CDM Forum Vancouver Island Conference Centre March 24, 2010 Neurodegenerative Diseases
New Complex Care Fee: Neurodegenerative Diseases Compensates for the time and skill required Most responsible physician re: longitudinal, co-ordinated care Development of care plan
Neurodegenerative Diseases Range of neurodegenerative disorders is vast: Alzheimer s and other dementias Parkinson s ALS MS Stroke Other brain, spinal injuries Etc.
Neurodegenerative Diseases: This presentation discusses Role of physicians and actions required : Initial Diagnosis Longitudinal evaluations & management Availability & Role of other resources: Other healthcare professionals Specialist clinics Community support resources Internet resources
Memory Where would we be without it?
Dementia including Alzheimer s Disease Initial Diagnosis : Role of physicians and actions required Careful history including collateral Review of risk factors (DM, BP,family history) Exclude confounding factors depression, medications, hypothyroid, delirium Administer standardised test: MMSE, MoCA Physical exam; Consider treatable differential NPH (triad ataxia, bladder & cognitive difficulties); chronic subdural etc. and test accordingly Brain imaging Do they meet standard diagnostic criteria? Obtain Specialist opinion local or UBC if complicated
Dementia How do we diagnose it? 1. Multiple cognitive deficits, manifested by both: Memory impairment & One or more of: aphasia, apraxia, agnosia or impaired executive functioning Representing significant decline from previous level of function & producing substantial disturbance of social or occupational function 2. Gradual onset & progressive 3. Not occurring in the course of a delirium, or better accounted for by psychiatric disorder such as Depression or Schizophrenia 4. Development of Cognitive deficits not due to: other CNS disorders (CVA, NPH, Tumour, PD), systemic disorders (hypothyroid, HIV), medication use or Substance abuse e.g. alcohol
Dementia relative prevalence of common causes
AD & Dementias Management issues Early Stage: Develop a Care Plan To decide: 1. Frequency of visits 2.? ADTI eligible; if so GP or specialist?
ADTI.. Alzheimer's Drug Therapy Initiative Providing eligible B.C. residents with PharmaCare coverage of cholinesterase inhibitors used in the treatment of Alzheimer s disease and gathering evidence on the effectiveness of these medications.
MMSE LS mean change from baseline (±SE) DONEPEZIL IN MILD TO MODERATE AD Winblad et al, Neurology 2001, 57, 489-495 2.5 2 1.5 1 0.5 0-0.5-1 -1.5-2 -2.5-3 0 p=0.053 Donepezil Placebo 12 P<0.001 24 Study week p=0.019 36 p=0.001 52 P<0.001 Endpoint Clinical improvement donepezil n= 135 127 121 104 91 (135) placebo n= 137 128 120 105 98 (137) Baseline Clinical decline
AD & Dementias: Management issues Monitor & actively treat co-morbidities: BP DM Hyperlipidemias Monitor Cognitive decline MMSE 1-2x / year ADL
AD & Dementias: Management issues Patient/Care-giver monitoring: could include home BP recording, patient-identified target symptoms Assess Driving Enduring Power of Attorney Advance Directive Support network referral e.g. BC Alzheimers Society Initial information gathering patient, family Patient & caregiver support
AD & Dementias: Management issues 3 Red Flags indicating need for careful review: Cognitive decline speeds up Hallucinations/delusions Atypical behaviours develop Focal impairments become more prominent e.g. dysphasia, motor function,
AD & Dementias: Management issues Changing behaviour Consider the possibilities: Depression; Medication side-effect Stroke Fall with subdural hematoma Non-AD dementia diagnoses
Differentiating features: Fronto-temporal Dementia Variability in function - Withdrawal/apathy Loss inhibitions Lack insight Repetitive behaviours Decreased/repetitive speech Relative memory preservation
Differentiating features: Lewy body dementia Hallucinations & delusions Sleep disturbance Visio-spatial function impaired > AD Episodic memory less severely vs. AD MoCA more sensitive than MMSE Unsteady and slow gait - motor features Cognitive decline more rapid than AD Some response to Ach-ase inhibitors
Alzheimer's and neurodegenerative dementias Canadian Consensus Guidelines Recommendations from 3 rd. Canadian Consensus Conference on the Diagnosis and Treatment of Dementia, held in March 2006 Published as a series of articles in the Canadian Medical Association Journal, 2008 e.g. Diagnosis & Treatment of mild to moderate dementia, CMAJ 2008;179(8), 787-93 All articles are available as free full text through CMAJ at www.cmaj.ca
Alzheimer's and neurodegenerative dementias Internet and Community Resources BC Alzheimer Society 604-681-6530, toll-free 1-800-667-3742, email info@alzheimerbc.org. http://www.alzheimerbc.org/ UBC Alzheimer s and related Disorders Clinic http://ubc-alzclinic.vch.ca/ Pick s dementia: inc. fronto-temporal, Lewy Body dementia & corticobasal degeneration http://www.pdsg.org.uk/clinical_information/
Parkinson s Disease:Initial Diagnosis Role of physicians & actions required Consider PD with Tremor or slowness plus at least one other major feature on history/examination 1. Resting tremor (pill-rolling) 2. Bradykinesia 3. Cog-wheel rigidity 4. Impaired postural reflexes Refer for specialist opinion/recommendation in all cases
73 years old; PD x 9 years Wife complains that he is a very restless sleeper: Rx l-dopa CR 200/50 qid; ropinerole 1mg tid o/e mild tremor & rigidity; poor clock-face drawing 66% 34% 1. Lewy-body dementia 2. Restless legs syndrome 0% 0% 3. Dopamine induced dyssomnia 4. REM sleep disorder Lewy-body deme... Restless legs... Dopamine induc... REM sleep diso...
