Neurological Sciences

Foreign accent syndrome (FAS) is characterized by new onset speech that is perceived as foreign. Available data from acquired cases suggests focal brain damage in language and sensorimotor brain networks, but little remains known about abnormal functional connectivity in idiopathic cases of FAS without structural damage. Here, connectomic analyses were completed on three patients with idiopathic FAS to investigate unique functional connectivity abnormalities underlying accent change for the first time. Machine learning (ML)-based algorithms generated personalized brain connectomes based on a validated parcellation scheme from the Human Connectome Project (HCP)...

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BACKGROUND: POLR3-related leukodystrophy is a group of rare neurodegenerative disorders characterized by degeneration of the white matter with different combinations of major clinical features. CASE: An 18-year-old lady was admitted for no menstruation since childhood. She gradually developed slight symptoms, such as choking after drinking water and unsteady walking in the last 2 years. Furthermore, her test scores and response capability were far lower than that of her peers...

BACKGROUND: Chitinase -3-like 1-protein (CHI3L1) is a glycoside secreted by monocytes, microglia, and activated astrocytes. Its distribution in inflammatory lesions denotes its role in astrocytic response to modulate CNS inflammation. In multiple sclerosis (MS), CHI3L1 levels have been found to be influenced by disease severity, activity, and progression. We aimed to measure CSF level of CHI3L1 in patients with MS and correlate its level with disability measures for a possible role as a biomarker for disease progression...

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OBJECTIVE: Patients diagnosed with autoimmune encephalitis (AE) require admission to the neurological intensive care unit (ICU) when they exhibit clinical manifestations such as status epilepticus, central hypoventilation, and severe involuntary movements. In order to determine the predictors of ICU admission and prognosis for patients with AE admitted to the neurological ICU, we analyzed the clinical characteristics of this patient population. METHODS: This retrospective study analyzed 123 patients admitted to the First Affiliated Hospital of Chongqing Medical University between 2012 and 2021 who were diagnosed with AE based on serum and/or cerebrospinal fluid (CSF) AE-related antibody positivity...

INTRODUCTION: Procedural perceptual-motor learning of sequences (PPMLS) provides perceptual-motor skills in many activities of daily living. Based on behavioral and neuroimaging results, theoretical models of PPMLS postulate that the cortico-striatal loop, the cortico-cerebellar loop and the hippocampus are specifically involved in the early stage of PPMLS while the cortico-striatal loop would be specifically involved in the late stage of PPMLS. Hence, current models predict that the early stage of PPMLS should be impaired in Parkinson's disease (PD: lesion of the cortico-striatal loop), in cerebellar disease (CD: lesion of the cortico-cerebellar loop) and in Alzheimer's disease (AD: lesion of the hippocampus), whereas the late stage of PPMLS should be specifically impaired in PD...

BACKGROUND: Diagnosis of Parkinson's disease (PD) is associated with a vast number of challenges. This study aimed to assess the overlap of PD patients' transcriptomes in the substantia nigra (SN) with peripheral blood mononuclear cells (PBMCs) to discover potential biomarkers for diagnosis. METHODS: GEO data were used to select genes with significant changes in expression level in the SN region and eligible studies. Also, transcriptome data related to blood of PD patients with other neurodegenerative diseases (ND) was considered...

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BACKGRO UND: Ocular abnormalities in myasthenia gravis (MG) are characterized by severely limited movements and rapid saccades. Data about eye motility of MG patients whose ocular movements are apparently normal are lacking. Our study assessed the eye movement parameters in MG patients without clinical eye motility dysfunctions and investigated the effects of neostigmine administration on the eye motility in these patients. MATERIALS: In this longitudinal study, we screened all patients diagnosed with MG referring to the Neurologic Clinic of the University of Catania between October 1, 2019, and June 30, 2021...

OBJECTIVES: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is hallmarked by age-dependent accumulation of microangiopathy with antiplatelet medications commonly used for stroke prevention though without known therapeutic benefit. Our objective was to identify whether antiplatelet therapy impacted the incidence of acute ischemic stroke (AIS) or intracerebral hemorrhage (ICH) in those with reported CADASIL. MATERIALS AND METHODS: Owing to the rarity of the disease, we performed a retrospective study of anonymized data from the international TriNetX Research Network (Oct 2015 through January 2021)...

BACKGROUND: People with Parkinson's disease (PD) often complain about handwriting difficulties. Currently, there is no consensus on the rehabilitative treatment and outcome measures for handwriting rehabilitation in PD. OBJECTIVES: This study aims to investigate evidence on handwriting rehabilitation in people with PD, examining characteristics of interventions and outcomes. METHODS: A scoping review was conducted according to Arksey and O'Malley's framework and PRISMA-ScR List...

OBJECTIVE: To evaluate the tolerability of clobazam in patients with drug-resistant epilepsy aged 50 years and older. METHODS: We performed a single center, retrospective chart review of patients at least 50 years of age with drug resistant epilepsy of any type who started clobazam as an add on therapy. Retention rate, safety, and tolerability at 6 and 12 months and last follow-up, and the discontinuation rate due to side effects were analyzed. RESULTS: A total of 26 patients met inclusion criteria...

BACKGROUND: When making decisions, one often faces a trade-off between immediate and long-term rewards. In these situations, people may prefer immediate over later rewards, even if immediate rewards are smaller than later ones; a phenomenon known as temporal discounting. In this study, we, for the first time, assessed temporal discounting in three populations: participants with manifest Huntington disease (HD), participants with premanifest HD, and control participants. METHODS: Using the temporal discounting task, we invited participants to choose between small immediate amount of money vs...

We report a patient with early-onset hereditary sensory and autonomic neuropathy type 1A (HSAN-1A) who developed a distinct phenotype, with tongue fasciculation and atrophy, due to a mutation at serine 331 in the SPTLC1 gene. HSAN-1A manifestation causing tongue fasciculation and atrophy have been rarely found. Our report adds to the growing evidence of the existence of an overlap between hereditary neuropathy and motor neuron disease caused by pathogenic p.S331Y variant in SPTLC1 gene.

BACKGROUND: While amyotrophic lateral sclerosis (ALS) incidence has increased during the last decades, structured evidence on increased prevalence is lacking. After reporting a significant yearly increase of ALS incidence over a 10-year period, we checked for increased prevalence in Southern Sardinia over a quinquennium. METHODS: ALS patients (El Escorial Criteria) recruited from the study area and followed at ALS Centre, University of Cagliari, were included. Prevalence was computed for January 1, 2015 and January 1, 2019 and was calculated for the overall ALS population as well as for tracheostomized and non-tracheostomized patients...

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OBJECTIVE: This systematic review aimed to compare the effects of immersive and non-immersive virtual reality on upper extremity function in stroke survivors by employing a network meta-analysis approach. DATA SOURCES: MEDLINE, Embase, CINAHL Plus, APA PsycINFO, and Scopus were searched. Virtual reality was used for upper extremity rehabilitation; dose-matched conventional rehabilitation was used for comparison. Fugl-Meyer Assessment was used to assess upper extremity function...

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