LEWY BODY DEMENTIA

Hena Jawaid

PGY-III

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Also known as ….

Cortical lewy body disease Lewy body variant of Alzheimer’s

disease Diffuse lewy body disease senile dementia of Lewy type

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History Of Disease

Frederic Lewy (1885–1950) was first to discover the abnormal protein deposits ("Lewy body inclusions") in the mid 1900s after the discovery of alpha-synuclein staining that highlighted Lewy bodies in the cortex of post mortem brains of a subset of dementia patients

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LBD - hena jawaid

History (cont.)

Attention was drawn to DLB following the 2008 death of actress Estelle Getty, who had previously been diagnosed with both Parkinson's and Alzheimer's diseases before her true condition was discovered. Fellow Golden Girls cast members noted, years earlier, Getty had severe trouble remembering her lines during the filming of the show.

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Anatomy

Presence of lewy body in cerebral cortex , also seen in Substantia nigra.

The key protein is Alpha subnuclein (proteinicous cytoplasmic inclusions and Ubiquitin (regulatory protein that directs proteins to recycling).

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LBD - hena jawaid

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Anatomy (Cont.)

Neocortex – decreased choline transferase Hippocampal atrophy Severe nigrostriatal dopaminergic

degeneration occurs in DLB, but not in Alzheimer's disease or most other dementia subtypes*

Resource – McKeith I et al, Lancet Neurol. 2007 Apr. Sensitivity and specificity of dopamine transporter imaging with 123I-FP-CIT SPECT in dementia with Lewy bodies: a phase III, multicentre study.

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Anatomy (Recent Evidence)

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Because unmodified alpha-synuclein is degraded by the proteasome in a ubiquitin-independent manner, these data suggest that accumulation of modified alpha-synuclein is a disease-specific event leading to aberrant ubiquitination.

Resource- Tofaris GK et al, J Biol Chem. 2003 Nov

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Clinical Features - key

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Progressive cognitive decline – roads, maps and stairs

Cognitive fluctuations Formed and defined visual hallucination Motor features of Parkinsonism

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Clinical Features - Supporting

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Falls Syncope Adverse reaction to antipsychotics

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Clinical Features - Rare

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Cerebrovascular disease stroke

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Difference

Alzheimer’s1. Plaques and tangles2. Memory loss3. Normal moves4. No sleep with

association5. Hallucination in

advance stages only

Lewy Body1. Alpha – synuclein2. Decreased

attention3. Parkinsonism4. REM sleep behavior

disorder is a risk factor

5. Visual hallucinationLBD - hena jawaid

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LBD - hena jawaid

NON-PHARMACOLOGICAL INTERVENTIONS

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Ways To Manage LBD

Become informed. Learn as much as you can about LBD and how it is likely to affect your loved one specifically, given his or her health history, age, and lifestyle.

Create a routine. It may help people with Lewy Body

Dementia to have predictable routines, especially around meal times and sleep times.

Establish a night-time ritual. Try to establish bedtime rituals that are calming and away from the noise of television, meal cleanup, and active family members. Limit caffeine, discourage napping, and encourage exercise.

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Ways To Manage LBD

Modify tasks. Break tasks into easier steps and focus on success, not failure.

Walk together. Taking a walk with the patient with LBD is a win-win activity. Being outdoors and exercising is vital for the health and state of mind—of both of you.

Strengthen senses. Have a doctor evaluate each the patient’s five senses in order to identify and treat any abnormalities. Then ask about exercises to improve them.

Make behavioral changes. To help minimize the risk of fall-related injuries, you can help stabilize blood pressure. Help your loved one stay well hydrated, exercise, take in adequate sodium (salt), avoid prolonged bed rest, and stand up slowly.

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PHARMACOLOGICAL INTERVENTIONS

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Recent Evidences

There is limited evidence about specific interventions but available data suggest only a partial response of motor symptoms to levodopa: severe sensitivity to typical and atypical antipsychotics in approximately 50%, and improvements in attention, visual hallucinations, and sleep disorders with cholinesterase inhibitors.

Resource - McKeith IG, Neurology. 2005 Dec 27;65(12)

Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium.

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Pharmacological Management There is no cure for DLB. Acetylcholinesterase inhibitors, such as

donepezil and rivastigmine, are primarily used to treat the cognitive symptoms of DLB, but they may also be of some benefit in reducing the psychiatric and motor symptoms.

Some individuals with DLB may benefit from the use of levodopa for their rigidity and loss of spontaneous movement.

Resource -www.ninds.nih.gov/disorders/dementiawithlewybodies/dementiawithlewybodies.htm

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References

www.helpguide.org/elder/lewy_body_disease.htm

en.wikipedia.org/wiki/Dementia_with_Lewy_bodies

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THANK YOU