Amyotrophic Lateral Sclerosis

A 62-year-old woman with hypothyroidism, dyslipemia, and a depressive syndrome first consulted our rheumatology unit in July 2002. She had a 6-month history of seropositive rheumatoid arthritis treated with corticosteroids and NSAIDs. Physical examination revealed 7 NSJ, 12 NTJ, and ESR was 107 mm/h. Methotrexate therapy was started (to 15 mg s.c./w, maximum-tolerated dose) and hydroxychloroquine (400 mg/d) was added later with good response (1 NSJ, 2 NTJ, and ESR, 8 mm/ h ). Methotrexate was replaced with cyclosporine A (100 mg/d) in January 2006 because of fatty liver (elevated gammaglutamyl transpeptidase and compatible ultrasound scan), which was then replaced with azathioprine (50 mg/d) in August 2007 due to increased creatinine levels. In September 2007, after liver enzyme had normalized and a new polyarthritis outbreak (ESR, 79 mm/h) methotrexate treatment was reestablished in combination with adalimumab (40 mg, s.c./eow). In September 2008, she underwent a total left hip replacement. Two attempts to optimize anti-TNF therapy failed because of flares. In February 2010, methotrexate was replaced with hydroxychloroquine (400 mg/d). In April 2010, a basal cell carcinoma of the left eyelid and nose was excised. Being fully informed of the ACR 2008 guidelines, she decided to continue anti-TNF therapy with close monitoring and remained in remission.

Amyotrophic Lateral Sclerosis

In April 2012, after three years on adalimumab and sustained remission, the patient complained of progressively worsening muscle weakness; despite walking with a cane, she had frequent falls. In February 2015 she had difficulties swallowing that impaired her ability to eat and drink, accompanied by weight loss (17 kg in 6 months), dysarthria, and slurred speech. She had no urinary or fecal incontinence. Physical examination revealed global atrophy in the muscles of the arms, legs, and hand, and weakness; cachexia; hypomotility and fasciculations of the tongue; and hyperreflexia of the arms and normoreflexia of the legs. The cranial nerves were not affected, and there was no cognitive impairment.

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