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Bent spine syndrome (BSS), an abnormal trunk anteroflexion of at least 45° that increases during walking and abates in supine position, is described in parkinsonism and myopathies.1 We report a 56-year-old man with genetically proven late-onset Pompe disease (LOPD) showing BSS (figure 1 and video 1 at Neurology.org). He complained of axial weakness that progressively forced him to walk stretching his arms backward since his 20s. Whole-body muscle MRI revealed severe fatty replacement of lumbar paraspinal (figure 2) and iliopsoas muscles, and spirometry showed restrictive ventilatory defect. Periodic acid-Schiff–positive vacuoles and reduced acid α-glucosidase activity were demonstrated in muscle biopsy. While lumbar hyperlordosis is a typical feature of LOPD, other trunk abnormalities may appear early in the disease. LOPD must be included in the differential diagnosis of BSS.2
Footnotes
Republished from Neurology 2017;89(3):e21-e22. doi: 10.1212/WNL.0000000000004119
Supplemental data at Neurology.org
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- © 2017 American Academy of Neurology
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