LEWY BODY DISEASE. Dr Kathryn Nicholson Dip Physio, Grad Cert Ergo, Grad Dip T&D, M Ass&Eval, PhD

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1 LEWY BODY DISEASE Dr Kathryn Nicholson Dip Physio, Grad Cert Ergo, Grad Dip T&D, M Ass&Eval, PhD

2 LEARNING OBJECTIVES Following the session today you will have increased awareness of ; Lewy bodies & the DLB PD PPD conundrum The general presentation of LBD Communication Challenges in care

3 PRIMARY REFERENCES O Brien, McKeith, Ames, Chiu (2006) Eds Dementia with Lewy Bodies and Parkinson s Disease Dementia. London Taylor & Francis Nicholson K. A. (2010) Dementia with Lewy bodies: The Caring Experience. PhD Thesis University of Melbourne.

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5 WHAT IS A LEWY BODY? Named after FH Lewy who first described them in 1912 Spherical intraneuronal cytoplasmic inclusions within selectively vulnerable neurons the main component being alpha synuclein (α synuclein) a protein found in the normal brain. Affect both cholinergic and dopaminergic transmitter systems Found in cortex, limbic structures, diencephalon & brain stem

6 LOCATION OF LEWY BODIES Nolte J (2002) The Human Brain Mosby Inc

7 PROGRESSIVE NEUROCOGNITIVE DISORDERS (NCD) DSM Alzheimer's Disease Vascular NCD Frontotemporal NCD Lewy Body Dementia Parkinson's Disease HIV Infection Substance Induced NCD Huntington's Disease Prion Disease (CJD) Other medical conditions & not elsewhere classified

8 LEWY BODY DISORDERS A spectrum Dementia with Lewy bodies Parkinson s disease Parkinson s disease dementia

9 PD, PDD & DLB If dementia occurs within 12 months of the onset of extrapyramidal symptoms, diagnosis = (possible) DLB If the clinical history of parkinsonism is longer than 12 months before onset of cognitive decline, PDD will be the more appropriate diagnostic label DLB & PPD may be the same

10 WHY THE CONFUSION? 1817 James Parkinson s essay on Shaking Palsy 1912 Lewy described PD pathology 1915 Subsequently named Lewy bodies 1961 First report of cortical LB s in dementia 1990s Alpha synuclein immunocyto-chemistry led to the ability to stain Lewy bodies 1995 Dementia with Lewy Bodies 1 st Consortium on DLB (McKeith et al.) nd CDLB 2000s ongoing research rd CDLB

11 ADDING TO THE CONFUSION EXTRACT FROM Menza, M.D et al Citalopram Treatment of Depression in Parkinson's Disease: The Impact on Anxiety, Disability, and Cognition J Neuropsychiatry Clin Neurosci 16: , August 2004 Parkinson's disease is associated with subtle but widespread cognitive impairment, even in the absence of clinically apparent cognitive decline. Dementia, typically of a subcortical type, also occurs frequently in PD patients..the patients in our study were not significantly impaired at baseline (MMSE=29.1), so we were not able to test the hypothesis that treatment of depression can improve cognitive impairment in these patients.

12 Frontotemporal Dementia (FTD) Movement (Parkinson s Plus) FTDParkinson-17 (mutations in the MAPT microtubule-associated protein tau) gene on chromosome 17 autosomal dominant rare 100 families worldwide CBS corticobasal syndrome PSP progressive supranuclear palsy MSA

13 GENERAL PRESENTATION A person with a Lewy body disorder can present with problems in the following domains: Cognitive Physical Neuropsychiatric Autonomic

14 REASONS FOR ASSESSMENT? Dementia something is just not right? Parkinson s disease Protracted Stigma associated with mental illness Capacity of POC to cover Autonomy Relationships

15 INFORMANT CONCERNS Behaviour characterised by attentional instability and apathy Memory loss often not the presenting complaint in a muddle concentration problems odd behaviours vivid nightmares

