title: Clinicopathological review of pallidonigroluysian atrophy.
abstract: Pallidonigroluysian atrophy is a rare neurodegenerative disease characterized by degeneration of the globus pallidus, substantia nigra, and subthalamic nucleus. Few studies have comprehensively documented the clinical and pathological features of pallidonigroluysian atrophy. A systematic review of all published cases of pallidonigroluysian atrophy in English since 1970 was performed. We also report a new case of pallidonigroluysian atrophy. Twenty-five cases of pathologically proven pallidonigroluysian atrophy were reviewed, 24 from the literature and 1 of our own. Average age of onset was 54.3 ± 14.3 years, and average duration of disease was 7.9 ± 5.8 years. The most common first symptom was gait or balance disturbance. Patients had a diversity of movement disorders, including chorea in 5 cases (20%). Nine cases (36%) had coexistent motor neuron disease. Almost all cases had gliosis, and many cases had iron-positive pigments in the pallidonigroluysian system. Tauopathy was absent to rare in this region. Widespread tau-negative, p62-positive glial inclusions, described in 1 previous case, were also present in our patient. As pallidonigroluysian atrophy has a diversity of clinical presentations, it is best defined neuropathologically. The relative lack of tauopathy and the presence of p62-positive glial inclusions or iron-positive pigments in the pallidonigroluysian region may help to distinguish pallidonigroluysian atrophy from similar disease entities.
authors.affiliation: Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Janice C
authors.initials: JC
authors.lastname: Wong
authors.forename: Melissa J
authors.initials: MJ
authors.lastname: Armstrong
authors.forename: Anthony E
authors.initials: AE
authors.lastname: Lang
authors.forename: Lili-Naz
authors.initials: LN
authors.lastname: Hazrati
mesh_terms: D000328:Adult; D000368:Aged; D001284:Atrophy / Q000150:complications; D016208:Databases, Factual / Q000706:statistics & numerical data; D005260:Female; D057180:Frontotemporal Dementia / Q000473:pathology*; D005917:Globus Pallidus / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D013378:Substantia Nigra / Q000473:pathology; D020531:Subthalamic Nucleus / Q000473:pathology; D024801:Tauopathies / Q000150:complications / Q000473:pathology