Dropped head syndrome as a presenting sign of scleromyositis

The isolated or prominent weakness of cervical extensor muscles is a relatively rare clinical sign known as "dropped head syndrome". It occurs in a variety of neuromuscular diseases. Here, we report the case of a 53 year old woman whose main symptom was a dropped head syndrome that led to the diagnosis of scleromyositis. Scleromyositis is a rare idiopathic inflammatory myopathy, which combines polymyositis and scleroderma symptoms. Although idiopathic inflammatory myopathies are listed as causes of dropped head syndrome, very few cases are reported in the literature. Furthermore, scleromyositis revealed by a dropped head syndrome has never been described. As this condition was totally reversed by a regiment of corticosteroids, it is thus of a diagnostic interest for neurologists to be aware of idiopathic inflammatory myopathies, including scleromyositis, as a potentially treatable aetiology of dropped head syndrome.