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Journals Journal of the Neurological Sc...

Journal of the Neurological Sciences

https://read.qxmd.com/read/38609815/corrigendum-to-incorporating-health-equity-in-the-neurology-curriculum-our-experience-journal-of-the-neurological-science-2023-120665
#1
Tamara B Kaplan, Christopher Doughty, LaShyra Nolan, Joshua Budhu
No abstract text is available yet for this article.
April 11, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38583390/successful-treatment-of-cranial-dystonia-using-a-zolpidem-melatonin-combination-a-video-case-series
#2
JOURNAL ARTICLE
Narendrakumar H Barad, Eoin Mulroy
INTRODUCTION: Cranial dystonias (CrD) are challenging to treat. Oral pharmacotherapy is often sub-optimal, while delicate anatomy and limited availability of skilled botulinum toxin injectors makes this approach risky, and often difficult to access; neurosurgical options e.g. deep brain stimulation, are high-risk in the elderly populations most affected. We observed significant improvement in CrD in 2 patients prescribed Zolpidem+Melatonin combination treatment for insomnia, and therefore trialled this treatment in a further 4 patients with CrD...
April 3, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38581740/trends-from-two-decades-of-orphan-designations-in-paediatric-rare-neuromuscular-diseases
#3
REVIEW
Dinah M Duarte, Maria Beatriz da Silva Lima, Bruno Sepodes
Rare diseases are characterized by substantial unmet need mostly because the majority have limited, or no treatment options and a large number also affect children. Since the inception of EU orphan regulation in 2000 the European Medicines Agency Committee for Orphan Medicinal Products has received several applications for paediatric rare neuromuscular diseases (PERAN) however treatment options remain limited. Here we discuss the results form an observational, retrospective, cross-sectional study to characterize the currently authorised orphan medicinal products (OMP) and orphan designations (OD) given to products for PERAN in the last two decades...
April 3, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38579412/neuronal-intranuclear-inclusion-disease-in-new-zealand-a-novel-discovery
#4
JOURNAL ARTICLE
Tony Zhang, Andrew Chancellor, Bernard Liem, Clinton Turner, David Hutchinson, Edward Wong, Emma Glamuzina, Jae Beom Hong, James Cleland, Nicholas Child, Richard H Roxburgh, Shilpan Patel, Yi-Chung Lee, Yi-Chu Liao, Neil E Anderson
Neuronal intranuclear inclusion disease, caused by a GGC repeat expansion in the 5'-untranslated region of NOTCH2NLC, is a rare neurodegenerative condition with highly variable clinical manifestations. In recent years, the number of reported cases have increased dramatically in East Asia. We report the first four genetically confirmed cases of neuronal intranuclear inclusion disease in New Zealand, all having Polynesian ancestry (three New Zealand Māori and one Cook Island Māori). Phenotypically, they resemble cases reported from recent large East Asian cohorts...
