, Volume 19, Issue 5, pp 321-328

Neuropsychological and neuroimaging correlates in corticobasal degeneration

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Abstract

The aim of this study was to correlate neuropsychological and neuroimaging findings in corticobasal degeneration (CBD). Three patients with clinical criteria for CBD were examined by means of neuropsychological tests, brain magnetic resonance imaging (MRI), and flow and metabolism neuroimaging techniques. Neuropsychological assessment revealed impairment in executive functions, manual dexterity and motor programming with significant asymmetry between upper limbs. Ideomotor and oral apraxia were also detected, and memory deficits were observed in one patient. MRI revealed cortical dilation of the frontal and perirolandic regions, symmetrical in one case and asymmetrical in the other two cases. An increased T2 signal intensity in the posterolateral putamen and substantia nigra ipsilateral to the cortical atrophy was observed in one patient. Asymmetries of both frontal and parietal cortices and basal ganglia were detected in all three patients by 18-fluorodeoxyglucose positron emission tomography; temporal region hypometabolism was associated in one patient. These cortical and subcortical asymmetries were observed in two patients by single photon emission tomography with the tracer technetium Tc 99m hexamethyl propylenamine oxime; cortical asymmetry was observed in only one patient. The results showed that functional neuroimaging findings correlated well with neuropsychological aspects in CBD. Neuroimaging and neuropsychological correlations may contribute toward understanding anatomical and functional abnormalities associated with this neurodegenerative disorder.

Sommario

Nel presente studio vengono riportate le correlazioni neuropsicologiche e neuroradiologiche in tre pazienti affetti da una rara malattia neurodegenerativa, la degenerazione cortico-basale (DCB). Lo studio neuropsicologico ha mostrato un decadimento nelle funzioni esecutive, nella destrezza manuale e programmazione rnotoria con una asimmetria tra i due arti superiori e un'aprassia ideo-motoria e bucco facciale. La risonanza magnetica nucleare (RMN) dell'encefalo ha evidenziato una dilatazione corticale in sede frontale e peri-rolandica, con asimmetria controlaterale al lato, più affetto in due casi, e simmetrica in un caso; inoltre un aumentato segnale T2 in sede di putamen e della sostanza nera ipsilaterale all'atrofia corticale è stato osservato in un paziente. La PET con tracciante 18-fluorodeossiglucosio ha evidenziato un ipometabolismo asimmetrico sia della corteccia frontale e parietale the dei gangli della base. La SPECT con tracciante 99-tecnezio ha evidenziato un'asimmetria corticale e sottocorticale in due casi; in un caso è stata osservata un'asimmetria solo in sede corticale. Le nostre osservazioni sui pazienti con DCB hanno mostrato una precisa correlazione tra aspetti neuropsicologici e neuroradiologici. Tali correlazioni possono dare un contributo allo studio delle alterazioni anatomiche e funzionali associate con questo disturbo neurodegenerativo.