Acute Motor Axonal Neuropathy

Introduction[edit | edit source]

Acute motor axonal neuropathy (AMAN) is classified as a subtype of Guillain–Barré syndrome (GBS)[1]. The disease was first discovered in China and was nicknamed 'chinese paralytic disease'[1]. AMAN is the most common cause of flaccid paralysis in China[2]. Pathology in AMAN patients are usually limited to the motor nerve, with macrophages destroying axons while myelin remains intact, a contrast to AIDP GBS (most common form of GBS) which is known as a demyelinating disease[1]. While AMAN mainly involves axonal damage to motor nerve fibres; acute inflammatory demyelinating polyradiculoneuropathy (AIDP) involves both motor and sensory nerve fibre damage due to demyelination[1].

AMAN has an estimated prevalence of 30–65% in Asia, Central and South America[3].

Aetiology[edit | edit source]

AMAN is linked to Campylobacter jejuni and Zika virus infection[4][5].

Clinical features[edit | edit source]

AMAN presents similarly in terms of clinical features as well as CSF findings to GBS[1].

  • Weakness of limbs (earliest symptom is weakness of lower limbs)
  • Weakness of respiratory muscles
  • Gradual weakening and finally, absence of tendon reflexes
  • Bulbar palsy in 61% of patients[1]

However, it presents without any sensory symptoms, unlike GBS[6]. A neurophysiological screen in patients with AMAN should also show no evidence of demyelination, unlike GBS[6].

References[edit | edit source]

  1. 1.0 1.1 1.2 1.3 1.4 1.5 Lv J, Zhaori G. Collaborative studies of U.S.–China neurologists on acute motor axonal neuropathy - PMC (nih.gov) Pediatr Investig. 2022 Mar 22;6(1):1-4. doi: 10.1002/ped4.12316. PMID: 35382424; PMCID: PMC8960912.
  2. McKhann GM, Cornblath DR, Griffin JW, Ho TW, Li CY, Jiang Z, Wu HS, Zhaori G, Liu Y, Jou LP, Liu TC. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Annals of neurology. 1993 Apr;33(4):333-42.
  3. Van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, Van Doorn PA. Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nature Reviews Neurology. 2014 Aug;10(8):469-82.
  4. Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, Asbury AK, Blaser MJ, McKhann GM. Guillain-Barre syndrome in northern China Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995 Jun 1;118(3):597-605.
  5. Watrin L, Ghawché F, Larre P, Neau JP, Mathis S, Fournier E. Guillain–Barré syndrome (42 cases) occurring during a Zika virus outbreak in French Polynesia. Medicine. 2016 Apr 1;95(14):e3257.
  6. 6.0 6.1 Al-Saffar A, Al-Fatly B. Acute Motor Axonal Neuropathy in Association with Hepatitis E. Front Neurol. 2018 Feb 9;9:62. doi: 10.3389/fneur.2018.00062. PMID: 29479336; PMCID: PMC5811470.