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The international Dementia With Lewy Bodies Consortium has updated its guideline for diagnosing and treating this disease. “The updated clinical criteria and associated biomarkers hopefully will lead to earlier and more accurate diagnosis, and that is key to helping patients confront this challenging illness and maximize their quality of life,” said Bradley Boeve, MD, Professor of Neurology at Mayo Clinic in Rochester, Minnesota, and a coauthor. The guideline was published online ahead of print June 7 in Neurology. 
The new guideline is a refinement of the consortium’s previous guideline, which was published in December 2005. To develop the new document, the consortium solicited and reviewed reports from four multidisciplinary expert working groups. The consortium also held a meeting in which patients and caregivers participated.
The revised consensus criteria distinguish between the clinical features and diagnostic biomarkers of the disease. The core clinical features are fluctuation in cognition, attention, and arousal; visual hallucinations (eg, of people, children, or animals); parkinsonism; and REM sleep behavior disorder, according to the authors. The criteria now list hypersomnia and hyposmia as supportive clinical features.
Direct biomarker evidence of Lewy body pathology is not available, said the authors. Indicative indirect biomarkers include reduced dopamine transporter uptake in basal ganglia demonstrated by single-photon emission computerized tomography or PET imaging, reduced uptake on metaiodobenzylguanidine (MIBG) myocardial scintigraphy, and polysomnographic confirmation of REM sleep without atonia.
A neurologist can diagnose probable dementia with Lewy bodies if two or more of its core clinical features are present, with or without the presence of indicative biomarkers. This diagnosis also is warranted if only one core clinical feature is present, but one or more indicative biomarkers are present.
The document incorporates new information about previously reported aspects of dementia with Lewy bodies and gives greater diagnostic weight to REM sleep behavior disorder and 123iodine-MIBG myocardial scintigraphy. The authors describe the diagnostic role of other neuroimaging, electrophysiologic, and laboratory investigations. They recommend minor modifications to pathologic methods and criteria to take account of Alzheimer’s disease neuropathologic change, to add previously omitted Lewy-related pathology categories, and to include assessments for substantia nigra neuronal loss.
Because the literature contains few randomized controlled trials, the guideline’s recommendations about clinical management are based on expert opinion. Cholinesterase inhibitors can improve cognition, global function, activities of living, and neuropsychiatric symptoms, according to the authors. Nonpharmacologic management strategies should be developed and tested, they added.
“There remains a pressing need to understand the underlying neurobiology and pathophysiology of dementia with Lewy bodies, to develop and deliver clinical trials with both symptomatic and disease-modifying agents, and to help patients and carers worldwide to inform themselves about the disease, its prognosis, best available treatments, ongoing research, and how to get adequate support,” the authors concluded.
—Erik Greb
Suggested Reading
McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017 Jun 7 [Epub ahead of print].
The international Dementia With Lewy Bodies Consortium has updated its guideline for diagnosing and treating this disease. “The updated clinical criteria and associated biomarkers hopefully will lead to earlier and more accurate diagnosis, and that is key to helping patients confront this challenging illness and maximize their quality of life,” said Bradley Boeve, MD, Professor of Neurology at Mayo Clinic in Rochester, Minnesota, and a coauthor. The guideline was published online ahead of print June 7 in Neurology.
The new guideline is a refinement of the consortium’s previous guideline, which was published in December 2005. To develop the new document, the consortium solicited and reviewed reports from four multidisciplinary expert working groups. The consortium also held a meeting in which patients and caregivers participated.
The revised consensus criteria distinguish between the clinical features and diagnostic biomarkers of the disease. The core clinical features are fluctuation in cognition, attention, and arousal; visual hallucinations (eg, of people, children, or animals); parkinsonism; and REM sleep behavior disorder, according to the authors. The criteria now list hypersomnia and hyposmia as supportive clinical features.
Direct biomarker evidence of Lewy body pathology is not available, said the authors. Indicative indirect biomarkers include reduced dopamine transporter uptake in basal ganglia demonstrated by single-photon emission computerized tomography or PET imaging, reduced uptake on metaiodobenzylguanidine (MIBG) myocardial scintigraphy, and polysomnographic confirmation of REM sleep without atonia.
