ABSTRACT
Progressive multifocal leukoencephalopathy (PML) is a demyelination disease caused by opportunistic infection of the ubiquitous, usually nonpathogenic neurotropic papovavirus (JC virus). The virus infects and destroys myelin-producing oligodendrocytes, thereby causing patchy areas of demyelination in the cerebral white matter. It is exclusively a disease of immunosuppressed individuals. We report a case of an immunocompetent child patient with pathologically-proven PML and with a survival over 3 years after diagnosis. Serial follow up of neuroimaging study including brain MRI, MRS, SPECT and PET was obtained.
Subject(s)
Child , Humans , Brain , Demyelinating Diseases , Diagnosis , Disease-Free Survival , Follow-Up Studies , Leukoencephalopathy, Progressive Multifocal , Magnetic Resonance Imaging , Neuroimaging , Oligodendroglia , Opportunistic Infections , Tomography, Emission-Computed, Single-PhotonABSTRACT
Sjogren's syndrome is a slowly progressive autoimmune disorder that predominantly affects major exocrine glands, and may also involve the central nervous system (CNS). It is sometimes very difficult to differentiate the CNS Sjogren's syndrome from multiple sclerosis. Here, we report two cases of Sjogren's syndrome who developed variable neurological symptoms mimicking the relapsing-remitting form of multiple sclerosis. There had been several relapses during the course of interferon-beta treatment but no relapses have occurred after steroid maintenance therapy.