Determining the Utility of the Guillain-Barré Syndrome Classification Criteria

Background@#and Purpose Several variants of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) exist, but their frequencies vary in different populations and do not always meet the inclusion criteria of the existing diagnostic criteria. However, the GBS classification criteria by Wakerley and colleagues recognize and define the clinical characteristics of each variant. We applied these criteria to a GBS and MFS cohort with the aim of determining their utility. @*Methods@#Consecutive GBS and MFS patients presenting to our center between 2010 and 2020 were analyzed. The clinical characteristics, electrophysiological data, and antiganglioside antibody profiles of the patients were utilized in determining the clinical classification. @*Results@#This study classified 132 patients with GBS and its related disorders according to the new classification criteria as follows: 64 (48.5%) as classic GBS, 2 (1.5%) as pharyngeal-cervical-brachial (PCB) variant, 7 (5.3%) as paraparetic GBS, 29 (22%) as classic MFS, 3 (2.3%) as acute ophthalmoparesis, 2 (1.5%) as acute ataxic neuropathy, 2 (1.5%) as Bickerstaff brainstem encephalitis (BBE), 17 (12.9%) as GBS/MFS overlap, 4 (3%) as GBS/BBE overlap, 1 (0.8%) as MFS/PCB overlap, and 1 (0.8%) as polyneuritis cranialis. The electrodiagnosis was demyelinating in 55% of classic GBS patients but unclassified in 79% of classic MFS patients. Anti-GM1, anti-GD1a, anti-GalNAc-GD1a, and anti-GD1b IgG ganglioside antibodies were more commonly detected in the axonal GBS subtype, whereas the anti-GQ1b and anti-GT1a IgG ganglioside antibodies were more common in classic MFS and its subtypes. @*Conclusions@#Most of the patients in the present cohort met the criteria of either classic GBS or MFS, but variants were seen in one-third of patients. These findings support the need to recognize variants of both syndromes in order to achieve a more-complete case ascertainment in GBS.

Determining the Utility of the Guillain-Barré Syndrome Classification Criteria

Background@#and Purpose Several variants of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) exist, but their frequencies vary in different populations and do not always meet the inclusion criteria of the existing diagnostic criteria. However, the GBS classification criteria by Wakerley and colleagues recognize and define the clinical characteristics of each variant. We applied these criteria to a GBS and MFS cohort with the aim of determining their utility. @*Methods@#Consecutive GBS and MFS patients presenting to our center between 2010 and 2020 were analyzed. The clinical characteristics, electrophysiological data, and antiganglioside antibody profiles of the patients were utilized in determining the clinical classification. @*Results@#This study classified 132 patients with GBS and its related disorders according to the new classification criteria as follows: 64 (48.5%) as classic GBS, 2 (1.5%) as pharyngeal-cervical-brachial (PCB) variant, 7 (5.3%) as paraparetic GBS, 29 (22%) as classic MFS, 3 (2.3%) as acute ophthalmoparesis, 2 (1.5%) as acute ataxic neuropathy, 2 (1.5%) as Bickerstaff brainstem encephalitis (BBE), 17 (12.9%) as GBS/MFS overlap, 4 (3%) as GBS/BBE overlap, 1 (0.8%) as MFS/PCB overlap, and 1 (0.8%) as polyneuritis cranialis. The electrodiagnosis was demyelinating in 55% of classic GBS patients but unclassified in 79% of classic MFS patients. Anti-GM1, anti-GD1a, anti-GalNAc-GD1a, and anti-GD1b IgG ganglioside antibodies were more commonly detected in the axonal GBS subtype, whereas the anti-GQ1b and anti-GT1a IgG ganglioside antibodies were more common in classic MFS and its subtypes. @*Conclusions@#Most of the patients in the present cohort met the criteria of either classic GBS or MFS, but variants were seen in one-third of patients. These findings support the need to recognize variants of both syndromes in order to achieve a more-complete case ascertainment in GBS.

Neuropathic Pain after Adolescent Idiopathic Scoliosis Correction Surgery

Methods@#Forty AIS patients were recruited. Demographic, preoperative, and postoperative data were recorded. The magnitude and characteristics of postoperative pain were assessed using the painDETECT questionnaire through telephone enquiries at intervals of 2, 6, 12, and 24 weeks. Statistical analyses were followed by Pearson correlation test to determine the relationship between pain scores at 6, 12, and 24 weeks with the risk factors. @*Results@#Based on the painDETECT questionnaire, 90% of the patients had nociceptive pain, and 10% had a possible neuropathic pain component at 2 weeks postoperatively as per a mean painDETECT score of 7.1±4.5. Assessments at 6, 12, and 24 weeks showed that no patients had neuropathic pain with painDETECT scores of 4.4±3.2, 2.9±2.9, and 1.5±2.0, respectively. There was a significant correlation between total postoperative morphine use during 48 hours after the surgery and a tendency to develop neuropathic pain (p=0.022). @*Conclusions@#Chronic neuropathic pain was uncommon in AIS patients who had undergone PSF surgery. Higher opioid consumption will increase the possibility of developing chronic neuropathic pain.

