Wilson's disease, a reversible dementia: Case report

Cognitive deficits related to major organ failure: The potential role of neuropsychological testing

“…CT and MRI show variable evidence of amelioration of brain lesions (usually basal ganglia lesions) after onset of treatment Rothfus et al, 1988). Some studies have reported slight to moderate improvements in intellectual functioning after treatment (Goldstein et al, 1968;Rosselli et al, 1987), although the lack of control for practice effects in these studies may have confounded the results. Some patients show an initial (and sometimes irreversible) deterioration upon initiation of pharmacotherapy, in spite of excretion of copper from the body.…”

“…Characteristically, the changes are subcortical, particularly affecting the basal ganglia, cerebellum, and brainstem Cummings and Benson, 1983;Rosselli et al, 1987;Rothfus et al, 1988). There has been very little evidence of cortical involvement, although occasionally cortical changes have been reported to be more marked than basal ganglia changes (Starosta-Rubinstein et aL, 1987).…”

“…However, in a further study of the memory deficits in neurologically impaired WD patients, Isaacs-Glaberman et al (1989) concluded that the pattern of impaired recall and intact recognition suggested similarities to other conditions, such as Huntington's disease, which involve subcortical damage. Rosselli et aL (1987) reported on a patient with WD who evidenced impaired performance on the Wechsler Adult Intelligence Scale (WAIS) and WMS before treatment. They claimed that neuropsychological testing indicated improvement of her dementia-like symptoms after treatment.…”

Cognitive impairment in Wilson's disease

“…13 Several clinical accounts have since described cases of WD which showed cognitive abnormalities leading to functional impairment, corresponding to clinical features that resemble a dementia syndrome. 3,29,30 All these studies demonstrated motor and cognitive improvement after initiating clinical treatment for WD. This observation led to WD being categorized as a reversible dementia, both after clinical treatment 29,30 or after hepatic transplant.…”

“…3,29,30 All these studies demonstrated motor and cognitive improvement after initiating clinical treatment for WD. This observation led to WD being categorized as a reversible dementia, both after clinical treatment 29,30 or after hepatic transplant. 31 Cognitive abnormalities may persist even after treatment, as discussed previously.…”