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J Neurol. 2018 Sep;265(9):1989-1997. doi: 10.1007/s00415-018-8955-z. Epub 2018 Jun 26.

Beyond 35 years of Parkinson's disease: a comprehensive clinical and instrumental assessment.

Author information

1
Department of Neuroscience "Rita Levi Montalcini", University of Turin, Via Cherasco 15, 10124, Turin, Italy. al.romagnolo@gmail.com.
2
Department of Neuroscience "Rita Levi Montalcini", University of Turin, Via Cherasco 15, 10124, Turin, Italy.
3
Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Avenida Professor Egas Moniz, 1649-028, Lisbon, Portugal.
4
Department of Neurology, Gardner Family Center for Parkinson's Disease and Movement Disorders, University of Cincinnati, Cincinnati, OH, USA.
5
Department of Psychology, University of Turin, Via Verdi 10, 10124, Turin, Italy.
6
Medical Genetics Laboratory, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
7
Parkinson Institute, ASST "G.Pini-CTO", Via Bignami 1, Milan, 20133, Italy.

Abstract

BACKGROUND:

We sought to characterize the clinical, neuropsychological, electrophysiological, and neuroimaging features of Parkinson's disease (PD) after over 35 years since the onset of motor symptoms.

METHODS:

Five consecutively consenting PD patients treated with subthalamic nucleus deep brain stimulation (STN-DBS) were recruited in a cross-sectional study of motor (Unified PD Rating Scale section-III), non-motor (Non-Motor Symptoms Scale), autonomic (Scale for Outcome in PD-Autonomic), and neuropsychological features associated with the very advanced phase of PD. In addition, patients underwent neurophysiological (autonomic tests and nerve conduction studies) and neuroimaging (brain MRI, 123I-FP-CIT SPECT, and 123I-MIBG myocardial scintigraphy) studies, as well as a genetic analysis of 34 genes and single nucleotide polymorphisms associated with PD.

RESULTS:

There was a sustained motor response to L-dopa (range 14.4-35.6%), STN-DBS (23.3-38.4%), and L-dopa plus STN-DBS (37.8-63.0%). There were mild-to-moderate non-motor symptoms (range 19-83 on a scale of 0 to 360) and autonomic dysfunction (8-28 on a scale of 0-69). Two patients were demented, one had mild cognitive impairment, and two were cognitively preserved. Three patients had a sensory-axonal peripheral neuropathy and two a moderate-to-severe autonomic neuropathy. All cases showed a complete nigro-striatal dopaminergic denervation and a severe cardiovascular noradrenergic denervation. The brain MRI revealed only moderate frontal atrophy. The genetic tests were unremarkable.

CONCLUSIONS:

Even after more than 35 years of disease, L-dopa  and STN-DBS remain effective on PD cardinal symptoms. Although axial, autonomic, and neuropsychological features may become key determinants of disability, some patients maintain a satisfactory quality of life, without significant motor and non-motor impairment.

KEYWORDS:

Deep brain stimulation; Late stage; Parkinson’s disease

PMID:
29943201
DOI:
10.1007/s00415-018-8955-z
[Indexed for MEDLINE]

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