title: Sensorimotor polyneuropathy in patients with SMA type-1: electroneuromyographic findings.
abstract: BACKGROUND
  Generally, spinal muscular atrophy (SMA) is believed to be a pure motor neuron disease. We retrospectively evaluated our electrodiagnostic findings in SMA type 1 patients to demonstrate co-existence of sensorimotor neuropathies.
  
  
  METHODS
  Electroneuromyographic (ENMG) studies in 15 patients (11 boys, 4 girls) were reviewed independently by 2 neurophysiologists. Upper extremity findings were compared with normal right arm controls.
  
  
  RESULTS
  Patient ages ranged from 1.5 to 26 months. Four SMA patients (26.7%) had decreased sensory nerve action potentials (SNAPs) or sensory nerve conduction velocities. Of them, median SNAPs could not be elicited in 3, and sural SNAPs could not be elicited in 2. Compound muscle action potential amplitudes were severely decreased in 14 (93.3%) and normal in 1.
  
  
  CONCLUSIONS
  Survival motor neuron 1 (SMN1) gene analysis should be considered if clinical features are consistent with SMA, even if pathological or electrophysiological findings demonstrate peripheral sensorimotor polyneuropathies.
authors: Akdeniz University Hospital, H Block, Department of Child Neurology, Antalya, Turkey. oduman@akdeniz.edu.tr
authors: Ozgur
authors: O
authors: Duman
authors: Hilmi
authors: H
authors: Uysal
authors: Karen L
authors: KL
authors: Skjei
authors: Ferah
authors: F
authors: Kizilay
authors: Sibel
authors: S
authors: Karauzum
authors: Senay
authors: S
authors: Haspolat
mesh_terms: D000200:Action Potentials / Q000502:physiology; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004576:Electromyography / Q000379:methods; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011115:Polyneuropathies / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology*; D012189:Retrospective Studies; D014897:Spinal Muscular Atrophies of Childhood / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology*