Neuropathological Correlates of Dysarthria in Progressive Supranuclear Palsy

• Published in: Archives of neurology (Chicago) Vol. 58; no. 2; pp. 265 - 269
• Main Authors: Kluin, Karen J, Gilman, Sid, Foster, Norman L, Sima, Anders A. F, D'Amato, Constance J, Bruch, Leslie A, Bluemlein, Laurie, Little, Roderick, Johanns, Jewel
• Format: Journal Article
• Language: English
• Published: Chicago, IL American Medical Association 01.02.2001
• Subjects: Aged; Aged, 80 and over; Biological and medical sciences; Corpus Striatum - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dysarthria - diagnosis; Female; Humans; Linear Models; Male; Medical sciences; Middle Aged; Neurology; Retrospective Studies; Substantia Nigra - pathology; Subthalamic Nucleus - pathology; Supranuclear Palsy, Progressive - pathology; Human; Nervous system diseases; Pathophysiology; Supranuclear ophthalmoplegia; Language disorder; Cerebral disorder; Progressive; Pathology; Eye disease; Central nervous system disease; Degenerative disease; Oculomotor syndrome; Neurological disorder; Brain stem syndrome; Dysarthria
• ISSN: 0003-9942; 2168-6149; 1538-3687; 2168-6157
• DOI: 10.1001/archneur.58.2.265

BACKGROUND The dysarthria of progressive supranuclear palsy consists of prominent hypokinetic and spastic components with less prominent ataxic components. OBJECTIVE To correlate the types of dysarthria with neuropathological changes in patients with progressive supranuclear palsy. DESIGN AND METHODS In 14 patients with progressive supranuclear palsy, we correlated the perceptual speech findings with the neuropathological findings. A dysarthria assessment was performed a mean ± SD of 31 ± 15 months (range, 10-53 months) before death. The deviant speech dimensions were rated on a scale of 0 (normal) to 3 (severe). The neuropathological examination consisted of semiquantitative analysis of neuronal loss and gliosis by investigators (A.A.F.S., and L.A.B.) blinded to the clinical findings. Correlation and linear regression analysis were used to correlate the severity of the hypokinetic, spastic, and ataxic components with the degree of neuronal loss and gliosis in predetermined anatomical sites. RESULTS All patients had hypokinetic and spastic dysarthria, and 9 also had ataxic components. The severity of the hypokinetic components was significantly correlated with the degree of neuronal loss and gliosis in the substantia nigra pars compacta (r = 0.61, P = .02) and pars reticulata (r = 0.64, P = .01) but not in the subthalamic nucleus (r = 0.51, P = .07) or the striatum or globus pallidus (/r/<0.34, P>.20). The severity of the spastic and ataxic components was not significantly correlated with the neuropathological changes in the frontal cortex (r = 0.20, P = .50) and cerebellum (/r/<0.28, P>.33), respectively. CONCLUSION The hypokinetic dysarthria of progressive supranuclear palsy may result from degenerative changes in the substantia nigra pars compacta and pars reticulata and not from changes in the striatum or globus pallidus.Arch Neurol. 2001;58:265-269-->