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LEWY BODY DEMENTIA
Hena Jawaid
PGY-III
Also known as ….
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Cortical lewy body disease
Lewy body variant of Alzheimer’s disease
Diffuse lewy body disease
senile dementia of Lewy type
LBD - hena jawaid
History Of Disease
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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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History (cont.)
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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
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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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Anatomy (Cont.)
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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 alphasynuclein 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
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1.
2.
3.
4.
5.
Alzheimer’s
Plaques and tangles
Memory loss
Normal moves
No sleep with association
Hallucination in advance
stages only
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2.
3.
4.
5.
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Lewy Body
Alpha – synuclein
Decreased attention
Parkinsonism
REM sleep behavior
disorder is a risk factor
Visual hallucination
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NON-PHARMACOLOGICAL INTERVENTIONS
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Ways To Manage LBD
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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.
LBD - hena jawaid
Ways To Manage LBD
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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.
LBD - hena jawaid
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PHARMACOLOGICAL INTERVENTIONS
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Recent Evidences
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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.
LBD - hena jawaid
Pharmacological Management
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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
LBD - hena jawaid
References
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www.helpguide.org/elder/lewy_body_disease.htm
en.wikipedia.org/wiki/Dementia_with_Lewy_bodie
s
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THANK YOU
LBD - hena jawaid