LAUSR

Objectives ALS is a progressive neurodegenerative disease. Our aim was to examine the correlation of the severity of symptoms and the findings of electromyography (EMG), electroneurography (ENG) and to find parameters in estimating prognosis. Methods EMG/ENG findings were analysed between the 1st of January 2015 and 31st of December 2016. Twenty-eight patients fulfilled the International ALS Guideline’s criteria. In 4 patients repeated ENG/EMG confirmed the diagnosis at 3–6 months. Single fiber EMG was done to measure fiber density and jitter in15 patients. Results From symptom onset 57% of the patients were referred within 6 months. From the patients 36% had upper limb, 25% bulbar, 18% lower limb and 21% generalized onset of ALS. Despite this EMG revealed pathological findings generalized. Fasciculation, positive sharp waves and fibrillation were detected in all patients, and polyphasic potential in 90% of the patients. The findings were already moderate or severe in 60% of the patients. Increased fiber density and jitter was detected in 7 patients. Discussion Despite the typical clinical symptoms only 2/3 of the patients were referred within 6 months for examination. Even in patients referred early and the symptoms were only mild and localized the neurophysiological findings were already severe. Fasciculation and fibrillation in clinically not involved muscles are early characteristic signs in ALS. Conclusions Follow-up examinations are important if the diagnosis is uncertain. SF EMG examination may be helpful by estimating the prognosis. Significance Clinical neurophysiological examinations are important not only in diagnostics, but by following the progression too.