Neuropediatrics 2008; 39 - P100
DOI: 10.1055/s-0029-1215869

Acute motor and sensory axonal neuropathy (AMSAN) in a 7-year-old boy. A cases report

U Kotzaeridou 1, D Vater 1, F Ebinger 1, J Pietz 1
  • 1Universität Heidelberg, Zentrum fuer Kinder und Jugendmedizin, Heidelberg, Germany

Acute motor-sensory neuropathy (AMSAN) is a variant of the Guillain-Barré Syndrom and has been characterized within the last few years in more detail. Characteristics of this disease are acute onset with weakness of distal extremities, areflexia.

Cases report: We describe here the case of a 7 year old patient with AMSAN. After infection of the upper airways the young patient developed acute symptoms of vegetative dysregulation, somnolence, tetraplegia, and respiratory failure requiring an artificial respirator. During onset of respiratory failure the patient suffered from a Grandmal seizure. The cerebrospinal fluid showed 14 cells, minimally elevated albumen, and distinct oligoclonal antibodies. Electroneurography of motor neurons indicated the clinical picture of axonal neuropathy. First treatment consisted of high doses of immunoglobulin. Furthermore, repeated plasmapheresis with subsequent administration of immunoglobins as response to pronounced heart rate variability, which was consistent with the underlying autonomous dysregulation. Due to increased mycoplasma antibody titers, antibiotic treatment with erythromycin was initiated. Later on, the patient exhibited a slow but steady improvement in his symptoms, with amelioration of the sensory deficits and restoration of his ability to communicate. Following that there was further improvement to his neurologic status with regards to the biceps reflex and motor strength to the extremities.

Before being discharged he was able to move his extremities, but he was not able to sit or ambulate.

Conclusion: Guillain Barre syndrome can be manifested in various ways: As an acute demyelinating polyneuropathy, as an acute motor axonal neuropathy and as an acute motor-sensory axonal neuropathy (AMSAN). The later carries a worse prognosis than the typical Guillain Barre. Our patient showed an impressive and unexpected response following an aggressive treatment with immunoglobulin and plasmapheresis.