Lewy Body Composite Risk Score Form and Instructions
Lewy Body Composite Risk Score Form and Instructions
Purpose of Use
The Lewy body dementias, composed of two related disorders: Dementia with Lewy bodies (DLB) and
Parkinsons disease dementia (PDD) are a challenge to diagnose, particularly outside of expert centers. One
of the great challenges in differential diagnosis of neurodegenerative disorders is attributing clinical symptoms
to specific pathologies to guide treatment choices and discuss prognosis and clinical course. While PDD
provides a potentially easier route to diagnosis because the cognitive disorder begins in face of an established
movement disorder and criteria have defined a mild cognitive impairment (MCI) state, DLB is a more difficult
entity to diagnose with delays in diagnosis approaching 18 months leading to significant burden to patients and
caregivers. Patients with DLB are often misdiagnosed. While consensus criteria for DLB have excellent
specificity (79-100%), there is no standardized way to assess or operationalize many of the cognitive and
behavioral symptoms which markedly decreases sensitivity in clinical practice (range 12-88%). We developed
the Lewy Body Composite Risk Score (LBCRS) to improve the ability to detect DLB and PDD in clinic and
research populations and increase the likelihood of determining whether Lewy bodies are contributing
pathology to the cognitive diagnosis. The LBCRS was derived from clinical features in autopsy-verified cases
of healthy controls, Alzheimers disease (AD), DLB, and PD with and without dementia. The LBCRS was tested
in a consecutive series of 256 patients compared with the Clinical Dementia Rating and gold standard
measures of cognition, motor symptoms, function, and behavior. The LBCRS increases diagnostic probability
that Lewy body pathology is contributing to the dementia syndrome and should improve clinical detection and
enrollment for clinical trials.
Administration and Scoring Guidelines
The questions are completed by a clinician after interview with patient and caregiver and a complete physical
and neurological exam. The operationalization of physical findings as being present for at least 6 months or
symptoms occurring at least 3 times over the past 6 months permitted the scoring of the LBCRS by totaling the
sum of signs and symptoms rated as present. In the context of a patient with cognitive impairment, the LBCRS
can increase the probability that Lewy bodies are a significant contributor to the clinical diagnosis.
The LBCRS has 10 Yes/No questions; 4 questions cover motor symptoms while 6 questions cover non-motor
symptoms. The clinician rates the presence or absence of physical signs and can elicit information from either
the patient or caregiver regarding the presence or absence of symptoms.
Interpretation of the QDRS
A screening test in itself is insufficient to diagnose a dementing disorder. The LBCRS is, however, quite
sensitive to suggesting that there is a high probability that Lewy bodies are a contributing pathology to the
underlying cognitive decline either as a single pathology or as a mixed dementia. The LBCRS discriminates
DLB, PDD and MCI due to Lewy body disease from other forms of cognitive impairment.
The LBCRS is scored on a continuous scale with a range of 0-10. Based on receiver operator characteristic
curves from 265 individuals included in the development and validation samples, LBCRS scores differentiate
with the following cut-points:
Non Lewy Body Case
0-2
Probable Lewy Body Case
3-10
Using the cutoff of 3 or greater, the LBCRS was able to discriminate:
DLB vs. AD
DLB vs any dementia
Area Under Curve
0.94 (0.90-0.97)
0.94 (0.91-0.98)
Sensitivity
94.2
97.9
Specificity
78.2
86.1
Positive Likelihood Ratio
4.1
7.0
Negative Likelihood Ratio
0.08
0.02
research, defined as investigator-initiated clinical research that is not funded or supported, in whole or in part,
by any for-profit entity (collectively, the Purpose). The Purpose specifically excludes any use, reproduction,
publication, and/or distribution of the LBCRS for any other reason or purpose, including without limitation (a)
the sale, distribution, publication, or transfer of the LBCRS for any consideration or commercial value; (b) the
creation of any derivative works of the LBCRS, including translations thereof; (c) the use of the LBCRS as a
marketing tool for the promotion or sale of any drug; (d) incorporation of the LBCRS in an electronic medical
record application software; and/or (e) any use of the LBCRS in connection with research or clinical trials that
are supported, in whole or in part, by any for-profit entity.
All copies of the Lewy Body Composite Risk Score (LBCRS) should include the following notice: "Reprinted
with permission. Copyright 2015. Lewy Body Composite Risk Score: A Rapid Method to Improve the Clinical
Detection of Lewy Body Dementia is a copyrighted instrument of James E. Galvin. All Rights Reserved."
Individuals or corporations intending to use the LBCRS for any use other than the Purpose stated above,
including clinical trial or commercial purposes, must obtain Dr. Galvins prior written permission. Dr. Galvin is
employed by the Charles E. Schmidt College of Medicine, Florida Atlantic University. Please contact James E.
Galvin, MD, MPH (galvinj@health.fau.edu) for more information.
Copyright 2015: The Lewy Body Composite Risk Score is a copyrighted instrument of James E. Galvin. All
Rights Reserved.
Copyright 2015 The Lewy Body Composite Risk Score James E. Galvin
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