Autonomic failure in Guamanian neurodegenerative disease

Phillip Anson Low, J. Eric Ahlskog, Ronald Carl Petersen, Stephen C. Waring, Conception Esteban-Santillan, Leonard T. Kurland

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Autonomic impairment is minor in idiopathic amyotrophic lateral sclerosis (ALS) and Alzheimer's-type dementia (D) and is usually not marked in Parkinson's disease. The autonomic status of Guamonian parkinsonism (P), ALS, and parkinsonism-dementia complex (PDC) is essentially unknown. We therefore evaluated the autonomic nervous system in Guamanian D, ALS, P, and PDC. Cardiovagal, adrenergic, and postganglionic sudomotor functions were quantitated in 16 patients and 14 paired household controls. Patients comprised PDC (N = 11), D (N = 2), P (N = 2), and ALS (N = 1). Autonomic deficit was expressed on a composite autonomic scoring scale (CASS) and its subsets that corrects for the effects of age and gender. CASS severity was rated from 0 to 10 and the maximal subset scores were 3, 3, and 4 for postganglionic sudomotor, cardiovagal, and adrenergic deficits, respectively. CASS scores for mild, moderate, and severe autonomic failure are 1 to 3, 4 to 6, and 7 to 10, respectively. Symptoms were scored by an Autonomic Symptom Profile (ASP). The affected patients were older than and had a sex distribution different from paired controls (64.2 ± 8.0 versus 53.1 ± 13.5; p < 0.01; male/female = 9/7 versus 2/12; p = 0.045). CASS scores were markedly increased over paired controls (6.2 ± 2.3 versus 1.9 ± 1.3; p < 0.001), and involvement was generalized by system. There were deficits in sudomotor, cardiovagal, and adrenergic function. Orthostatic hypotension occurred in 6 of 16 patients and 2 of 14 paired controls. Guamanian patients had more autonomic dysfunction than non-Guamanian Parkinson's disease. ASP scores were higher in patients than controls and regressed with CASS. These differences persisted when corrected for the confounding effects of age, gender, and diabetes. We conclude that Guamanian patients have autonomic failure to a greater extent than non-Guamanian Parkinson's disease or ALS. This autonomic failure suggests multisystem autonomic involvement similar to but less severe than in multiple system atrophy.

Original languageEnglish (US)
Pages (from-to)1031-1034
Number of pages4
JournalNeurology
Volume49
Issue number4
StatePublished - Oct 1997

Fingerprint

Neurodegenerative Diseases
Amyotrophic Lateral Sclerosis
Parkinsonian Disorders
Adrenergic Agents
Parkinson Disease
Dementia
Multiple System Atrophy
Orthostatic Hypotension
Sex Distribution
Autonomic Nervous System
Alzheimer Disease

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Low, P. A., Ahlskog, J. E., Petersen, R. C., Waring, S. C., Esteban-Santillan, C., & Kurland, L. T. (1997). Autonomic failure in Guamanian neurodegenerative disease. Neurology, 49(4), 1031-1034.

Autonomic failure in Guamanian neurodegenerative disease. / Low, Phillip Anson; Ahlskog, J. Eric; Petersen, Ronald Carl; Waring, Stephen C.; Esteban-Santillan, Conception; Kurland, Leonard T.

In: Neurology, Vol. 49, No. 4, 10.1997, p. 1031-1034.

Research output: Contribution to journalArticle

Low, PA, Ahlskog, JE, Petersen, RC, Waring, SC, Esteban-Santillan, C & Kurland, LT 1997, 'Autonomic failure in Guamanian neurodegenerative disease', Neurology, vol. 49, no. 4, pp. 1031-1034.
Low PA, Ahlskog JE, Petersen RC, Waring SC, Esteban-Santillan C, Kurland LT. Autonomic failure in Guamanian neurodegenerative disease. Neurology. 1997 Oct;49(4):1031-1034.
Low, Phillip Anson ; Ahlskog, J. Eric ; Petersen, Ronald Carl ; Waring, Stephen C. ; Esteban-Santillan, Conception ; Kurland, Leonard T. / Autonomic failure in Guamanian neurodegenerative disease. In: Neurology. 1997 ; Vol. 49, No. 4. pp. 1031-1034.
@article{6be455062efb4a6a956d4a2b6f393461,
title = "Autonomic failure in Guamanian neurodegenerative disease",
abstract = "Autonomic impairment is minor in idiopathic amyotrophic lateral sclerosis (ALS) and Alzheimer's-type dementia (D) and is usually not marked in Parkinson's disease. The autonomic status of Guamonian parkinsonism (P), ALS, and parkinsonism-dementia complex (PDC) is essentially unknown. We therefore evaluated the autonomic nervous system in Guamanian D, ALS, P, and PDC. Cardiovagal, adrenergic, and postganglionic sudomotor functions were quantitated in 16 patients and 14 paired household controls. Patients comprised PDC (N = 11), D (N = 2), P (N = 2), and ALS (N = 1). Autonomic deficit was expressed on a composite autonomic scoring scale (CASS) and its subsets that corrects for the effects of age and gender. CASS severity was rated from 0 to 10 and the maximal subset scores were 3, 3, and 4 for postganglionic sudomotor, cardiovagal, and adrenergic deficits, respectively. CASS scores for mild, moderate, and severe autonomic failure are 1 to 3, 4 to 6, and 7 to 10, respectively. Symptoms were scored by an Autonomic Symptom Profile (ASP). The affected patients were older than and had a sex distribution different from paired controls (64.2 ± 8.0 versus 53.1 ± 13.5; p < 0.01; male/female = 9/7 versus 2/12; p = 0.045). CASS scores were markedly increased over paired controls (6.2 ± 2.3 versus 1.9 ± 1.3; p < 0.001), and involvement was generalized by system. There were deficits in sudomotor, cardiovagal, and adrenergic function. Orthostatic hypotension occurred in 6 of 16 patients and 2 of 14 paired controls. Guamanian patients had more autonomic dysfunction than non-Guamanian Parkinson's disease. ASP scores were higher in patients than controls and regressed with CASS. These differences persisted when corrected for the confounding effects of age, gender, and diabetes. We conclude that Guamanian patients have autonomic failure to a greater extent than non-Guamanian Parkinson's disease or ALS. This autonomic failure suggests multisystem autonomic involvement similar to but less severe than in multiple system atrophy.",
author = "Low, {Phillip Anson} and Ahlskog, {J. Eric} and Petersen, {Ronald Carl} and Waring, {Stephen C.} and Conception Esteban-Santillan and Kurland, {Leonard T.}",
year = "1997",
month = "10",
language = "English (US)",
volume = "49",
pages = "1031--1034",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Autonomic failure in Guamanian neurodegenerative disease

