A case of central nervous system graft-versus-host disease following allogeneic stem cell transplantation

Graft-versus-host disease (GVHD) is a serious complication of allogeneic stem cell transplantation (SCT). Here, we report a rare case of GVHD involving the central nervous system (CNS). A 35-year-old woman was diagnosed with myelodysplastic syndrome unclassifiable and underwent allogeneic peripheral blood SCT for disease progression to myelodysplastic syndrome with excess blasts-2. One year following SCT, she experienced chronic oral and hepatic GVHD symptoms, which were managed with oral steroids and tacrolimus. Sixteen months after SCT, she developed sudden-onset, generalized, tonic-clonic-type seizures. Magnetic resonance imaging and cerebrospinal fluid evaluation showed multiple discrete white lesions and elevated IgG levels. Brain biopsy revealed periventricular plaques with the destruction of axons, representing a demyelinating disease of the CNS. We diagnosed the case as CNS GVHD. Neurologic symptoms gradually improved with methylprednisolone pulse therapy and total plasma exchange combined with a calcineurin inhibitor; the brain lesions nearly disappeared after decreasing steroid maintenance dosage, and were completely resolved 1 year after the onset of CNS GVHD. The patient is CNS GVHD-symptom-free, 3-year post-transplantation. Thus, CNS GVHD should be considered in cases of newly developed neurologic symptoms in SCT recipients showing evidence of preceding chronic GVHD.