This is a short — albeit filled with medical terminology — summary of Dementia with Lewy bodies (DLB). The authors of the review article are Lithuanian geriatricians.
The abstract notes that the differential diagnoses focuses on:
* “other later life dementia syndromes” – an example is Alzheimer’s Disease or vascular dementia
* “other parkinsonian diseases (Parkinson’s disease, progressive supranuclear palsy, corticobasal degeneration)” – within the pool of 100+ families I have helped accomplish brain donation, two families with a clinical diagnosis of DLB ended up with a neuropathology report that said PSP. I have yet to see anyone with CBD on the neuropathology report.
* “primary psychiatric illnesses” – not sure what these would be other than major depression or psychosis (hallucinations and delusions).
The paper mentions one additional disorder that DLB can be confused with:
* Creutzfeldt-Jacob disease. In that same pool of 100+ families, one family had a member with a clinical diagnosis of DLB and a CJD diagnosis upon brain autopsy.
There were a few new things for me in the full paper, including:
1. “Prevalence rates reported in studies by neurologists (0%–2.8%) were different from those reported by other specialists, such as psychiatrists and geriatricians (3.6%–30.5%). It may be due to a different emphasis on clinical phenotypes while diagnosing DLB: psychiatrists pay more attention to psychiatric symptoms and neurologists to neurological ones.”
2. “Timely and accurate diagnosis is very important due to several reasons: 1) neuroleptic sensitivity; 2) effectiveness of the treatment with cholinesterase inhibitors; and 3) some patients may respond to dopaminergic treatment of parkinsonism.”
3. “The loss of consciousness, which is characteristic of DLB, can be explained by temporal slow-wave transients revealed in EEG.”
4. “Currently, there are no evidence-based guidelines available when the treatment with antidementia drugs should be discontinued. The expert group recommends the following procedure: neuropsychological testing should be repeated after 3 to 6 months, and withdrawal of antidementia medication should be considered if the deterioration of cognitive functions is documented. If a significant decrease in the cognitive function is observed after 3-year treatment, antidementia drugs should be discontinued.”
5. “Bonelli with colleagues studied the effect of levodopa in patients with PD, PDD, and DLB and showed that motor improvement was similar in 3 groups studied, although the proportion of patients with an improvement of 10% and more was greater in PD than PDD and DLB.”
6. “A study by Ballard in 1998 reported that severe neuroleptic sensitivity occurred only in DLB (compared with AD) and was seen in 29% of cases, in all cases occurring within 2 weeks’ administration of a new neuroleptic or a dose change.”
I’ve copied the abstract below.
Medicina (Kaunas). 2012;45(1):1-8.
Dementia with lewy bodies: the principles of diagnostics, treatment, and management.
Macijauskiene. J, Lesauskaite. V.
Department of Geriatrics, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania.
Dementia with Lewy bodies was first recognized as a separate entity about 30 years ago. The prevalence varies from 0% to 5% in the general population, and this disease accounts for 0% to 30.5% of all dementia cases. Dementia with Lewy bodies is considered the second most common cause of degenerative dementia after Alzheimer’s disease.
The disease is characterized by alpha-synuclein immunoreactive protein deposits in both neurons and glial cells. The protein deposits are especially prominent in dopaminergic neurons, where they can be detected using conventional histological stains, such as hematoxylin and eosin, and are commonly referred to as Lewy bodies.
The diagnosis of dementia with Lewy bodies is based on the presence of dementia as well as 2 of the following 3 core diagnostic features: 1) fluctuating cognition, 2) visual hallucinations, and 3) movement disorder.
Diagnostic tests include laboratory data, structural and functional imaging, and electroencephalography.
Differential diagnosis of dementia with Lewy bodies focuses on other later life dementia syndromes, other parkinsonian diseases (Parkinson’s disease, progressive supranuclear palsy, corticobasal degeneration), and primary psychiatric illnesses.
There is type 1b evidence to support treatment with cholinesterase inhibitors. Glutamatergic and dopaminergic therapies are used as well. Standard neuroleptics are contraindicated, and atypical agents should be used cautiously. Nonpharmacologic measures – therapeutic environment, psychological and social support, physical activity, behavioral management strategies, caregivers’ education and support, and different services – could be suggested.
PubMed ID#: 22481369 (see pubmed.gov for this abstract only)
cholinesterase inhibitors = Aricept, Exelon, Razadyne
glutamatergic therapies = Namenda
dopaminergic therapies = Sinemet, Selegiline