Acute Motor and Sensory Axonal Neuropathy (AMSAN) in a 15-Year-Old Boy Presenting with Severe Pain and Distal Muscle Weakness

Abstract

Acute motor and sensory axonal neuropathy (AMSAN) is a recently described subtype of Guillain-Barré syndrome characterized by acute onset of distal weakness, loss of deep tendon reflexes and sensory symptoms. Electrophysiological studies show mildly reduced nerve conduction velocities combined with a marked reduction of muscle action and sensory nerve action potentials. Here, we report a 15-year-old boy who suffered from severe burning and knife-like pain that increased over a period of three months and resulted in a disrupted sleep pattern and suicidal intentions as well as marked loss of weight. In addition, he developed muscle weakness in his hands and feet. Neurophysiological and histopathological studies revealed AMSAN. Marked improvement of his condition was achieved by treatment with intravenous immunoglobulins, high-dose methylprednisolone, and a combination of gabapentin, antidepressants, and an oral morphine.

Key words

AMSAN - children - IVIG - pain management

References

• 1 Alam T A, Chaudhry V, Cornblath D R. Electrophysiological studies in the Guillain-Barré syndrome: Distinguishing subtypes by published criteria.  Muscle Nerve. 1998;  21 1275-1279
• 2 Backonja M, Beydoun A, Edwards K R, Schwartz S L, Fonseca V, Hes M. et al . Gabapentin for the symptomatic treatment of painful neuropathy in patients with diabetes mellitus: a randomized controlled trial.  JAMA. 1998;  280 1831-1836
• 3 Chowdhury D, Arora A. Axonal Guillain-Barré syndrome: a critical review.  Acta Neurol Scand. 2001;  103 267-277
• 4 Griffin J W, Li C Y, Ho T W, Tian M, Gao C Y, Xue P. et al . Pathology of motor-sensory axonal Guillain-Barré syndrome.  Ann Neurol. 1996;  39 17-28
• 5 Hahn K, Arendt G, Braun J S, von Giesen H J, Husstedt I W, Maschke M. et al . A placebo-controlled trial of gabapentin for painful HIV-associated sensory neuropathies.  J Neurol. 2004;  251 1260-1266
• 6 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
• 7 Khatri A, Pearlstein L. Pain in Guillain-Barré syndrome.  Neurology. 1997;  49 1474
• 8 Korinthenberg R, Monting J S. Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study.  Arch Dis Child. 1996;  74 281-287
• 9 Massaro M E, Rodriguez E C, Pociecha J, Arroyo H A, Sacolitti M, Taratuto A L. et al . Nerve biopsy in children with severe Guillain-Barré syndrome and inexcitable motor nerves.  Neurology. 1998;  51 394-398
• 10 Mendell J R, Sahenk Z. Painful sensory neuropathy.  N Engl J Med. 2003;  348 1243-1255
• 11 Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group . Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome.  Lancet. 1997;  349 225-230
• 12 Sindrup S H, Gram L F, Brosen K, Eshoj O, Mogensen E F. The selective serotonin reuptake inhibitor paroxetine is effective in the treatment of diabetic neuropathy symptoms.  Pain. 1990;  42 135-144
• 13 Sindrup S H, Jensen T S. Pharmacologic treatment of pain in polyneuropathy.  Neurology. 2000;  55 915-920
• 14 Sladky J T. Guillain-Barré syndrome. Jones HR, de Vivo DC, Darras BT Neuromuscular Disorders of Infancy, Childhood, and Adolescents. Philadelphia; Butterworth, Heinemann 2003: 407-424
• 15 Yuki N, Kuwabara S, Koga M, Hirata K. Acute motor axonal neuropathy and acute motor-sensory axonal neuropathy share a common immunological profile.  J Neurol Sci. 1999;  168 121-126