title: Neuropsychophysiological findings in a case of long-standing overt ventriculomegaly (LOVA).
abstract: Long-standing overt ventriculomegaly in adults (LOVA) is a clinical entity characterized by chronic hydrocephalus with infant onset, slow evolution and clinical disturbances during adulthood. Few cases are reported in literature describing the evident contrast between the severity of hydrocephalus and the relatively spared neurological functioning and cognitive aspects. The authors describe a 59-year-old man with congenital hydrocephalus complaining of persistent gait impairment. Neurological examination showed a mild paraparesis, severe higher cortical function impairment but relatively sparing of daily living activity. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a very remarkable ventriculomegaly compressing the brain cortex but sparing the cerebellum and the brainstem. Brain Single Photon Emission Computer Tomography (SPECT) showed a prevalent cerebellar perfusion as well. Neuropsychological testing was consistent with severe cognitive deterioration and attention disorders. Language and praxis functions seemed to be preserved. Auditory oddball ERPs (P300) showed morphological abnormalities especially of late components. This case report demonstrates in vivo the level of adaptation to which human brain can reach under chronic mechanic stress conditions. The striking poor cerebral parenchyma representation and the relatively spared language and praxic abilities account for a functional reorganization of residual structures due to the neural plasticity.
authors.affiliation: Institute of Clinical Neurology, University of Sassari, Viale San Pietro, 10, 07100 Sassari, Italy.
authors.forename: Edoardo Domenico Giorgio
authors.initials: ED
authors.lastname: Canu
authors.forename: Immacolata
authors.initials: I
authors.lastname: Magnano
authors.forename: Kai Stephan
authors.initials: KS
authors.lastname: Paulus
authors.forename: Maria Rita
authors.initials: MR
authors.lastname: Piras
authors.forename: Maurizio
authors.initials: M
authors.lastname: Conti
authors.forename: Salvatore
authors.initials: S
authors.lastname: Costantino
authors.forename: Susanna
authors.initials: S
authors.lastname: Nuvoli
authors.forename: Isidoro
authors.initials: I
authors.lastname: Aiello
mesh_terms: D002552:Cerebral Ventricles / Q000473:pathology*; D014695:Cerebral Ventriculography; D003071:Cognition / Q000502:physiology; D004569:Electroencephalography / Q000379:methods; D005071:Evoked Potentials / Q000502:physiology; D005500:Follow-Up Studies; D020233:Gait Disorders, Neurologic / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D006849:Hydrocephalus / Q000473:pathology* / Q000503:physiopathology*; D021621:Imaging, Three-Dimensional / Q000379:methods; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination / Q000379:methods; D009483:Neuropsychological Tests; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods