Rapidly progressive neurodegenerative dementias

Keith Anthony Josephs, J. Eric Ahlskog, Joseph E Parisi, Bradley F Boeve, Brian A. Crum, Caterina Giannini, Ronald Carl Petersen

Research output: Contribution to journalArticle

83 Citations (Scopus)

Abstract

Backgound:Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death. Objective: To characterize patients with a neurodegen-erative disease and a rapidly progressive course to death. Design, Setting, and patients: Using a text word search for "rapid" and "dementia" in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between January 1, 2000, and September 30, 2007, with brain autopsy (N = 96) at a tertiary care medical center. Of these 96 patients, we included only those with disease duration of less than 4 years to death and with histological diagnosis of a neu-rodegenerative disease. Main Outcome Measures: Rapidly progressive dementia with death sooner than 4 years after onset and pathological diagnosis at our institution of a neurode-generative disease. Results: We included 22 patients (10 men). Although 8 cases (36%) had Creutzfeldt-Jakob disease (CJD), the rest had frontotemporal lobar degeneration with motor neuron degeneration (5 cases [23%]), a tauopathy (progressive supranuclear palsy or corticobasal degeneration) (4 cases [18%]), diffuse Lewy body disease (3 cases [14%]), or Alzheimer disease (2 cases [9%]). All of the patients with CJD died 12 months or sooner after onset, whereas the others had an illness duration longer than 12 months. Notably, all of the 3 patients with diffuse Lewy body disease but no others initially experienced a transient postoperative or illness-associated encephalopa-thy, then relative normality for 2 years, and finally a rapidly progressive dementia and decline to death in 4 to 12 months. Conclusions: Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegen-erative dementia, frontotemporal lobar degeneration with motor neuron degeneration, diffuse Lewy body disease, tauopathies, and Alzheimer disease can also cause a rapidly progressive dementia. If illness duration is beyond 12 months, a non-CJD neurodegenerative disease may be more likely than CJD to be the diagnosis.

Original languageEnglish (US)
Pages (from-to)201-207
Number of pages7
JournalArchives of Neurology
Volume66
Issue number2
DOIs
StatePublished - Feb 2009

Fingerprint

Dementia
Creutzfeldt-Jakob Syndrome
Lewy Body Disease
Frontotemporal Lobar Degeneration
Tauopathies
Nerve Degeneration
Motor Neurons
Medical Record Linkage
Progressive Supranuclear Palsy
Tertiary Care Centers
Neurodegenerative Diseases
Autopsy
Alzheimer Disease
Outcome Assessment (Health Care)
Brain
Degeneration

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Rapidly progressive neurodegenerative dementias. / Josephs, Keith Anthony; Ahlskog, J. Eric; Parisi, Joseph E; Boeve, Bradley F; Crum, Brian A.; Giannini, Caterina; Petersen, Ronald Carl.

In: Archives of Neurology, Vol. 66, No. 2, 02.2009, p. 201-207.

Research output: Contribution to journalArticle

@article{1faf6f7fe8624f34ac3b579e76475983,
title = "Rapidly progressive neurodegenerative dementias",
abstract = "Backgound:Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death. Objective: To characterize patients with a neurodegen-erative disease and a rapidly progressive course to death. Design, Setting, and patients: Using a text word search for {"}rapid{"} and {"}dementia{"} in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between January 1, 2000, and September 30, 2007, with brain autopsy (N = 96) at a tertiary care medical center. Of these 96 patients, we included only those with disease duration of less than 4 years to death and with histological diagnosis of a neu-rodegenerative disease. Main Outcome Measures: Rapidly progressive dementia with death sooner than 4 years after onset and pathological diagnosis at our institution of a neurode-generative disease. Results: We included 22 patients (10 men). Although 8 cases (36{\%}) had Creutzfeldt-Jakob disease (CJD), the rest had frontotemporal lobar degeneration with motor neuron degeneration (5 cases [23{\%}]), a tauopathy (progressive supranuclear palsy or corticobasal degeneration) (4 cases [18{\%}]), diffuse Lewy body disease (3 cases [14{\%}]), or Alzheimer disease (2 cases [9{\%}]). All of the patients with CJD died 12 months or sooner after onset, whereas the others had an illness duration longer than 12 months. Notably, all of the 3 patients with diffuse Lewy body disease but no others initially experienced a transient postoperative or illness-associated encephalopa-thy, then relative normality for 2 years, and finally a rapidly progressive dementia and decline to death in 4 to 12 months. Conclusions: Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegen-erative dementia, frontotemporal lobar degeneration with motor neuron degeneration, diffuse Lewy body disease, tauopathies, and Alzheimer disease can also cause a rapidly progressive dementia. If illness duration is beyond 12 months, a non-CJD neurodegenerative disease may be more likely than CJD to be the diagnosis.",
author = "Josephs, {Keith Anthony} and Ahlskog, {J. Eric} and Parisi, {Joseph E} and Boeve, {Bradley F} and Crum, {Brian A.} and Caterina Giannini and Petersen, {Ronald Carl}",
year = "2009",
month = "2",
doi = "10.1001/archneurol.2008.534",
language = "English (US)",
volume = "66",
pages = "201--207",
journal = "Archives of Neurology",
issn = "0003-9942",
publisher = "American Medical Association",
number = "2",

