Dropped head with positive intravenous edrophonium, progressing to myasthenia gravis.

Abstract

'Dropped head syndrome' (DHS) may be associated with a variety of neurological diseases. The absence of neurological clues to the underlying cause of DHS can make management particularly challenging. We review six patients who presented with only DHS, responded to intravenous edrophonium and turned out to have myasthenia gravis (MG) including similar patients who were previously documented. Six patients presented with neck weakness and three had bulbar symptoms. Acetylcholine receptor (AchR) was positive in four patients. One patient had thymoma. The interval from the onset of DH to the presentation of typical MG features was shorter in patients who tested positive for anti-Ach antibody (1-2 months) than in patients who tested negative for anti-AchR antibody (13 months, 4 years). Our results suggest that patients with DHS responding to intravenous edrophonium might turn out to have MG and such patients might respond to a combination of anticholinesterase agents and steroids.

• Authors: Nobuhiro Sawa, Hiroshi Kataoka, Nobuyuki Eura, Satoshi Ueno
• Journal: BMJ case reports (BMJ Case Rep) Vol. 2013 (Jan 31 2013) ISSN: 1757-790X [Electronic] England
• PMID: 23376659 (Publication Type: Case Reports, Journal Article)

Chemical References

• Cholinesterase Inhibitors
• Edrophonium

Topics

• Aged
• Aged, 80 and over
• Cholinesterase Inhibitors (administration & dosage, therapeutic use)
• Disease Progression
• Edrophonium (administration & dosage, therapeutic use)
• Female
• Head
• Humans
• Injections, Intraventricular
• Muscle Weakness (drug therapy, etiology)
• Myasthenia Gravis (complications, diagnosis)
• Neck Muscles (drug effects, physiopathology)
• Syndrome