Myopathic dropped head syndrome: a syndrome of mixed aetiology.
We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.
Goh
K J
KJ
Division of Neurology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Wong
K T
KT
Tan
C T
CT
D000328:Adult; D019416:Head Movements / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology* / Q000473:pathology* / Q000503:physiopathology; D009135:Muscular Diseases / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D009334:Neck Muscles / Q000473:pathology* / Q000503:physiopathology