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The value of comparing mortality of Guillain-Barré syndrome across different regions

Wong AH, Umapathi T, Shahrizaila N, Chan YC, Kokubun N, Fong MK, et al.

J Neurol Sci, 2014 Sep 15;344(1-2):60-2.
PMID: 24993467 DOI: 10.1016/j.jns.2014.06.021

To study the clinical profile of Guillain-Barré syndrome (GBS) patients who died in 4 Asian countries in order to understand factors underlying any variation in mortality.
Serial nerve conduction studies provide insight into the pathophysiology of Guillain-Barré and Fisher syndromes

Shahrizaila N, Goh KJ, Kokubun N, Abdullah S, Yuki N

J Neurol Sci, 2011 Oct 15;309(1-2):26-30.
PMID: 21849173 DOI: 10.1016/j.jns.2011.07.042

The electrodiagnosis of Guillain-Barré syndrome (GBS) can be broadly divided into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Fisher syndrome (FS) is a variant of GBS, although the underlying neuropathy of FS has yet to be established. Serial nerve conduction studies (NCS) can provide further insight into the likely pathophysiology by further subtyping of GBS and FS. We present a patient with an initial diagnosis of AIDP in whom repeated NCS revealed the AMAN variant. This led us to investigate serial NCS in five patients with GBS, FS and FS/GBS overlap presenting over a period of a year. Three patients with AIDP showed a gradual increase in distal motor latencies during the acute phase of illness. NCS of two patients with FS and FS/GBS overlap showed no demyelinating features suggesting underlying axonal neuropathy in this group of patients. The importance of serial NCS in establishing the underlying pattern of neuropathy in GBS and FS is further emphasized in this study. Larger studies incorporating serial NCS are required to confirm the observations seen in our case series especially when pathological studies are often not justified in this group of patients.
DYT1 mutations amongst adult primary dystonia patients in Singapore with review of literature comparing East and West

Jamora RD, Tan EK, Liu CP, Kathirvel P, Burgunder JM, Tan LC

J Neurol Sci, 2006 Aug 15;247(1):35-7.
PMID: 16631205

Dystonia is a heterogenous group of movement disorders whose clinical spectrum is very wide. At least 13 different genes and gene loci have been reported. While a 3-bp deletion in the DYT1 gene is the most frequent cause of early limb-onset, generalized dystonia, it has also been found in non-generalized forms of sporadic dystonia. An 18-bp deletion in the DYT1 gene has also been reported.
Comparative effects of alpha-tocopherol and gamma-tocotrienol against hydrogen peroxide induced apoptosis on primary-cultured astrocytes

Mazlan M, Sue Mian T, Mat Top G, Zurinah Wan Ngah W

J Neurol Sci, 2006 Apr 15;243(1-2):5-12.
PMID: 16442562

Oxidative stress is thought to be one of the factors that cause neurodegeneration and that this can be inhibited by antioxidants. Since astrocytes support the survival of central nervous system (CNS) neurons, we compared the effect of alpha-tocopherol and gamma-tocotrienol in minimizing the cytotoxic damage induced by H(2)O(2), a pro-oxidant. Primary astrocyte cultures were pretreated with either alpha-tocopherol or gamma-tocotrienol for 1 h before incubation with 100 microM H(2)O(2) for 24 h. Cell viability was then assessed using the MTS assay while apoptosis was determined using a commercial ELISA kit as well as by fluorescent staining of live and apoptotic cells. The uptake of alpha-tocopherol and gamma-tocotrienol by astrocytes were also determined using HPLC. Results showed that gamma-tocotrienol is toxic at concentrations >200 microM but protects against H(2)O(2) induced cell loss and apoptosis in a dose dependent manner up to 100 microM. alpha-Tocopherol was not cytotoxic in the concentration range tested (up to 750 microM), reduced apoptosis to the same degree as that of gamma-tocotrienol but was less effective in maintaining the viable cell number. Since the uptake of alpha-tocopherol and gamma-tocotrienol by astrocytes is similar, this may reflect the roles of these 2 vitamin E subfamilies in inhibiting apoptosis and stimulating proliferation in astrocytes.
Photophobia in a blind eye after removal of a progressive orbital optic glioma with denervation

Loh CK, Weis B, van Velthoven V, Reiff C, Rössler J

J Neurol Sci, 2015 Nov 15;358(1-2):522-4.
PMID: 26474792 DOI: 10.1016/j.jns.2015.09.375

Optic glioma (OPG) accounts for 4-8% of all brain tumors in children. En-block removal of intraorbital tumor is recommended in cases with disfiguring exophthalmos and impaired vision. Surgical resection of intraorbital optic nerve (ON) poses the risks of permanent ptosis and globe atrophy. We present here the case of a 4-year-old boy with exophthalmos and near blindness due to an intraorbital OPG. Despite chemotherapy he showed progressive exophthalmos and vision loss. Bony orbital decompression with ON transection temporally reduced his exophthalmos. OPG resection was required later for recurrence of his exophthalmos secondary to tumor progression. Post operatively, he had preserved oculomotor nerve functions but developed globe ischemia. Unusually, his ischemic globe caused him to have pain and severe photophobia, which later lead to enucleation. Photophobia has been reported in blind patients. Animal models and MRI functional imaging showed activation of trigeminal pathway during photophobia in completely transected ON. However, the exact neuro-ophthalmology pathway requires further study.
Delayed facial palsy in Miller Fisher syndrome

Tan CY, Yuki N, Shahrizaila N

J Neurol Sci, 2015 Nov 15;358(1-2):409-12.
PMID: 26277343 DOI: 10.1016/j.jns.2015.08.009

Miller Fisher syndrome is characterised by the triad of ophthalmoplegia, ataxia and areflexia. However, facial palsy can occur during the course of the illness although development of facial palsy when other cardinal signs of Miller Fisher syndrome have reached nadir or improving, is unusual. This delayed appearance of facial palsy can be easily overlooked by the treating clinician. Here, we report four patients with Miller Fisher syndrome and delayed-onset facial palsy. We discuss the possible underlying reasons behind the delay in facial palsy.
Fulltext Neurological research & training after the easing of lockdown in countries impacted by COVID-19

Tan EK, Albanese A, Chaudhuri KR, Opal P, Wu YC, Chan CH, et al.

J Neurol Sci, 2020 11 15;418:117105.
PMID: 32980781 DOI: 10.1016/j.jns.2020.117105
Trends in stroke outcomes at hospital discharge in first-ever stroke patients: Observations from the Malaysia National Stroke Registry (2009-2017)

Chen XW, Shafei MN, Aziz ZA, Sidek NN, Musa KI

J Neurol Sci, 2019 Jun 15;401:130-135.
PMID: 31000206 DOI: 10.1016/j.jns.2019.04.015

BACKGROUND: Stroke outcomes could be a quality indicator across the continuum of care and inform stroke management policymaking. However, this topic has rarely to date been studied directly.
AIMS: We sought to investigate recent trends in stroke outcomes at hospital discharge among first-ever stroke patients.
METHODS: This was an analysis of data from the Malaysia National Stroke Registry. Patients aged 18 years or older documented as having a first episode of stroke in the registry were recruited. Subsequently, the comparison of proportions for overall and sex-specific stroke outcomes between years (from 2009 to 2017) was conducted. The primary outcome was modified Rankin Scale score, which was assessed at hospital discharge, and each patient was categorized as follows: 1) functional independence, 2) functional dependence, or 3) death for analysis.
RESULTS: This study included 9361 first-ever stroke patients. Approximately 36.2% (3369) were discharged in an independence state, 53.1% (4945) experienced functional dependence, and 10.8% (1006) patients died at the time of hospital discharge. The percentage of patients who were discharged independently increased from 23.3% in 2009 to 46.5% in 2017, while that of patients discharged in a disabled state fell from 56.0% in 2009 to 45.6% in 2017. The percentage of death at discharge was reduced from 20.7% in 2009 to 7.8% in 2017. These findings suggest that the proportions of stroke outcomes at hospital discharge have changed significantly over time (p 
The potential role of miRNA therapies in spinal muscle atrophy

Gandhi G, Abdullah S, Foead AI, Yeo WWY

J Neurol Sci, 2021 08 15;427:117485.
PMID: 34015517 DOI: 10.1016/j.jns.2021.117485

Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by low levels of full-length survival motor neuron (SMN) protein due to the loss of the survival motor neuron 1 (SMN1) gene and inefficient splicing of the survival motor neuron 2 (SMN2) gene, which mostly affects alpha motor neurons of the lower spinal cord. Despite the U.S. Food and Drug Administration (FDA) approved SMN-dependent therapies including Nusinersen, Zolgensma® and Evrysdi™, SMA is still a devastating disease as these existing expensive drugs may not be sufficient and thus, remains a need for additional therapies. The involvement of microRNAs (miRNAs) in SMA is expanding because miRNAs are important mediators of gene expression as each miRNA could target a number of genes. Hence, miRNA-based therapy could be utilized in treating this genetic disorder. However, the delivery of miRNAs into the target cells remains an obstacle in SMA, as there is no effective delivery system to date. This review highlights the potential strategies for intracellular miRNA delivery into target cells and current challenges in miRNA delivery. Furthermore, we provide the future prospects of miRNA-based therapeutic strategies in SMA.
Ischemic optic neuropathy as a model of neurodegenerative disorder: A review of pathogenic mechanism of axonal degeneration and the role of neuroprotection

Khalilpour S, Latifi S, Behnammanesh G, Majid AM, Majid AS, Tamayol A

J Neurol Sci, 2017 Apr 15;375:430-441.
PMID: 28320183 DOI: 10.1016/j.jns.2016.12.044

Optic neuropathy is a neurodegenerative disease which involves optic nerve injury. It is caused by acute or intermittent insults leading to visual dysfunction. There are number of factors, responsible for optic neuropathy, and the optic nerve axon is affected in all type which causes the loss of retinal ganglion cells. In this review we will highlight various mechanisms involved in the cell loss cascades during axonal degeneration as well as ischemic optic neuropathy. These mechanisms include oxidative stress, excitotoxicity, angiogenesis, neuroinflammation and apoptosis following retinal ischemia. We will also discuss the effect of neuroprotective agents in attenuation of the negative effect of factors involve in the disease occurrence and progression.
The professional practice and training of neurology in the Asian and Oceanian Region: A cross-sectional survey by the Asian and Oceanian Association of Neurology (AOAN)

Roxas A, Mehndiratta MM, Bornstein N, Macdonell R, Lim KS, Ng PW, et al.

J Neurol Sci, 2017 Nov 15;382:108-115.
PMID: 29111001 DOI: 10.1016/j.jns.2017.09.022

OBJECTIVE: To survey AOAN member countries regarding their organizational structure, postgraduate neurology training program, and resources for neurological care provision.
METHODOLOGY: A cross-sectional survey using a 36-item questionnaire was conducted among country representatives to AOAN from August 2015 to August 2016.
RESULTS: A total of 18/20 AOAN member countries participated in the survey. All the countries have organized association with regular meetings, election of officers and neurology training program. In 9/18 countries, professionals other than neurologists were eligible for affiliation. In 11/18 countries, prior Internal medicine training (or equivalent postgraduate housemanship) is prerequisite to neurology program. Recertification examination is not a practice, but submission of CME is required in 7/18 countries to maintain membership. 12/18 countries publish peer-reviewed journals with at least 1 issue per year. Subspecialty training is offered in 14/18 countries. The ratio of neurologist to population ranges from 1:14,000 to as low as 1:32 million with 9/18 having <1 neurologist per 100,000 population. 6/18 countries have at least 1 specialized center solely for neurological diseases. In government-funded hospitals, the lag time to be seen by a neurologist and/or obtain neuroimaging scan ranges from 1day to 3months. All except one country have several medical- and lay- advocacy or support groups for different neurological conditions.
IMPLICATIONS: The data generated can be used for benchmarking to improve neurological care, training, collaborative work and research in the field of neurosciences among the AOAN member countries. The paper presented several strategies used by the different organizations to increase their number of neurologists and improve the quality of training. Sharing of best practices, academic networking, exchange programs and use of telemedicine have been suggested.
World Health Organization (WHO)'s Intersectoral Global Action Plan (IGAP); implications for Asian and African countries

Wasay M, Younis S, Charway-Felli A, Basri H

J Neurol Sci, 2023 Jan 15;444:120499.
PMID: 36450220 DOI: 10.1016/j.jns.2022.120499
Fulltext Pre-existing neurological conditions and COVID-19 co-infection: Data from systematic reviews, meta-analyses, and scoping reviews

Boruah AP, Thakur KT, Gadani SP, Kothari KU, Chomba M, Guekht A, et al.

J Neurol Sci, 2023 Dec 15;455:120858.
PMID: 37948972 DOI: 10.1016/j.jns.2023.120858

BACKGROUND: Pre-existing neurological diseases have been identified as risk factors for severe COVID-19 infection and death. There is a lack of comprehensive literature review assessing the relationship between pre-existing neurological conditions and COVID-19 outcomes. Identification of high risk groups is critical for optimal treatment and care.
METHODS: A literature review was conducted for systematic reviews, meta-analyses, and scoping reviews published between January 1, 2020 and January 1, 2023. Literature assessing individuals with pre-existing neurological diseases and COVID-19 infection was included. Information regarding infection severity was extracted, and potential limitations were identified.
RESULTS: Thirty-nine articles met inclusion criteria, with data assessing >3 million patients from 51 countries. 26/51 (50.9%) of countries analyzed were classified as high income, while the remaining represented middle-low income countries (25/51; 49.0%). A majority of evidence focused on the impact of cerebrovascular disease (17/39; 43.5%) and dementia (5/39; 12.8%) on COVID-19 severity and mortality. 92.3% of the articles (36/39) suggested a significant association between neurological conditions and increased risk of severe COVID-19 and mortality. Cerebrovascular disease, dementia, Parkinson's disease, and epilepsy were associated with increased COVID severity and mortality.
CONCLUSION: Pre-existing neurological diseases including cerebrovascular disease, Alzheimer's disease and other dementias, epilepsy, and Parkinson's disease are significant risk factors for severity of COVID-19 infection and mortality in the acute infectious period. Given that 61.5% (24/39) of the current evidence only includes data from 2020, further updated literature is crucial to identify the relationship between chronic neurological conditions and clinical characteristics of COVID-19 variants.