A
12-year-old boy came to the emergency department with
progressive weakness for 10-h while he was seated in his classroom.
He described it as a stinging sensation in his arms that progressed
to numbness
and weakness to the level of his upper chest. He has not
urinated for several hours and he wakes only with assistance. He has
moderate hypertonia in his upper extremities and his lower
extremities are flaccid.
Formal strength testing reveals (R/L): biceps 4/4, triceps 3/3, and
wrist extension 4/4, with all other muscle groups tested being grade
0. He has a sensory level at T2, and deep tendon reflexes are absent
throughout.
MRI of the spine shows an ill-defined intramedullary hyperintensity
extending from C5 to T2 without significant cord expansion or
enhancement. MRI of the head is normal. CSF studies are normal, and
no oligoclonal bands are detected.
What is the most appropriate treatment at this time?
A. IVIG 0.5 g/kg I.V. daily × 4 days
B. Methylprednisolone 500 mg I.V.B.I.D × 5 days
C. Prednisone 40 mg P.O. daily with a slow taper
D. Cyclophosphamide 800 mg/m2 I.V. every 4 weeks
E. Interferon-β − 1a 30 μg I.M. every week
CHECK RESULT ON MONDAY
Correct Answer is: B
ReplyDeleteTransverse myelitis (TM) is a heterogeneous disorder. It occurs as an idiopathic or secondary to a wide array of inflammatory (post-infectious, demyelinating), metabolic, and vascular processes.
Inflammatory demyelinating lesions, as seen in clinically isolated syndrome, multiple sclerosis, and neuromyelitis optica, are the most common cause of TM. Acute treatment for those demyelinating conditions is a high-dose, intravenous corticosteroids for 3–5 days. A subsequent oral steroid taper over 4–6 weeks is recommended for patients with acute disseminated encephalomyelitis. Long-term therapy with immune-suppressants may decrease the relapse rate for patients with NMO. Non-demyelinating forms of transverse myelitis must be treated according to the underlying etiology, e.g. parenteral cyanocobalamin (vitamin B12) supplementation for subacute combined degeneration.
REFERENCES
Brinar VV, Habek M, et al. Current concepts in the diagnosis of transverse myelopathies. Clin Neurol Neurosurg. 2008;110:919–27.
Pohl D. Epidemiology, immuno-pathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions. Curr Opin Neurol. 2008;21:366–72.