April 3, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38608413/predicting-post-surgical-outcomes-in-idiopathic-normal-pressure-hydrocephalus-using-clinically-important-changes-from-the-cerebrospinal-fluid-tap-test
#5
JOURNAL ARTICLE
Ryan M Gallagher, Jodie L Marquez, Shabnam Dal, Peter G Osmotherly
OBJECTIVE: Patients diagnosed with idiopathic Normal Pressure Hydrocephalus (iNPH) typically experience symptom improvements after undergoing a cerebrospinal fluid-tap test (CSF-TT), These improvements are recognized as indicative of potential improvements following surgical intervention. As gait disturbance is the most common iNPH symptom, gait improvements are of predominant interest. The purpose of this study was to examine if clinically important changes in gait and balance from CSF-TT predict meaningful changes following surgery...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38583391/diagnostic-value-of-lower-extremity-ultrasonographic-nerve-enlargement-for-differentiating-demyelinating-charcot-marie-tooth-disease-from-chronic-inflammatory-demyelinating-polyneuropathy
#6
JOURNAL ARTICLE
Hiroyuki Naito, Takamichi Sugimoto, Akemi Hironaka, Masahiro Nakamori, Yu Yamazaki, Kazuhide Ochi, Hirofumi Maruyama
BACKGROUND AND PURPOSE: We previously reported that nerve enlargement assessment by nerve ultrasonography of the intermediate upper limb is applicable for distinguishing demyelinating Charcot-Marie-Tooth disease (CMT) from chronic inflammatory demyelinating polyneuropathy (CIDP). However, differences in the severity and distribution patterns of lower extremity nerve enlargement have not been established for either disease. Therefore, we examined the utility of lower extremity nerve ultrasonography for differentiating between CMT and CIDP...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38581741/hypothalamic-involvement-in-multiple-system-atrophy-a-structural-mri-study
#7
JOURNAL ARTICLE
Jacopo Pasquini, Michael J Firbank, Laura Best, Victoria Foster, Debra Galley, Vincenzo Silani, Roberto Ceravolo, George Petrides, David J Brooks, Kirstie N Anderson, Nicola Pavese
OBJECTIVE: To investigate hypothalamic atrophy and its clinical correlates in multiple system atrophy (MSA) in-vivo. BACKGROUND: MSA is characterized by autonomic dysfunction and parkinsonian/cerebellar manifestations. The hypothalamus regulates autonomic and homeostatic functions and is also involved in memory and learning processes. METHODS: 11 MSA, 18 Parkinson's Disease (PD) and 18 Healthy Controls (HC) were included in this study. A validated and automated hypothalamic segmentation tool was applied to 3D-T1-weighted images acquired on a 3T MRI scanner...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38581739/psilocybin-pulse-regimen-reduces-cluster-headache-attack-frequency-in-the-blinded-extension-phase-of-a-randomized-controlled-trial
#8
JOURNAL ARTICLE
Emmanuelle A D Schindler, R Andrew Sewell, Christopher H Gottschalk, L Taylor Flynn, Yutong Zhu, Brian P Pittman, Nicholas V Cozzi, Deepak C D'Souza
BACKGROUND: In a recent randomized, double-blind, placebo-controlled study, we observed a nonsignificant reduction of attack frequency in cluster headache after pulse administration of psilocybin (10 mg/70 kg, 3 doses, 5 days apart each). We carried out a blinded extension phase to consider the safety and efficacy of repeating the pulse regimen. METHODS: Eligible participants returned to receive a psilocybin pulse at least 6 months after their first round of study participation...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38579416/bilateral-vestibulopathy-as-the-initial-presentation-of-canvas
#9
JOURNAL ARTICLE
Carlos R Gordon, Roy Zaltzman, Dario Geisinger, Zohar Elyoseph, Yoav Gimmon
Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowly progressing autosomal recessive ataxic disorder linked to an abnormal biallelic intronic (most commonly) AAGGG repeat expansion in the replication factor complex subunit 1 (RFC1). While the clinical diagnosis is relatively straightforward when the three components of the disorder are present, it becomes challenging when only one of the triad (cerebellar ataxia, neuropathy or vestibular areflexia) manifests. Isolated cases of Bilateral Vestibulopathy (BVP) or vestibular areflexia that later developed the other components of CANVAS have not been documented...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38579415/a-systematic-review-of-randomized-controlled-trials-of-endovascular-therapy-for-stroke-on-mortality-and-disability
