Neurodegenerative 'overlap' syndrome: Clinical and pathological features of Parkinson's disease, motor neuron disease, and Alzheimer's disease

Ryan J. Uitti, Kenneth Berry, Osamu Yasuhara, Andrew Eisen, Howard Feldman, Patrick L. McGeer, Donald B. Calne

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Parkinson's disease (PD), Alzheimer's disease (AD), and motor neuron disease (MND) share epidemiological, clinical, and pathological features. Few studies have reported comprehensively on individuals who demonstrate a neurodegenerative 'overlap' syndrome, comprising idiopathic parkinsonism, dementia, and motor neuron dysfunction. We describe clinical, electrophysiological, and pathological features in six patients with neurodegenerative 'overlap' syndrome. All had cardinal features of PD (duration 6-26 years), and any mixture of dementia (slowly advancing), fasciculations, hyperreflexia, Babinski signs and mild atrophy and weakness of distal muscles (slowly progressive). EMG often demonstrated a lack of denervation in conjunction with abnormal MEPs (high thresholds). Patients had either 6FD-PET or pathological studies consistent with PD. Pathological studies also demonstrated moderate numbers of neurofibrillary tangles and plaque formation, typically with sparing of motor neurons in the spinal cord. We conclude that neurodegenerative 'overlap' syndrome may represent forme frustes of traditionally accepted diagnostic categories. Patients with parkinsonism, fasciculations, hyperreflexia and mild atrophy are unlikely to demonstrate active denervation on EMG; their prognosis is better than for classical MND. Neurodegenerative overlap syndrome (clinicopathological mixtures of PD, AD, and MND) may develop in some individuals as a reflection of common etiology, pathogenesis or susceptibility.

Original languageEnglish (US)
Pages (from-to)21-34
Number of pages14
JournalParkinsonism and Related Disorders
Volume1
Issue number1
StatePublished - Jul 1995
Externally publishedYes

Fingerprint

Motor Neuron Disease
Parkinson Disease
Alzheimer Disease
Fasciculation
Abnormal Reflexes
Parkinsonian Disorders
Motor Neurons
Denervation
Atrophy
Dementia
Babinski's Reflex
Neurofibrillary Tangles
Muscle Weakness
Spinal Cord

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Geriatrics and Gerontology

Cite this

Uitti, R. J., Berry, K., Yasuhara, O., Eisen, A., Feldman, H., McGeer, P. L., & Calne, D. B. (1995). Neurodegenerative 'overlap' syndrome: Clinical and pathological features of Parkinson's disease, motor neuron disease, and Alzheimer's disease. Parkinsonism and Related Disorders, 1(1), 21-34.

Neurodegenerative 'overlap' syndrome : Clinical and pathological features of Parkinson's disease, motor neuron disease, and Alzheimer's disease. / Uitti, Ryan J.; Berry, Kenneth; Yasuhara, Osamu; Eisen, Andrew; Feldman, Howard; McGeer, Patrick L.; Calne, Donald B.

In: Parkinsonism and Related Disorders, Vol. 1, No. 1, 07.1995, p. 21-34.

Research output: Contribution to journalArticle

Uitti, Ryan J. ; Berry, Kenneth ; Yasuhara, Osamu ; Eisen, Andrew ; Feldman, Howard ; McGeer, Patrick L. ; Calne, Donald B. / Neurodegenerative 'overlap' syndrome : Clinical and pathological features of Parkinson's disease, motor neuron disease, and Alzheimer's disease. In: Parkinsonism and Related Disorders. 1995 ; Vol. 1, No. 1. pp. 21-34.
@article{8def70faf0e442fdaea19a96b50fc28e,
title = "Neurodegenerative 'overlap' syndrome: Clinical and pathological features of Parkinson's disease, motor neuron disease, and Alzheimer's disease",
abstract = "Parkinson's disease (PD), Alzheimer's disease (AD), and motor neuron disease (MND) share epidemiological, clinical, and pathological features. Few studies have reported comprehensively on individuals who demonstrate a neurodegenerative 'overlap' syndrome, comprising idiopathic parkinsonism, dementia, and motor neuron dysfunction. We describe clinical, electrophysiological, and pathological features in six patients with neurodegenerative 'overlap' syndrome. All had cardinal features of PD (duration 6-26 years), and any mixture of dementia (slowly advancing), fasciculations, hyperreflexia, Babinski signs and mild atrophy and weakness of distal muscles (slowly progressive). EMG often demonstrated a lack of denervation in conjunction with abnormal MEPs (high thresholds). Patients had either 6FD-PET or pathological studies consistent with PD. Pathological studies also demonstrated moderate numbers of neurofibrillary tangles and plaque formation, typically with sparing of motor neurons in the spinal cord. We conclude that neurodegenerative 'overlap' syndrome may represent forme frustes of traditionally accepted diagnostic categories. Patients with parkinsonism, fasciculations, hyperreflexia and mild atrophy are unlikely to demonstrate active denervation on EMG; their prognosis is better than for classical MND. Neurodegenerative overlap syndrome (clinicopathological mixtures of PD, AD, and MND) may develop in some individuals as a reflection of common etiology, pathogenesis or susceptibility.",
author = "Uitti, {Ryan J.} and Kenneth Berry and Osamu Yasuhara and Andrew Eisen and Howard Feldman and McGeer, {Patrick L.} and Calne, {Donald B.}",
year = "1995",
month = "7",
language = "English (US)",
volume = "1",
pages = "21--34",
journal = "Parkinsonism and Related Disorders",
issn = "1353-8020",
publisher = "Elsevier BV",
number = "1",

