Concomitant transverse myelitis and acute motor axonal neuropathy in an adolescent.

Children's Neurosciences Centre, Royal Children's Hospital, Melbourne, Victoria, Australia.
Pediatric Neurology (Impact Factor: 1.42). 12/2007; 37(5):378-81. DOI:10.1016/j.pediatrneurol.2007.05.020
Source: PubMed

ABSTRACT A 14-year-old boy presented with acute paraparesis with sensory and sphincter disturbance. Imaging and neurophysiologic studies were diagnostic for transverse myelitis with acute motor axonal neuropathy. He was treated with both intravenous immunoglobulin and high-dose corticosteroids, and he made a slow and incomplete recovery. Acute motor axonal neuropathy is an unusual variant of Guillain-Barré syndrome. Concomitant transverse myelitis and acute motor axonal neuropathy were not previously reported in childhood, although there are reports of coexisting transverse myelitis and other variants of Guillain-Barré syndrome. Such dual lower motor neuron pathology may be associated with a poorer outcome, and indicates simultaneous central and peripheral immune-mediated injury. There may be a place for combined therapy with immunoglobulin and corticosteroids in patients with transverse myelitis and inflammatory neuropathy.

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    ABSTRACT: Guillain-Barré syndrome is a rapidly progressive symmetrical muscle weakness associated with acute inflammatory disease. Transverse myelitis (TM) is the inflammation of the spinal cord characterized by rapidly evolving muscle weakness in the lower extremities, defects in sensory level and sphincter dysfunction. Guillain-Barré syndrome, and TM association occurs very rarely in childhood. A 7-year-old girl presented with complaints of neck pain, spout-style vomiting, cough, shortness of breath, and acute paraparesis with sensory and sphincter disturbance. The patient was intubated because of increased respiratory distress. A positive direct fluorescein antigen test in bronchoalveolar lavage confirmed Legionella pneumophila infection. Imaging and neurophysiologic studies were diagnostic for TM with acute motor and sensory axonal neuropathy. She was treated with a combination of high-dose methylprednisolone and intravenous immunoglobulins, and we observed incomplete recovery. The presented case is the first child with concomitant TM and acute motor and sensory axonal neuropathy related to L. pneumophila infection.
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    ABSTRACT: Guillain-Barré syndrome (GBS) and transverse myelitis may occur coexistently in the pediatric population. This may be explained by a shared epitope between peripheral and central nervous system myelin. Coexistent transverse myelitis, myositis, and acute motor neuropathy in childhood have not been previously described. We describe a 14-year-old female patient with transverse myelitis, myositis, and GBS following Mycoplasma pneumoniae infection. She presented with weakness and walking disability. Weakness progressed to involve all extremities and ultimately, she was unable to stand and sit. Based on the clinical findings, a presumptive diagnosis of myositis was made at an outside institution because of high serum creatine kinase level. The patient was referred to our institution for further investigation. Magnetic resonance imaging of spine revealed enhancing hyperintense lesions in the anterior cervicothoracic spinal cord. The electromyography revealed acute motor polyneuropathy. Serum M. pneumoniae IgM and IgG were positive indicating an acute infection. Repeated M. pneumoniae serology showed a significant increase in Mycoplasma IgG titer. The patient was given intravenous immunoglobulin for 2 days and clarithromycin for 2 weeks. She was able to walk without support after 2 weeks of hospitalization. This paper emphasizes the rarity of concomitant myositis, transverse myelitis, and GBS in children.
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