#10
JOURNAL ARTICLE
Kazutaka Uchida, Leon A Rinkel, Johanna M Ospel, William K Diprose, Mayank Goyal
BACKGROUND: Endovascular treatment (EVT) has become standard of care for patients with anterior circulation large vessel occlusion (LVO) stroke, with indications having recently expanded to late time-window and large ischemic core patients. There is conflicting evidence on whether EVT reduces mortality or only disability. We performed a meta-analysis of randomized controlled trials (RCTs) to assess the effect of EVT on mortality and severe disability. METHODS: We systematically searched PubMed, Web of Science, Scopus, and Embase on November 15, 2023, to identify phase 3 RCTs comparing EVT to best medical treatment (BMT) in patients with anterior circulation LVO stroke in a common effects meta-analysis...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38579414/sex-differences-in-treatment-effect-in-neuroprotectant-trials-for-acute-ischemic-stroke-a-systematic-review
#11
JOURNAL ARTICLE
Sahily Reyes-Esteves, Donna K George, Brett Cucchiara
BACKGROUND: Pre-clinical data suggest sex differences in mechanisms of cerebral ischemic injury. This might result in differential outcomes of putative neuroprotectants by sex, though little systematic data is available to assess this. METHODS: We performed a systematic review of multicenter randomized controlled trials published from January 1980-June 2022 enrolling >100 subjects and testing neuroprotectants in acute ischemic stroke (AIS). For each trial, reported treatment effect by sex was extracted...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38579413/pathogenesis-and-management-of-low-pressure-hydrocephalus-a-narrative-review
#12
REVIEW
Shanshan Duan, Jin Hu
Patients diagnosed with low-pressure hydrocephalus typically present with enlarged ventricles and unusually low intracranial pressure, often measuring below 5 cmH2 O or even below atmospheric pressure. This atypical presentation often leads to low recognition and diagnostic rates. The development of low-pressure hydrocephalus is believed to be associated with a decrease in the viscoelasticity of brain tissue or separation between the ventricular and subarachnoid spaces. Risk factors for low-pressure hydrocephalus include subarachnoid hemorrhage, aqueduct stenosis, prior cranial radiotherapy, ventricular shunting, and cerebrospinal fluid leaks...
April 2, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38580483/nerve-ultrasound-aids-sural-nerve-biopsy-in-sarcoid-neuropathy
#13
LETTER
Amuro Kondo, Keiichi Hokkoku, Taiji Mukai, Yudai Uchida, Takashi Chiba, Asuka Kitamura, Akatsuki Kubota, Yuki Hatanaka, Masahiro Sonoo, Jun Shimizu, Shunsuke Kobayashi
No abstract text is available yet for this article.
April 1, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38574438/delayed-levodopa-responsive-parkinsonism-following-acute-midbrain-injury
#14
JOURNAL ARTICLE
Tali Friedman-Korn, Caroline Weill, Simona Ben-Haim, David Arkadir
Acute midbrain injury may cause both hyperkinetic movement disorders and parkinsonism. The temporal interval between the insult and the emergence of hyperkinetic disorders can last years. A delayed appearance of parkinsonism, on the other hand, was rarely described. We present three cases of male patients (50-, 58- and 28-year-old) who developed levodopa-responsive parkinsonism 20, 8 and two years, respectively, after acute brain insult involving the midbrain. Insults included subcortical intracerebral hemorrhage dissecting into the midbrain, embolic basilar occlusion and trauma...
April 1, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38569376/cerebrospinal-fluid-glial-fibrillary-acidic-protein-in-contrast-to-amyloid-beta-protein-is-associated-with-disease-symptoms-in-huntington-s-disease
#15
JOURNAL ARTICLE
Sara Korpela, Jimmy Sundblom, Henrik Zetterberg, Radu Constantinescu, Per Svenningsson, Martin Paucar, Valter Niemelä
INTRODUCTION: Huntington's disease (HD) is a hereditary neurodegenerative disease, currently lacking disease-modifying treatments. Biomarkers are needed for objective assessment of disease progression. Evidence supports both complex protein aggregation and astrocyte activation in HD. This study assesses the 42 amino acid long amyloid beta (Aβ42) and glial fibrillary acidic protein (GFAP) as potential biomarkers in the cerebrospinal fluid (CSF) of HD mutation carriers. METHODS: CSF from participants was obtained from three sites in Sweden...
