گزارش موردي اسكلروز اميوتروفيك كناري

Report of a Case of Amyotrophic Lateral Sclerosis

Introduction:Amyotrophic lateral sclerosis (ALS) is the benchmark of the motor neuron disorders. ALS encompasses two conditions: progressive bulbar palsy and progressive atrophy. Assistant The classic from of ALS is the most prevalent motor neuron disease. The incidence of ALS is approximately 1.6 to 2.4 cases Sciences per 100000 population, but this varies with age. The mean age of onset is 55 years old. Distal asymmetrical weakness commonly develops as the early symptom, which is limited to only one limb. The disease progresses rapidly to affect the muscles of the trunk and those innervated by the cranial nerves. Sensation is generally spared. The diagnosis of ALS is based on the presence of a progressive disorder with the characteristic combination of upper and lower motor neuron involvement and confirmed by electro- diagnostic studies. Case: The patient is a man, 54 years old, who referred with progressive weakness and atrophy in right and left upper limbs begun 3 years and 4 months ago, respectively. In physical examination, manual muscle testing of right and left upper limb muscles were 1/5 and 3-4/5 respectively, Normal sensation and gait, muscle stretch reflexes of upper and lower limbs were 0.2 and 3/2 , positive Babinski and Hoffman reflexes and tongue fasciculations were observed. There was mild spasticity in his lower limbs. MRIʹs of brain and cervical spin were normal. In nerve conduction study, amplitude of compound muscle action potential of upper limbs decreased or was absent and in needle electromyography there were denervation in tested upper limbs muscles and fasciculations in tongue muscles.Conclusion: Diagnosis of motor neuron diseases results in appropriate treatment and prevention of unnecessary procedures of diagnosis.