degenerave neurological diseases · 2018-07-09 · degenerave neurological diseases a brief...

Degenera'veNeurologicalDiseases

ABriefOverviewofMul'pleSclerosis&Parkinson’sDisease

ReturntoWork&Rehabilita'onChallenges

AustralasianLifeUnderwri'ngandClaimsAssocia'onRehabilita'onMedicalSeminarApril192018

DrDavidRosen|PrinceofWalesPrivateHospital

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Outline • Neurodegenerative diseases • MS and PD

•  Definition •  Pathology •  Presentation •  Some key symptoms •  Therapy •  Clinical Course and prognosis

• Case studies to illustrate rehabilitation issues • Questions

Dr David Rosen | mlcoa • nsw | Prince of Wales Private Hospital

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PD, MS Rehabilitation and Work - Aims •  For each condition

•  Who gets it and when •  Why do they get it •  How is it diagnosed and treated •  What does it do to them over time symptoms •  What is the role of rehab and when to apply it

•  For any neurodegenerative disease decisions about work and rehabilitation rest on a complex interaction between the host, the environment and the disease

ALUCA - Thursday19 April 2018 Dr David Rosen | mlcoa • nsw | Prince of Wales Private Hospital

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Case Histories • Parkinson’s disease

•  The Electrician •  The Opera Singer •  The Psychiatrist & Musician

• Multiple Sclerosis •  The Journalist •  The Wool Trader


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The Electrician - History •  46 y/o left handed father of 4; Telstra electrician •  6 y h/o slowly progressive right upper limb tremor

•  Left side unaffected •  Attributed to work stress / dissatisfaction with Telstra •  Changed jobs back to work as an electrician

• Symptoms worse with stress; unchanged after 8 months; •  Unable to do things fast; tasks easier if he thinks rather than ‘auto-

pilot’ • Wife and workmates have noticed his tremor • History of mild anxiety • Denies other motor / non motor symptoms

•  Parasomnia, restless legs syndrome, constipation, difficulty with gait, balance, difficulty moving in bed, bulbar symptoms, cognition


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The Opera Singer - History •  64 year old left handed acclaimed Australian Opera Singer. •  18 m h/o impaired dexterity, balance, posture, sinus, bladder Sxs •  2 y h/o urinary problems

•  Urgency, enuresis, nocturia, poor flow, terminal dribbling, low volume, •  Previous few months, symptoms affecting movement, on left side.

•  Truncal stiffness; difficulty straightening up during rehearsals •  Balance problems; stooped posture during Tosca performances; aware that he

might topple into the orchestra when he moved suddenly or changed position. •  Occasional left hand and forearm tremor •  Trouble alighting from a deep settee and rolling in bed. •  Mild sialorrhea •  Longstanding hyposmia.

•  Voice husky •  Friends and family told him he is not moving well.


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The Psychiatrist & Musician - History • One year history

•  Subtle appreciation of right hand tremulousness •  Occasional twitches •  Difficulty writing - “becoming more cramped and more illegible and

difficult to complete medical records as easily”. •  Difficulty cello playing - executing quick alternating movements •  More recently gait difficulty

•  Decrease in the arm swing on the right •  A sense that the right leg is ‘dragging’ •  Gait changes noticed by others – unsurprising considering her job

•  Initially unsure as to the presence of these symptoms •  Attributed to recently diagnosed hypertension.

•  Father had Parkinson’s disease diagnosed at 60.


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The Journalist - History •  33 F Journalist; 1 month painful reduction in vision left eye • Arrived with MR scan

•  Results consistent with optic neuritis and white matter demyelination (did not quite reach criteria for MS diagnosis).

•  Three days IV methylprednisolone • Vision worse after a hot bath. Denies Lhermitte’s • Other symptoms

•  Left foot paraesthesia present for a few days, then resolved •  Mild unsteadiness on and off for the last couple of years •  Two years fatigue,

• PMH - anxiety, PTSD (stress as a Police reporter), tension-type headaches


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The Wool Trader – History & Examn • Presented aged 34; 5y history of non-specific symptoms

•  Visual disturbance, paraesthesia, fatigue, unsteady gait.

