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lewy_body_dementia of psycitarit disease
- 1. Dementia with Lewy Bodies Leonard Griffiths Thursday 22nd Nov 07
- 2. Case: Mrs ME 86♀ Admitted 2/10/07 with 2/12 Hx worsening mobility • Leg weakness • No altered sensation of pain Fall noted 6/52 prior to admission • ‘mechanical sounding’ • No LOC/HI/CP/palpitations/SOB • Recalled incident
- 3. Case: Mrs ME Intermittent confusion for 5/52 prior to admission • Treated for UTI (although no urinary signs or symptoms)
- 4. Case: Mrs ME PMH • ‘Registered blind’ • Osteoporosis • Angina • Hypertension • Asthma • Episode of jaundice 40 yrs ago
- 5. PTWR Diagnosed ‘weakness of legs’ • ?musuloskeletal • ?UTI
- 6. Abnormal results WBC 11.5; Neut 8.2 Na+ 128 • U Osmo 566 • U Na+ <20 • P Osmo 282 • P Na+ 132 • not SIADH
- 7. Abnormal results Urine dip • Blood + • Leuk +++ • Protein + • Nitrites + Lab +ve for nitrites and leukocytes • Culture ‘mixed growth suggesting contam.’
- 8. Abnormal results AMSE • 8/10 • MMSE 20/28 • GDS 3/15 • i.e. not depressed CT brain • Moderate small vessel disease • Small L occipital infarct
- 9. SHO WR 6/10/07 Thought pt had low mood d/w consultant ref to Ψ liaison • Felt that ‘new environment and poor eyesight a significant factor in agitation’
- 10. Consultant WR 9/10/07 Cogwheeling Tremor Short shuffling gait ‘Leans backwards’ • Impression: Parkinsonism • 1/52 domperidone commenced • Ref. to PD consultant
- 11. SHO WR 11/10/07 Hallucinating • “Horse woman of the year” • Believed someone gave her eye drops (not prescribed)
- 12. PD consultant review “Tricky mixed picture which is difficult to disentangle” • Hallucinations • Although ?Charles Bonnet in relation to reduced eyesight • Some reduced memory • ?DLB but has cerebrovascular disease • Parkinsonism – but not typical PD • ‘tremor rather fine’ • ‘balance back’ Try madopar, ‘but suspect little to be gained from medication’
- 13. SHO WR 15/10/07 Hallucinating • Relatives • Monkey
- 14. Progress… 18/10/07 madopar commenced 22/10/07 more confused and hallucinations worsening madopar stopped & rivastigmine commenced 30/10/07 – paranoia and aggressive
- 15. Progress… 6/11/07 – hallucinations worse at night quetiapine added 16/11/07 – mood even lower venlafaxine added Now awaiting community hospital bed
- 16. Diagnosis Clinical features reflect anatomical distribution of pathology rather than its nature Therefore subtle clinical features not helpful
- 17. What is Dementia? ICD-10 definition 1. A decline in memory to an extent that it interferes with everyday activities, or makes independent living either difficult or impossible. 2. A decline in thinking, planning and organizing day-to-day things, again to the above extent. 3. Initially, preserved awareness of the environment, including orientation in space and time. 4. A decline in emotional control or motivation, or a change in social behaviour, as shown in one or more of the following: emotional lability, irritability, apathy or coarsening of social behaviour, as in eating, dressing and interacting with others. • Maj, M.. 2002., Dementia, Second Edition [online]. 2nd Edition. Wiley. Available from: http://mil.ingramdigital.com/Browse/open.asp?ID=10147&loc=Cover 15 November 2007
- 18. Wikipedia definition Dementia is the progressive decline in cognitive function due to damage or disease in the brain beyond what might be expected from normal ageing. • http://en.wikipedia.org/wiki/Dementia
- 20. Dementia Alzheimer's Vascular Lewy body Alcohol Pure vascular Frontotemporal lobar degenerations Creutzfeldt-Jakob disease Dementia pugilistica Moyamoya disease
- 21. Nomenclature Diffuse Lewy body Disease Cortical Lewy body Disease Lewy Body Dementia Senile Dementia Of Lewy Type Lewy Body Variant of Alzheimer's Disease Dementia with Lewy Bodies (preferred)
- 22. Dementia with Lewy Bodies Describes several common disorders causing dementia The main features of these conditions are: • development of dementia with features overlapping with those of Alzheimer's disease • development of features of Parkinson's disease • fluctuation in severity of condition on a day-to-day basis • early development of hallucinations
- 23. Neuropathology Degeneration of substantia nigra Degeneration of the cortical areas of the brain with many or all of the features seen in Alzheimer's disease Remaining nerve cells contain abnormal structures called ‘Lewy bodies’
- 24. Lewy Bodies Abnormal aggregation of proteins, including • alpha-synuclein, neurofilament and ubiquitin • PD • Dementia with Lewy bodies • MSA • Amyotrophic lateral sclerosis • Hallervorden-Spatz syndrome Core, body, halo Variations in shape Synucleopathies
- 28. Tonus und Bewegund (Muscle Tone and Movement)
- 31. Alpha-synuclein Abundant CNS protein Composed of 140 amino acids Alpha form of synuclein is the only form capable of aggregating into fibrillar structures in vitro Beta-synuclein is not localized in Lewy bodies, it may have a role in regulating alpha-synuclein metabolism or aggregation
- 33. Normal role of alpha-synuclein Synaptic plasticity Negative regulation of dopamine neurotransmission Protection at nerve terminals during injury Trafficking of cargo in the ER/Golgi complex
