title: Clinical features and SPECT scanning in presumed cortico-basal ganglionic degeneration.
abstract: Eight patients presented with a strikingly similar core syndrome of asymmetric upper limb rigidity and dystonia (which was maximal in the non-dominant limb in seven), accompanied by ipsilateral ideomotor apraxia, cortical sensory disturbane, and sensory reflex myoclonus. The clinical diagnosis in each case was of presumed cortico-basal ganglionic degeneratioan. The clinical features in our cases matched those of other series closely, except for a higher prevalence of dementia (presumably explained by referral bias), and a lower incidence of oculomotor abnormalities. Seven of the eight patients underwent (59)Tc-HMPAO SPECT scanning, which in each case revealed contralateral fronto-parietal perfusion defects out of proportion to the focal or generalised atrophy demonstrated on MRI scans. SPECT scanning appears to be a useful confirmatory investigation in patients presenting with this uncommon but distinctive clinical syndrome, and in separating them from patients with other causes of asymmetric rigid syndromes, such as Parkinsons's disease.
authors.affiliation: Department of Neurology, Royal Melbourne Hospital, Parkville, Victoria, 3050, Australia; Department of Neuropsychiatry, Royal Melbourne Hospital, Parkville, Victoria, 3050, Australia.
authors.forename: E
authors.initials: E
authors.lastname: Storey
authors.forename: M
authors.initials: M
authors.lastname: Lichtenstein
authors.forename: P
authors.initials: P
authors.lastname: Desmond
authors.forename: J
authors.initials: J
authors.lastname: Lloyd