Subcutaneous immunoglobulin therapy in a patient with myopathic dropped head syndrome and common variable immunodeficiency.
Prominent neck extension weakness is an uncommon clinical entity, also termed dropped-head syndrome, that may be part of a generalized neuromuscular disorder. We report here the case of a woman with dropped-head syndrome and pulmonary arterial hypertension secondary to systemic sclerosis. Subsequently, she developed common variable immunodeficiency and subcutaneous immunoglobulin therapy was started. After two months from the start of therapy we did not observe any improvement in the degree of flexion of the head, although the clinical examination shows an improvement in neck extensor muscle strength. Subcutaneous immunoglobulin therapy could be a possible therapeutic option for the treatment of myopathic neck extensor weakness.
Sapienza University of Rome, Department of Clinical Medicine, Clinical Immunology Unit, Scleroderma Center, Rome, Italy.
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Rosato
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Molinaro
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Pisarri
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Salsano
D000368:Aged; D017074:Common Variable Immunodeficiency / Q000628:therapy*; D005260:Female; D006801:Humans; D007116:Immunization, Passive / Q000379:methods*; D055104:Infusions, Subcutaneous; D009334:Neck Muscles / Q000473:pathology*; D009468:Neuromuscular Diseases / Q000628:therapy*; D012595:Scleroderma, Systemic / Q000150:complications*