ENeurologicalSci

A 74-year-old man developed orthostatic syncope, a feeling of food stuck in his chest, and postprandial vomiting 3 years before presentation. Examination revealed severe orthostatic hypotension and cerebellar ataxia, and he was diagnosed with multiple system atrophy (MSA) with predominant cerebellar ataxia. Videofluoroscopic examination of swallowing showed lower oesophageal stricture and barium stagnation within the oesophagus. Oesophagogastroduodenoscopy revealed hypercontraction of the lower oesophagus, and high-resolution oesophageal manometry showed premature contractions of the lower oesophagus and decreased oesophageal peristalsis...

BACKGROUND: Acute hemorrhagic leukoencephalitis (AHLE) is a very rare demyelinating disease with rapid fulminant inflammation of the white matter. Although the exact etiology is unknown, AHLE usually manifests post a viral or bacterial infection and less often seen post vaccination for measles or rabies. AHLE has a very poor prognosis and a high mortality rate. Owing to the rarity of this entity there is not clear consensus on the proper line of management. In this report, we present a case of AHLE as a para-infectious sequel to COVID-19 in a young patient...

BACKGROUND: Chronic foot pain, including conditions such as plantar fasciitis, presents a significant challenge to patients and healthcare providers. Traditional treatments often offer limited relief, prompting exploration of alternative therapies. Transcranial direct current stimulation (tDCS) has emerged as a noninvasive brain stimulation technique with potential for alleviating chronic pain syndromes. METHODS: A review was conducted following the JBI methodology and adhering to PRISMA guidelines...

BACKGROUND AND OBJECTIVES: The aim of this study is to provide a comprehensive characterization of a large Estonian family spanning five generations with seventeen individuals affected by spastic paraplegia associated with a novel variant in the receptor expression-enhancing protein-1 ( REEP1 ) gene. METHODS: Comprehensive clinical evaluation, neuroimaging, and neurophysiological studies were performed on six patients who provided oral and written consent. Whole-exome sequencing was performed on the index case...

No abstract text is available yet for this article.

INTRODUCTION: 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. METHODS: Three databases (October 2021; MEDLINE, SCOPUS, and Web of Science), Cambridge University Press, and Annals of Neurology were searched...

No abstract text is available yet for this article.

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...

BACKGROUND: Acute spontaneous intracerebral haemorrhage is a devastating form of stroke. Prognostication after ICH may be influenced by clinicians' subjective opinions. PURPOSE: 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. METHOD: We included clinicians' predictions of 6-month outcome in the first 48 h for 52 adults with ICH and compared to actual 6-month outcome using descriptive statistics and multilevel binomial logistic regression...

•We report the first case of IgG4-related pachyleptomeningitis.•Our case showed also an inflammatory pseudotumor on the side ipsilateral to the pachyleptomeningitis.•The pachyleptomeningitis is probably due to inflammation from the dural pseudotumor spreading along the adjacent meninges.

BACKGROUND: 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. METHODS: 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...

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...

COVID-19 is primarily classified as a respiratory disorder; however, various neurological symptoms have been reported in COVID-19 patients. Neurological manifestations may be the initial signs of COVID-19 and can develop in patients of different age groups and with or without underlying disease. COVID-19 causes a broad range of complications in the central nervous system. These include headaches, altered mental status, dizziness, seizures, cerebrovascular events, encephalitis, and other encephalopathies. Moreover, a broad spectrum of peripheral nervous system symptoms such as olfactory and gustatory dysfunctions, neuropathy, visual impairments, neuralgia, cranial nerves palsy, and muscle involvement could manifest as symptoms...

BACKGROUND: Dandy-Walker malformation or syndrome is the most common posterior fossa malformation. It is commonly associated with other congenital anomalies such as cardiac defects; however, association with a giant occipital meningocele is extremely rare, as only around 34 cases have been described. CASE DESCRIPTION: We report a case of a 2-month-old female infant who presented with a midline, gigantic mass in the back of the head. It was first discovered on a prenatal ultrasound...

BACKGROUND: The characteristics of functional limb weakness (FLW) as one of the manifestations of functional neurological disorder after vaccination against coronavirus disease 2019 (COVID-19) remain controversial. METHODS: In this descriptive case series, we aimed to elucidate the characteristics of Japanese patients with FLW who claimed muscle weakness after COVID-19 vaccination among patients who visited our outpatient clinic between 1 June 2021 and 31 December 2022...

BACKGROUND: There is severe shortage of neurologists in sub-Saharan Africa. Tele-neurology consultations (TNC) can bridge this service gap, but there is very little published evidence on TNC in our setting, which we addressed through our study. METHODS: We prospectively enrolled patients at our neurology outpatients from October 2020 to October 2021. We administered a post-TNC questionnaire which captured satisfaction/acceptability using Likert scales. A sub-group of participants who also did in-person consultations (IPC) were additionally administered post-IPC questionnaires...

OBJECTIVES: To determine the stroke risk profile of dwellers of Nnewi community in Nigeria. METHODS: This was a cross-sectional survey involving consecutively recruited community-dwelling adults without a previous history of stroke. The Modified Framingham Stroke Risk Score (MFSRS) was used to evaluate the stroke risk profile of the participants. Data was analysed using descriptive and inferential statistics at an alpha level of 0.05. RESULT: 310 individuals (mean age = 37...

BACKGROUND: Greater blood pressure variability has detrimental effects on clinical outcome after a stroke; its effects are controversial and have not been evaluated in Sub-Saharan Africa (SSA). METHODS: We conducted a prospective study of patients with CT head confirmed ischemic and hemorrhagic strokes admitted to a tertiary hospital within 7 days of onset of unilateral neurological deficits. Blood pressure variability indices, standard deviation (SD) and coefficient of variation (CV) of systolic and diastolic blood pressure between day 0 and day 7, were calculated with a subsequent modified Rankin Scale (mRS) score on day 14 post-stroke...

BACKGROUND: The coexistence of persistent trigeminal artery (PTA) and Moyamoya disease (MMD) has been reported. If their pathogenesis is related and if PTA is protective or harmful in MMD remains unknown as these are rare cerebrovascular anomalies. CASE PRESENTATION: A 35-year-old woman with sudden global aphasia whose CT head and CT angiography of head and neck showed a hypodensity in the left posterior middle cerebral artery (MCA), a possible left proximal internal carotid artery occlusion, and a left PTA with hypoplasia of vertebral and basilar arteries...

The correlation between Down syndrome and Dandy-Walker syndrome is an exceptionally uncommon occurrence. To date, only four cases have been documented. All previously reported cases involved individuals under the age of 37 months, with prenatal or birth diagnoses. Additionally, most of these cases displayed a limited life expectancy and experienced poor developmental outcomes. In this report, we present the first-ever instance of an 11-year-old male patient, previously undiagnosed with Dandy-Walker syndrome, who presented with acute intracranial hypertension...