The particular value of clinical, radiological and electromyographical features is compared in 42 patients with motor deficit related to cervicarthrosic myelopathy or amyotrophic lateral sclerosis. The initial onset of the disease was identical (motor deficit and long tracts pathways involvement). Three different groups were identifyed according to the evolution: -- Group I: (13 cases): true lateral amyotrophic sclerosis which were not operated on. -- Group II (10 cases): myelopathy called "cervicarthrosic" because of radiological findings which were operated on but had the same steady worsened course as a lateral amyotrophic sclerosis. -- Group III (19 cases): cervical myelopathy which had surgery. The operation brought about stabilization or fairly good recovery over the 18 months following at least. From a clinical aspect, the "Lhermitte sign" or objective sensitive deficit are strongly significant for cervical myelopathy. On the contrary, diffuse fasciculations specially in the tongue seem to be mostly found in lateral amyotrophic sclerosis, whereas they are restricted into the paralysed area in cervical myelopathy. Electromyographic examination is decisive: simple activity with high frequency motor units (increased amplitude and polyphasic waves) or "preponderant potentials" into a cranial nerve territory or three segments of the lower limbs are frequently found in lateral amyotrophic sclerosis. These electromyographic features are less significant in the upper limbs. The neuroradiological findings lonely cannot assert definitely the cervicarthrosic origin of the myelopathy but visualize the conflicting situation between the spinal cord and the cervical canal and allow to choose the surgical procedure.
Download full-text PDF |
Source |
---|
Acta Neuropathol Commun
January 2025
Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College (PUMC) and Chinese Academy of Medical Science (CAMS), Beijing, China.
Mutations in the ANXA11 gene, encoding an RNA-binding protein, have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but the underlying in vivo mechanisms remain unclear. This study examines the clinical features of ALS patients harboring the ANXA11 hotspot mutation p.P36R, characterized by late-onset motor neuron disease and occasional multi-system involvement.
View Article and Find Full Text PDFCNS Neurosci Ther
January 2025
Jiujiang Clinical Precision Medicine Research Center, Jiujiang, Jiangxi, China.
Background: Adenosine deaminase action on RNA 1 (ADAR1) can convert the adenosine in double-stranded RNA (dsRNA) molecules into inosine in a process known as A-to-I RNA editing. ADAR1 regulates gene expression output by interacting with RNA and other proteins; plays important roles in development, including growth; and is linked to innate immunity, tumors, and central nervous system (CNS) diseases.
Results: In recent years, the role of ADAR1 in tumors has been widely discussed, but its role in CNS diseases has not been reviewed.
Sci Rep
January 2025
Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E. Chicago Ave, Chicago, IL, 60611, USA.
Corticospinal motor neurons (CSMN), located in the motor cortex of the brain, are one of the key components of the motor neuron circuitry. They are in part responsible for the initiation and modulation of voluntary movement, and their degeneration is the hallmark for numerous diseases, such as amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegia, and primary lateral sclerosis. Cortical hyperexcitation followed by in-excitability suggests the early involvement of cortical dysfunction in ALS pathology.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Protein Science, SciLifeLab, KTH Royal Institute of Technology, Stockholm, Sweden.
Accurate diagnosis and monitoring of neurodegenerative diseases require reliable biomarkers. Cerebrospinal fluid (CSF) proteins are promising candidates for reflecting brain pathology; however, their diagnostic utility may be compromised by natural variability between individuals, weakening their association with disease. Here, we measured the levels of 69 pre-selected proteins in cerebrospinal fluid using antibody-based suspension bead array technology in a multi-disease cohort of 499 individuals with neurodegenerative disorders including Alzheimer's disease (AD), behavioral variant frontotemporal dementia, primary progressive aphasias, amyotrophic lateral sclerosis (ALS), corticobasal syndrome, primary supranuclear palsy, along with healthy controls.
View Article and Find Full Text PDFNat Commun
January 2025
Department of Neurology, Columbia University Irving Medical Center, New York, NY, USA.
Mitochondrial function is modulated by its interaction with the endoplasmic reticulum (ER). Recent research indicates that these contacts are disrupted in familial models of amyotrophic lateral sclerosis (ALS). We report here that this impairment in the crosstalk between mitochondria and the ER impedes the use of glucose-derived pyruvate as mitochondrial fuel, causing a shift to fatty acids to sustain energy production.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!