[S2-1B-1] Childhood Transverse Myelitis: Current concepts and future directions
Transverse myelitis is an acute inflammatory disorder of the spinal cord, accounting for 10-20% of children with acute flaccid paralysis. Disability risk is significant, with 20-40% losing independent mobility and a further 20% having longstanding bowel or bladder incontinence.
Diagnosis rests on identifying clinical signs and symptoms related to the spinal cord, demonstrating spinal cord inflammation and exclusion of competing differential diagnosis through careful cerebrospinal fluid analysis and neuroimaging.
However, advances in neuroimaging and challenging clinical cases unveil our incomplete understanding of this condition as the borders between trauma, infection and inflammation seem to blur. In some children, the spectrum of concurrent inflammation spans brain, spinal cord and even peripheral nerves, making a clear phenotypic neuroimmune diagnosis virtually impossible.
There is a general opinion that early treatment with corticosteroids limits eventual disability and the search for an alternate diagnosis should not delay the initiation of immunomodulatory therapy. However, the diagnosis of transverse myelitis should be fairly certain before escalation of immunomodulatory therapy in children with severe disease. Some children with infectious myelitis and vascular disorders may show an impressive recovery following corticosteroid therapy.
Transverse myelitis may also be the sentinel event in a relapsing inflammatory demyelinating disorder (multiple sclerosis, neuromyelitis optica and MOG antibody spectrum disease), and the initial diagnostic evaluation and subsequent follow up should include consideration and surveillance for these disorders.
Diagnosis rests on identifying clinical signs and symptoms related to the spinal cord, demonstrating spinal cord inflammation and exclusion of competing differential diagnosis through careful cerebrospinal fluid analysis and neuroimaging.
However, advances in neuroimaging and challenging clinical cases unveil our incomplete understanding of this condition as the borders between trauma, infection and inflammation seem to blur. In some children, the spectrum of concurrent inflammation spans brain, spinal cord and even peripheral nerves, making a clear phenotypic neuroimmune diagnosis virtually impossible.
There is a general opinion that early treatment with corticosteroids limits eventual disability and the search for an alternate diagnosis should not delay the initiation of immunomodulatory therapy. However, the diagnosis of transverse myelitis should be fairly certain before escalation of immunomodulatory therapy in children with severe disease. Some children with infectious myelitis and vascular disorders may show an impressive recovery following corticosteroid therapy.
Transverse myelitis may also be the sentinel event in a relapsing inflammatory demyelinating disorder (multiple sclerosis, neuromyelitis optica and MOG antibody spectrum disease), and the initial diagnostic evaluation and subsequent follow up should include consideration and surveillance for these disorders.