Rituximab - associated immune myelopathy

a simple transfusion over erythrocytapheresis early in the course of ACS is lacking. To our knowledge, no randomized control trial exists comparing simple transfusion therapy with erythrocytapheresis for ACS. Further, the potential benefits versus risks of repeated simple transfusions in this patient population should be evaluated carefully. Of 5 patients, 4 had a positive fluid balance prior to their neurologic event, 3 with subsequent hypertension and reversible posterior leukoencephalopathy syndrome. In regard to our definition of silent cerebral infarct, we defined a silent infarct as a lesion consistent with infarction on magnetic resonance imaging without evidence of a focal neurologic finding lasting longer than 24 hours. This definition is based on the one used by a standard neurologic textbook that defines a stroke as a “sudden occurrence of a nonconvulsive, focal neurologic deficit” and distinguishes strokes from reversible transient ischemic attacks based upon duration of symptoms lasting less than 24 hours.2 Additionally, our definition of silent cerebral infarct is one that we have used previously.3 In our case series, none of the 3 patients determined to have a silent cerebral infarct had evidence of a focal neurologic deficit persisting longer than 24 hours. Patient 1 had a headache (a nonfocal neurologic symptom); patient 2 had generalized and multifocal seizures and transient, mild right-arm weakness and left-eye deviation lasting less than one hour (focal findings resolved in one hour); and patient 3 had evidence of diffuse deconditioning (nonfocal) after being intubated for several months. All patients had normal neurologic examination after being examined by a pediatric neurologist. Regardless of the definition of silent cerebral infarcts, children with sickle cell anemia and ACS have a high incidence of cerebral infarcts, placing them at increased risk for further neurologic morbidity4,5 and associated cognitive impairment.6 In the setting of severe ACS following erythrocytapheresis, we recommend consideration be given for assessment of occult neurologic morbidity.