In this clinical study the presentation, electromyography and nerve conduction studies were described in different types of motor neurone disease in 28 Sudanese patients seen at El Shaab and Khartoum Teaching Hospitals. The three major clinical subtypes encountered were amyotrophic lateral sclerosis (n = 19), progressive bulbar palsy (n = 7) and progressive muscular atrophy (n = 2). Family history of the disease was found in four patients and those mainly presented with bulbar symptoms. Sudanese patients were found to have an earlier age of onset and a better prognosis of the disease than their caucasian counterparts.
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Ann Neurol
January 2025
Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA.
Objective: Approximately 20% of familial cases of amyotrophic lateral sclerosis (ALS) are caused by mutations in the gene encoding superoxide dismutase 1 (SOD1). Epidemiological data have identified traumatic brain injury (TBI) as an exogenous risk factor for ALS; however, the mechanisms by which TBI may worsen SOD1 ALS remain largely undefined.
Methods: We sought to determine whether repetitive TBI (rTBI) accelerates disease onset and progression in the transgenic SOD1 mouse ALS model, and whether loss of the primary regulator of axonal degeneration sterile alpha and TIR motif containing 1 (Sarm1) mitigates the histological and behavioral pathophysiology.
J Alzheimers Dis
January 2025
Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Background: White matter hyperintensities (WMH) are prominent neuroimaging markers of cerebral small vessel disease (CSVD) linked to cognitive decline. Nevertheless, the pathophysiological mechanisms underlying WMH remain unclear.
Objective: This study aimed to assess the structural decoupling index (SDI) as a novel metric for quantifying the brain's hierarchical organization associated with WMH in cognitively normal older adults
Methods: We analyzed data from 112 cognitively normal individuals with varying WMH burdens (43 high WMH burden and 69 low WMH burden).
ACS Chem Neurosci
January 2025
Department of Neurology, Multi-Omics Research Center for Brain Disorders,The First Affiliated Hospital, Hengyang Medical School, University of South China, Hengyang, Hunan 421001, China.
Brachial plexus root avulsion (BPRA) is often caused by road collisions, leading to total loss of motor function in the upper limb. At present, effective treatment options remain limited. Edaravone (EDA), a substance that eliminates free radicals, exhibits numerous biological properties, including neuroprotective, antioxidant and anti-inflammatory effects.
View Article and Find Full Text PDFJ Cent Nerv Syst Dis
January 2025
School of Pharmacy, National Defense Medical Center, Taipei, Taiwan.
Background: Parkinson's disease (PD) is one of the most common neurodegenerative disorders. Previous research has confirmed that isofraxidin can reduce macrophage expression and inhibit peripheral inflammation. However, its effects on the central nervous system remain underexplored.
View Article and Find Full Text PDFSci Rep
January 2025
Dept. of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.
Primary lateral sclerosis (PLS) is a motor neuron disease (MND) which mainly affects upper motor neurons. Within the MND spectrum, PLS is much more slowly progressive than amyotrophic laterals sclerosis (ALS). `Classical` ALS is characterized by catabolism and abnormal energy metabolism preceding onset of motor symptoms, and previous studies indicated that the disease progression of ALS involves hypothalamic atrophy.
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