73 years old; PD x 9 years Wife complains that he is a very restless sleeper: What is the likeliest diagnosis? 4. REM sleep disorder
Parkinson s Disease: Management Plan Early Decisions need on: Frequency of follow-up with GP & specialist When to initiate Rx and which Rx : decide after input from the patient and specialist Later Decisions may include: Changing medication freq./dosage/type Cognitive evaluations (MoCA) Driving fitness (reaction speed, set shifting ) Role of surgery
Longitudinal evaluations: When your PD patient is not responding as expected to L-Dopa/agonist Rx Consider: 1. Is patient compliant? 2. Is timing of doses inappropriate? 3. is increasing tremor actually dopamineinduced dyskinesia? 4. Are patient/care-givers expectations realistic? 5. Is there something else going on? Depression is common in PD and spouse.
When your PD patient is not responding as expected to L-Dopa Rx, consider these Parkinson s variants : Progressive supranuclear palsy vertical gaze abnormalities, axial rigidity, falls during the early stages of disease, swallowing dysfunction, pseudobulbar palsy, dysarthria, cognitive impairment, rapid progression. (The Gait of the Dancing Bear) Multiple system atrophy Postural hypotension and autonomic dysfunction (Shy Drager variant), cerebellar dysfunction (OPCA), Striatonigral degeneration variant. Falls, swallowing dysfunction, dysarthria, rapid progression,
When your PD patient is not responding as expected to L- Dopa Rx, consider these Parkinson s variants : Diffuse Lewy body disease. Early dementia, hallucinations with L-dopa therapy, fluctuating level of alertness, sensitivity to extrapyramidal side effects of neuroleptics Corticobasal degeneration. unilateral presentation Apraxia, cortical sensory signs, myoclonus, dystonia, cognitive impairment, (Alien hand syndrome),
Parkinson s Disease: Management Resources Local neurologists Pacific Parkinson's Research Centre, University of British Columbia, Purdy Pavilion, 2221 Wesbrook Mall, Vancouver, BC, Canada V6T 2B5 Neurosurgery: Dr.Honey http://www.drhoney.org/ Local Mental Health Geriatricians, Psychogeriatrics OT, PT, families and friends Parkinson Society of BC www.parkinson.bc.ca Victoria Epilepsy and Parkinson's Disease Association www.vepc.bc.ca
ALS : Role of physicians and actions required in Initial Diagnosis If you are considering this Diagnosis: 1. Classical limb presentation UMN + LMN findings 2. Bulbar presentation in elderly may progress rapidly 3. Requires urgent neuro evaluation + EMG; MRI may be required but not so urgently to exclude other Contact a neurologist with EMG/Neurophysiology training by phone/fax to ask for an early appt. OR Contact Dr. Hannah R. Briemberg @ Neuromuscular Diseases Unit in Vancouver
ALS : Management plan Requires expertise & a co-ordinated approach; Confidence regarding the diagnosis Standardised management protocol, according to disease pattern and stage Respiratory assessment Advance directive Follow-up in Vancouver after initial assesment.
