Chlorambucil- Induced progressive multifocal leucoencephalopathy - A case report

Abstract

Progressive multifocal leucoencephalopathy (PML) is a fatal demyelinating disease of the CNS caused by reactivation of the John-Cunningham (JC) polyomavirus. Only few cases of Chlorambucil-induced PML in chronic lymphocytic leukemia (CLL) are described in literature. To present a clinical case of PML in a patient with CLL in remission after treatment with Chlorambucil misdiagnosed with ischemic stroke. Somatic and neurological assessment, blood and CSF examination, immunologic tests and Magnetic Resonance Imaging (MRI) were performed. A 57-year-old patient with CCL in remission, who had been treated with Chlorambucil, suffered blurred vision, handwriting difficulties, posture instability and speech disorders. Following a Computer tomography of the head, the patient was diagnosed with ischemic stroke. However, due to the progressive neurological deterioration, the patient was admitted to the Neurology Clinic of University Hospital Pleven. On admission, bilateral amblyopia, horizontal and rotatory nystagmus, severe right-sided hemiparesis, facial asymmetry, dysarthria and dysphagia were established. The MRI revealed multiple sub- and supra-tentorial white matter lesions, highly likely due to leukoencephalopathy. CSF showed hyper proteinorachie and positive PCR for the JC-virus. Despite the 21-day intensive care treatment, the patient ultimately had a fatal outcome. PML is a rare disease that is difficult to diagnose, especially in cases with non-typical clinical presentation. In immunocompromised patients with deteriorating neurological signs MRI and JC-virus identification results allow early diagnosis of PML, improving the patient’s chance of survival.