Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: Probable manganese neurotoxicity

Kevin J. Klos, J. Eric Ahlskog, Keith Anthony Josephs, Robert D. Fealey, Clayton T. Cowl, Neeraj Kumar

Research output: Contribution to journalArticle

75 Citations (Scopus)

Abstract

Background: An atypical form of parkinsonism has been described in patients with chronic liver disease, associated with increased T1 signal in the basal ganglia on magnetic resonance imaging. The magnetic resonance imaging signal changes are characteristic of manganese accumulation, which has been neuropathologically confirmed. Manganese neurotoxicity may result in additional neurologic findings besides parkinsonism. Objective: To fully characterize patients with chronic central nervous system symptoms and chronic liver failure associated with basal ganglia T1 hyperintensity. Design: Prospective and retrospective case study. Setting: Mayo Clinic, Rochester, Minn. Participants: Eight patients referred for neurologic evaluation and studied prospectively, and 7 additional retrospectively identified patients who had been examined by Mayo Clinic neurologists. Main Outcome Measures: Neurologic syndromes identified. Results: Three syndromes were recognized in these 15 patients with liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: (1) isolated parkinsonism, (2) gait ataxia plus other neurologic findings (ataxia-plus), and (3) cognitive impairment with psychiatric features. All but 1 patient had elevated blood manganese levels. Ammonia levels were normal in most, and the neurologic syndromes did not appear to reflect the well-known toxic-metabolic encephalopathy of liver disease. Conclusions: Chronic liver failure may result in heterogeneous neurologic syndromes that cut across a variety of liver diseases. We selected cases on the basis of evidence of brain manganese accumulation, and this may be a crucial component of these syndromes. Further studies are necessary to explore this issue.

Original languageEnglish (US)
Pages (from-to)1385-1390
Number of pages6
JournalArchives of Neurology
Volume62
Issue number9
DOIs
StatePublished - Sep 2005

Fingerprint

End Stage Liver Disease
Manganese
Basal Ganglia
Nervous System
Magnetic Resonance Imaging
Parkinsonian Disorders
Liver Diseases
Neurologic Manifestations
Neurotoxicity Syndromes
Metabolic Brain Diseases
Gait Ataxia
Liver Failure
Ataxia
Ammonia
Psychiatry
Chronic Disease
Central Nervous System
Retrospective Studies
Outcome Assessment (Health Care)
Syndrome

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging : Probable manganese neurotoxicity. / Klos, Kevin J.; Ahlskog, J. Eric; Josephs, Keith Anthony; Fealey, Robert D.; Cowl, Clayton T.; Kumar, Neeraj.

In: Archives of Neurology, Vol. 62, No. 9, 09.2005, p. 1385-1390.

Research output: Contribution to journalArticle

Klos, Kevin J. ; Ahlskog, J. Eric ; Josephs, Keith Anthony ; Fealey, Robert D. ; Cowl, Clayton T. ; Kumar, Neeraj. / Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging : Probable manganese neurotoxicity. In: Archives of Neurology. 2005 ; Vol. 62, No. 9. pp. 1385-1390.
@article{838f82c26eaf4f8b8e50bce7fb3a4880,
title = "Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: Probable manganese neurotoxicity",
abstract = "Background: An atypical form of parkinsonism has been described in patients with chronic liver disease, associated with increased T1 signal in the basal ganglia on magnetic resonance imaging. The magnetic resonance imaging signal changes are characteristic of manganese accumulation, which has been neuropathologically confirmed. Manganese neurotoxicity may result in additional neurologic findings besides parkinsonism. Objective: To fully characterize patients with chronic central nervous system symptoms and chronic liver failure associated with basal ganglia T1 hyperintensity. Design: Prospective and retrospective case study. Setting: Mayo Clinic, Rochester, Minn. Participants: Eight patients referred for neurologic evaluation and studied prospectively, and 7 additional retrospectively identified patients who had been examined by Mayo Clinic neurologists. Main Outcome Measures: Neurologic syndromes identified. Results: Three syndromes were recognized in these 15 patients with liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: (1) isolated parkinsonism, (2) gait ataxia plus other neurologic findings (ataxia-plus), and (3) cognitive impairment with psychiatric features. All but 1 patient had elevated blood manganese levels. Ammonia levels were normal in most, and the neurologic syndromes did not appear to reflect the well-known toxic-metabolic encephalopathy of liver disease. Conclusions: Chronic liver failure may result in heterogeneous neurologic syndromes that cut across a variety of liver diseases. We selected cases on the basis of evidence of brain manganese accumulation, and this may be a crucial component of these syndromes. Further studies are necessary to explore this issue.",
author = "Klos, {Kevin J.} and Ahlskog, {J. Eric} and Josephs, {Keith Anthony} and Fealey, {Robert D.} and Cowl, {Clayton T.} and Neeraj Kumar",
year = "2005",
month = "9",
doi = "10.1001/archneur.62.9.1385",
language = "English (US)",
volume = "62",
pages = "1385--1390",
journal = "Archives of Neurology",
issn = "0003-9942",
publisher = "American Medical Association",
number = "9",

