eNeurologicalScieNeurologicalSci Vol 34

March 2024

Review Article

A systematic review of the association between the age of onset of spinal bulbar muscular atrophy (Kennedy's disease) and the length of CAG repeats in the androgen receptor gene

Dante J. Bellai, Mark G. Rae

  • Article 100495
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  • https://doi.org/10.1016/j.ensci.2024.100495

    Spinal bulbar muscular atrophy (SBMA) is an X-linked recessive motor neuron disorder caused by the presence of ≥38 CAG repeats in the androgen receptor gene. Existing literature indicates a relationship between CAG repeat number and the onset age of some motor symptoms of SBMA. This review explores the effect of larger versus shorter CAG repeats on the age of weakness onset in male SBMA patients.


Original Articles

Psychometric validation of the Arabic multiple sclerosis resiliency scale: Uncovering resilience factors in Lebanese MS patients for clinical and research advancements

Nour Yaktine, Souha Fares, Samia J. Khoury, Hala Darwish

  • Article 100489
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  • https://doi.org/10.1016/j.ensci.2023.100489

    Multiple sclerosis (MS) is an autoimmune demyelinating disease that involves the central nervous system (CNS). Individuals with Multiple Sclerosis (MS) may experience difficulty adapting to their diagnosis as the unpredictable nature of the disease can be challenging to cope with.

    The purpose of this study is twofold. First, we have culturally adapted and analyzed the Arabic version of the Multiple Sclerosis Resiliency Scale (MSRS) psychometric properties. Second, we aimed to explore resilience in a sample of Lebanese patients with MS in the face of the chronic disease and financial hardship that Lebanon is going through to evaluate their strengths and struggles.

Doctors and nurses subjective predictions of 6-month outcome compared to actual 6-month outcome for adult patients with spontaneous intracerebral haemorrhage (ICH) in neurocritical care: An observational study

Siobhan Mc Lernon, Daniel Frings, Louise Terry, Rob Simister, ... David J. Werring

  • Article 100491
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  • https://doi.org/10.1016/j.ensci.2023.100491

    Acute spontaneous intracerebral haemorrhage is a devastating form of stroke. Prognostication after ICH may be influenced by clinicians' subjective opinions.


    To evaluate subjective predictions of 6-month outcome by clinicians' for ICH patients in a neurocritical care using the modified Rankin Scale (mRS) and compare these to actual 6-month outcome.

    Early prognostication is challenging. Doctors and nurses were most likely to correctly predict poor outcome but tended to err on the side of optimism for mortality, suggesting an absence of clinical nihilism in relation to ICH.

Effect of istradefylline on postural abnormalities in patients with Parkinson's disease: An association study of baseline postural angle measurements with changes in Unified Dystonia Rating Scale total score

Makio Takahashi, Toshio Shimokawa, Jinsoo Koh, Takao Takeshima, ... Hidefumi Ito

  • Article 100493
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  • https://doi.org/10.1016/j.ensci.2023.100493

    In our previous study, istradefylline treatment in patients with Parkinson's disease (PD) improved postural abnormalities (PAs), as seen from a decrease in the mean Unified Dystonia Rating Scale (UDRS) total score from week 0 to week 24. A subgroup analysis based on baseline clinical characteristics investigated the association between improvement in the UDRS total score and istradefylline treatment. However, the association between an objective assessment of PAs and improvement in the UDRS total score is unclear. This ad hoc analysis investigated the association between improvement in the UDRS total score after istradefylline treatment and baseline trunk and neck angles, objective assessments of PAs, measured from patients' photographs taken in the previous study.


Case Reports

Cytomegalovirus and varicella zoster virus coinfection-associated central hypoventilation syndrome (Ondine's Curse)

Marine Isakadze, Benjamin Zwain, Alan J. Velander, Jesus Lovera

  • Article 100488
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  • https://doi.org/10.1016/j.ensci.2023.100488

    Central hypoventilation syndrome (CHS) is a rare condition resulting from damage to the respiratory centers in the central nervous system (CNS). It can be congenital or acquired and can cause hypoventilation, inadequate gas exchange, and respiratory failure, often during sleep but sometimes even while awake. CHS can lead to respiratory failure and life-threatening complications if not identified promptly. In this report, we present a rare case of a patient with human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS), who developed CHS likely due to an opportunistic infection by cytomegalovirus (CMV) and varicella zoster virus (VZV), manifesting as a lesion in the medullary respiratory nuclei. After treatment with ganciclovir, the patient showed clinical improvement, and his medullary lesion resolved.


Letters to the Editor

FLAIR hyperintense cortical lesions in anti-myelin oligodendrocyte glycoprotein antibody-associated encephalitis with seizure following SARS-CoV-2 mRNA vaccination

Fumitaka Yoshino, Yuichi Ozaki, Tomoya Shibahara, Mikiaki Matsuoka, ... Hiroshi Nakane

IgG4-related pachyleptomeningitis with inflammatory pseudotumor

Koki Suezumi, Taira Uehara, Akihiko Taira, Naoki Akamatsu, ... Hiroyuki Murai

Behr's syndrome mimicking a case of hereditary spastic paraparesis

Rajesh Verma, Rajarshi Chakraborty