Effect of mindfulness meditation on brain-computer interface: fMRI perspective

@#This study observed the functional changes in brain activity while performing real and imagery movement using functional MRI (fMRI); and to compare the fMRI changes of motor imagery before and after mindfulness meditation (MM) training for correlation with actual brain computer interface (BCI) performance. Thirty-eight participants completed a randomized control trial consisting of 2 groups (MM and non-intervention control groups) to study the effect of MM on BCI performance. The MM group participated in a 4-week MM intervention programme. Out of the 38 cohorts, five participants from the MM group and five from the control group were fMRI scanned for real and imagery movement of right hand, left hand and both feet, before and after intervention. Statistical parametric mapping was used for post processing and analysis of fMRI data. The MM group showed a significant improvement in BCI performance compared to the control group. The fMRI results showed activation of right hand, left hand and both feet motor imagery at fronto-parietal regions before MM training (p <0.05, family wise error). After MM training, the fMRI results revealed a focused activation in 3 out of 4 of the trained subjects during right hand motor imagery, 2 out of 4 of the trained subjects during both feet motor imagery and 1 out of 4 of the trained subjects during left hand motor imagery, compared to the control group. This is also correlated with the improvement of BCI accuracy of the intervention group after MM training. Mindfulness meditation improves BCI performance and is correlated with focused activation of the fronto-parietal region in fMRI during motor imagery.

Predictors of functional outcome in patients with stroke thrombolysis in a tertiary hospital in Malaysia

INTRODUCTION@#Intravenous (IV) thrombolysis with alteplase (rt-PA) is effective in ischaemic stroke. The primary objective was to evaluate predictors of functional outcome in acute ischaemic stroke (AIS) patients treated with IV rt-PA. The secondary objective was to assess the outcome with the modified Rankin scale (mRS). We also examined the predictive value of the Totaled Health Risks in Vascular Events (THRIVE) score.@*METHODS@#AIS patients treated with IV rt-PA from February 2012 to August 2016 were recruited. Demographic data, National Institutes of Health Stroke Scale (NIHSS) scores, timing and neuroradiological findings were recorded. Patients received a dose of 0.9 mg/kg IV rt-PA within 4.5 hours of symptom onset. mRS score was evaluated at discharge and three months, and good and poor clinical outcomes were defined as scores of 0-2 and 3-6, respectively. Baseline THRIVE scores were assessed.@*RESULTS@#36 patients received IV rt-PA. 20 (55.6%) patients had an mRS score of 0-2 at three months. Based on THRIVE score, 86.1% had a good or moderately good prognosis. On univariate analysis, poor outcome was associated with NIHSS score before rt-PA (p = 0.03), THRIVE score (p = 0.02), stroke subtype (p = 0.049) and diabetes mellitus (DM; p = 0.06). Multiple logistic regression showed that outcome was significantly associated with NIHSS score before rt-PA (p = 0.032) and DM (p = 0.010).@*CONCLUSION@#Our newly developed Malaysian IV rt-PA service is safe, with similar outcomes to the published literature. Functional outcome after thrombolysis was associated with baseline NIHSS score and DM.

Chorea in Sporadic Creutzfeldt-Jakob Disease

No abstract available.

MR neurography of median nerve using diffusion tensor imaging (DTI) and its efficacy to diagnose carpal tunnel syndrome in Malaysian population

@#Objective: This study evaluates the feasibility of diffusion tensor imaging(DTI) in assessing median nerve by measuring diffusion parameters such as fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD) at different sites of median nerve and evaluating their differences in patients with and without carpal tunnel syndrome (CTS) in local setting. Methods: A prospective cross sectional study was performed with 9 female patients diagnosed with CTS by clinical evaluation and nerve conduction study and 8 age and sex matched normal patients. Magnetic resonance imaging (MRI) wrist was performed with pre-set axial PD and DTI protocol on a 3T MRI, images post-processed using 3D SLICER software to generate median nerve tract and measure diffusion parameters FA, MD, AD and RD in segments and focal points. Results: The FA values were significantly lower in CTS patients, 0.454 (± 0.065), p< 0.002 and demonstrates negative correlation with disease severity, r = - 0.510, p = 0.002.The mean MD, 1.090 (± 0.178) and mean RD, 0.834 (± 0.128) is higher in CTS patients, p = 0.041 and p = 0.014 respectively. They show an increasing trend with increasing disease severity. Negative correlation was noted between the FA values and age groups. FA cut of value of ≤ 0.487 with sensitivity 70.6 % and specificity 76.5%, is suggested for diagnosing CTS.Conclusion: MR neurography using DTI can be utilised to detect CTS. Patients with CTS demonstrate lower FA and higher MD and RD values.

The prevalence and associated factors of neuropathic pain symptoms in a cohort of multi-ethnic Malaysian patients with diabetes mellitus

Objective: To determine prevalence and factors associated with neuropathic pain symptoms in a multiethniccohort of Malaysian adult diabetic patients. Methods: This was aprospective cross-sectionalobservational study of hospital-based diabetic outpatients in Malaysia. Subjects were interviewedfor their demographic data and medical history. The painDETECT questionnaire was used to screenfor neuropathic pain symptoms and pain intensity was assessed using the numeric pain rating scale(NPRS). Neuropathy symptoms and signs were assessed using the Neuropathy Symptom Score(NSS) and Neuropathy Disability Score (NDS). Results:Of 242 patients,140 (58%) were women,with a mean age of 61 + 11.4 years (range 21 to 81). Ninety nine(40.9%) were Malay, 64 (26.4%)Chinese, 76 (31.4%) Indian and three (1.2%) were Eurasian. Mean duration of diabetes was 15.9+ 9.8years (range 1 to 53) and 232 (95.9%) patients had Type II diabetes. Peripheral neuropathy,based onNSS and NDS criteria, was found in 83 (34.3%). Thirteen (5.4%) patients were found to likely haveneuropathic pain symptoms and this was independently associated with peripheral neuropathy ((OR)= 3.40, 95% confidence interval (CI): 1.04, 11.14) and Indian ethnicity (OR = 5.44, 95% CI: 1.50,19.57)). Patients with neuropathic pain had higher average pain intensity scores.Conclusions: The prevalence of neuropathic pain symptoms in a Malaysian DM patient cohort waslow and was associated with the severity of neuropathy symptoms and Indian ethnicity. The causesfor ethnic differences are unknown and could be due socio-cultural or physiological differences inneuropathic pain perception.

Spontaneous remission without progression to limbic encephalitis in a patient with LGi1 seropositive faciobrachial dystonic seizure

Faciobrachial dystonic seizures are pathognomonic of leucine-rich glioma inactivated-1 (LGi1) antibody, non-paraneoplastic limbic encephalitis. Faciobrachial dystonic seizures usually precede limbic encephalitis by about a month. It is unknown whether, if untreated, faciobrachial dystonic seizures inevitably progress to limbic encephalitis. We present an LGi1 seropositive patient with a year’s history of faciobrachial dystonic seizures, who achieved remission spontaneously without immunotherapy or antiepileptic drug treatment, and did not develop evidence of limbic encephalitis over a three-year follow-up.

Cold allodynia as the presenting symptom in a case of acquired neuromyotonia (Isaacs syndrome) with multiple autoantibodies

We report a patient who presented with severe cold-induced allodynia and hyperhidrosis, and found to have acquired neuromyotonia (Isaacs syndrome) with high voltage-gated potassium channel (VGKC) antibody titre,positive contactin-associated protein 2 (CASPR2) and leucine-rich glioma-inactivated 1 (LGI1) antibodies. The patient also had positive anti-dsDNA and acetylcholine receptor (AChR) antibodies without clinical features of SLE or myasthenia gravis, suggesting a strong underlying autoimmune tendency. CT thorax showed no thymoma. Her symptoms improved with intravenous immunoglobulin infusion but recurred despite maintenance oral corticosteroids and carbamazepine. She has since been on regular IVIG infusions. Cold allodynia is an unusual presentation in acquired neuromyotonia.

Single mitochondrial DNA deletions in chronic progressive external ophthalmoplegia (CPEO) and Kearns-Sayre syndrome (KSS) patients from a multiethnic Asian population

Mitochondrial DNA (mtDNA) deletions are a major cause of chronic progressive external ophthalmoplegia (CPEO) and Kearns-Sayre syndrome (KSS). We analyzed single mtDNA deletions in 11 CPEO and one KSS patients by means of Southern blot and long polymerase chain reaction (PCR) assays. The deletion sizes ranged from 3.4 kb to 6.9 kb whereas the heteroplasmy level varied from 18.8% to 85.5%. Two unique deletions sized 4320 bp and 4717 bp were found. This study represents the first genetic screen of mtDNA disorders in Malaysia, and it follows the data seen in other published reports on CPEO and KSS genetic aetiology.