• Significantly exacerbated by exercise •  Ceased serious exercise two years previously

•  Intermittent symptoms; ?? periods of recovery •  No definite acute relapses

• Mildly anxious, slightly fatuous affect, thin body habitus • Mild left arm incoordination • Signs of mild paraparesis; possible sensory level at T10 • Mild left optic disc pallor, mildly decreased vision and

hearing on the left


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Neurodegenerative Diseases •  Progressive

•  Loss of structure or function of neurons, including death of neurons. •  Examples of neurodegenerative diseases include

•  Motor Neuron Disease or Amyotrophic Lateral Sclerosis •  Parkinson’s disease •  Alzheimer's disease •  Several genetically inherited conditions

•  Huntington’s disease (HD) Spinocerebellar ataxia (SCA) Spinal muscular atrophy (SMA) •  (Multiple sclerosis)

•  Common features •  Cause often unknown •  Often late onset •  Incurable (almost invariably) •  Progressive degeneration and/or death of neuron cells •  Progressive disability •  Common or overlapping sub-cellular pathology

•  atypical protein aggregates •  induced cell death


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Neurodegenerative diseases General Comments

• Effective rehabilitation / return to work strategies depend on •  Correct diagnosis •  Prognosis and time scale (tempo) of the condition •  Understanding the patient •  Effective symptom management / treatment •  Symptoms are subjective and often difficult to measure and treat

•  Pain and other sensory symptoms •  Sleep quality •  Fatigue •  Cognitive dysfunction •  Mood dysregulation •  Social dysfunction


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Prognosis in Neurodegenerative Disease • Diagnosis often determines prognosis •  Tempo / timescale of disease progression

•  Takes time to assess this

• Knowledge of the patient •  Co morbidity and past medical history •  Expectations – of the patient •  Social circumstances and support networks •  Education •  Personality and psychological factors •  Motivation


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Neurodegenerative Disease - Tempo • Uniphasic vs Multiphasic Illnesses

•  Rehabilitation & return to work expectations will vary •  Stroke – uniphasic; prognosis depends on initial severity •  Multiple sclerosis – often multiphasic; initial severity not a good

guide to medium term function; highly variable course •  Parkinson’s disease – insidious onset; fairly predictable course

• Similar levels of disability, very different prognosis • Progressive vs Static Conditions

•  Stroke – static disability at maximal medical improvement •  MS and PD – concept of MMI does not apply •  MS - disease modifying therapy alters natural history •  PD – symptomatic treatment only; variable individual course


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Neurodegenerative Disease - Tempo • Uniphasic uncomplicated with full recovery

•  e.g. mild TBI

• Uniphasic with no or incomplete recovery •  e.g. disabling stroke. Typically abrupt onset variable initial

impairment optimal recovery usually depends on medical and allied input

•  Well established and understood causes and natural hx


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Neurodegenerative disease - Tempo •  Neurodegenerative diseases are progressive

•  eg MND, AD, PD most cases of MS •  Insidious onset; relentless course over years •  Often cause is unknown •  Often no treatment to modify natural history BUT many effective

symptomatic treatments •  Work capacity depends on

•  Patient factors •  Type of work •  Type of disability •  Motivation

•  Objective measures of disease may not correlate with functional capacity

•  Many symptoms common to different neurodegenerative diseases •  Fatigue, dyscognitive and psychological symptoms


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Neurodegenerative disease - Rehabilitation • Philosophy of rehabilitation still applies

•  Restore optimal function •  Goal setting, learning, adapting, training •  Use of aids, need for home and workplace modifications •  Management of expectations

• Common Barriers to Optimal Function in NDD •  Disease pathology •  Neurological symptoms •  Neurological impairments •  Motivation •  Occupation •  Social and Psychological •  Access to treatment


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Onset and Progression of Neurological Disease


Different diseases, different time courses

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What is MS? •  An immune mediated disease of the central nervous system (CNS). An abnormal response of immune system is directed against the CNS (brain, spinal cord and optic nerves).

•  The exact antigen or target that the immune cells are sensitised to attack, remains unknown

•  Chronic neuro-inflammation and neuro-degeneration the pathomorphological hallmarks of the disease

•  MS is the leading cause of non-traumatic disability among young and middle-aged adults in the western world.

•  MS gives rise to a great variety of clinical symptoms, impairments and disabilities

MS is a Central Nervous System disease


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MS - Who gets it? • Prevalence

•  > 23,000 Australians living with MS •  Affects 1 in 1000 people •  Two to three times as many women as men •  Peak diagnosis: 20–40 years of age

• Genetic susceptibility: •  Caucasians •  First-degree relatives 3% •  Monozygotic twins 25%

• Cause •  Exact Cause Unknown •  Genetic & Environmental factors interact


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MS subtypes – Progression (Tempo) https://upload.wikimedia.org/wikipedia/commons/3/3c/Ms_pr...

