III. Epidemiology

  1. Accounts for 20% of Dementia cases
    1. Second most common cause
  2. Mean age of onset: 75 to 80 years old

IV. Pathophysiology

  1. Lewy body and Lewy neurites
    1. Eosinophilic cytoplasmic inclusions (alpha-synuclein)
      1. Parkinsonism: Substantia Nigra, locus ceruleus
      2. Lewy Body Dementia: Frontotemporal cortex
  2. Other changes specific to Lewy Body Dementia
    1. Basal forebrain degeneration (CholinergicNeurons)
    2. Nigra degeneration (Dopaminergic Neurons)
    3. Serotonergic Neurons also affected
  3. Comparison with Alzheimer's Disease changes
    1. Amyloid Plaques in Alzheimer's and Lewy Body Dementia
    2. Neurofibrillary tangles seen in Alzheimer's only

V. Clinical features

  1. Dementia (cognitive function decline)
  2. Core features (2 features required for probable case)
    1. Recurrent Visual Hallucinations
    2. Fluctuating cognition, alertness and attention
      1. Normal performance in stimulating activity
      2. Transient periods of "blankness"
    3. Parkinsonism
      1. Rigidity, Bradykinesia and altered gait are common
      2. Mask-like faces are also seen
      3. Resting Tremor less common than Parkinson's Disease
  3. Manifesting signs and symptoms
    1. Syncope
    2. Recurrent falls
    3. Transient loss of consciousness
    4. Delusions
    5. Major Depression
    6. REM Sleep disorder
      1. Associated with Nightmares of being chased

VI. Differential Diagnosis

  1. See Dementia
  2. Alzheimer's Disease differentiating features
    1. Memory loss is more prominent than in DLB
    2. Visual Hallucinations less common in Alzheimer's
    3. Parkinsonism uncommon in Alzheimer's Disease
    4. Minimal cortical atrophy in Lewy Body Dementia
    5. Alzheimer's: MRI involves hippocampus, Temporal Lobe

VII. Diagnostic Testing

  1. Rule-out other cause
    1. See Dementia for diagnostic testing
  2. Mini-Mental State Exam (MMSE) findings
    1. Difficult attention and construction
    2. Difficult clock drawing
    3. Memory appears unaffected in early DLB

VIII. Management

  1. See Dementia Management
  2. Avoid Anticholinergic Medications
  3. Cholinesterase Inhibitors
    1. Effects
      1. Improve apathy and anxiety
      2. Reduce Hallucinations and Delusions
      3. Improve cognition
    2. Agents
      1. Rivastigmine (Exelon)
      2. Donepezil (Aricept)
      3. Galantamine (Reminyl)
  4. Antiparkinsonism Medications
    1. Levodopa-Carbidopa (Sinemet)
      1. Start with lowest dose of a single agent
      2. Goal: Improve mobility without inducing Psychosis
  5. REM Sleep Behavior Disorder
    1. Clonazepam 0.25 to 1 mg orally at bedtime
  6. Orthostatic Hypotension
    1. Fluids and increase Sodium in diet
    2. Rise from lying or sitting slowly
    3. Avoid prolonged bed rest
  7. Anti-Psychotic medications (Neuroleptics)
    1. Cholinesterase Inhibitors may reduce Psychosis
    2. High sensitivity to Neuroleptics in DLB
      1. May severely exacerbate Parkinsonism symptoms
        1. Rigidity
        2. Sedation
      2. Effects on Parkinsonism may be irreversible
      3. Effects may be life-threatening
        1. Example: Neuroleptic Malignant Syndrome
      4. Use Neuroleptics with caution and Informed Consent
    3. Agents
      1. Risperidone 0.25 mg PO bid (maximum 1 mg bid)
      2. Olanzapine 2.5 mg PO daily (maximum 10 mg daily)
      3. Quetiapine 25 mg PO bid (maximum 150 mg/day)
    4. Contraindicated agents: Older, type 2 Antipsychotics
      1. Avoid Haloperidol, fluphenazine, chlorpromazine

IX. Resources

  1. Lewy Body Dementia Association
    1. http://www.lewybodydisease.org/

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Ontology: Lewy Body Disease (C0752347)