- 34. Alpha-synuclein in disease ‘Ubiquitinated’ with no loss of proteasome function, suggesting there is an excessive accumulation of alpha- synuclein that overwhelms the proteolytic machinery (Tofaris, et al. 2003). This may promote the formation of Lewy bodies
- 35. Presenting Features DLB Dementia normally presenting feature Minority present with parkinsonism Some with psychiatric disorder without dementia Others with orthostatic hypotension, falls or transient disturbances of consciousness Sporadic (rarely familial)
- 36. Common Features Fluctuation in cognitive performance and functional ability Variations in attention and level of consciousness Visual hallucinations in two-thirds
- 37. Gelder, Michael G.; Lopez-Ibor, Juan Jose; Andreasen, Nancy C.. 2003., New Oxford Textbook of Psychiatry, Volume 1 [online]. New Edition. Oxford University Press. Available from: http://www.myilibrary.com/Browse/open.asp?ID=14714&loc=416 15 November 2007
- 38. Sensitive signs for ‘bedside’ diagnosis Psychiatric vs. cognitive symptoms • Hallucinations • Delusions Fluctuant cognitive state
- 39. Hallucinations Repeated visual hallucinations are present in about two-thirds of patients • vivid, colourful, and sometimes fragmented figures of people and animals • often complex, detailed and rapidly moving • can involve scenes and bizarre situations • can start with misinterpretations and are usually short • often occur at night • usually not distressing to the patient
- 40. Treatment No cure Cognitive symptoms acetylcholinesterase inhibitors, such as donepezil and rivastigmine • May reduce psychiatric and motor symptoms Rigidity levodopa
- 41. Summary Third most common dementia Central feature is progressive cognitive decline • Pronounced fluctuations • Recurrent visual hallucinations • Parkinsonism Symptoms caused by Lewy Bodies comprised of bits of alpha-synuclein
Editor's Notes
- PATHOLOGICAL FEATURES Several key areas of the brain undergo degeneration in this form of disease There is degeneration of an area in the brain stem called the substantia nigra as would be seen in Parkinson's disease. Normally the substantia nigra is populated by nerve cells which contain a dark-brown pigment called neuromelanin. The cells of the substantia nigra are responsible for making the neurotransmitter dopamine. In both Parkinson's disease and Lewy body dementia these cells die and so the substantia nigra appears abnormally pale in comparison to normal. Remaining nerve cells contain abnormal structures called Lewy bodies. which are a pathological hallmark of the disease process. Degeneration of the cortical areas of the brain with many or all of the features seen in Alzheimer's disease Degeneration of the cortical areas of the brain with formation of abnormal structures inside nerve cells called cortical Lewy bodies which can be detected by immunochemical staining for the protein ubiquitin. N
- Dementia is usually, but not always, the presenting feature; a minority of patients present with parkinsonism alone, some with psychiatric disorder in the absence of dementia, and others with orthostatic hypotension, falls, or transient disturbances of consciousness. Fluctuation in cognitive performance and functional ability, which is based in variations in attention and level of consciousness, is the most characteristic feature of DLB It is usually evident on a day-to-day basis, and often apparent within much shorter periods. The marked amplitude between best and worst performance distinguishes it from the minor day-to-day variations that commonly occur in dementia of any aetiology. Repeated visual hallucinations are present in about two-thirds of patients. They take the form of vivid, colourful, and sometimes fragmented figures of people and animals,
- Dementia with Lewy bodies (DLB) is one of the most common types of progressive dementia. The central feature of DLB is progressive cognitive decline, combined with three additional defining features: (1) pronounced “fluctuations” in alertness and attention, such as frequent drowsiness, lethargy, lengthy periods of time spent staring into space, or disorganized speech; (2) recurrent visual hallucinations, and (3) parkinsonian motor symptoms, such as rigidity and the loss of spontaneous movement. People may also suffer from depression. The symptoms of DLB are caused by the build-up of Lewy bodies – accumulated bits of alpha-synuclein protein -- inside the nuclei of neurons in areas of the brain that control particular aspects of memory and motor control. Researchers don’t know exactly why alpha-synuclein accumulates into Lewy bodies or how Lewy bodies cause the symptoms of DLB, but they do know that alpha-synuclein accumulation is also linked to Parkinson's disease, multiple system atrophy, and several other disorders, which are referred to as the "synucleinopathies." The similarity of symptoms between DLB and Parkinson’s disease, and between DLB and Alzheimer’s disease, can often make it difficult for a doctor to make a definitive diagnosis. In addition, Lewy bodies are often also found in the brains of people with Parkinson's and Alzheimer’s diseases. These findings suggest that either DLB is related to these other causes of dementia or that an individual can have both diseases at the same time. DLB usually occurs sporadically, in people with no known family history of the disease. However, rare familial cases have occasionally been reported.