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Dropped Head: An Unusual Presentation of Dermatomyositis To the Editor: Muscle weakness is a cardinal feature of idiopathic inflammatory myopathies that triggers further investigations and proper diagnosis by high serum levels of muscle enzymes, characteristic electromyography (EMG), and muscle biopsy1. Most patients with bilateral, symmetric, progressive, proximal weakness leading to difficulty getting up out of a chair or combing their hair are usually diagnosed within weeks or months from onset of complaints, while those presenting with atypical weakness may go undiagnosed, and subsequently untreated, for years. We describe a case of dermatomyositis with prominent weakness of axial musculature and resultant dropped head syndrome. While neck flexor involvement with difficulty in raising one's head from a pillow is a known manifestation of inflammatory myopathy2, a weakness of neck extensors with dropped head has not been previously reported, to our knowledge, as a presenting feature of dermatomyositis. A 66-year-old Caucasian woman first presented in March, 2003, with a 4-month history of difficulty in maintaining her head erect. Her muscle strength was estimated as 2/5 for neck extensors and 4/5 for deltoid muscles, with other muscle groups normal. EMG revealed C6 radiculopathy with no myopathic changes. Computer tomography (CT) and later magnetic resonance imaging (MRI) of the C-spine showed degenerative changes of the C3-C7 segments with minimal spinal stenosis, which was not felt to explain the patient's complaints. Final diagnosis was deferred. In September 2003 the patient was reevaluated because of worsening weakness of the neck with complete inability to hold her head up and proximal weakness of both upper and lower extremities. She had a waddling gait with dropped head and declined her torso in a sway back posture to allow her to see where she was walking. The review of systems revealed mild dysphagia, Raynaud's phenomenon, and weight loss of about 20 kg since onset of symptoms 9 months earlier. Her medications were amitriptyline 25 mg, cimetidine 400 mg, and calcium carbonate 600 mg. Physical examination was remarkable for complete head drop, weakness of the neck extensors 0/5, left deltoid 3/5, right deltoid 4/5 and bilateral iliopsoas 3/5, violaceous rash over her forehead, upper back, chest, thighs, metacarpophalangeal and proximal interphalangeal joints, and lesions compatible with Gottron papules. She had periungular telangiectasia and taut skin on her distal fingers. Routine laboratory studies, including complete blood count, serum creatinine, electrolytes, proteins, thyroid and liver function tests, were normal. CPK level was 419 U/l (normal range up to 100 U/l), lactate dehydrogenase was 344 U/l (normal range up to 240 U/l). C-reactive protein was 9.3 (normal up to 6) and erythrocyte sedimentation rate was 55 mm/h. Antinuclear antibody test was weakly positive in a nucleolar pattern, while extractable nuclear antigen (ENA) screen including anti-Jo-1 antibodies, as well as rheumatoid factor and antibodies to acetylcholine receptor were negative; serum complement levels C3 and C4 were normal. Extensive investigations to rule out underlying malignancy were negative. EMG of the right deltoid, splenius capitus, and semispinalis muscles showed no spontaneous activity with fast recruitment of small, short, and polyphasic units. A biopsy of the left deltoid muscle was performed and revealed atrophic degenerative and necrotic fibers with foci of inflammatory infiltrate, composed of both T and B cells, compatible with dermatomyositis. Dermatomyositis was diagnosed and the treatment with 1 mg/kg/day steroids and 15 mg of methotrexate (MTX) weekly was started. Two months later, monthly intravenous immunoglobulin (IVIG) was added to her treatment due to failure to improve. The patient reported gradual improvement in muscle strength since starting IVIG, which allowed tapering of her steroid dose. On reevaluation in June, 2004, she was able to hold her head straight up with no help and had returned to an active lifestyle. Her rash had disappeared and serum muscle enzyme levels were normal. Restricted or isolated weakness of the extensors of the neck is a relatively rare condition, designated the dropped head syndrome, generally seen as a part of generalized neuromuscular disorder such as myasthenia gravis or amyotrophic lateral sclerosis3-5. The dropped head is not widely described as a manifestation of rheumatic disease, despite the presence of inflammatory myopathies in the list of differential diagnoses4. Reported cases of dropped head plus syndrome, characterized by the predominant weakness of neck extensors with restricted involvement of other proximal musculature, and biopsy varying from scattered interstitial inflammatory infiltrate to rich lymphocytic infiltration, may represent an early stage of polymyositis. These patients may have a good response to glucocorticosteroids and/or azathioprine with improvement of their muscle strength6-8. This case shows that the dropped head syndrome may also be an early and disabling symptom of dermatomyositis, still reversible with aggressive treatment. Awareness of such an unusual manifestation of inflammatory myopathy should advance diagnosis and antiinflammatory therapy and improve the overall prognosis of such patients.
GLEB SLOBODIN, MD, Department of Internal Medicine; MICHAEL ROZENBAUM, MD, Department of Rheumatology; BOAZ WELLER, MD, Department of Neurology; NINA BOULMAN, MD; ITZHAK ROSNER, MD, Department of Rheumatology, Bnai Zion Medical Center, Bruce Rapoport Faculty of Medicine, Technion, Haifa, Israel. REFERENCES 1. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975;292:344-7. 2. Oddis CV. Idiopathic inflammatory myopathies. In: Wortmann RL, editor. Diseases of skeletal muscle. Baltimore: Williams & Wilkins; 2000:45-86. 3. Lange DJ, Fetell MR, Lovelace RE, Rowland LP. The floppy head syndrome. Ann Neurol 1986;20:133. 4. Beekman R, Tijssen CC, Visser LH, Schellens RL. Dropped head as a presenting symptom of primary hyperparathyroidism. J Neurol 2002;249:1738-9. 5. Mahjneh I, Marconi G, Paetau A, Saarinen A, Salmi T, Somer H. Axial myopathy: an unrecognized entity. J Neurol 2002;249:730-4. 6. Rose MR, Levin KH, Griggs RC. The dropped head plus syndrome: quantitation of response to corticosteroids. Muscle Nerve 1999;22:115-8. 7. Goh KJ, Wong KT, Tan CT. Myopathic dropped head syndrome: a syndrome of mixed aetiology. J Clin Neuroscience 2000;7:334-6. 8. Biran I, Cohen O, Diment J, Peyser A, Bahnof R, Steiner I. Focal, steroid responsive myositis causing dropped head syndrome. Muscle Nerve 1999;22:769-71. |