Neuropediatrics 2000; 31(3): 162-163
DOI: 10.1055/s-2000-7493
Letter to the Editor

Georg Thieme Verlag Stuttgart · New York

A Rare Variant of Guillain-Barré Syndrome with Acute Motor Axonal Neuropathy (AMAN) in a Caucasian Boy

N. Nagdyman1 , F. Behse2 , M. Schülke3
  • 1 Department of Neonatology, Charité University Hospital, Humboldt University Berlin, Germany
  • 2 Department of Neurology, Charité University Hospital, Humboldt University Berlin, Germany
  • 3 Department of Neuropediatrics, Charité University Hospital, Humboldt University Berlin, Germany
Further Information

Publication History

Publication Date:
31 December 2000 (online)

Sir,

Guillain-Barré syndrome (GBS) is no longer considered a single entity. It is generally accepted as an acute immuno-mediated, predominantly motor neuropathy of the peripheral nervous system [[3], [7]]. In most cases the pathogenesis appears to reflect an inflammatory autoimmune attack on myelin-forming cells that results in demyelination [[2]]. However, axonal damage with varying degrees of Wallerian-like degeneration may also occur in 3 to 16 % of GBS patients [[7]]. These patients are generally more severely affected and tend to recover more slowly [[5]]. A separate clinical subset of axonal GBS with a peak incidence in summer - termed acute motor axonal neuropathy (AMAN) - has been reported almost exclusively in Chinese children [[4]]. Its characteristic features comprise rapidly progressing ascending paresis, axonal degeneration of predominantly intramuscular motor nerve terminals, while not involving the sensory nerves, and rapid recovery. Campylobacter jejuni infections seem to play a causative role [[1], [7]].

We report on a 16-month old Caucasian boy who presented with AMAN 3 weeks after a gastroenteritis. He was admitted to hospital after a 2-day history of unsteady gait and finally the inability to walk. Besides a symmetric flaccid paralysis of both lower limbs, he showed cranial nerve involvement with severe ptosis and facial weakness (Fig. [1 a]). However, brain stem function, conjugate ocular movements and muscle strength of the upper extremities remained completely unimpaired. Deep tendon reflexes were absent in the lower limbs but normal in the upper limbs. Pain sensation and bladder function were preserved. Within one week the patient developed a bilateral equinus foot. Respiratory function, as monitored by blood gas analysis and continuous S aO2 measurements, was unimpaired. An intracranial or intraspinal mass were excluded by cranial and spinal MRI.

Fig. 1 a Bilateral ptosis and facial weakness in a 16-month old boy with an acute motor axonal neuropathy on admission.

Fig. 1 b The same child 5 months later.

Initial laboratory tests failed to reveal any evidence of an ongoing infection and the CSF analysis was normal. Only 5 days later was an elevated protein content (244 mg/dl) and a mononuclear pleocytosis measured (40 cells/µl). Serum antibody titers against Campylobacter jejuni were marginally raised (1 : 20). Serological tests excluded acute infections with Borrelia burgdorferi and other neurotropic or entero viruses. A TensilonTM test and anti-acetylcholine receptor antibody studies were negative. Stool investigations excluded intestinal colonization with Clostridium botulinum. Ten days after disease onset we detected rising serum titers for anti-ganglioside antibodies (anti-GM1, -GD1 b, -GQ1 b), for antibodies against myelin-associated glycoprotein and against myelin basic protein. Motor and sensory nerve-conduction velocity and F-wave studies performed on days 2 and 10 after admission were normal in the median, tibial and sural nerves. Only the compound motor action potential of the abductor hallucis muscle decreased from 4 to 2 mV between days 2 and 10. Needle EMG of the anterior tibial muscle performed 15 days after the onset of clinical symptoms revealed pathologically spontaneous activity. At forced contraction, a decreased recruitment of normally formed action potentials was observed. The EEG, brainstem evoked potentials and a decrement test of the ulnar nerve were normal.

He was treated with a single high-dose of γ-globulin (1 g/kg) and after 2 weeks the symptoms started to disappear. Deep tendon reflexes returned faintly on day 16. The patient was discharged from hospital after 19 days. He was able to stand without support and one week later at follow-up he was able to walk independently. Ptosis was markedly reduced albeit still present. Five months later we saw a normal child (Fig. [1 b]) with normal deep tendon reflexes but a residual bilateral equinus foot of 10 degrees.

The main differential diagnoses, such as transverse myelitis (normal bladder function, preserved sensation, normal spinal MRI), poliomyelitis (previous immunization, no specific IgM antibodies), Miller Fisher syndrome (absence of extraocular muscle and bulbar symptoms and of ataxia) and a myasthenic crisis (normal TensilonTM and decrement tests, negative anti-AChR antibodies) were ruled out. Demyelinating GBS and AMAN are difficult to differentiate on purely clinical grounds [[4]]. In this case, only electrophysiological and the immunological studies provided the diagnosis. Electrodiagnostic findings characteristic for demyelinating GBS are delayed or absent F-waves and increased distal latencies [[7]]. These features were missing in this case. Only diminishing compound motor action potentials, decreased recruitment on needle EMG and signs of denervation led us to suspect the diagnosis of AMAN. Recent studies into the pathophysiology of AMAN revealed products of complement activation and immunoglobulins on the nodal axolemma and in the periaxonal space of myelinated motor fibers [[6]]. Axonal loss was most pronounced in the motor nerve terminals and intramuscular axons. Since AMAN is often preceded by Campylobacter jejuni infections, Ho et al hypothesized that bacterial ganglioside-like epitopes might trigger the production of autoantibodies that cross-react with nerve targets. The motor nerve terminals that are not shielded by a blood/nerve barrier might be particularly susceptible to autoimmune attack [[1]]. Axonal damage restricted to the terminal portion of the motor nerve might, therefore, explain the rapid recovery. In this case the Campylobacter jejuni titers were only marginally positive, but elevated serum anti-GM1 antibodies and the clinical history of previous gastroenteritis are strongly suggestive of an immune-mediated pathogenesis. The predominance of AMAN in the Chinese population might serve as an example of how the genetic background of an individual influences his/her susceptibility to a certain disease phenotype.

References

  • 1 Ho T W, Hsieh S T, Nachamkin I, Willison H J, Sheikh K, Kiehlbauch J. et al . Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection.  Neurology. 1997;  48 717-724
  • 2 Hughes R A, Rees J H. Guillain-Barré syndrome.  Curr Opin Neurol. 1994;  7 386-392
  • 3 McFarlin D E. Immunological parameters in Guillain-Barré syndrome.  Ann Neurol. 1990;  27 (Suppl) S25-S29
  • 4 McKhann G M, Cornblath D R, Griffin J W, Ho T W, Li C Y, Jiang Z. et al . Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China.  Ann Neurol. 1993;  33 333-342
  • 5 Korinthenberg J, Schulte-Mönting J. Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study.  Arch Dis Child. 1995;  74 281-287
  • 6 Rees J H, Soudain S E, Gregson N A, Hughes R A. Campylobacter jejuni infection and Guillain-Barré syndrome.  N Engl J Med. 1995;  333 1374-1379
  • 7 Trojaborg W. Acute and chronic neuropathies: New aspects of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, an overview and an update.  Electroenc Clin Neurophysiol. 1998;  107 303-316

M.D. Nicole Nagdyman

Department of Neonatology Charité University Hospital Humboldt University Berlin

Augustenburger Platz 1

13353 Berlin

Germany

Email: E-mail: nicole.nagdyman@charite.de

    >