Definition (MEDLINEPLUS)

Lewy body disease is one of the most common causes of dementia in the elderly. Dementia is the loss of mental functions severe enough to affect normal activities and relationships. Lewy body disease happens when abnormal structures, called Lewy bodies, build up in areas of the brain. The disease may cause a wide range of symptoms, including

  • Changes in alertness and attention
  • Hallucinations
  • Problems with movement and posture
  • Muscle stiffness
  • Confusion
  • Loss of memory

Lewy body disease can be hard to diagnose, because Parkinson's disease and Alzheimer's disease cause similar symptoms. Scientists think that Lewy body disease might be related to these diseases, or that they sometimes happen together.

Lewy body disease usually begins between the ages of 50 and 85. The disease gets worse over time. There is no cure. Treatment focuses on drugs to help symptoms.

NIH: National Institute of Neurological Disorders and Stroke

Definition (NCI) A progressive form of dementia characterized by the presence of protein deposits called Lewy bodies in the midbrain and cerebral cortex, and loss of cholinergic and dopaminergic neurons. The signs and symptoms overlap with Alzheimer and Parkinson disease.
Definition (MSH) A neurodegenerative disease characterized by dementia, mild parkinsonism, and fluctuations in attention and alertness. The neuropsychiatric manifestations tend to precede the onset of bradykinesia, MUSCLE RIGIDITY, and other extrapyramidal signs. DELUSIONS and visual HALLUCINATIONS are relatively frequent in this condition. Histologic examination reveals LEWY BODIES in the CEREBRAL CORTEX and BRAIN STEM. SENILE PLAQUES and other pathologic features characteristic of ALZHEIMER DISEASE may also be present. (From Neurology 1997;48:376-380; Neurology 1996;47:1113-1124)
Definition (PSY) Neurodegenerative disease marked by the presence of Lewy body cells in the cerebral cortex and brain stem. Symptoms often include dementia, parkinsonianism, and striking fluctuations in cognitive performance.
Concepts Disease or Syndrome (T047)
MSH D020961
ICD9 331.82
ICD10 G31.83
SnomedCT 230277007, 192808003, 230276003, 230275004, 80098002, 312991009
English Diffuse Lewy Body Disease, Lewy Body Disease, Diffuse, CLBD - Cortical Lewy body dis, DLBD - Diffuse Lewy body dis, SDLT - Sen dement Lewy bdy typ, Senile dement of Lewy bdy type, LEWY BODY DEMENTIA, DIFFUSE LEWY BODY DISEASE, DEMENTIA, LEWY BODY, DLB, LEWY BODY DIS, LEWY BODY DIS CORTICAL, LEWY BODY DIS DIFFUSE, DIFFUSE LEWY BODY DIS, CORTICAL LEWY BODY DIS, dementia with Lewy bodies, dementia with Lewy bodies (diagnosis), Dementia with Lewy bodies, Cortical Lewy Body Disease, Lewy Body Disease, Cortical, Dementia, Lewy Body, Lewy Body Type Senile Dementia, Lewy Body Disease, Dementia w Lewy bodies, Lewy body disease, Lewy Body Disease [Disease/Finding], bodies dementia lewy, body lewy dementia, bodies dementias lewy, body dementia lewis, body dementia lewy, bodies disease lewy, body dementia lewi, body disease lewy, body diseases lewy, body disease lewi, body disease lewis, diffuse lewy body disease, lewy bodies dementia, dementia lewy bodies, dementia with lewy bodies, lewy body dementia, lewy body disease, Dementia with lewy bodies, Lewy Body Dementia, Lewy body disease (disorder), senile dementia with Lewy bodies, senile dementia with Lewy bodies (diagnosis), dementia with lewy bodies senile, CLBD - Cortical Lewy body disease, Cortical Lewy body disease, DLBD - Diffuse Lewy body disease, Diffuse Lewy body disease, LBD - Lewy body disease, SDLT - Senile dementia of the Lewy body type, Senile dementia of the Lewy body type, Lewy body variant of Alzheimer's disease, Dementia of the Lewy body type, Lewy body dementia, Diffuse Lewy body disease (disorder), Senile dementia of the Lewy body type (disorder), Dementia with Lewy Bodies
Swedish Lewykropp-sjukdom
Czech difuzní nemoc Lewy body, demence Lewy body, Nemoc s difuzními Lewyho tělísky, Demence s Lewyho tělísky, demence s Lewyho tělísky, nemoc Lewy body
Spanish Enfermedad Difusa por Cuerpos de Lewy, Demencia con cuerpos de Lewys, Demencia por cuerpos de Lewy, Enfermedad por Cuerpos de Lewy Difusos, demencia senil tipo cuerpos de Lewy (trastorno), demencia senil tipo cuerpos de Lewy, enfermedad difusa de cuerpos de Lewy (trastorno), enfermedad difusa de cuerpos de Lewy, variante de la enfermedad de Alzheimer con cuerpos de Lewy, Demencia con Cuerpos de Lewy, Enfermedad por Cuerpos de Lewy
Finnish Lewynkappaledementia
Russian LEVI TELETS NALICHIIA BOLEZN', LEVI TELETS NALICHIIA BOLEZN' DIFFUZNAIA, LEVI TELETS NALICHIIA DEMENTSIIA, DEMENTSIIA PRI NALICHII LEVI TELETS, ДЕМЕНЦИЯ ПРИ НАЛИЧИИ ЛЕВИ ТЕЛЕЦ, ЛЕВИ ТЕЛЕЦ НАЛИЧИЯ БОЛЕЗНЬ, ЛЕВИ ТЕЛЕЦ НАЛИЧИЯ БОЛЕЗНЬ ДИФФУЗНАЯ, ЛЕВИ ТЕЛЕЦ НАЛИЧИЯ ДЕМЕНЦИЯ
Italian Malattia diffusa a corpi di Lewy, Demenza con corpi di Lewy, Malattia dei corpi di Lewy
French Maladie à corps de Lewy diffuse, Démence sénile de type corps de Lewy, Démence à corps de Lewy, Maladie à corps de Lewy
Dutch diffuse Lewy-lichaampjes-aandoening, dementie met Lewy-lichaampjes, Dementie, Lewy-lichaampjes, Diffuse Lewy-lichaampjes-ziekte, Lewy-lichaampjes-ziekte
German Lewy-Koerper-Demenz, diffuse Lewy-body-Krankheit, Diffuse Lewy-Korpuskel-Krankheit, Lewy-Korpuskel-Krankheit, Demenz, Lewy-Korpuskel-
Portuguese Doença difusa de corpos de Lewy, Demência com corpos de Lewy, Doenças por Corpos de Lewy Difusos, Demência com Corpos de Lewy, Demência por Corpos de Lewy, Doença Difusa por Corpos de Lewy, Doença por Corpos de Lewy
Japanese びまん性レヴィ小体病, ビマンセイレヴィショウタイビョウ, レヴィ小体型認知症, レヴィショウタイガタニンチショウ, Lewy小体病, びまん性Lewy小体病, びまん性リューイ小体疾患, びまん性レビー小体疾患, びまん性レビー小体病, びまん性レビ小体疾患, びまん性レヴィー小体疾患, びまん性レヴィ小体疾患, びまん性レーヴィ小体疾患, リューイ小体性痴呆, リューイ小体疾患-びまん性, リューイ小体疾患, レビー小体性痴呆, レビー小体疾患-びまん性, レビー小体疾患, レビー小体病, レビ小体性痴呆, レビ小体疾患-びまん性, レビ小体疾患, レヴィー小体性痴呆, レヴィー小体疾患-びまん性, レヴィー小体疾患, レヴィ小体性痴呆, レヴィ小体疾患-びまん性, レヴィ小体疾患, レーヴィ小体性痴呆, レーヴィ小体疾患-びまん性, レーヴィ小体疾患, 痴呆-リューイ小体性, 痴呆-レビー小体性, 痴呆-レビ小体性, 痴呆-レヴィー小体性, 痴呆-レヴィ小体性, 痴呆-レーヴィ小体性
Polish Choroba ciał Lewy'ego
Hungarian Diffúz Lewy-testes betegség, Lewy-testekkel kísért dementia
Norwegian Lewylegemedemens, Demens med lewylegeme, Sykdom med lewylegemer