ALS : Management plan: Issues that will arise Counselling re: diagnosis, prognosis and need for advance decisions on intubation Vancouver ALS Clinic and ALS Society of BC Need for equipment: suction; meds. To reduce salivary secretions. Mobility aids; suitable bed; communication devices. Support Groups: http://alsbc.ca/ Also: support@alsbc Dr. Hannah R. Briemberg, Clinical Assistant Professor Neuromuscular Diseases Unit Tel: (604) 875-4247 Fax: (604) 875-4405 8th floor, Diamond Centre, 2775 Laurel Street, Vancouver BC Canada V5Z 1M9
MS: Role of physicians and actions required in Initial Diagnosis Considerations: MS the great mimic MRI high sensitivity; specificity? not so much ( multifocal areas of T2 hyperintensity are seen in the white matter; consider demyelinating disease, cerebrovascular ischemia, vasculitis or inflammatory white matter disease ) Disease modifying therapy effective with early diagnosis
2005 Revisions to the McDonald Diagnostic Criteria for MS. Ann Neurol 2005;58:840 846 The McDonald Criteria integrating MRI assessment with clinical and other paraclinical methods have been extensively assessed and used since 2001. New evidence and consensus strengthen the role of these criteria in the multiple sclerosis diagnostic workup to demonstrate: 1. dissemination of lesions in time, 2. clarify the use of spinal cord lesions, 3. simplify diagnosis of primary progressive disease.
The 2005 Revisions to the McDonald Diagnostic Criteria for Multiple Sclerosis : Additional Data Needed for MS Diagnosis 1. Two or more attacks; objective clinical evidence of two or more lesions 2. Two or more attacks; objective clinical evidence of one lesion & dissemination in space, demonstrated by: MRI or Two or more MRI-detected lesions consistent with MS, plus positive CSF or Await further clinical attack implicating a different site 3. One attack: objective clinical evidence of two or more lesions Dissemination in time, demonstrated by MRI or Second clinical attack 4. One attack; objective clinical evidence of one lesion (monosymptomatic presentation; clinically isolated syndrome)********* Dissemination in space, demonstrated by: MRI or Two or more MRI-detected lesions consistent with MS plus positive CSF and Dissemination in time, demonstrated by: MRI or Second clinical attack ETC ETC ETC
MS: Role of physicians and actions required in Initial Diagnosis Rush to order MRI may not be the best option. Expertise required for timely diagnosis & initiation of appropriate Rx Consider early referral to MS trained neurologist VIHA MS Clinic referral appropriate only after diagnosis made Charmaine Campbell RN MSCN
48 year old; MS diagnosed x 22 years In extended care; mobile in motorised wheelchair. c/o increasing generalised weakness x 3 weeks; less able to assist with transfers o/e Afebrile; catheterised; new finding sustained ankle clonus. 43% 26% 20% 1. Bladder infection 11% 2. Acute MS relapse 3. Cervical cord compression 4. Bilateral peroneal nerve palsies Bladder infect... Acute MS relap... Cervical cord... Bilateral pero...
48 year old; MS x 22 years c/o increasing generalised weakness x 3 weeks; Most likely diagnosis is: 1. Bladder infection
MS: Management issues; many issues arising throughout long and variable course Initial learning re: MS Education re: disease modifying therapies Detection and appropriate Rx of relapses Transition to progressive disease in some Symptomatic Rx s Cognitive issues Mood issues Mobility issues
MS: Acute symptomatic deterioration: acute relapse or something else? Acute relapse acute worsening of function lasting at least 24 hours, usually several days or weeks, then improvement that lasts at least 1 month Can be entirely new neurologic sx or prior symptom/dysfunction recurring. Probably not a relapse Symptoms of minutes to hours in duration Aggravation of preexisting sx in presence of acute infection, increased body temp. Aggravation preexisting spinal sx in presence focal irritation e.g. pressure ulcers, constipation, Symptoms/signs that don t improve with time.
MS: Resources UBC trained Neurologists VIHA MS Clinic, Royal Jubilee Hospital (Charmaine Campbell next up) North Island MS support: Judy Spencer, Community Services Coordinator at (250) 286-0999 in Campbell River or toll free at 1-888-299-2025 Central Island MS support; Nanaimo, 1-888-844-2047 info.centralisland@mssociety.ca Multiple Sclerosis Society Of Canada
Cerebrovascular Disease; other brain injuries, etc : Role of physicians and actions required Initial assessment & follow-up: On a case-by-case basis Cause, nature, extent and risk factors all need to be determined Neurology opinion will probably be needed initially Care plan, follow-up frequency and need for medical and community supports according to nature and extent of disability
Many Neurodegenerative disorders 1. Alzheimer s and other dementias 2. Parkinson s 3. ALS 4. MS 5. Stroke, Other brain, spinal injuries 6. Etc. Chronic seizure disorder, Down s Syndrome, low grade Gliomas/other tumors Role of physicians & actions required in initial Diagnosis & continuing Management. Other Resources specialists & clinics Other medical resources Community support resources Internet resources
THE END
Mild cognitive impairment criteria Memory complaint, preferably corroborated by an informant Objective memory impairment Normal general cognitive function Intact activities of daily living Not demented