The spectrum of elderly myopathies in an Asian population

Myopathies, although presenting more commonly in the younger age group, can occur and contribute signifi cantly to disability in the elderly. To describe the spectrum of elderly myopathies, we reviewed 52 elderly patients (> 65 years) from the University of Malaya Medical Centre muscle biopsy databank, constituting 6.8% of 759 adult patients (> 18 years) who underwent muscle biopsy between 1992 and 2012. Commonest were the infl ammatory myopathies (41/52, 78.8%), of which 43.9% had dermatomyositis; 23.9% polymyositis; 14.6% sporadic inclusion body myositis; 9.8% undifferentiated myositis and 2.4% overlap myositis. Seven patients (13.4%) had genetic myopathy; 2 muscular dystrophy and 5 chronic progressive external ophthalmoplegia, while 4 patients (7.7%) had drug-associated myopathy, 3 with statins. Malignancies were seen in 9.8% of infl ammatory myopathies at diagnosis. Both acquired and genetic myopathies are seen in elderly Malaysians of all ethnicities and should not be misdiagnosed as some are potentially treatable and/or associated with malignancy.

MRI findings of orbicularis oculi hypertrophy due to heavy resistance training on the inferior orbital rim

Increase in cross-sectional muscle area of major muscle groups associated by heavy resistance training has been well documented. However, there has been no published article of changes in the inferior orbicularis oculi muscle when heavy resistance training is applied. We present a case of inferior orbicularis hypertrophy detected on MRI in a gentleman who practised heavy resistance training using his lower eyelids.

Late-onset Nipah virus encephalitis 11 years after the initial outbreak: A case report

Nipah virus infection is known to cause late-onset and relapsed encephalitis, in addition to an acute encephalitic illness. This is a report of a 35 years old woman, who had exposure to the Nipah virus infection during the 1999 Malaysian outbreak, was positive for Nipah IgG by immunofl uorescence, and had multiple small hyperintense lesions in brain MRI typically seen in acute Nipah encephalitis patients, indicating asymptomatic Nipah virus infection. She subsequently developed acute encephalitis after 11 years, manifesting as diplopia, internuclear opthalmoplegia and epileptic seizures with pleocytosis in cerebrospinal fl uid examination. She had another episode of relapsed encephalitis a year later, with seizures, memory impairment, chorea and new lesions in MRI brain. This patient is unusual in the long incubation of 11 years before manifesting with late-onset Nipah encephalitis.

The frequency of common mitochondrial DNA mutations in a cohort of Malaysian patients with specifi c mitochondrial encephalomyopathy syndromes

A cohort of Malaysian patients with clinico-pathological diagnosis of three specifi c mitochondrial encephalomyopathy syndromes comprising of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS), myoclonus epilepsy with ragged-red fi bers (MERRF) and Leigh syndrome were studied to determine the frequency of their common mitochondrial DNA mutations. The ‘hot-spot’ point mutations for MELAS, MERRF and Leigh syndrome were screened. In the absence of common point mutations, screening of large-scale deletions as well as sequencing of tRNALeu and tRNALys genes were performed. Of 22 patients studied, nine m.3243A>G mutations, four m.8344A>G mutations, one m.8993T>G mutation and one deletion were identifi ed (65% detection rate). While the m.3243A>G mutation was closely associated with MELAS, the m.8344A>G was more heterogenous, being seen in one MERFF, two isolated mitochondrial myopathies and one Leigh syndrome patient. Screening for m.8993T>G in Leigh syndrome has a low yield as unsurprisingly Leigh syndrome has considerable genetic heterogeneity.

Karaoke, power failure and carbon monoxide poisoning

Accidental carbon monoxide poisoning in countries with cold climates is commonly related to indoor heating. This condition appears to be relatively uncommon in tropical Asian countries and therefore the diagnosis may be unsuspected. We report a case of a Malaysian patient who presented with a severe, and ultimately fatal, delayed (biphasic) neuropsychiatric syndrome due to carbon monoxide poisoning. The diagnosis was made only when a history compatible with carbon monoxide poisoning subsequently surfaced, and neuroimaging demonstrated the typical pallidal lesions, associated with marked leukoencephalopathy, seen in this condition. Our case is unique because the poisoning occurred in the setting of indoor operation of a portable electricity generator in a karaoke centre because of power failure. Karaoke is a highly popular form of entertainment in many parts of Asia and we suggest that a high index of suspicion of carbon monoxide poisoning is required in this setting.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis: A series of ten cases from a university hospital in Malaysia

