Possible central nervous graft-versus-host reaction after stem cell transplantation by chronic myeloid leukemia

Peripheral and central nervous deficits are common problems of matched unrelated donor stem cell transplantation. About 6% of patients die after stem cell transplantation due to neurologic complications. Causes are metabolic encephalopathies, CNS-infections, cerebrovascular complications, drug toxicity, recurrence of malignancy or therapy induced leukencephalopathy.

Case report: A 17 year old was diagnosed with chronic myeloid leukemia in 9/2002. Hematological remission was achieved. 3/2003 she received a matched unrelated donor stem cell transplantation. Transplantation procedure was tolerated well. She developed a mild (grade I) graft versus host disease (GvHD) of the skin/intestine. In controls there was no sign of recurrence of the CML. In 6/2003 she developed first neurologic deficits. In 8/2003 she deteriorated rapidly and developed a severe tetraspastic paresis, weak atrophic muscles (especially hand muscles), hyperreflexia and cloni, positive Babinski sign, muscle fasciculation, circumscribed decreased sensitivity, involvement of cranial nerves (inability to speak, to swallow, reduced tongue movement/facial expression) with normal intelligence. Clinical symptoms resembled an amyotrophic lateral sclerosis (ALS). Exhaustive evaluation (MRI of brain/spine, lab studies, exclusion of polio) revealed no possible aetiology for her clinical picture resembling an ALS. High dose cortison lead to a significant but short neurological improvement.

Discussion: Neurological deficits are common sequelae of stem cell transplantation. All reported cases have a defined etiology or pathological imaging studies. Because of chronic GvHD of the skin/intestine in our patient, the neurologic defecits may be caused by a chronic central nervous GvHD. Despite negativ imaging studies, the regular improvement of the neurologic deficits after high dose cortison therapy supports the diagnosis of a chronic central nervous GvHD. Our patient has most likely a lesion of the anterior horn. This kind of lesion has not been identified as a complication of stem cell transplantation.