}

TY - JOUR

T1 - Neurodegenerative 'overlap' syndrome

T2 - Clinical and pathological features of Parkinson's disease, motor neuron disease, and Alzheimer's disease

AU - Uitti, Ryan J.

AU - Berry, Kenneth

AU - Yasuhara, Osamu

AU - Eisen, Andrew

AU - Feldman, Howard

AU - McGeer, Patrick L.

AU - Calne, Donald B.

PY - 1995/7

Y1 - 1995/7

N2 - Parkinson's disease (PD), Alzheimer's disease (AD), and motor neuron disease (MND) share epidemiological, clinical, and pathological features. Few studies have reported comprehensively on individuals who demonstrate a neurodegenerative 'overlap' syndrome, comprising idiopathic parkinsonism, dementia, and motor neuron dysfunction. We describe clinical, electrophysiological, and pathological features in six patients with neurodegenerative 'overlap' syndrome. All had cardinal features of PD (duration 6-26 years), and any mixture of dementia (slowly advancing), fasciculations, hyperreflexia, Babinski signs and mild atrophy and weakness of distal muscles (slowly progressive). EMG often demonstrated a lack of denervation in conjunction with abnormal MEPs (high thresholds). Patients had either 6FD-PET or pathological studies consistent with PD. Pathological studies also demonstrated moderate numbers of neurofibrillary tangles and plaque formation, typically with sparing of motor neurons in the spinal cord. We conclude that neurodegenerative 'overlap' syndrome may represent forme frustes of traditionally accepted diagnostic categories. Patients with parkinsonism, fasciculations, hyperreflexia and mild atrophy are unlikely to demonstrate active denervation on EMG; their prognosis is better than for classical MND. Neurodegenerative overlap syndrome (clinicopathological mixtures of PD, AD, and MND) may develop in some individuals as a reflection of common etiology, pathogenesis or susceptibility.

AB - Parkinson's disease (PD), Alzheimer's disease (AD), and motor neuron disease (MND) share epidemiological, clinical, and pathological features. Few studies have reported comprehensively on individuals who demonstrate a neurodegenerative 'overlap' syndrome, comprising idiopathic parkinsonism, dementia, and motor neuron dysfunction. We describe clinical, electrophysiological, and pathological features in six patients with neurodegenerative 'overlap' syndrome. All had cardinal features of PD (duration 6-26 years), and any mixture of dementia (slowly advancing), fasciculations, hyperreflexia, Babinski signs and mild atrophy and weakness of distal muscles (slowly progressive). EMG often demonstrated a lack of denervation in conjunction with abnormal MEPs (high thresholds). Patients had either 6FD-PET or pathological studies consistent with PD. Pathological studies also demonstrated moderate numbers of neurofibrillary tangles and plaque formation, typically with sparing of motor neurons in the spinal cord. We conclude that neurodegenerative 'overlap' syndrome may represent forme frustes of traditionally accepted diagnostic categories. Patients with parkinsonism, fasciculations, hyperreflexia and mild atrophy are unlikely to demonstrate active denervation on EMG; their prognosis is better than for classical MND. Neurodegenerative overlap syndrome (clinicopathological mixtures of PD, AD, and MND) may develop in some individuals as a reflection of common etiology, pathogenesis or susceptibility.

UR - http://www.scopus.com/inward/record.url?scp=0003375492&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0003375492&partnerID=8YFLogxK

M3 - Article

AN - SCOPUS:0003375492

VL - 1

SP - 21

EP - 34

JO - Parkinsonism and Related Disorders

JF - Parkinsonism and Related Disorders

SN - 1353-8020

IS - 1

ER -