RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164088279 T1 Wohlfart-Kugelberg-Welander Syndrome T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164088279 RD 2024/03/29 AB It is an autosomal recessive juvenile spinal muscular atrophy characterized by slowly progressive muscular weakness and atrophy of the upper and lower limbs. It is caused by the degeneration of anterior horn cells (spinal motor neurons). The onset of the disease is during childhood, particularly between the ages of 2 and 17 years. Early symptoms consist of atrophy and weakness of the proximal muscle of the extremities (mainly legs), followed by thoracic muscles. Should not be confused with muscular dystrophy syndromes.