Doenças Autoimunes do Sistema Nervoso

A evolução recente do conhecimento no campo das doenças do sistema nervoso mediadas imunologicamente tem trazido novas perspectivas nos aspectos de diagnóstico e tratamento. Paralelamente, um melhor conhecimento das especificidades moleculares dos autoanticorpos envolvidos tem também sido possível. Este trabalho pretende rever alguns conceitos actuais nesta área.

Spectrum of Neurologic Complications in Chronic Lymphocytic Leukemia

Neurologic disease is believed to be an unusual complication during the course of chronic lymphocytic leukemia. Nevertheless, it has already been proven in autopsy series that the incidence of occult nervous system infiltration is much higher than was previously expected. The advent of more potent drugs to treat this lymphoproliferative disorder has brought a new hope for a possible cure in the future. However, an appropriate systemic treatment for central nervous system infiltration of this disease is still lacking. Also, due to the potent immunosuppressive properties of the agents used in the up-front treatment, for example, the purine nucleoside analogues, we have witnessed an increase in the incidence of opportunistic infections, with progressive multifocal leukoencephalopathy being one of the most serious. The goal of this review is to summarize the spectrum of neurologic derangements linked to chronic lymphocytic leukemia and to raise clinicians’ awareness to recognize the possibility of such associations.

Non-Progressive Leukoencephalopathy with Bilateral Temporal Cysts

We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. Cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.