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Clinicopathological review of pallidonigroluysian atrophy
Author(s) -
Wong Janice C.,
Armstrong Melissa J.,
Lang Anthony E.,
Hazrati LiliNaz
Publication year - 2013
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.25232
Subject(s) - atrophy , tauopathy , pathology , gliosis , medicine , chorea , pathological , globus pallidus , extrapyramidal disorder , disease , neuroscience , psychology , basal ganglia , neurodegeneration , central nervous system
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. © 2012 Movement Disorder Society