AU - Low, Phillip Anson

AU - Ahlskog, J. Eric

AU - Petersen, Ronald Carl

AU - Waring, Stephen C.

AU - Esteban-Santillan, Conception

AU - Kurland, Leonard T.

PY - 1997/10

Y1 - 1997/10

N2 - Autonomic impairment is minor in idiopathic amyotrophic lateral sclerosis (ALS) and Alzheimer's-type dementia (D) and is usually not marked in Parkinson's disease. The autonomic status of Guamonian parkinsonism (P), ALS, and parkinsonism-dementia complex (PDC) is essentially unknown. We therefore evaluated the autonomic nervous system in Guamanian D, ALS, P, and PDC. Cardiovagal, adrenergic, and postganglionic sudomotor functions were quantitated in 16 patients and 14 paired household controls. Patients comprised PDC (N = 11), D (N = 2), P (N = 2), and ALS (N = 1). Autonomic deficit was expressed on a composite autonomic scoring scale (CASS) and its subsets that corrects for the effects of age and gender. CASS severity was rated from 0 to 10 and the maximal subset scores were 3, 3, and 4 for postganglionic sudomotor, cardiovagal, and adrenergic deficits, respectively. CASS scores for mild, moderate, and severe autonomic failure are 1 to 3, 4 to 6, and 7 to 10, respectively. Symptoms were scored by an Autonomic Symptom Profile (ASP). The affected patients were older than and had a sex distribution different from paired controls (64.2 ± 8.0 versus 53.1 ± 13.5; p < 0.01; male/female = 9/7 versus 2/12; p = 0.045). CASS scores were markedly increased over paired controls (6.2 ± 2.3 versus 1.9 ± 1.3; p < 0.001), and involvement was generalized by system. There were deficits in sudomotor, cardiovagal, and adrenergic function. Orthostatic hypotension occurred in 6 of 16 patients and 2 of 14 paired controls. Guamanian patients had more autonomic dysfunction than non-Guamanian Parkinson's disease. ASP scores were higher in patients than controls and regressed with CASS. These differences persisted when corrected for the confounding effects of age, gender, and diabetes. We conclude that Guamanian patients have autonomic failure to a greater extent than non-Guamanian Parkinson's disease or ALS. This autonomic failure suggests multisystem autonomic involvement similar to but less severe than in multiple system atrophy.

AB - Autonomic impairment is minor in idiopathic amyotrophic lateral sclerosis (ALS) and Alzheimer's-type dementia (D) and is usually not marked in Parkinson's disease. The autonomic status of Guamonian parkinsonism (P), ALS, and parkinsonism-dementia complex (PDC) is essentially unknown. We therefore evaluated the autonomic nervous system in Guamanian D, ALS, P, and PDC. Cardiovagal, adrenergic, and postganglionic sudomotor functions were quantitated in 16 patients and 14 paired household controls. Patients comprised PDC (N = 11), D (N = 2), P (N = 2), and ALS (N = 1). Autonomic deficit was expressed on a composite autonomic scoring scale (CASS) and its subsets that corrects for the effects of age and gender. CASS severity was rated from 0 to 10 and the maximal subset scores were 3, 3, and 4 for postganglionic sudomotor, cardiovagal, and adrenergic deficits, respectively. CASS scores for mild, moderate, and severe autonomic failure are 1 to 3, 4 to 6, and 7 to 10, respectively. Symptoms were scored by an Autonomic Symptom Profile (ASP). The affected patients were older than and had a sex distribution different from paired controls (64.2 ± 8.0 versus 53.1 ± 13.5; p < 0.01; male/female = 9/7 versus 2/12; p = 0.045). CASS scores were markedly increased over paired controls (6.2 ± 2.3 versus 1.9 ± 1.3; p < 0.001), and involvement was generalized by system. There were deficits in sudomotor, cardiovagal, and adrenergic function. Orthostatic hypotension occurred in 6 of 16 patients and 2 of 14 paired controls. Guamanian patients had more autonomic dysfunction than non-Guamanian Parkinson's disease. ASP scores were higher in patients than controls and regressed with CASS. These differences persisted when corrected for the confounding effects of age, gender, and diabetes. We conclude that Guamanian patients have autonomic failure to a greater extent than non-Guamanian Parkinson's disease or ALS. This autonomic failure suggests multisystem autonomic involvement similar to but less severe than in multiple system atrophy.

UR - http://www.scopus.com/inward/record.url?scp=0030669244&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0030669244&partnerID=8YFLogxK

M3 - Article

C2 - 9339685

AN - SCOPUS:0030669244

VL - 49

SP - 1031

EP - 1034

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 4

ER -