MyMedR

Survival and prognostic factors of motor neuron disease in a multi-ethnic Asian population

Goh KJ, Tian S, Shahrizaila N, Ng CW, Tan CT

Amyotroph Lateral Scler, 2011 Mar;12(2):124-9.

PMID: 21039118 DOI: 10.3109/17482968.2010.527986

Our objective was to determine the survival and prognostic factors of motor neuron disease (MND) in a multi-ethnic cohort of Malaysian patients. All patients seen at a university medical centre between January 2000 and December 2009 had their case records reviewed for demographic, clinical and follow-up data. Mortality data, if unavailable from records, were obtained by telephone interview of relatives or from the national mortality registry. Of the 73 patients, 64.4% were Chinese, 19.2% Malays and 16.4% Indians. Male: female ratio was 1.43: 1. Mean age at onset was 51.5 + 11.3 years. Onset was spinal in 75.3% and bulbar in 24.7% of the patients; 94.5% were ALS and 5.5% were progressive muscular atrophy (PMA). Overall median survival was 44.9 + 5.8 months. Ethnic Indians had shorter interval from symptom onset to diagnosis and shorter median survival compared to non-Indians. On Cox proportional hazards analysis, poor prognostic factors were bulbar onset, shorter interval from symptom onset to diagnosis and worse functional score at presentation. In conclusion, age of onset and median survival duration are similar to previous reports in Asians. Clinical features and prognostic factors are similar to other populations. In our cohort, ethnic Indians had more rapid disease course accounting for their shorter survival.

Myopathic dropped head syndrome: a syndrome of mixed aetiology

Goh KJ, Wong KT, Tan CT

J Clin Neurosci, 2000 Jul;7(4):334-6.

PMID: 10938615

We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.

Fulltext Clinical features of Nipah virus encephalitis among pig farmers in Malaysia

Goh KJ, Tan CT, Chew NK, Tan PS, Kamarulzaman A, Sarji SA, et al.

N Engl J Med, 2000 Apr 27;342(17):1229-35.

PMID: 10781618 DOI: 10.1056/NEJM200004273421701

BACKGROUND: Between September 1998 and June 1999, there was an outbreak of severe viral encephalitis due to Nipah virus, a newly discovered paramyxovirus, in Malaysia.
METHODS: We studied the clinical features of the patients with Nipah virus encephalitis who were admitted to a medical center in Kuala Lumpur. The case definition was based on epidemiologic, clinical, cerebrospinal fluid, and neuroimaging findings.
RESULTS: Ninety-four patients with Nipah virus infection were seen from February to June 1999 (mean age, 37 years; ratio of male patients to female patients, 4.5 to 1). Ninety-three percent had had direct contact with pigs, usually in the two weeks before the onset of illness, suggesting that there was direct viral transmission from pigs to humans and a short incubation period. The main presenting features were fever, headache, dizziness, and vomiting. Fifty-two patients (55 percent) had a reduced level of consciousness and prominent brain-stem dysfunction. Distinctive clinical signs included segmental myoclonus, areflexia and hypotonia, hypertension, and tachycardia and thus suggest the involvement of the brain stem and the upper cervical spinal cord. The initial cerebrospinal fluid findings were abnormal in 75 percent of patients. Antibodies against Hendra virus were detected in serum or cerebrospinal fluid in 76 percent of 83 patients tested. Thirty patients (32 percent) died after rapid deterioration in their condition. An abnormal doll's-eye reflex and tachycardia were factors associated with a poor prognosis. Death was probably due to severe brain-stem involvement. Neurologic relapse occurred after initially mild disease in three patients. Fifty patients (53 percent) recovered fully, and 14 (15 percent) had persistent neurologic deficits.
CONCLUSIONS: Nipah virus causes a severe, rapidly progressive encephalitis with a high mortality rate and features that suggest involvement of the brain stem. The infection is associated with recent contact with pigs.

Oculopharyngeal muscular dystrophy with PABPN1 mutation in a Chinese Malaysian woman

Goh KJ, Wong KT, Nishino I, Minami N, Nonaka I

Neuromuscul Disord, 2005 Mar;15(3):262-4.

PMID: 15725589

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disorder of middle age presenting as progressive dysphagia and eyelid ptosis, due to short expansions of the GCG trinucleotide repeat (from GCG6 to GCG8-13) in the polyadenylate binding-protein nuclear 1 (PABPN1) gene. OPMD is rarely seen in Asians and morphologically and/or genetically confirmed cases have been reported in Japanese kindreds only. We report a 64 year old Chinese-Malaysian woman who presented with progressive dysphagia and bilateral ptosis for about 6 years. Her mother and elder brother (both deceased) were believed to be affected. Muscle histopathology revealed angulated fibres with rimmed vacuoles. Genetic analysis showed repeat expansion in one allele to (GCG)9 while normal in the other (GCG)6. This is the first non-Japanese Asian family with genetically confirmed OPMD.

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

Goh KJ, Abdullah S, Wong WF, Yeap SS, Shahrizaila N, Tan CT

Neurology Asia, 2014;19(4):409-412.

MyJurnal

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.

Effects of demographic and physical factors on nerve conduction study values of healthy subjects in a multi-ethnic Asian population

Fong SY, Goh KJ, Shahrizaila N, Wong KT, Tan CT

Muscle Nerve, 2016 08;54(2):244-8.

PMID: 26790132 DOI: 10.1002/mus.25029

INTRODUCTION: We investigated the effects of demographic and physical factors on nerve conduction studies (NCS) in a multi-ethnic Asian population.

METHODS: One hundred sixty-six healthy Malaysians of different ethnicities (51.2% women, aged 21-77 years) underwent NCS using a standard protocol. Correlations of various factors to NCS were determined, and multiple linear regression analysis was used to develop predictive equations for each parameter.

RESULTS: Age and ethnicity were the commonest independent factors influencing NCS followed by gender, height, weight, and body mass index. Increasing age predicted a reduction in lower limb motor and all sensory nerve action potential amplitudes and decrease in motor and sensory (except sural) conduction velocities. Ethnic Indians had slower motor and sensory conduction velocities in several nerves and also had differences in action potential amplitudes.

CONCLUSIONS: NCS parameters in multi-ethnic Malaysians were influenced independently by various demographic and physical factors, including ethnicity. Muscle Nerve 54: 244-248, 2016.

Systemic lupus erythematosus may have an early effect on peripheral nerve function in patients without clinical or electrophysiological neuropathy: comparison with age- and gender-matched controls

Fong SY, Raja J, Wong KT, Goh KJ

Rheumatol Int, 2021 02;41(2):355-360.

PMID: 32488429 DOI: 10.1007/s00296-020-04610-8

Asymptomatic electrophysiological peripheral neuropathy is described in systemic lupus erythematosus (SLE) patients. To determine if SLE could have an even earlier effect on peripheral nerve function even before the development of electrophysiological abnormalities, we compared nerve conduction studies (NCS) of SLE patients without electrophysiological or clinical peripheral neuropathy with healthy controls. Consecutive SLE patients without clinical neuropathy (or other known causes of neuropathy) underwent sensory and motor NCS of all four limbs. Results of 61 patients without electrophysiological criteria of neuropathy were compared with age- and gender-matched controls. Although still within the laboratory's range of normal values, significant differences were found in several NCS parameters between patients and controls. SLE patients had lower amplitudes for ulnar, fibular, and tibial compound muscle action potentials (CMAP) and sural sensory nerve action potentials (SNAP); slower conduction velocities for median, ulnar, and fibular motor nerves, and median, ulnar and sural sensory nerves. SLE patients also had longer minimum F-wave latencies for median, ulnar, fibular, and tibial nerves. H reflexes were more often absent in patients. Correlations were found between the number of disease relapses and motor conduction velocities of the fibular and tibial nerves. SLE may have early effect on peripheral nerve function in patients even before they develop electrophysiological or clinical neuropathy.

Longitudinal extensive transverse myelitis with cervical epidural haematoma following dengue virus infection

Fong CY, Hlaing CS, Tay CG, Abdul Kadir KA, Goh KJ, Ong LC

Eur. J. Paediatr. Neurol., 2016 May;20(3):449-53.

PMID: 26900103 DOI: 10.1016/j.ejpn.2016.01.012

Longitudinal extensive transverse myelitis associated with dengue infection is rare with no reported paediatric cases.

Bickerstaff's brainstem encephalitis with overlapping Guillain-Barré syndrome: Usefulness of sequential nerve conduction studies

Fong CY, Aung HWW, Khairani A, Gan CS, Shahrizaila N, Goh KJ

Brain Dev, 2018 Jun;40(6):507-511.

PMID: 29459060 DOI: 10.1016/j.braindev.2018.02.001

Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.

Fulltext A survey on patients' disease perception and the impact of the COVID-19 pandemic on persons living with amyotrophic lateral sclerosis in Malaysia

Edgar S, Abdul-Aziz NA, Loh EC, Capelle D, Goh KJ, Latif LA, et al.

Neurodegener Dis Manag, 2021 08;11(4):307-314.

PMID: 34284643 DOI: 10.2217/nmt-2021-0004

Aim: To investigate the patients' perception of their disease, its management and the impact of the COVID-19 pandemic on persons living with amyotrophic lateral sclerosis (ALS) in Malaysia. Patients & methods: An online survey comprising 42 questions was conducted on ALS patients during the peak of the COVID-19 pandemic. Results: Responses were received from 37/60 (62%) participants with ALS directly or through their caregivers. During the COVID-19 pandemic, two-thirds of patients were negatively impacted by the sudden disruption to their hospital appointments, rehabilitation sessions and reduced social interactions. Conclusion: This study provided insight into patients' perception of their care and management of ALS in Malaysia which will facilitate in implementing changes that can improve care to persons living with this devastating illness.

Mutation analysis of SOD1, C9orf72, TARDBP and FUS genes in ethnically-diverse Malaysian patients with amyotrophic lateral sclerosis (ALS)

Edgar S, Ellis M, Abdul-Aziz NA, Goh KJ, Shahrizaila N, Kennerson ML, et al.

Neurobiol Aging, 2021 12;108:200-206.

PMID: 34404558 DOI: 10.1016/j.neurobiolaging.2021.07.008

Recent studies have identified SOD1, FUS, TARDBP and C9orf72 as major ALS-related genes in both European and Asian populations. However, significant differences exist in the mutation frequencies of these genes between various ancestral backgrounds. This study aims to identify the frequency of mutations in the common causative ALS genes in a multi-ethnic Malaysian cohort. We screened 101 Malaysian ALS patients including 3 familial and 98 sporadic cases for mutations in the coding regions of SOD1, FUS, and TARDBP by Sanger sequencing. The C9orf72 hexanucleotide repeat expansion was screened using the repeat-primed polymerase chain reaction assay. Mutations were found in 5.9% (6 of 101) of patients including 3.0% (3 of 101) of patients with the previously reported SOD1 missense mutations (p.V48A and p.N87S) and 3.0% (3 of 101) of patients with the C9orf72 repeat expansion. No mutations were found in the FUS and TARDBP genes. This study is the first to report the mutation frequency in an ethnically diverse Malaysian ALS population and warrants further investigation to reveal novel genes and disease pathways.

The presence of Nipah virus in respiratory secretions and urine of patients during an outbreak of Nipah virus encephalitis in Malaysia

Chua KB, Lam SK, Goh KJ, Hooi PS, Ksiazek TG, Kamarulzaman A, et al.

J Infect, 2001 Jan;42(1):40-3.

PMID: 11243752

To study the excretion of Nipah virus in the upper respiratory secretions and urine of infected patients in relation to other clinical features.

Fatal encephalitis due to Nipah virus among pig-farmers in Malaysia

Chua KB, Goh KJ, Wong KT, Kamarulzaman A, Tan PS, Ksiazek TG, et al.

Lancet, 1999 Oct 9;354(9186):1257-9.

PMID: 10520635

Between February and April, 1999, an outbreak of viral encephalitis occurred among pig-farmers in Malaysia. We report findings for the first three patients who died.

High mortality in Nipah encephalitis is associated with presence of virus in cerebrospinal fluid

Chua KB, Lam SK, Tan CT, Hooi PS, Goh KJ, Chew NK, et al.

Ann Neurol, 2000 Nov;48(5):802-5.

PMID: 11079547

During the outbreak of Nipah virus encephalitis in Malaysia, stored cerebrospinal fluid (CSF) samples from 84 patients (27 fatal and 57 nonfatal cases) were cultured for the virus. The virus was isolated from 17 fatal cases and 1 nonfatal case. There were significant associations between CSF virus isolation and mortality as well as clinical features associated with poor prognosis. In addition, there was a positive linear correlation of CSF virus isolation with age. There was no significant association between CSF virus isolation and the character of the CSF, presence of Nipah-specific antibody in the serum or CSF, duration of illness before collection of samples, or sex or ethnicity of the patients. This study suggests that high viral replication in the central nervous system may be an important factor for high mortality.

Treatment of acute Nipah encephalitis with ribavirin

Chong HT, Kamarulzaman A, Tan CT, Goh KJ, Thayaparan T, Kunjapan SR, et al.

Ann Neurol, 2001 Jun;49(6):810-3.

PMID: 11409437

Nipah virus, a newly identified paramyxovirus caused a severe outbreak of encephalitis in Malaysia with high fatalities. We report an open-label trial of ribavirin in 140 patients, with 54 patients who were managed prior to the availability of ribavirin or refused treatment as control. There were 45 deaths (32%) in the ribavirin arm; 29 deaths (54%) occurred in the control arm. This represents a 36% reduction in mortality (p = 0.011). There was no associated serious side effect. This study suggests that ribavirin is able to reduce the mortality of acute Nipah encephalitis.

