Clinical characteristics and neurophysiology features of amyotrophic lateral sclerosis patients at Sanglah Hospital Denpasar

I Made Odie Lastrawan,Komang Arimbawa,Ni Made Dwita Pratiwi

doi:10.37897/rjn.2021.3.11

Abstract

Background. Amyotrophic lateral sclerosis (ALS) is a one of motor neurons disease. The Incidents in Europe are 2.6 per 100,000 people per year and in the United States, more than 5,600 are diagnosed each year. The death was reportedly 2 per 100,000 people per year. In Sanglah Hospital there are no study that described the characteristics clinical and neurophysiology of ALS at Sanglah Hospital. Objective. To find the characteristics of ALS based on clinical and neurophysiology of ALS at Sanglah Hospital in terms of demography, clinical, Nerve Conduction Studies (NCS) and Electromyography (EMG). Methods. Retrospective descriptive study using medical records of patients in neurology polyclinic at Sanglah Hospital from January until December, 2018. Results. From 14 ALS patients with average age of 47 years old, men and women same amount, high school 71.4%, unemployed 50 %, and married 92.8%. Symptoms first appeared with an average age of 42 years with complaints of lower limb weakness 64.3%. The diagnosis of ALS with the symptom UMN and LMN on bulbar and 2 spinal region 71.4%, and symptom with the UMN and LMN on 3 spinal region 28.6%. Results of motor and sensory NCS were normal, type axonal and mixed neuropathy. Characteristics of EMG were PSW +4, fibrillation +4, MUAP: high amplitude (giant potential), widened duration, polyphasic phase, incomplete IP recruitment in all patients (100%) although in different muscles of the examined patient. Conclusions. Characteristics from 14 patients ALS in 2018 at Sanglah Hospital indicate that patients have a good prognosis.

Highlights

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that attacks motor neurons
The inclusion criteria were all neurological patients with ALS suspicion who came to the polyclinic and who underwent an ENMG examination at Sanglah Hospital Denpasar
Data were obtained in the form of the ALS incidence rate in Sanglah Hospital for the period January until December 2018, with total of 14 patients

Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that attacks motor neurons. Lateral sclerosis indicates hardening of the corticospinal tracts lateral and anterior where the motor neurons in the area degenerate through the process of gliosis. Through this combination of terms, it can be illustrated that in this disease a mixed lesion of upper motor neuron and lower motor neuron occurs (1-3). The incidence of ALS in Europe is around 2.6 people per 100,000 per year (4). ALS is classified as a rare disease designated by the FDA as an “orphan” disease (affecting less than 200,000 people in the United States), but it is the most common motor neuron disease. Characteristics from 14 patients ALS in 2018 at Sanglah Hospital indicate that patients have a good prognosis

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Discussion

Conclusion