A neurologist can diagnose probable dementia with Lewy bodies if two or more of its core clinical features are present, with or without the presence of indicative biomarkers. This diagnosis also is warranted if only one core clinical feature is present, but one or more indicative biomarkers are present.
The document incorporates new information about previously reported aspects of dementia with Lewy bodies and gives greater diagnostic weight to REM sleep behavior disorder and 123iodine-MIBG myocardial scintigraphy. The authors describe the diagnostic role of other neuroimaging, electrophysiologic, and laboratory investigations. They recommend minor modifications to pathologic methods and criteria to take account of Alzheimer’s disease neuropathologic change, to add previously omitted Lewy-related pathology categories, and to include assessments for substantia nigra neuronal loss.
Because the literature contains few randomized controlled trials, the guideline’s recommendations about clinical management are based on expert opinion. Cholinesterase inhibitors can improve cognition, global function, activities of living, and neuropsychiatric symptoms, according to the authors. Nonpharmacologic management strategies should be developed and tested, they added.
“There remains a pressing need to understand the underlying neurobiology and pathophysiology of dementia with Lewy bodies, to develop and deliver clinical trials with both symptomatic and disease-modifying agents, and to help patients and carers worldwide to inform themselves about the disease, its prognosis, best available treatments, ongoing research, and how to get adequate support,” the authors concluded.
—Erik Greb
Suggested Reading
McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017 Jun 7 [Epub ahead of print].
The international Dementia With Lewy Bodies Consortium has updated its guideline for diagnosing and treating this disease. “The updated clinical criteria and associated biomarkers hopefully will lead to earlier and more accurate diagnosis, and that is key to helping patients confront this challenging illness and maximize their quality of life,” said Bradley Boeve, MD, Professor of Neurology at Mayo Clinic in Rochester, Minnesota, and a coauthor. The guideline was published online ahead of print June 7 in Neurology.
The new guideline is a refinement of the consortium’s previous guideline, which was published in December 2005. To develop the new document, the consortium solicited and reviewed reports from four multidisciplinary expert working groups. The consortium also held a meeting in which patients and caregivers participated.
The revised consensus criteria distinguish between the clinical features and diagnostic biomarkers of the disease. The core clinical features are fluctuation in cognition, attention, and arousal; visual hallucinations (eg, of people, children, or animals); parkinsonism; and REM sleep behavior disorder, according to the authors. The criteria now list hypersomnia and hyposmia as supportive clinical features.
Direct biomarker evidence of Lewy body pathology is not available, said the authors. Indicative indirect biomarkers include reduced dopamine transporter uptake in basal ganglia demonstrated by single-photon emission computerized tomography or PET imaging, reduced uptake on metaiodobenzylguanidine (MIBG) myocardial scintigraphy, and polysomnographic confirmation of REM sleep without atonia.
A neurologist can diagnose probable dementia with Lewy bodies if two or more of its core clinical features are present, with or without the presence of indicative biomarkers. This diagnosis also is warranted if only one core clinical feature is present, but one or more indicative biomarkers are present.
The document incorporates new information about previously reported aspects of dementia with Lewy bodies and gives greater diagnostic weight to REM sleep behavior disorder and 123iodine-MIBG myocardial scintigraphy. The authors describe the diagnostic role of other neuroimaging, electrophysiologic, and laboratory investigations. They recommend minor modifications to pathologic methods and criteria to take account of Alzheimer’s disease neuropathologic change, to add previously omitted Lewy-related pathology categories, and to include assessments for substantia nigra neuronal loss.
Because the literature contains few randomized controlled trials, the guideline’s recommendations about clinical management are based on expert opinion. Cholinesterase inhibitors can improve cognition, global function, activities of living, and neuropsychiatric symptoms, according to the authors. Nonpharmacologic management strategies should be developed and tested, they added.
“There remains a pressing need to understand the underlying neurobiology and pathophysiology of dementia with Lewy bodies, to develop and deliver clinical trials with both symptomatic and disease-modifying agents, and to help patients and carers worldwide to inform themselves about the disease, its prognosis, best available treatments, ongoing research, and how to get adequate support,” the authors concluded.
—Erik Greb
Suggested Reading
McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017 Jun 7 [Epub ahead of print].