SQSTM1 and Restrictive ventilatory defect: Twelve patients (9 VCP, 2 SQSTM1 + TIA1, 1 TFG) had pulmonary function tests at median of 15 years (range 5–22) after disease onset: 6 showed restrictive ventilatory defect (FVC 70–80% of predicted in 3 patients, FVC <70% of predicted in 3 patients) with median vital capacity of 2.1 L (range 1.8–3.8); the other 6 had reduced maximum inspiratory or expiratory pressure suggestive of neuromuscular weakness.