Abstract:OBJECTIVE: To investigate the clinical and electrophysiological characteristics and prognosis of acute motor axonal neuropathy (AMAN) in children in South China. METHODS: The clinical and electrophysiological data of 6 children with AMAN was analyzed, and they were followed up. RESULTS: The mean age of onset was 4.4 years. Most patients came from rural areas and 5 cases had a history of prodromal infection. There were no seasonal differences in clinical onset among the patients. The most common first symptom was muscle weakness, and the mean time from onset to the most severe disease status was 4.2 days. Nerve conduction test results revealed that all patients showed significantly lower amplitude of motor nerve action potential, only 22.3%-73.4% of the lower limit of normal. Injury to the nerves of distal extremities was more serious than injury to the nerves of proximal extremities (P0.05). Motor nerve conduction velocity and sensory nerve conduction velocity were normal. All patients received intravenous immunoglobulin (IVIG). Of the 6 AMAN patients, 4 could walk independently after a follow-up of 3 months to 1 year. CONCLUSIONS: AMAN in children occurs mostly in rural areas. There is no seasonal difference in the clinical onset of the disease. Muscle weakness is the most common first symptom and the worst status of AMAN appears in the early stage of the disease. Electrophysiological examination provides important information for the diagnosis of AMAN. Some children with AMAN regain the ability to walk independently 1 year after onset. Early application of IVIG treatment may help recovery of neural function.
LIU Chen-Tao,ZHANG Guo-Yuan,WANG Guo-Li et al. Clinical and electrophysiological characteristics, and prognosis of acute motor axonal neuropathy in children[J]. CJCP, 2013, 15(3): 192-195.
[2]Hughes RA, Cornblath DR. Guillain-Barre syndrome[J]. Lancet, 2005, 366(9497): 1653-1666.
[3]Nagasawa K, Kuwabara S, Misawa S, Fujii K, Tanabe Y, Yuki N, et al. Electrophysiological subtypes and prognosis of childhood Guillain-Barré syndrome in Japan[J]. Muscle Nerve, 2006, 33(6): 766-770.
[5]Lee JH, Sung IY, Rew IS. Clinical presentation and prognosis of childhood Guillain-Barré syndrome[J].J Paediatr Child Health, 2008, 44(7-8): 449-454.
[6]Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, et al. An acute axonal form of Guillain-Barré polyneuropathy[J]. Brain, 1986, 109 (Pt 6): 1115-1126.
[7]Lee JH, Sung IY, Rew IS. Clinical presentation and prognosis of childhood Guillain-Barre syndrome[J]. J Pediatr Child Health, 2008, 44(7-8): 449-454.
[8]Nishimoto Y, Susuki K, Yuki N. Serologic marker of acute motor axonal neuropathy in childhood[J]. Pediatr Neurol, 2008, 39(1): 67-70.
[10]Hiraga A, Mori M, Ogawara K, Kojima S, Kanesaka T, Misawa S, et al. Recovery patterns and long term prognosis for axonal Guillain-Barré syndrome[J].J Neurol Neurosurg Psychiatry, 2005, 76(5): 719-722.
[11]Tamura N, Kuwabara S, Misawa S, Kanai K, Nakata M, Sawai S, et al. Time course of axonal regeneration in acute motor axonal neuropathy[J]. Muscle Nerve, 2007, 35(6): 793-795.
[12]Kuwabara S, Mori M, Ogawara K, Hattori T, Yuki N. Indicators of rapid clinical recovery in Guillain-Barré syndrome[J]. J Neurol Neurosurg Psychiatry, 2001, 70(4): 560-562.