Giuseppe Lanza1, Maurizio Papotto2, Giovanni Pennisi3, Rita Bella3, Raffaele Ferri4. 1. Department of Neurology I.C., Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina (EN), Italy. Electronic address: giuseppelanza2003@yahoo.it. 2. Department of Neurorehabilitation, Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina (EN), Italy. 3. Department of Neurosciences, University of Catania, Catania, Italy. 4. Department of Neurology I.C., Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina (EN), Italy.
Abstract
BACKGROUND: Vascular progressive supranuclear palsy (vPSP) is an uncommon akinetic-rigid syndrome characterized by asymmetric lower body involvement, predominant corticospinal and pseudobulbar signs, urinary incontinence, cognitive impairment, and increased frequency of stroke risk factors, together with neuroimaging evidence of vascular disease. CASE REPORT: We report a case of a patient with a PSP-like phenotype and marked cognitive impairment who significantly worsened after a generalized epileptic seizure that occurred a few months after its clinical onset. RESULTS: Signs of widespread ischemic subcortical vascular disease, together with atrophy of the midbrain tectum, corpus callosum, and cerebral cortex, were evident on brain magnetic resonance imaging. CONCLUSIONS: vPSP is a condition that should be considered when a patient presents with a gradually progressive clinical picture suggestive of idiopathic PSP associated with neuroimaging evidence of cerebrovascular disease. The occurrence of epileptic seizures has not been reported before in vPSP but they might trigger the onset or precipitate the course of the PSP-like disorders.
BACKGROUND:Vascular progressive supranuclear palsy (vPSP) is an uncommon akinetic-rigid syndrome characterized by asymmetric lower body involvement, predominant corticospinal and pseudobulbar signs, urinary incontinence, cognitive impairment, and increased frequency of stroke risk factors, together with neuroimaging evidence of vascular disease. CASE REPORT: We report a case of a patient with a PSP-like phenotype and marked cognitive impairment who significantly worsened after a generalized epileptic seizure that occurred a few months after its clinical onset. RESULTS: Signs of widespread ischemic subcortical vascular disease, together with atrophy of the midbrain tectum, corpus callosum, and cerebral cortex, were evident on brain magnetic resonance imaging. CONCLUSIONS: vPSP is a condition that should be considered when a patient presents with a gradually progressive clinical picture suggestive of idiopathic PSP associated with neuroimaging evidence of cerebrovascular disease. The occurrence of epileptic seizures has not been reported before in vPSP but they might trigger the onset or precipitate the course of the PSP-like disorders.
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