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This patient has the brachial diplegia variant of ALS with severe weakness and wasting of his upper extremities but little to no lower motor neuron involvement of his lower extremities, progressive over two years. Profuse fasciculations are rippling through his shoulder girdle and particularly in the pectoral muscles. There is atrophy of both upper extremities. Notice the guttering of the hands due to atrophy of the intrinsic muscles. The legs are spared.

Fasciculations are a characteristic feature of motor neuron disease. They can occur in any chronic denervating process but when this widespread and when accompanied by such weakness and atrophy the diagnosis of motor neuron disease is inescapable. Early in the disease ultrasound may detect fasciculations that are not as obvious by clinical examination.


Campbell WW. Clinical signs in neurology : a compendium. Philadelphia: Wolters Kluwer Health, 2016.

Campbell WW. DeJong's the neurologic examination, 7th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2013. de Carvalho M, Swash M. Origin of fasciculations in amyotrophic lateral sclerosis and benign fasciculation syndrome. JAMA Neurol. 2013;70:1562-5.

Mills KR. Characteristics of fasciculations in amyotrophic lateral sclerosis and the benign fasciculation syndrome. Brain. 2010;133:3458-69.

Swash M, Carvalho Md. Muscle ultrasound detects fasciculations and facilitates diagnosis in ALS. Neurology. 2011;77:1508-9.