16 DEMENTIA MEMORY LOSS Immediate memory Short term or working memory (AD) Declarative or explicit memory Episodic history memory unique (AD) Semantic cultural memory knowledge (FTD) Procedural or implicit memory (LBD) Habits Automatic behaviours

17 Nicholson K A (2010) PhD Thesis

18 MR ROBIN GROVE

19 MR ROBIN GROVES

20 LBD DEMENTIA - Subcortical defined in DLB criteria as progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function. Implicit memory loss Executive dysfunction Attentional deficits - apathy Drive reward dysfunction Visuo perceptual / constructional problems

21 WHAT HAPPENS AT A ROUNDABOUT? drivinghelp.com

22 CARERS VOICES I don t know what happened but he refused to take the kids in the car 6 years before he was diagnosed He got halfway there and wanted to come home because he just felt he was a danger to himself, he said, and everybody else. He was very angry (when I said I wouldn t get in the car with him) and he went to bed. Next morning he came out and said you are right He said it was a relief not to drive.

23 VISUO-SPATIAL DEFICITS MMSE (FOLSTEIN et al)

24 OTHER FEATURES Fluctuating cognition Visual hallucinations Parkinsonism REM sleep behaviour disorder Autonomic dysfunction

25 AUTONOMIC DYSFUNCTION Orthostatic hypotension Syncope & falls Impotence Urinary dysfunction (often nocturnal) Constipation Temperature dysfunction Cardiac arrhythmias

26 DISCUSSION POINTS

27 MANAGEMENT Person centered care Advance care planning Ongoing medical management Cholinesterase inhibitors should always be offered PD meds may not be as effective as expected Neuroleptic sensitivity is a major concern

28 COMMUNICATION & LBD People with LBD usually have Insight into their situation The ability to rationalise A knowledge of current affairs & the world around them Connectedness to their family & friends HOWEVER

29 COMMUNICATION & LBD People with LBD may Need extra time to think & respond Be overwhelmed in a noisy or crowded environment Talk gibberish on a bad day because of their fluctuations Speak very softly because of loss of pharyngeal mobility Lose speech because of motor impairment Be unable to interpret gestural communication

30 COMMUNICATION & LBD ALWAYS assume that a person with LBD Can respond appropriately if given time Can understand what is being said to them or about them Is competent and involve them in decision making

31 BPSDs & LBD Most are related to: The site of the pathology in the brain Fluctuations Insight Is the CAUsED model as relevant for LBD as it is for AD?

32 RESPITE & LBD Family carers struggle to accept respite In home respite is a challenge for both carer an POC Often the activities at day respite are inappropriate or the environment is too stressful RACF respite can resulted in addition carer burden

33 PALLIATIVE CARE Chronic illness in the elderly typically follows one of the three trajectories. Source: Lynn & Adamson (2003)

34 THE LAST DAYS People with LBD may Know they are dying Have fluctuating periods ranging from being lucid to unconsciousness Acknowledge presence of family and friends Be unable to eat and drink Be unable to control their body temperature Understand what is being said

35 PREVALENCE PD is already cited as the 2 nd most prevalent degenerative disease of ageing DLB is now cited as the 2 nd most prevalent dementia in the ageing population accounting for about 1 in 5 cases It is not the same as AD and we need to understand the differences People with this disease & their carers need our support

36 $$ First study to investigate comparisons of the cost of care between AD and DLB was undertaken in Sweden in 2007 by Boström, Jönsson, Minthon, & Londos (Intern J of Geriatric Psychiatry) The overall DLB costs of care (45,800 USD) were twice the costs of AD care (22,200 USD). Costs of care in DLB patients with apathy was almost three times as high as in AD patients with apathy

37 A PERSON WITH LBD MAY Remain in the world & connected Need time, space & one on one person centred care Find respite inappropriate and challenging With time have increased falls, more autonomic dysfunction, swallowing problems, and require intense palliative care.

38 ALZHEIMER S AUSTRALIA Lewy Body Disease Resource Further information kathryn.nicholson@ihsipl.com.au Lewy Body Dementia Association USA (2008) Lewy Body Society UK (2010) lewybody.org

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