March 30, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38569375/long-term-cognitive-and-neurovascular-changes-after-carotid-endarterectomy
#16
JOURNAL ARTICLE
Kristiina Relander, Marja Hietanen, Petra Ijäs, Krista Nuotio, Pirkka Vikatmaa, Suvi M Koskinen, Marianne Ala-Kauhaluoma, Teemu I Paajanen, Jussi Virkkala, Perttu J Lindsberg, Lauri Soinne
BACKGROUND: Carotid endarterectomy (CEA) has been associated with both cognitive decline and improvement, but the underlying neurovascular mechanisms are unclear. The aim of this study was to investigate the relationship between neurovascular indices and cognitive changes after CEA. METHODS: We studied 55 patients with severe (≥70%) symptomatic or asymptomatic carotid stenosis before and six months after CEA. A wide array of neuropsychological tests was arranged in eight cognitive domains and cognitive functions specific to hemisphere ipsilateral to operation...
March 28, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38594137/continuing-the-search-for-fluid-biomarkers-to-enhance-huntington-s-disease-staging-and-therapeutic-response
#17
EDITORIAL
Eilrayna Gelyana, Rebecca Khamishon, Jee Bang
No abstract text is available yet for this article.
March 27, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38599028/early-neurological-changes-following-endovascular-therapy-for-acute-stroke-due-to-intracranial-atherosclerotic-disease
#18
JOURNAL ARTICLE
Kazutaka Uchida, Hiroshi Yamagami, Nobuyuki Sakai, Koji Iihara, Hirotoshi Imamura, Akira Ishii, Yuji Matsumaru, Chiaki Sakai, Tetsu Satow, Fumihiro Sakakibara, Manabu Shirakawa, Shinichi Yoshimura
BACKGROUND: Endovascular therapy (EVT) reduces functional disability in patients with acute large vessel occlusion (LVO). However, the early neurological change after EVT may be limited in patients with intracranial atherosclerotic disease (ICAD). METHODS: We analyzed the Japanese Registry of NeuroEndovascular Therapy (JR-NET) 4 which was a retrospective, nationwide, multicenter registry of patients with LVO between 2015 and 2019. We compared the early neurological change, efficacy and safety of EVT for acute LVO in ICAD and other etiologies...
March 26, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38531241/primary-headache-disorders-among-people-living-with-hiv-in-lusaka-zambia
#19
JOURNAL ARTICLE
Curi Kim, Mashina Chomba, Arnold Phiri, Sombo Fwoloshi, Cassidy W Claassen, Lottie Hachaambwa, Deanna Saylor
OBJECTIVE: We completed a cross-sectional survey study to determine headache prevalence and its association with HIV characteristics among people living with HIV (PLHIV) in Lusaka, Zambia. BACKGROUND: Headaches are common but their association with HIV status is unknown. METHODS: The HARDSHIP survey, a headache epidemiology questionnaire previously validated in Zambia, was distributed during a 3-month period to 3666 consecutive adult PLHIV attending routine clinic appointments at the Adult Infectious Diseases Centre at the University Teaching Hospital in Lusaka, Zambia...
March 21, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38527411/superficial-small-cerebellar-infarcts-in-cerebral-amyloid-angiopathy-on-3%C3%A2-t-mri-a-preliminary-study
#20
JOURNAL ARTICLE
Yuichiro Ii, Hidehiro Ishikawa, Akisato Nishigaki, Takaya Utsunomiya, Naoko Nakamura, Yoshinori Hirata, Hirofumi Matsuyama, Hiroyuki Kajikawa, Keita Matsuura, Kana Matsuda, Masaki Shinohara, Seiya Kishi, Ryota Kogue, Maki Umino, Masayuki Maeda, Hidekazu Tomimoto, Akihiro Shindo
BACKGROUND: Strictly superficial cerebellar microbleeds and cerebellar superficial siderosis have been considered markers of advanced cerebral amyloid angiopathy (CAA), but there are few studies on cerebellar ischemic lesions in CAA. We investigated the presence of superficial small cerebellar infarct (SCI) ≤15 mm and its relation to magnetic resonance imaging (MRI) markers in patients with probable CAA. METHODS: Eighty patients with probable CAA were retrospectively evaluated...
March 21, 2024: Journal of the Neurological Sciences
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