Spinocerebellar Ataxia Type 2 [PDF]
1. Introduction: The autosomal dominant cerebellar ataxias (ADCA) are a clinically, pathologically and genetically heterogeneous group of neurodegenerative disorders caused by degeneration of cerebellum and its afferent and efferent connections.
Auburger, Georg +3 more
core +1 more source
ABSTRACT Objective Facioscapulohumeral muscular dystrophy type 1 (FSHD1) is a progressive neuromuscular disorder with no approved treatments. Identifying reliable biomarkers is critical to monitor disease severity, activity, and progression. Interleukin‐6 (IL‐6) has been proposed as a candidate biomarker, but longitudinal validation is limited ...
Jonathan Pini +13 more
wiley +1 more source
The structural and functional integrity of peripheral nerves depends on the glial-derived signal desert hedgehog [PDF]
We show that desert hedgehog ( dhh), a signaling molecule expressed by Schwann cells, is essential for the structural and functional integrity of the peripheral nerve.
Jessen, KR +6 more
core
Co-cultures with stem cell-derived human sensory neurons reveal regulators of peripheral myelination [PDF]
Effective bidirectional signalling between axons and Schwann cells is essential for both the development and maintenance of peripheral nerve function. We have established conditions by which human induced pluripotent stem cell-derived sensory neurons can
Bennett, David L.H. +5 more
core +2 more sources
The Relationship Between Inflammation and Central Nervous System in Multiple Sclerosis
ABSTRACT Aim Multiple sclerosis is an autoimmune demyelination disease that is seen especially in the young population and has a progressive course, causing motor, sensory, and cognitive deficits. In the literature, the pathogenesis of MS disease and the interconnection between the immune and central nervous system in the disease have not been fully ...
Gamze Ansen +5 more
wiley +1 more source
An autopsy study of a familial oculopharyngeal muscular dystrophy (OPMD) with distal spread and neurogenic involvement [PDF]
An 81-year-old man from a family with a history of oculopharyngeal muscular dystrophy (OPMD) involving 6 members over 4 generations is described. The patient first noted drooping of his eyelids at the age of 65.
Aarli +49 more
core +1 more source
Lessons Learned: Quality Analysis of Optical Coherence Tomography in Neuromyelitis Optica
ABSTRACT Introduction Optical coherence tomography (OCT)‐derived retina measurements are markers for neuroaxonal visual pathway status. High‐quality OCT scans are essential for reliable measurements, but their acquisition is particularly challenging in eyes with severe visual impairment, as often observed in neuromyelitis optica spectrum disorders ...
Hadi Salih +40 more
wiley +1 more source
Wallerian Degeneration in Peripheral Nerve Disease
Wallerian-like degeneration can be regarded as the final shared pathway of the most prevalent diseases of the peripheral nervous system, including many degenerative, metabolic, heritable, toxic, inflammatory and ischaemic disorders. The cellular responses that take place during Wallerian degeneration, including the elaboration of neurotrophins, are ...
V, Chaudhry, J D, Glass, J W, Griffin
openaire +3 more sources
Autosomal Recessive Spastic Ataxia of Charlevoix‐Saguenay in Two Half‐Siblings
ABSTRACT Autosomal recessive spastic ataxia of Charlevoix‐Saguenay (ARSACS) is caused by biallelic pathogenic variants in the SACS gene. We report the clinical, radiologic and neurophysiologic features of a pair of half‐siblings who presented with progressive cerebellar ataxia, peripheral neuropathy and upper motor neuron signs.
Dennis Yeow +6 more
wiley +1 more source
Radioprotective Effects of Vitis vinifera L. Seed against Radiation-Induced Short-Term Memory Loss in Young Adult Wistar Rats [PDF]
Introduction: Brain radiation therapy often results in short-term memory loss that progresses to cognitive impairment. This issue primarily arises from ionizing radiation generating reactive oxygen species, contributing to chronic oxidative stress that ...
Ayomide Abe +2 more
doaj +1 more source