Objective: To report on the incidence, and the clinical and laboratory features of patients seen at the University of Malaya Medical Centre with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Methods: The charts of all patients admitted to the adult neurology ward with encephalitis over an 18- month period from January 2010 to June 2011 were reviewed. Diagnosis of anti-NMDAR encephalitis was based on the presence of encephalitis plus antibody against the NMDAR. Two other paediatric patients with anti-NMDAR encephalitis seen over the same period were also included in this report. Results: There was a total of 10 patients with anti-NMDAR encephalitis seen over the study period. The mean age was 18.1 years (range 9-29 years). Eight patients were female, two male. Five were Malay and fi ve were Chinese. All patients had prominent psychiatric symptoms, followed by epileptic seizures. Nine patients had a movement disorder, orofacial dyskinesia being the commonest, and all had autonomic involvement. None had an underlying tumour. Treatments consisted of corticosteroid, plasma exchange and intravenous immunoglobulin (IVIG). The clinical outcome was variable, with full recovery (2), substantial recovery (3), partial recovery (4), and mortality (1) seen. Remarkably, the eight adult cases of anti-NMDAR encephalitis accounted for 50% of the 16 cases of encephalitis seen during the study period. Conclusion: Anti-NMDAR encephalitis may be a relatively common cause of adult encephalitis among certain Asian groups. None of our cases was paraneoplastic in origin.

Ultrasonography in the evaluation of carpal tunnel syndrome: Diagnostic criteria and comparison with nerve conduction studies

Ultrasound criteria for carpal tunnel syndrome (CTS) may vary in different populations. To determine the ultrasonographic criteria for CTS in a Malaysian population and compare its usefulness with nerve conduction studies (NCS), we studied patients clinically diagnosed with CTS and normal controls by ultrasonography. All patients also underwent standard NCS. Median nerve Cross-Sectional Area (CSA) and Flattening Ratio (FR) at 3 different levels – proximal to tunnel inlet, at tunnel inlet and tunnel outlet were measured. Receiver operator characteristic (ROC) analyses were used to calculate the optimal discriminatory threshold values for CTS. Of 54 CTS hands, NCS was positive in 85.2%. Median nerve CSA at all 3 levels, were signifi cantly greater in CTS hands.FR was signifi cantly greater at tunnel inlet. A CSA threshold of 0.1 cm2 proximal to and at tunnel inlet had sensitivities of 70.4% and 63% and specifi cities of 85.2% and 88.5 % respectively. CSA at tunnel outlet had lower specifi city. If CSA of 2 levels (viz. proximal to or at tunnel inlet) were considered together, sensitivity and specifi city improved to 81.5% and 83.3%. Qualitative loss of fascicular discrimination of the nerve proximal to the inlet had sensitivity and specifi city of 77.8% and 96.3%. The most useful ultrasonographic parameter was median nerve CSA either proximal to or at tunnel inlet. However, the sensitivities were lower compared to NCS. Qualitative appearance of the median nerve is a useful adjunct to diagnosis. In conclusion, ultrasonography play an important complementary role to NCS in the diagnosis of CTS.

Development of marked striatal hand with antiparkinsonian medication withdrawal during traditional (Ayurvedic) medicine treatment for Parkinson’s disease

Ayurveda is a traditional medical system used widely in India and increasingly worldwide. Here, we report on a patient with Parkinson’s disease (PD) who developed marked striatal hand with antiparkinsonian medication withdrawal during Ayurvedic medicine treatment for her PD. Although a direct role for the Ayurvedic medicines in inducing the hand deformity cannot be excluded, we propose that severe dopaminergic defi ciency (due to prolonged withdrawal of antiparkinsonian medications in the context of long-standing PD) was probably the main culprit in our patient.

Exercise induced cramps and myoglobinuria in dystrophinopathy – a report of three Malaysian patients

Dystrophinopathies commonly present as Duchenne or Becker muscular dystrophy but rare, unusual phenotypes have also been described. We have identifi ed three Malaysian boys with an unusual form of dystrophinopathy, presenting with exercise-induced cramps and myoglobinuria, but with no apparent muscle weakness. Immunohistochemistry for dystrophin and genetic analysis confi rmed the diagnosis. The frequency of this phenotype is unknown but there have been several case reports. Consistent with these reports, we also found that two of our patients had deletions in the rod domain of dystrophin, which has been suggested to be associated with this unusual manifestation