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Secondary progressive multiple sclerosis Initial relapsing-remitting multiple sclerosis that declines without periods of remission. Most patients with RRMS progress to SPMS

Primary progressive multiple sclerosis (20%) Steady increase in disability without attacks from the outset.

Relapsing-remitting multiple sclerosis (80%) Unpredictable attacks which may or may not leave permanent deficits followed by periods of remission.

Benign MS < 5% - does it exist?

CIS – clinically isolated syndrome RIS – radiologically isolated syndrom


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Progression of MS Subtypes

06-Sep-16

7

MS: Inflammatory & Neurodegenerative

Clinical Threshold

MRI lesion activity

Number of lesions

Total lesion load (T2 lesion volume)

CIS RRMS SPMSPre-clinical

McDonaldMS

PoserCDMS

Axonal Degeneration

Natural History of MS

Brain Volume

Sydney Neuroimaging Analysis Centre 2013


Different diseases, different time courses Where is your patient on the curve?

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What are the historical characteristics associated with each subtype of MS?

•  Clinically isolated syndrome (CIS)

•  Full MS diagnostic criteria not met at 1st relapsing event

•  Relapsing-remitting MS (RRMS): ~85% of pts with MS •  Repeated relapse episodes followed by recovery

•  Secondary progressive MS (SPMS): 50-60% of pts with RRMS •  First few years: recovery of previous functioning common

•  Over time: recovery diminishes, permanent disability occurs

•  Primary progressive MS (PPMS): ~15% of pts with MS •  Progressive disability accumulation from onset of disease

•  Disability accumulation can occur rapidly

•  Radiologically-isolated syndrome (controversial) •  Incidental MRI findings meet diagnostic criteria for MS w/o any

history or symptoms suggestive of MS

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MS Diagnosis - McDonald Criteria • No single MS diagnostic test or biomarker • Current MS diagnosis based on

•  2010 McDonald Criteria for Diagnosis of MS • Diagnosis can be made on clinical grounds alone

• Dissemination in SPACE and TIME • Role of MRI in diagnosis of MS

• Primary diagnostic and prognostic tool in evaluation • MRI - support, supplement or replace some clinical criteria • McDonald criteria may require confirmation based on MRI


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Prognosis Is there a way of predicting the future? Watch “The Simpsons”

image.jpg (PNG Image, 800 × 533 pixels) https://static.businessinsider.com/image/58244915dd0895fe3...

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In 2000, “Bart to the Future" an episode of “The Simpsons” predicted that Donald Trump would one day become US president. It is still not possible to predict the outcome in MS


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Poor Prognostic Factors •  Age > 40 years at disease onset •  Male •  Asian or African–American ethnicity •  Initial presentation with

•  motor, cerebellar or sphincter symptoms OR •  multifocal symptoms Incomplete recovery after initial attacks

•  Frequent attacks during the early years of the disease •  Short interval between the first two attacks •  Rapid disability progression •  Progressive disease from disease onset •  Short interval from the time of disease onset to the start of the

progressive phase •  Cognitive impairment at disease onset •  Presence of oligoclonal immunoglobulins in the CSF •  High disease burden or gadolinium lesion enhancement on initial MRI


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Progression of RR MS

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Trapp BD et al The Neuroscientist. 1999 relapses and impairment MRI burden of disease MRI activity — — brain volume

Preclinical Relapsing

pt4Mrtw1ftt1tt

Secondary Progressive

I Fig. 6. Brain atrophy and the natural history of multiple sclerosis (MS). Slices from magnetic resonance images from normal healthy control subject (age 31, male) (A); patient with relapsing-remitting multiple sclerosis (RR-MS) (age 36, female) with disease duration of 2 years (6); and secondary progressive disease course multiple sclerosis (SP-MS) (age 43, female) with disease duration of 19 years (C) Parenchymal brain volume decreases and ventricular volume increases with disease severity, as shown in B and C. D summarizes the relationship between clinical disability, brain atrophy, and various MR disease parameters measured over time. MRI activity (arrows) refers to either the presence of gadolinium-enhancing lesions or the appearance of new lesions on T2-weighted images. In RR-MS, there are typically many more inflammatory events marked by MRI activity than clinical relapses. Enhancement is not frequently observed in SP-MS. MRI burden of disease refers to lesion load measurement on normalized T2 images. As de-picted by the wavy line, this measurement varies considerably over time. Lesion load generally increases more rapidly during the RR phase and then levels off or even decreases during the SP phase, although clinical disability accumulates more rapidly in SP-MS. The red line represents the current hypothesis for the time course of brain atrophy, consistent with the MRI data shown above. Brain tissue loss occurs during both phases of disease but occurs at a more rapid rate during SP-MS. Demyelination and axonal loss during RR-MS contribute to a slow decrease in brain volume. The "switch" from RR-MS to SP-MS is hypothesized to occur when the brain volume has decreased to the point when compensatory mechanisms have been exhausted. This point can not be predicted by any of the other MR disease markers displayed here.

