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JAMA Neurology Clinical Challenge
March 2017

Improved Muscular Weakness During Asthma Exacerbation

Author Affiliations
  • 1CRMN Hôpital de la Citadelle, Liège, Belgium
  • 2Institute I-Motion, Armand Trousseau Hospital, Paris, France
JAMA Neurol. 2017;74(3):353-354. doi:10.1001/jamaneurol.2016.4069

A 48-year-old man attended the outpatient clinic for muscular weakness and restricted ability to walk, which had slowly increased since infancy. Medical history revealed muscle weakness noticeable since birth, but normal cognitive development. He first walked at age 18 months, but always had a waddling gait and hyperlordosis. The patient was examined at 3 neuromuscular centers. Electromyography (EMG), creatine phosphokinase levels, muscle tomodensitometry, and 3 muscle biopsies were normal. Results for acetylcholinesterase antibodies were negative. At age 48 years, the patient had increasing axial and proximal weakness, hyperlordosis (Figure, A), and difficulties rising from the floor. He was only able to walk 181 m in a 6-minute walking test. Peripheral facial weakness and bilateral eyelid ptosis were prominent. Clinical history also included asthma with several episodes of decompensation. During these episodes, the patient received oral albuterol, 2.5 mg every 6 hours, and inhaled steroids, 250 µg twice a day. Surprisingly, during an asthma attack, the patient was able to climb the stairs, rise from the floor, and walk several kilometers. His neuromuscular condition resumed to the initial level of weakness some weeks after the asthma attack.