Objective:

Evaluating the use of rEl Escorial criteria (rEEC) in electromyography (EMG) evaluations and reports for patients with suspected MND.

Background:

Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease affecting the anterior horn cells and corticospinal tract. The clinical heterogeneity and lack of definitive diagnostic markers often leads to a delay in diagnosis and treatment. World federation of neurology research group proposed the rEEC to promote early diagnosis and clinical trial participation.

Design/Methods:

The authors reviewed 1500 EMG reports from July 2022 to June 2023 at a single institution. If the key words, “motor neuron disease” were noted in the final impression, data regarding rEEC, needle EMG and the final diagnosis at the time of the most recent visit was collected.

Results:

25 EMG reports were identified that included “motor neuron disease” in the final impression. REEC were utilized in report terminology in 60 % (n=15). Among these cases, 20 % (n=3) were classified as definite ALS and 80 % (n=12) probable ALS. In cases without rEEC, (n=10, 40%), alternate diagnoses at the time of last visit included facial onset sensory motor neuronopathy, axonal neuropathy, polyradiculopathy and ischemic lumbosacral plexus (n=4). 6 of the cases without REEC reference were classified as definite ALS, ALS workup or atypical ALS. EMG evaluation in suspected MND included 3 limbs, thoracic, and bulbar regions in 80 % (n=20) of the total cases. This evaluation was not complete in 20 % of the cases; citing declined test (n=1), anticoagulation (n=2), and unspecified (n=1).

Conclusions:

This study demonstrated a need for consistency in reporting and performing EMG studies concerning for MND. Future research can assess if consistent reporting of REEC can reduce the time to diagnosis, time to referral to a multidisciplinary ALS clinic and time to starting treatment for ALS.