Recurrent asymptomatic demyelinating disease following 13-cis-retinoic acid exposure

Abstract

We report a case of multifocal demyelination within the central nervous system in a patient being treated for a left hemispheric gemnistocytic astrocytoma with radiation therapy and chemotherapy, comprising temozolomide (360 mg/day-days 1-5 every 28 days) and 13-cis-retinoic acid (100 mg/m(2)/day-separated into two doses administered every 12 h on days 1 through 21 every 28 days). Five months into her first round of chemotherapy, brain magnetic resonance imaging (MRI) demonstrated multifocal regions of T2 prolongation with associated gadolinium enhancement within the right cerebral hemisphere. Spectroscopic data were consistent with demyelination rather than neoplasia. Despite the incidentally identified radiological progression, new neurological symptoms were not described. Interval resolution of the demyelinating lesions was observed in the years following the discontinuance of her chemotherapy regimen with reactivation of the previously observed lesions and the development of new T2 foci 6 months into her second round of re-treatment for tumour progression 5 years later.

Figure 1

(A) Brain magnetic resonance imaging (MRI) study performed at the time of the patient’s initial diagnosis. Axial fluid attenuated inversed recovery (FLAIR) sequence demonstrating a large frontoparietal region of T2 prolongation with mass effect. (B) Axial FLAIR image obtained approximately 5 months post-exposure to temozolomide and 13-cis-retinoic acid demonstrating new regions of T2 prolongation in the superior aspect of the right frontal lobe, right perisylvian region, right peri-atrial white matter, and right frontoparietal white matter. (C and D) Axial post-contrast T1 images demonstrating gadolinium enhancement of the new right hemispheric lesions observed. (E) Axial FLAIR image demonstrating interval resolution of the previously identified regions of T2 prolonagtion. (F) Brain MRI study following the patient’s second round of chemotherapy treatment obtained >5 years after her initial treatment course. Axial FLAIR image obtained 6 months post-retreatment demonstrating reactivation of previous lesions (all not shown) and new foci of T2 prolongation (right frontal lobe and frontal horn). Post-surgical changes and high signal abnormalities are also observed in the left frontal lobe.