is restored in the intact nerve fibers (Fig. 1). Axonal transection begins at disease onset (Fig. 2), but is clin-ically silent in RR-MS because of the remarkable ability of the mammalian CNS to compensate for neuronal loss. The relapse rate in RR-MS is a poor predictor of con-version to SP-MS, because intermittent clinical relapses mark the presence but not the severity of the underlying

axonal pathology. Brain atrophy, therefore, may be a valuable predictor for conversion to SP-MS. Once a threshold of axonal transection is exceeded, MS patients enter an irreversible secondary progressive stage. Axonal loss from chronic demyelination (Fig. 3) may be irre-versibly progressive. In this view, MS can be considered as a neurodegenerative disease characterized by axonal

54 THE NEUROSCIENTIST Axonal Degeneration in MS


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Different diseases, different time courses Where is your patient on the curve?

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What is the overall approach to treatment of patients with MS? •  Multidisciplinary and comprehensive approach can

significantly improve quality of life of patients with MS •  Prevent and manage relapses

•  Disease modifying therapies (DMTs) •  Delay disease progression and reduce relapse rate with

medications •  Disease modifying therapies (DMTs)

•  Symptomatic therapies •  Use medication and nonmedical approaches for fatigue •  Treat spasticity and bladder dysfunction •  Assess cognitive functioning

•  Consider ways to help patients maximize daily function •  Management of lifestyle factors

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Fatigue Management •  Fatigue affects > 70% of people with MS • One of the most frequent and disabling symptoms in MS

• More than simple physical tiredness - lethargy • Can occur very early in MS – direct disease effect. • Can affect everyday activities and cognition • Sometimes sleep interrupted - night spasms or nocturia • High variability of the clinical presentation, consider

fatigue as a complex symptom composed of the three main components •  Asthenia/daytime tiredness, •  Pathological exhaustibility and •  Worsening of symptoms due to stress


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Lifestyle factors Recommendations for all people with MS •  Stop Smoking

•  Delay onset of SPMS/disability progression •  Reduce MRI disease activity (lesions, brain atrophy)

• Vitamin D supplementation •  Aim for the higher end of normal range •  Therapeutic effect of supplementing vitamin D unclear – ongoing

research

• Exercise •  Maintain condition & fitness; biologivcal reserve

• Healthy, balanced diet • Address other health issues


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MS Treatment - Summary • Disease modifying therapies (DMTs) • Symptomatic therapies • Management of lifestyle factors •  Therapies only available as part of clinical trials/research • Neuroprotective or reparative therapies

•  Not yet available


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Parkinson’s Disease: Overview • Chronic progressive neurodegenerative disorder

•  Causes motor and non-motor dysfunction • Prevalence - 0.3 % Australian population •  2nd only to Alzheimer’s Disease as the most common NDD • Aging population, burden of PD increases

–  8.7 million PD cases worldwide 20-25 years (individuals ≥50 y/o) –  Affects 1 – 2% of all persons > 65 yrs old –  Affects 4 – 5% of all persons > 85 yrs old

•  ~ 80,000 people in Australia live with PD •  > MS, ALS, &muscular dystrophy patients combined

• Usual age at onset – early 60s •  10% affected < 45 yrs old –’young onset Parkinson’s’ •  Lifetime risk men : 2.0%, women : 1.3%


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Parkinson’s Disease: Overview (II) • Degeneration of dopaminergic neurons

in the substantia nigra •  Cause unknown ? Genetic ? Toxin ?Trauma

• Symptoms due to insufficient dopamine •  3 main motor symptoms:

•  Tremors •  Rigidity •  Slowed motion (Bradykinesia) •  Also

•  Postural instability, dysarthria, shuffling gait • Non motor symptoms include:

•  Psychiatric, cognitive (Dementia), sleep disturbances, autonomic, sensory, pain, fatigue

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Diagnosis • No definitive tests for PD. PET scans can aid to determine

levels of dopamine. • Difficult to diagnose, many symptoms shared with other

disorders. • Medical history and neurological tests are conducted to

diagnose. •  Usually, if two of the cardinal symptoms are present.

