Dropped-head syndrome due to steroid responsive focal myositis: a case report and review of the literature.
Isolated severe weakness of the paraspinal musculature, either at the cervical level leading to a "dropped head syndrome" or at the thoracic level leading to a "bent-spine syndrome", is a rare disorder. Etiologically it may be present in a variety of neurological diseases including Parkinson's disease, multiple system atrophy, neuromuscular or motor neuron diseases, as well as non-inflammatory, inflammatory, dystrophic or metabolic myopathies. We present a previously healthy 74-year-old man with a 2-month history of progressive difficulty in lifting his chin off his chest. Magnetic resonance imaging and skeletal muscle biopsy revealed an isolated myositis of the neck extensor and trapezius muscles, which responded well to steroid therapy. This case and other rare reports obtained from a systematic review of the literature indicate that in a subgroup of patients "dropped head syndrome" or "bent spine syndrome" is caused by a myositis, which emphasizes the necessity to obtain a MRI examination, as well as a muscle biopsy to diagnose this potentially treatable disease.
Kastrup
Andreas
A
Department of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany. andreas.kastrup@medizin.uni-goettingen.de
Gdynia
Hans-Jürgen
HJ
Nägele
Thomas
T
Riecker
Axel
A
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D001379:Azathioprine / Q000008:administration & dosage; D001706:Biopsy; D018450:Disease Progression; D004487:Edema / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D005260:Female; D019416:Head Movements / Q000502:physiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D018908:Muscle Weakness / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D009133:Muscular Atrophy / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D009220:Myositis / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D009334:Neck Muscles / Q000187:drug effects / Q000473:pathology* / Q000503:physiopathology; D011239:Prednisolone / Q000008:administration & dosage; D013121:Spinal Curvatures / Q000209:etiology / Q000503:physiopathology; D013577:Syndrome; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine; D008775:Methylprednisolone