Clinical neurophysiologic findings in patients with rapidly progressive familial parkinsonism and dementia with pallido-ponto-nigral degeneration

被引:15
作者
Wszolek, ZK
Lagerlund, TD
Steg, RE
McManis, PG
机构
[1] Univ Nebraska, Med Ctr, Dept Internal Med, Neurol Sect, Omaha, NE 68198 USA
[2] Mayo Clin & Mayo Fdn, Dept Neurol, Rochester, MN 55905 USA
[3] Creighton Univ, Dept Neurol, Omaha, NE 68178 USA
[4] Nepean Hosp, Dept Neurol, Penrith, NSW, Australia
来源
ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY | 1998年 / 107卷 / 03期
关键词
clinical neurophysiology; electroencephalography; frontotemporal dementia and parkinsonism linked to chromosome 17q2122; pallido-ponto-nigral degeneration family;
D O I
10.1016/S0013-4694(98)00064-9
中图分类号
R318 [生物医学工程];
学科分类号
0831 ;
摘要
Objective: To present clinical electrophysiologic studies performed on the pallido-ponto-nigral degeneration (PPND) family linked to chromosome 17q21-22. Methods: Nine patients from this kindred were studied with 11 electroencephalograms (EEGs), 4 electroencephalographic background frequency analysis (BFA) studies, 4 electromyographic recordings (EMGs) including nerve conduction studies (NCSs), 4 electromyographic multichannel surface recordings (MSRs), one pattern visual evoked potential (VEP) study and one median nerve somatosensory evoked potential (SEP) study. Results: EEGs revealed normal findings early in the disease and diffuse slowing which became more prominent with disease progression. BFA studies demonstrated rapid decrease in mean parietal frequencies with disease progression. EMGs and NCSs showed no abnormalities. MSRs revealed action myoclonus and a dystonic process. Long loop reflexes were absent in resting hand muscles. VEPs and SEPs were normal. Conclusions: Clinical neurophysiologic studies were consistent with a cortical and subcortical degenerative process. With clinical deterioration, there is a progressive decline in the mean parietal frequency and background rhythms. Tremor studies were consistent with action myoclonus and a dystonic process and did not show parkinsonian features of resting tremor or agonist-antagonist cocontraction. There was no evidence of peripheral nerve involvement or slowing in central sensory pathways. Electrophysiologic findings are characteristic for this illness. (C) 1998 Elsevier Science Ireland Ltd. All rights reserved.
引用
收藏
页码:213 / 222
页数:10
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