JNNP: Overexcitement of split hand motor axons in spinal myeloid amyotrophic disorder.

The "split hand" sign refers to the common clinical symptoms of amyotrophic lateral sclerosis (ALS).
spinal myeloid amyotrophic disease (SBMA) is a rare adult seizure and hereditary motor neurone disease that has a genetic basis associated with repeated amplification of the androgen-incommonular (CAG) gene on the X chromosome.
muscle spasms and beam muscles are clinical symptoms of SBMA and are associated with excessive excitation of motor nerves.
this paper aims to study the excitability of split hand motor axons in complete spinal myeloid amyotrophic disease (SBMA) of cortical motor neurons.
: 35 patients treated with SBMA and not treated with bright propylene acetate.
in all of these patients, the first exon of the AR gene has an extended CAG trinucleotide repeat sequence.
neuro-excitement tests were conducted in 35 SBMA patients and 55 ALS patients, as well as 45 healthy subjects of age matching.
used a computer program (London Institute of Neurology) to perform multiple neuro-excitement measurements in the frontal nerves of the wrist.
CMAP from the AB muscles after a nerve stimulation in the middle of the wrist.
intensity duration constant (SDTC) is calculated by the relationship between stimulus intensity and duration to cause the target CMAP amplitude, using the Weiss formula.
a threshold to record 40% of the maximum CMAP amplitude.
results: ALS had a higher average examination age (p .lt;0.001), and all SBMA patients were male.
proportion of male patients in disease control is greater than in normal people (p .lt;0.001).
alSFRS-R scores were similar for patients with SBMA and ALS.
patients with ALS had a shorter course of illness than 2007 patients with SBMA.
CMAP amplitude of all three muscles in SBMA was lower than that of normal people (all, p .lt;0.001), but was comparable to ALS, while the CMAP amplitude of the control group for neuromuscular disease showed a moderate decrease.
found clinical symptoms of hand cleft hand in 57 percent of SBMA patients, 62 percent of ALS and 0 percent of the general population.
in SBMA, the relationship between nerve excitability and CMAP amplitude in muscles was analyzed.
there was no significant correlation between the excitability index and the CMAP magnitude, indicating that neuro-excitement changes were widespread, independent of the CMAP magnitude and disease stage.
results show that hand fractures are often found not only in ALS but also in SBMA patients using quantitative CMAP analysis, suggesting that both diseases have the same pathophysiology of motor neuron death.
Shibuya K, Misawa S, Uzawa A, et al Split hand and motor axonal hyperexcitability in spinal bulbar muscular atrophyJournal of Neurology, Neurosurgery and Psython Published Online First: 15 September 2020. doi: 10.1136/jnnp-2020-324026MedSci Original Source: MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Met Medical" or "Source: MedSci Original" are owned by Metz Medicine and are not reproduced by any media, website or individual without authorization, and must be reproduced with the words "Source: Mets Medicine".
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