Natural history of SMA IIIb: muscle strength decreases in a predictable sequence and magnitude

Neurology
Feza DeymeerMehves Poda

Abstract

To assess the natural progression of muscle weakness in spinal muscular atrophy (SMA) IIIb. Ten patients with SMA IIIb were followed for at least 10 years. Age at disease onset varied between 9 and 18 years. Patients were initially seen 2 to 10 years after disease onset. They were evaluated at approximately 2, 5, 10, 15, and 20 years of disease duration depending on the timing of their initial visit after onset. Medical Research Council (MRC) scale was used with particular attention to proximal muscles. The MRC grade declined with years in all of the muscles. The decline was usually not more than by one MRC grade for each 5-year period. There were 5-10 year periods when some muscles appeared to remain stationary. The succession of weakness was first triceps, then biceps and deltoid for upper extremity muscles and first thigh adductors, then iliopsoas, then quadriceps femoris, then hamstrings, thigh abductors, and gluteus maximus for lower extremity muscles. There was a remarkable uniformity between patients in the MRC grade for each muscle at each stage: in the first 5 years of the disease, triceps, iliopsoas, thigh adductors, and quadriceps femoris were the muscles which had noticeable weakness. These findings show that streng...Continue Reading

Citations

Feb 16, 2011·Archives of Neurology·Petra KaufmannUNKNOWN Pediatric Neuromuscular Clinical Research Network for Spinal Muscular Atrophy
Oct 19, 2012·Neurology·Petra KaufmannUNKNOWN Pediatric Neuromuscular Clinical Research Network for Spinal Muscular Atrophy (PNCR)
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Apr 14, 2012·Muscle & Nerve·Seward B RutkoveBasil T Darras
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Oct 21, 2016·Muscle & Nerve·Sally Dunaway YoungDarryl C De Vivo
Dec 14, 2011·Journal of Child Neurology·Sally DunawayDarryl C De Vivo
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Aug 6, 2020·Journal of Personalized Medicine·Piera SmeriglioMaria Grazia Biferi
Oct 9, 2019·Journal of Neuromuscular Diseases·Maggie C WalterBenedikt Schoser
Mar 14, 2020·Orphanet Journal of Rare Diseases·Hamish W Y WanMichelle A Farrar
Jul 16, 2020·Neurology·Feza Deymeer
Aug 1, 2020·Neurology·Camiel A WijngaardeW Ludo van der Pol
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Sep 17, 2009·Current Opinion in Neurology

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