Abstracts

Rationale: Recently graft-versus-host disease(GVHD) was observed to cause central nervous system (CNS) complications following allogenic stem cell transplants (allo-HSCT). However, very few patients have presented with intractable myoclonus epilepsy.Methods: We present clinical and radiographic findings in a 48 year old male patient with chronic GVHD, associated with intractable myoclonic seizures following an allo-HSCT.Results: A bone marrow Transplant was performed on 10/23/2008 following complete remission of Philadelphia chromosome positive acute myelogenous leukemia. The patient remained stable and tolerated the transplant well. He was diagnosed with chronic GVHD on 10/20/2011 (more than two year after stem cell transplant) the patient presented with significant irritation and thickening of the mucosae over the tongue and buccal surfaces. He was treated with topical oral Dexamethasone swish and spit and tacrolimus. In June 2012 the patient presented with myoclonic jerks involving his arms and legs. They caused occasional falls over, but he denied any loss of consciousness. In spite of Levetiracetam, he continued to have episodes multiple times per day. He was admitted to the neurology service after a myoclonic jerk that resulted in a fall with loss of consciousness. His neurological exam was unremarkable except for frequent myoclonic jerks of the extremities with brief alteration in awareness and responsiveness. Brain magnetic resonance imaging revealed a periventricular white matter lesions (figure 1). EEG showed frequent generalized 40-70 uV polyspike activity that occurred in runs lasting from 0.5 - 3 seconds, maximal in the frontocentral regions (Figure 2). This activity was seen spontaneously and during photic stimulation. His seizures have worsened and are intractable to levetiracetam, valproic acid, topiramate, lorazepam, clonazepam, zonisamide, and lacosamide. Perampanal improved his jerks by 40%. . Vagal Nerve Stimulation has not improved the frequency or severity of the myoclonic jerks. For chronic oral graft versus host disease the patient requires ongoing immunosuppression with tacrolimus, mycophenylate, and oral topical dexamethasone. Attempts to taper tacrolimus have resulted in problematic ulcerative oral inflammation.Conclusions: GVHD may be associated with CNS disorders the include pharmacoresistent myoclonus seizures. This observation may provide insight into mechanisms of myoclonus; Further research is needed to better understand causes and interventions for myoclonus associated wit GVHD.