Abstract
Motor neuron disease/amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder characterised by loss of upper motor neurons (including the Betz cells of the motor cortex), and lower motor neuron, anterior horn cells of the spinal cord and brainstem nuclei. 5–10% in ALS is hereditary and sporadic, with an incidence of 2–3 per 100,000. ALS is a fatal disease. The average survival of patients is 3–5 years. So far, the pathogenesis of ALS has been unclear. Although there are many drugs in the trial, there is no reversible treatment for motor neurons that are already degenerating. Although more and more researchers are investing a lot of energy in research, the time for identifying this disease is still very long. In the diagnosis of ALS patients, in order to obtain appropriate treatment and care, it is great significance of the concept of “the earlier the better” to diagnose. The purpose of this study was to explore factors for possible early diagnosis and potential therapeutic agents by reviewing the literature and collecting case data and discuss their potential for improvement. We retrospectively analyzed ALS patients from 23427 patients in department of neurology in Xi’an Gaoxin Hospital between December 2006 and March 2017. The conclusion displayed that early neurological electrophysiological examination combined with clinical features can improve the diagnosis of ALS.
Highlights
MND/ALS is an adult-onset neurodegenerative disorder characterised by loss of upper motor neurons (UMNs, including the Betz cells of the motor cortex), and lower motor neuron (LMNs, anterior horn cells of the spinal cord and brainstem nuclei) (Bäumer et al 2014)
Screening of limb muscles including deltoid, triceps, biceps brachii, abductor pollicis brevis (APB), first dorsal interosseous (FDI), abductor digiti minimi (ADM), quadriceps, tibialis anterior (TA) and musculus gastrocnemius (MG) ; Screening for extra muscles based on clinical performance
It improved 4 cases from clinically possible to clinically probable (7.1% of total cases); 6 cases from clinically probable to clinically definite (10.7% of total cases); 2 cases from clinically possible to clinically definite (3.6% of total cases) (Tables 1, 2, 3, 4). 39 males and 17 females patients were in this group of ALS
Summary
MND/ALS is an adult-onset neurodegenerative disorder characterised by loss of upper motor neurons (UMNs, including the Betz cells of the motor cortex), and lower motor neuron (LMNs, anterior horn cells of the spinal cord and brainstem nuclei) (Bäumer et al 2014). The term MND is largely synonymous with ALS, reflecting the observation that most patients demonstrate combined LMN-related loss. In the diagnosis of ALS patients, in order to obtain appropriate treatment and care, it is great significance of the concept of “the earlier the better” to diagnose. The purpose of this study was to explore potential therapeutic agents and factors for possible early diagnosis by reviewing the literature and collecting case data and discuss their potential for improvement
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