}

TY - JOUR

T1 - Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging

T2 - Probable manganese neurotoxicity

AU - Klos, Kevin J.

AU - Ahlskog, J. Eric

AU - Josephs, Keith Anthony

AU - Fealey, Robert D.

AU - Cowl, Clayton T.

AU - Kumar, Neeraj

PY - 2005/9

Y1 - 2005/9

N2 - Background: An atypical form of parkinsonism has been described in patients with chronic liver disease, associated with increased T1 signal in the basal ganglia on magnetic resonance imaging. The magnetic resonance imaging signal changes are characteristic of manganese accumulation, which has been neuropathologically confirmed. Manganese neurotoxicity may result in additional neurologic findings besides parkinsonism. Objective: To fully characterize patients with chronic central nervous system symptoms and chronic liver failure associated with basal ganglia T1 hyperintensity. Design: Prospective and retrospective case study. Setting: Mayo Clinic, Rochester, Minn. Participants: Eight patients referred for neurologic evaluation and studied prospectively, and 7 additional retrospectively identified patients who had been examined by Mayo Clinic neurologists. Main Outcome Measures: Neurologic syndromes identified. Results: Three syndromes were recognized in these 15 patients with liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: (1) isolated parkinsonism, (2) gait ataxia plus other neurologic findings (ataxia-plus), and (3) cognitive impairment with psychiatric features. All but 1 patient had elevated blood manganese levels. Ammonia levels were normal in most, and the neurologic syndromes did not appear to reflect the well-known toxic-metabolic encephalopathy of liver disease. Conclusions: Chronic liver failure may result in heterogeneous neurologic syndromes that cut across a variety of liver diseases. We selected cases on the basis of evidence of brain manganese accumulation, and this may be a crucial component of these syndromes. Further studies are necessary to explore this issue.

AB - Background: An atypical form of parkinsonism has been described in patients with chronic liver disease, associated with increased T1 signal in the basal ganglia on magnetic resonance imaging. The magnetic resonance imaging signal changes are characteristic of manganese accumulation, which has been neuropathologically confirmed. Manganese neurotoxicity may result in additional neurologic findings besides parkinsonism. Objective: To fully characterize patients with chronic central nervous system symptoms and chronic liver failure associated with basal ganglia T1 hyperintensity. Design: Prospective and retrospective case study. Setting: Mayo Clinic, Rochester, Minn. Participants: Eight patients referred for neurologic evaluation and studied prospectively, and 7 additional retrospectively identified patients who had been examined by Mayo Clinic neurologists. Main Outcome Measures: Neurologic syndromes identified. Results: Three syndromes were recognized in these 15 patients with liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: (1) isolated parkinsonism, (2) gait ataxia plus other neurologic findings (ataxia-plus), and (3) cognitive impairment with psychiatric features. All but 1 patient had elevated blood manganese levels. Ammonia levels were normal in most, and the neurologic syndromes did not appear to reflect the well-known toxic-metabolic encephalopathy of liver disease. Conclusions: Chronic liver failure may result in heterogeneous neurologic syndromes that cut across a variety of liver diseases. We selected cases on the basis of evidence of brain manganese accumulation, and this may be a crucial component of these syndromes. Further studies are necessary to explore this issue.

UR - http://www.scopus.com/inward/record.url?scp=25144510105&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=25144510105&partnerID=8YFLogxK

U2 - 10.1001/archneur.62.9.1385

DO - 10.1001/archneur.62.9.1385

M3 - Article

C2 - 16157745

AN - SCOPUS:25144510105

VL - 62

SP - 1385

EP - 1390

JO - Archives of Neurology

JF - Archives of Neurology

SN - 0003-9942

IS - 9

ER -