA1, SCA2 and MJD. Frequencies of ophthalmoparesis, nystagmus, hyperreflexia and areflexia were significantly different among the common SCAs, whilst frequency of slow saccade was not. In contrast to Caucasian patients, parkinsonism, dystonia, dementia, and facial fasciculation were uncommon in Thai patients. Multivariate logistic regression analysis demonstrated that ophthalmoparesis (p < 0.001) and sensory impairment (p = 0.025) were associated with the severity of the disease. CONCLUSIONS: We described clinical characteristics of the 4 most common SCAs in Thailand accounting for almost 90% of familial spinocerebellar ataxias. There were some different observations compared to Caucasian patients including earlier age at onset of SCA6 and the paucity of extrapyramidal features, cognitive impairment and facial fasciculation. Severity of the disease, size of the pathological CAG repeat allele, genetic background and somatic heterogeneity of pathological alleles may influence clinical expressions of these common SCAs.

Electrophysiological diagnosis and patterns of response to treatment of botulism with neuromuscular respiratory failure.

In this study we describe the electrophysiological findings in botulism patients with neuromuscular respiratory failure from major botulism outbreaks in Thailand. High-rate repetitive nerve stimulation testing (RNST) of the abductor digiti minimi (ADM) muscle of 17 botulism patients with neuromuscular respiratory failure showed mostly incremental responses, especially in response to >20-HZ stimulation. In the most severe stage of neuromuscular respiratory failure, RNST failed to elicit a compound muscle action potential (CMAP) of the ADM muscle. In the moderately severe stage, the initial CMAPs were of very low amplitude, and a 3-HZ RNST elicited incremental or decremental responses. A 10-HZ RNST elicited mainly decremental responses. In the early recovery stage, the initial CMAP amplitudes of the ADM muscle improved, with initially low amplitudes and an incremental response to 3- and 10-HZ RNSTs. Improved electrophysiological patterns of the ADM muscle correlated with improved respiratory muscle function. Incremental responses to 20-HZ RNST were most useful for diagnosis. The initial electrodiagnostic sign of recovery following treatment of neuromuscular respiratory failure was an increased CMAP amplitude and an incremental response to 10-20-HZ RNST. Muscle Nerve 40: 271-278, 2009.

An outbreak of botulism in Thailand: clinical manifestations and management of severe respiratory failure.

BACKGROUND: Northern Thailand's biggest botulism outbreak to date occurred on 14 March 2006 and affected 209 people. Of these, 42 developed respiratory failure, and 25 of those who developed respiratory failure were referred to 9 high facility hospitals for treatment of severe respiratory failure and autonomic nervous system involvement. Among these patients, we aimed to assess the relationship between the rate of ventilator dependence and the occurrence of treatment by day 4 versus day 6 after exposure to bamboo shoots (the source of the botulism outbreak), as well as the relationship between ventilator dependence and negative inspiratory pressure. METHODS: We reviewed the circumstances and timing of symptoms following exposure. Mobile teams treated patients with botulinum antitoxin on day 4 or day 6 after exposure in Nan Hospital (Nan, Thailand). Eighteen patients (in 7 high facility hospitals) with severe respiratory failure received a low- and high-rate repetitive nerve stimulation test, and negative inspiratory pressure was measured. RESULTS: Within 1-65 h after exposure, 18 of the patients with severe respiratory failure had become ill. The typical clinical sequence was abdominal pain, nausea and/or vomiting, diarrhea, dysphagia and/or dysarthria, ptosis, diplopia, generalized weakness, urinary retention, and respiratory failure. Most patients exhibited fluctuating pulse and blood pressure. Repetitive nerve stimulation test showed no response in the most severe stage. In the moderately severe stage, there was a low-amplitude compound muscle action potential with a low-rate incremented/high-rate decremented response. In the early recovery phase, there was a low-amplitude compound muscle action potential with low- and high-rate incremented response. In the ventilator-weaning stage, there was a normal-amplitude compound muscle action potential. Negative inspiratory pressure variation among 14 patients undergoing weaning from mechanical ventilation was observed. Kaplan-Meier survival analysis identified a shorter period of ventilator dependency among patients receiving botulinum antitoxin on day 4 (P=.02). CONCLUSIONS: Patients receiving botulinum antitoxin on day 4 had decreased ventilator dependency. In addition, for patients with foodborne botulism, an effective referral system and team of specialists are needed.

A 12-case series of Penicillium marneffei pneumonia.

BACKGROUND: Penicillium marneffei, an endemic fungus in Southeast Asia and southern China, is the cause of opportunistic infection in HIV-infected patients who may present with symptoms and signs of the lungs, and abnormal chest radiographs. However, only a few cases of pulmonary infection from this organism have been reported. OBJECTIVE: To study the clinical manifestations of patients with Penicillium marneffei pneumonia diagnosed by sputum or bronchoalveolar lavage (BAL) fluid culture MATERIAL AND METHOD: Retrospective descriptive study of patients who were diagnosed with Penicillium marneffei pneumonia at Maharaj Nakorn Chiang Mai Hospital from September 1999 to July 2004. RESULTS: Twelve patients (eight males, four females) were included with mean age of 36.1 years. Nine cases were HIV-infected. Their presenting symptoms included fever, cough, dyspnea and weight loss. Skin lesions, hepatomegaly and lymphadenopathy were extrapulmonary signs. Chest radiographs revealed diffuse reticulonodular, diffuse reticular, localized alveolar, localized reticular infiltration, and cavitary lesion. The diagnosis was made by cultures from the sputum in five cases and BAL fluid in the others. Co-infections with Streptococcus pneumoniae, Klebsiella pneumoniae, Mycobacterium tuberculosis, Cryptococcus neoformans, and Strongyloides stercoralis were found. Most of them were treated by intravenous amphotericin B followed by oral itraconazole, or oral itraconazole. CONCLUSION: Penicillium marneffei pneumonia has non-specific clinical manifestations, it cannot be excluded from other infections and may have co-infections. Physicians should include this infection in their differential diagnosis especially in immunocompromised patients.