Amyotropic Lateral Sclerosis

These two videos are from a middle-aged man presenting with progressive bilateral lower limb and right upper limb weakness for few months. In the first video, there are prominent fasciculations over the entire right upper limb. Whilst in the second video, fasciculations can also be noted over both lower limbs, but more prominent on the left leg. Limb power was reduced over the distal muscles of both lower limbs and right arm. There were in addition brisk reflexes throughout and bilateral upgoing plantar reflexes. The patient had absence of any sensory symptoms nor any bulbar symptoms. Family history for a significant neurodegenerative disorder was negative. Investigations including imaging of the cervical spine excluded presence of a significant cervical / lumbosacral radiculopathy causing the symptoms and subsequent nerve conduction and studies and electromyography confirmed the diagnosis of a sporadic, limb-onset amyotrophic lateral sclerosis.

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