}

TY - JOUR

T1 - Rapidly progressive neurodegenerative dementias

AU - Josephs, Keith Anthony

AU - Ahlskog, J. Eric

AU - Parisi, Joseph E

AU - Boeve, Bradley F

AU - Crum, Brian A.

AU - Giannini, Caterina

AU - Petersen, Ronald Carl

PY - 2009/2

Y1 - 2009/2

N2 - Backgound:Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death. Objective: To characterize patients with a neurodegen-erative disease and a rapidly progressive course to death. Design, Setting, and patients: Using a text word search for "rapid" and "dementia" in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between January 1, 2000, and September 30, 2007, with brain autopsy (N = 96) at a tertiary care medical center. Of these 96 patients, we included only those with disease duration of less than 4 years to death and with histological diagnosis of a neu-rodegenerative disease. Main Outcome Measures: Rapidly progressive dementia with death sooner than 4 years after onset and pathological diagnosis at our institution of a neurode-generative disease. Results: We included 22 patients (10 men). Although 8 cases (36%) had Creutzfeldt-Jakob disease (CJD), the rest had frontotemporal lobar degeneration with motor neuron degeneration (5 cases [23%]), a tauopathy (progressive supranuclear palsy or corticobasal degeneration) (4 cases [18%]), diffuse Lewy body disease (3 cases [14%]), or Alzheimer disease (2 cases [9%]). All of the patients with CJD died 12 months or sooner after onset, whereas the others had an illness duration longer than 12 months. Notably, all of the 3 patients with diffuse Lewy body disease but no others initially experienced a transient postoperative or illness-associated encephalopa-thy, then relative normality for 2 years, and finally a rapidly progressive dementia and decline to death in 4 to 12 months. Conclusions: Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegen-erative dementia, frontotemporal lobar degeneration with motor neuron degeneration, diffuse Lewy body disease, tauopathies, and Alzheimer disease can also cause a rapidly progressive dementia. If illness duration is beyond 12 months, a non-CJD neurodegenerative disease may be more likely than CJD to be the diagnosis.

AB - Backgound:Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death. Objective: To characterize patients with a neurodegen-erative disease and a rapidly progressive course to death. Design, Setting, and patients: Using a text word search for "rapid" and "dementia" in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between January 1, 2000, and September 30, 2007, with brain autopsy (N = 96) at a tertiary care medical center. Of these 96 patients, we included only those with disease duration of less than 4 years to death and with histological diagnosis of a neu-rodegenerative disease. Main Outcome Measures: Rapidly progressive dementia with death sooner than 4 years after onset and pathological diagnosis at our institution of a neurode-generative disease. Results: We included 22 patients (10 men). Although 8 cases (36%) had Creutzfeldt-Jakob disease (CJD), the rest had frontotemporal lobar degeneration with motor neuron degeneration (5 cases [23%]), a tauopathy (progressive supranuclear palsy or corticobasal degeneration) (4 cases [18%]), diffuse Lewy body disease (3 cases [14%]), or Alzheimer disease (2 cases [9%]). All of the patients with CJD died 12 months or sooner after onset, whereas the others had an illness duration longer than 12 months. Notably, all of the 3 patients with diffuse Lewy body disease but no others initially experienced a transient postoperative or illness-associated encephalopa-thy, then relative normality for 2 years, and finally a rapidly progressive dementia and decline to death in 4 to 12 months. Conclusions: Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegen-erative dementia, frontotemporal lobar degeneration with motor neuron degeneration, diffuse Lewy body disease, tauopathies, and Alzheimer disease can also cause a rapidly progressive dementia. If illness duration is beyond 12 months, a non-CJD neurodegenerative disease may be more likely than CJD to be the diagnosis.

UR - http://www.scopus.com/inward/record.url?scp=60549103244&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=60549103244&partnerID=8YFLogxK

U2 - 10.1001/archneurol.2008.534

DO - 10.1001/archneurol.2008.534

M3 - Article

VL - 66

SP - 201

EP - 207

JO - Archives of Neurology

JF - Archives of Neurology

SN - 0003-9942

IS - 2

ER -