Acute Respiratory Failure in a Patient with Stiff-Person Syndrome
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PRACTICAL PEARL
Acute Respiratory Failure in a Patient with Stiff-Person Syndrome Vincent Jachiet1 • Laurent Laine1 • Thierry Gendre2 • Carole Henry2 Daniel Da Silva1 • Etienne de Montmollin1
•
Ó Springer Science+Business Media New York 2016
Abstract Background Stiff-person syndrome (SPS) is a rare disorder characterized by progressive muscle stiffness, rigidity, and spasms involving the axial muscles. Acute respiratory distress has rarely been reported in this condition. Methods We report a case of a 49-year-old woman with autoimmune SPS diagnosed during an episode of acute respiratory failure secondary to repetitive episodes of apnea, requiring intensive care. Results Acute respiratory failure manifesting with apneic episodes is a life-threatening and unpredictable complication of SPS. Its pathophysiology is not well known. The two suggested mechanisms are as follows: (1) apnea due to muscle rigidity and paroxysmal muscle spasms, and (2) paroxysmal autonomic hyperactivity. Sudden and unexpected deaths have been reported in SPS, and all described cases have been associated with apnea. Thus, the onset of apnea during SPS should be considered a criterion of high severity and should lead to intensive care unit (ICU) admission for continuous monitoring. In patients with severe disease who are unresponsive to symptomatic treatment with benzodiazepines and baclofen, or in patients with life-threatening complications, early immunotherapy by intravenous immunoglobulins should be considered. Conclusion Onset of apneas during SPS should be considered as a signal of possible progression toward acute respiratory failure and sudden death, and should lead to & Etienne de Montmollin [email protected] 1
Intensive Care Unit, Centre Hospitalier de Saint-Denis, 2 Rue du Docteur Delafontaine, 93200 Saint Denis, France
2
Neurology Department, Centre Hospitalier de Saint-Denis, Saint Denis, France
ICU admission for continuous monitoring. Early immunotherapy should be started in such situations, including intravenous immunoglobulins as the first-line treatment. Keywords Stiff-person syndrome Intensive care unit Acute respiratory failure Apnea Intravenous immunoglobulins
Introduction Stiff-person spectrum disorder (SPSD) is a rare disease characterized by progressive muscle stiffness, rigidity, and spasms involving the axial muscles and results in severely impaired ambulation [1]. SPSD has an estimated prevalence of one to two cases per million, with an incidence of one case per million per year [2]. Apart from classic stiff-person syndrome (SPS), clinical presentations include stiff-limb syndrome (SLS) and progressive encephalomyelitis with rigidity and myoclonus. There can also be overlaps between SPSD and ataxic, epileptic and encephalitic syndromes [3]. There is an important phenotypical variability of SPSD, with several associated autoantibodies; and a classification based on likely etiology has been proposed [4]. Autoimmune SPS (70 %) is defined by the autoantibody positivity in the absence of unde
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