JNNP: Neuropathy Around ALS: Esothypetic Association

Amyotrophic lateral sclerosis (ALS) is a rare progressive neurodegenerative disease that mainly affects upper and lower motor neurons, but also causes multiple systemic conditions, especially related to changes in cognitive function.
have described mild fibrous dysfunction in the past, as recent studies have confirmed.
in a multi-center study, 88 ALS patients were surveyed, 12.5 percent of whom developed sensory multiple neuropathy (PNP), independent of age, course and site of onset.
the study was to re-understand the prevalence and risk factors for assessing PNP in ALS patients.
a large number of variables, including genetic mutations.
conducted a forward-looking follow-up of ALS patients in Lisbon (January 2015-January 2018) and was continuously enrolled in the OnWebDuals register to test the effects of clinical characteristics and genotypes on PNP prevalence.
we include patients over the age of 18, according to the revised El Escorial standard, and comply with Awaji electrophysiological guidelines, possibly or explicitly ALS.
excluding patients with known PNP, significant lower limb edema, monoclonal cglobulin disease, and incomplete neurophysiological examination.
record age, sex, site of onset, course of illness, prediagnostic weight loss, history of diabetes or cancer, past history of chemotherapy or exposure to neurotoxic drugs, and mutations in SOD1 and C9orf72.
duration of the disease is the time between the onset of muscle weakness and neurophysiological examination, which is performed at the time of diagnosis or during a confirmation assessment by a patient referred from another centre.
as part of the evaluation program, standardized neurotranscing studies were conducted.
motion conduction studies include bipolenal fibula nerves and right-hand ruler nerves (far-end incubation period and velocity), including F-waves (incubation period and duration).
two-sided fibula nerves (far end of the calf, back of the foot) record the sensory actional motor (SNAP) (conduction speed and amplitude), and if one or both of these abnormalities occur, the two sides record the fibula nerve.
is stimulated and recorded by surface electrodes, and the skin temperature remains above 30 degrees C.
SNAP amplitude we included 339 ALS patients, of whom 191 were male (56.3%), spine type (n=243,71.7%), elyse type (n=75,22 .1%), respiratory type (n=8,2.4%), shaft type (n=6,1.8%), cognitive type (n=5,1.5%) and whole body type (n=2,0.6%).
clinical and neurophysiological data were complete for each patient, while SOD1 and C9Orf72 mutations were tested in 130 and 233 patients, respectively.
across the study population, 29 patients had PNP (8.6 percent, G2), while 310 patients did not have PNP (91.4 percent, G1).
of all patients in phase G2 showed mild or moderate amplitude reduction, with 19 cases of sensory conduction speed approaching the critical value and 10 cases of slightly lower sensory conduction speed.
no patient has symptoms of neuropathy.
all demographic variables were similar between groups except gender, age and frequency of respiratory seizures.
G2 is characterized by a higher proportion of males (54.5% G1 vs 75.9% G2, p-0.027) and older (61.00± 13.5% G1 vs 67.31±13.3% G2, p-0.01), higher frequency of respiratory illness (p-0.024).
Logistic regression, including all variables defined in the method section, confirms that age (p-0.014), sex (p-0.024), and breathing (p-0.037) are independent predictors of PNP.
year, the risk of PNP increased by 1.044 (CI 1.009 to 1.08), women reduced PNP risk by 0.644 (CI 0.145 to 0.874), and respiratory seizures increased PNP risk by 5.4 (CI 1.108 to 26.156).
8.6% of ALS patients have PNP, because ALS patients tend to have cold limbs, and by normalizing the skin temperature, it is not possible to guarantee the normal propagation rate of the motion prodistant along the axis.
history of diabetes, weight loss at diagnosis, cancer, chemotherapy or SOD1 and C9orf72 HRE mutations were not associated with PNP.
incidence of PNP is most pronounced in older men.
can also have many potential causes, namely high metabolic status and nutritional factors, unresolted exoaphic hypoxia can lead to external nerve damage in this group of patients.
further limitations of this study are the small number of patients with respiratory diseases, the lack of genetic testing in some patients, and the lack of detailed information about nutritional status and neurophysiological follow-up examinations.
, however, the most complete assessment of PNP risk factors in ALS is presented, especially by including genetic discovery and precise physiological characteristics, as well as standardized neurophysiological data.
men with respiratory amyotrophic lateral sclerosis had a higher risk of PNP.
follow-up study of these patients is necessary to understand the possible role of hypoxia in causing peripheral neurological dysfunction.
de Carvalho M, Gromicho M, Andersen P, et al Peren neuropathy in ALS: phenotype associationJournal of Neurology, Neurosurgery and Psython Published Online First: 28 December 2020. doi: 10.1136/jnnp-2020-325 164MedSci Original Source: MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Mets Medicine" or "Source: MedSci Originals" are owned by Mets Medicine and are not authorized to be reproduced by any media, website or individual, and are authorized to be reproduced with the words "Source: Mets Medicine".
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