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20-September-2008 10:08:30 - Dementia with Lewy bodies May 2008 Dementia with Lewy bodies Classification and external resources ICD-10 G31.8 ICD-9 331.82 DiseasesDB 3800 eMedicine neuro/91 MeSH D020961 Dementia with Lewy bodies DLB is a type of dementia characterized by abnormal levels of Lewy bodies, a nervous system protein. DLB is sometimes described as second only to Alzheimer's disease as a cause of dementia.12 Current estimates are that about 60-to-75% of diagnosed dementias are of the Alzheimer's and mixed Alzheimer's and vascular dementia type, 10-to-15% are Lewy bodies type, with the remaining types being of an entire spectrum of dementias including frontotemporal lobar degeneration, alcoholic dementia, pure vascular dementia, etc. Contents 1 Causes 2 Presentation 3 History 4 Clinical features 5 Treatment 6 Nomenclature 7 References 8 External links Causes The causes are not yet well understood, but a locus at PARK11 has been described.3 Presentation Dementia with Lewy bodies exhibits clinical overlap between Alzheimer's disease and Parkinson's disease. Pathologically, it is characterized by development of abnormal proteinaceous alpha-synuclein cytoplasmic inclusions, called Lewy bodies, throughout the brain. These inclusions have similar structural features to classical Lewy bodies seen subcortically in Parkinson's disease. Additionally, there is a loss of dopamine-producing neurons in the substantia nigra similar to that seen in Parkinson's disease, and a loss of acetylcholine-producing neurons in the basal nucleus of Meynert and elsewhere similar to that seen in Alzheimer's disease. Cerebral atrophy or shrinkage also occurs as the cerebral cortex degenerates. Autopsy series have revealed that the pathology of DLB is often concomitant with the pathology of Alzheimer's disease. That is, when Lewy body inclusions are found in the cortex, they often co-occur with Alzheimer's disease pathology found primarily in the hippocampus, including: neurofibrillary tangles abnormally phosphorylated tau protein, senile plaques deposited beta-amyloid protein, and granulovacuolar degeneration. Within DLB, the loss of cholinergic acetylcholine-producing neurons is thought to account for the degradation of cognitive and emotional functioning as in Alzheimer's disease, while the loss of dopaminergic dopamine-producing neurons is thought to account for the degradation of motor control as in Parkinson's disease. Thus, DLB is similar in some ways to both the dementia resulting from Alzheimer's disease and Parkinson's disease. In fact, it is often confused in its early stages with Alzheimer's disease and/or vascular dementia multi-infarct dementia. The overlap of neuropathologies and presenting symptoms cognitive, emotional, and motor may make an accurate differential diagnosis difficult to reach. History Dementia with Lewy bodies is not a DSM-IV recognized diagnosis. Note: DSM-IV was published in 1994. It is, however, briefly mentioned in the DSM-IV-TR published in 2000 under Dementia Due to Other General Medical Conditions. In 1996, a consortium of scientists initially proposed and later revised diagnostic guidelines. Central features of DLB include progressive cognitive decline, typically with impairments in memory, visual-spatial abilities, and/or attention. Clinical features Core features include fluctuating cognition with variations in attention and alertness, recurrent visual hallucinations typically early in the disease, and motor features of parkinsonism. DLB patients also often experience repeated falls, syncope fainting, transient loss of consciousness, and hypersentivity to neuroleptic medications. Generally, DLB is diagnosed when cognitive symptoms develop within a year or two of movement disorder/Parkinsonian symptoms. Recent research suggests that presence of sleep disturbance may also be useful in differentiating DLB from other forms of dementia. Treatment The treatment of DLB, as with Parkinson's disease, involves striking a balance between treating the motor and emotive/cognitive symptoms. Treatment of the movement portion of the disease can typically result in worsening hallucinations and psychosis, while treatment of the hallucinations and psychosis can result in worsening movement symptoms. The use of cholinesterase inhibitors represents the treatment of choice. This improves symptoms, but does not cure the disease. The use of memantine may be recommended, and may represent a means to slow or prevent the decline of cognitive function, although strong evidence to support or disprove this is lacking. Nomenclature Dementia with Lewy bodies DLB is also known under a variety of other names including, Lewy body dementia LBD, diffuse Lewy body disease DLBD, cortical Lewy body disease CLBD, and senile dementia of Lewy type. All incorporate the name Lewy, as Dr. Frederic Lewy 1885-1950 was first to discover the abnormal protein deposits Lewy body inclusions in the early 1900s.4 References ^ Heidebrink JL 2002. Is dementia with Lewy bodies the second most common cause of dementia?. J Geriatr Psychiatry Neurol 15 4: 182-7. PMID 12489913. ^ Zaccai J, McCracken C, Brayne C November 2005. A systematic review of prevalence and incidence studies of dementia with Lewy bodies. Age Ageing 34 6: 561-6. doi:10.1093/ageing/afi190. PMID 16267179. ^ Bogaerts V, Engelborghs S, Kumar-Singh S, et al September 2007. A novel locus for dementia with Lewy bodies: a clinically and genetically heterogeneous disorder. Brain 130 Pt 9: 2277-91. doi:10.1093/brain/awm167. PMID 17681982. ^ Lewy body dementia at Who Named It External links Laboratory for Alzheimer's and Parkinson's Disease Research - Prof. Dr. Christian Haass dementiawithlewybodies at NINDS Dementia Research News from ScienceDaily Lewy Body Dementia Association, Inc. The Lewy Body Society, UK and Europe's only dementia with Lewy bodies charity. FTD Support Forum The Association for Frontotemporal Dementias UCSF Memory and Aging Center - FTD Resources and FTD Info Mayo Clinic - FTD Info Pick's Disease Support Group Online Frontotemporal Dementia Caregiver Support Center v d e Pathology of the nervous system, primarily CNS G00-G47, 320-349 Inflammatory Meningitis Arachnoiditis - Encephalitis - Myelitis - Encephalomyelitis Acute disseminated - Tropical spastic paraparesis - Cavernous sinus thrombosis Systemic atrophies Huntington's disease - Spinocerebellar ataxia Friedreich's ataxia, Ataxia telangiectasia, Herary spastic paraplegia - Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio-Londe - MND ALS, PMA, PBP, PP, PLS Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia/Dyskinesia Spasmodic torticollis, Meige's, Blepharospasm - Essential tremor - Myoclonus - Lafora - Chorea Choreoathetosis - Restless legs - Stiff person Other degenerative/ demyelinating diseases dementia: Alzheimer's - Pick's - Dementia with Lewy bodies - Frontotemporal lobar degeneration mitochondrial disease: Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis - Marchiafava-Bignami disease - CAMFAK syndrome - Alpers' Seizure/epilepsy Focal - Generalised - Status epilepticus - Myoclonic epilepsy Headache Migraine Familial hemiplegic - Cluster - Vascular - Tension Vascular Transient ischemic attack Amaurosis fugax, Transient global amnesia Cerebrovascular disease MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke Sleep disorders Insomnia - Hypersomnia - Sleep apnea Obstructive, Ondine's curse - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase Intracranial hypertension Hydrocephalus Normal pressure - Idiopathic intracranial hypertension Other encephalopathy Brain herniation - Cerebral edema - Reye's Other spinal cord disease Syringomyelia - Syringobulbia - Morvan's syndrome - Spinal cord compression Retrieved from http://en..org/wiki/Dementia_with_Lewy_bodies Categories: Neurological disorders | Cognitive disordersHidden category: Articles needing additional references from May 2008 Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Nederlands Polski Português Suomi This page was last modified on 31 July 2008, at 08:39
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