Idiopathic paroxysmal kinesigenic choreoathetosis: precipitation of attacks by vestibular stimulation

Chew NK, Tan CT, Goh KJ

J Clin Neurosci, 2002 Sep;9(5):604-5.

PMID: 12383430

A 24-year-old woman presented with a 3.5-year history of paroxysmal dystonia that was precipitated by sudden movement, especially when she started to walk. It was characterised by shrugging of shoulders, flexion of the neck and thoracic spine, and stiffness of the right leg followed by falls. Each attack lasted for less than 5min. Inadequate sleep and stress were exacerbating factors. There was no similar family history. Physical examination and investigations were normal. The following manoeuvres that caused vestibular stimulation precipitated attacks: turning her head from side to side while standing still, sitting still on a rotating chair and an ice-water caloric test. She had partial responses to phenytoin and levodopa, and a good response to haloperidol. Vestibular stimulation as a precipitating factor in paroxysmal kinesigenic choreoathetosis has not been reported previously.

Delayed post-irradiation bulbar palsy in nasopharyngeal carcinoma

Chew NK, Sim BF, Tan CT, Goh KJ, Ramli N, Umapathi P

Neurology, 2001 Aug 14;57(3):529-31.

PMID: 11502928

In a hospital series of 70 patients on follow-up after radiotherapy for nasopharyngeal carcinoma, 14 patients (20%) developed delayed post-irradiation bulbar palsy 1 to 18 years after radiotherapy (mean 5.5 years). Functional disability was moderate to severe. Three patients had aspiration pneumonia with one mortality. Post-irradiation bulbar palsy was a common complication and probably resulted from direct neuronal damage.

Acute flaccid paralysis with chronic cough

Chai CH, Yuki N, Nor HM, Goh KJ, Shahrizaila N

Pract Neurol, 2012 Oct;12(5):328-31.

PMID: 22976064 DOI: 10.1136/practneurol-2011-000205

Fulltext Soil CO2 and CH4 fluxes from different forest types in tropical peat swamp forest

Busman NA, Melling L, Goh KJ, Imran Y, Sangok FE, Watanabe A

Sci Total Environ, 2023 Feb 01;858(Pt 2):159973.

PMID: 36347298 DOI: 10.1016/j.scitotenv.2022.159973

Information on temporal and spatial variations in soil greenhouse gas (GHG) fluxes from tropical peat forests is essential to predict the influence of climate change and estimate the effects of land use on global warming and the carbon (C) cycle. To obtain such basic information, soil carbon dioxide (CO2) and methane (CH4) fluxes, together with soil physicochemical properties and environmental variables, were measured at three major forest types in the Maludam National Park, Sarawak, Malaysia, for eight years, and their relationships were analyzed. Annual soil CO2 fluxes ranged from 860 to 1450 g C m⁻2 yr⁻1 without overall significant differences between the three forest sites, while soil CH4 fluxes, 1.2-10.8 g C m⁻2 yr⁻1, differed. Differences in GHG fluxes between dry and rainy seasons were not necessarily significant, corresponding to the extent of seasonal variation in groundwater level (GWL). The lack of significant differences in soil CO2 fluxes between the three sites could be attributed to set-off between the negative and positive effects of the decomposability of soil organic matter as estimated by pyrophosphate solubility index (PSI) and GWL. The impact of El-Niño on annual CO2 flux also varied between the sites. The variation in soil CH4 fluxes from the three sites was enhanced by variations in temperature, GWL, PSI, and soil iron (Fe) content. A positive correlation was observed between the annual CH4 flux and GWL at only one site, and the influence of soil properties was more pronounced at the site with the lowest GWL and the highest PSI. Variation in annual CH4 fluxes was controlled more strongly by temperature where GWL was the highest and GWL and plant growth fluctuations were the least. Inter-annual variations in soil CO2 and CH4 fluxes confirmed the importance of long-term monitoring of these at multiple sites supporting different forest types.

Fulltext Corrigendum to "Soil CO2 and CH4 fluxes from different forest types in tropical peat swamp forest" [Sci. Total Environ. 858 (2023) 159973]

Busman NA, Melling L, Goh KJ, Imran Y, Sangok FE, Watanabe A

Sci Total Environ, 2024 Feb 20;912:168867.

PMID: 38040577 DOI: 10.1016/j.scitotenv.2023.168867

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