Longitudinal Extensive Transverse Myelitis following T-ALL

Background/Purpose: Acute transverse myelitis is an acquired demyelinating disease of the central nervous system with different etiologies. Besides infections autoimmune demyelination plays a major role. Clinical presentation includes motor, sensory and autonomic symptoms.

Methods: We saw a 10-year-old girl, who presented to our department 3 weeks after the end of chemotherapy for T-ALL. She complained of vomiting and drowsiness and had signs of upper respiratory tract infection with low grade fever. On examination the patient was somnolent and had paraplegia of the lower extremities with bladder and bowel disturbance. She had meningismus and showed left-sided ptosis, double-vision and nystagmus. Symptoms worsened to loss of reflexes and spastic paraparesis with positive Babinski sign. MRI showed rhombencephalitis and transverse myelitis with extensive signal changes and swelling of the entire spinal cord without contract enhancement. EEG showed diffuse slowing of background activity.

Results: As long as investigations for leukemia relapse and acute infection were pending the patient received antibiotic and virostatic treatment and immunoglobulins. Having excluded these differential diagnoses we started steroid pulse therapy. Paraparesis and brainstem symptoms improved, but bladder- and bowel disturbance persisted. Upon escalation therapy of plasmapheresis she was symptom free and MRI normalized. She is in constant remission concerning the T-ALL and has no residual neurological deficit.

Conclusion: We propose that longitudinal extensive transverse Myelitis in this patient was caused by an immune reconstitution syndrome triggered by the upper respiratory tract infection after the end of chemotherapy for T-ALL.