Multiple Sclerosis Presenting as Posterior Cortical Atrophy
Journal Title: Dual Diagnosis: Open Access - Year 2017, Vol 2, Issue 2
Abstract
Posterior cortical atrophy (PCA) is a rare neurodegenerative syndrome, characterized by insidious and gradual visual impairment in the absence of primary ocular disease [1]. Pathologic studies have revealed that PCA is most often the clinical expression of an atypical form of Alzheimer pathology [2], named the visual variant. More rarely, PCA may be associated to other neurodegenerative etiologies, such as corticobasal degeneration, dementia with Lewy bodies, prion disease (Creutzfeldt-Jakob disease), and subcortical gliosis [1,3-5]. The clinical phenotype may vary according to the etiology, and may include elements of the Balint’s syndrome (optic ataxia, oculomotor apraxia, simultagnosia). Patients complain having inconsistent difficulties to distinguish lines, to read, to estimate distances, and to identify objects. Visual hallucinations are often reported. Clinical onset of PCA is usually observed during the 5th or 6th decades. Diagnosis is usually evoked on a very peculiar neurovisual pattern during cognitive testing and comforted by posterior (occipito-temporo-parietal atrophy on MRI, with clear cortical hypometabolism in the same regions on perfusion PET ou SPECT scans. [6-8]. We describe the case of a patient presenting with PCA syndrome, which was related to an atypical presentation of multiple sclerosis.
Authors and Affiliations
Ehrlé Nathalie, Cason Nia, Vaunaize Jean, Garcia Tony, Bakchine Serge
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