Hematopoietic stem cell transplantation (HSCT) is an effective treatment modality for many life-threatening diseases that were once considered incurable. 1,2 This therapy has evolved rapidly during the last few decades due to immunologic advances and progress in stem cell technology; currently, more than 60,000 HSCTs are carried out annually worldwide. 2 In the first few years of using this therapy, patients survived only for a few months, despite successful grafts and remissions of the malignancies, due to complications that arose soon after transplantation, including severe bleeding or severe infections caused by thrombocytopenia or neutropenia, respectively. 2 Recently, transplantation-related mortality soon after transplantation decreased because of the introduction of reducedintensity conditioning regimens and more effective anti-infectious agents. 3,4 Unfortunately, complications arising a longer period after transplantation, such as graft-versus-host disease (GVHD), has begun to emerge. These are even more problematic than early onset complications, because poor progress has been made in decreasing late transplantation-related mortalities. 3 Here, we report a case of chronic GVHD (cGVHD) of the central nervous system (CNS) presenting as hemichorea-hemiballismus.
Case ReportA 45-year-old woman with acute myelogenous leukemia underwent total body irradiation (13.2 Gy) and chemotherapy while awaiting an HLA-haploidentical (5 of 10 compatible alleles) HSCT from her daughter. The transplant was performed with no complications, and she continued post-transplantation cyclophosphamide and fludarabine to facilitate a successful graft. At 104 days post-transplantation, the patient presented with a red and itchy eye and jaundice but no skin rash or diarrhea. She was diagnosed with ocular and hepatic GVHD Type 1; methylprednisolone was initiated, and she achieved complete remission.The patient continued immunosuppressant therapy with prednisolone, and the dose was tapered until Day 219 posttransplantation, at which point the hematologist decided to suspend it, because there was no recurrence of GVHD, and the probability of a later recurrence was low.On Day 253 post-transplantation, the patient was admitted to the emergency room with new-onset, right, involuntary, choreic movements in the distal upper limb associated with flailing and ballistic movements of the right side of the body with no other symptoms (Video S1). A brain magnetic resonance image (MRI) was obtained and, on a fluid-attenuated inversion recovery (FLAIR) sequence, revealed a focal lesion in the right occipital lobe and a lesion in the left globus pallidus. The imaging study did not show any evidence of subthalamic compromise (Fig. 1A, B). Cerebrospinal fluid (CSF) analysis was normal (0 leukocytes; 0 red blood cells; glucose. 50 mg/dL; proteins, 35 mg/dL; Indian ink, potassium hydroxide, Gram, and latex for capsular antigens were negative). Haloperidol and clonazepam were initiated with improvement of symptoms, and the patient was discharged.Fifteen ...