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Diagnosis of Parkinson’s disease •  Early Pre (non) Motor Signs of PD •  Probable early signs (strong association)

–  Constipation –  Olfactory deficit –  REM sleep behavior disorder –  Depression

•  Possible early signs (possible association) –  Apathy –  Fatigue, Excessive daytime sleepiness –  Anxiety –  Orthostatic hypotension –  Pain –  Restless legs syndrome –  Sexual dysfunction –  Urinary dysfunction

PD, Parkinson’s disease; REM, rapid eye movement. Chaudhuri et al. Lancet Neurol. 2006;5(3):235-245. Tolosa et al. Neurology. 2009;72(7 suppl):S12-S20

.


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PD - Cardinal Motor Symptoms

PD = Parkinson’s disease. Olanow et al. Neurology. 2009;72(suppl 4):S1-S136.

Early PD Advanced PD

Bradykinesia (slowed movement)

Rigidity (stiffness)

Rest tremor

Postural instability


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Treatment – Parkinson’s Disease

• No cure for PD. • Treatment can be divided into two stages.

• Early and Later stages • Early stage

• Onset of symptoms, treated with physical therapy and medications (Levodopa, dopamine agonists, etc)

• Later stage • Usually after having received 5+ years of levodopa

treatment. •  “Wearing-off” and “On/Off” effect develops, other

medication in conjunction levodopa is commenced. • MAO-B and COMT inhibitors.

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Treatment – Physical Therapy • Regular exercise

•  Recommended throughout the life of disorder. •  Helps maintain and improve mobility and strength. •  Physical exercise aids in rigidity relief, muscle strength and flexibility,

balance, etc. •  Caution is advised to avoid sudden movements or strenuous

activities – fall could result in serious injury.

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Treatment – Drug Therapy •  Levodopa (L-DOPA)

•  Preferred medication to control major symptoms. •  Usually administered at the early onset of disorder. •  Drug is well tolerated and side affects are limited.

Levodpa

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Dopamine

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Onset of motor fluctuations: When? •  10% per year after L-dopa starts •  ‘Honeymoon’ for ~ 5 years • Patient profiles identical after 10–14 years treatment,

regardless how dopaminergic therapy began • What are motor complications primarily related to?

•  Duration of levodopa therapy? •  Disease-related factors?

• Major risk factors are •  Age and •  Disease duration but •  Not L-dopa exposure

•  'Don't delay, start today’ •  Delaying levodopa does not delay motor complications


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What to tell patients? • Early presentation - diagnosis may not be clear

•  Wait and see • All treatment is symptomatic / no cure • Outline of the course of the disease • Management options

•  Side effects of medication • Parkinson’s disease Society (NSW) • Non drug treatments and lifestyle

•  Exercise, rehabilitation, diet, • Driving • Cognitive impairment

•  Maintaining intellectual pursuits, hobbies, social interaction


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Stages of Parkinson’s Disease

Do

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Severi

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isease

Stages of Parkinson’s Disease1,2

Abnormal rate of dopaminergic neuron deterioration begins

Onset of symptoms

Diagnosis Maintenance

Complex Palliative

Death

Symptomatic threshold

Presymptomatic phase of disease c 4- 10 years

Disease can be managed without dopaminergic therapy for c 1 year

Time

Symptoms/motor complications can be managed with dopaminergic therapy for c 5-10 years

Motor complications develop c 5-10 years

Cognitive decline

Dopaminergic neuron number and function Severity of the disease

Adapted from Olanow 2001 & Baker 200D3 r P Ramesh, Parkinson's TRACS May24th2013

Abnormal rate of dopaminergic neuron deterioration begins

Onset of symptoms Diagnosis

Maintenance Complex Palliative Death

Presymptomatic phase ~ 5 - 10 y

PD can be Mx without DA Rx for ~ 1y after Dx

Sxs /motor Cxs Mx with DA Rx for ~ 5-10y

Cognitive decline Motor Cx develop ~ 5-10 y Time

Dopaminergic neuron number and function Severity of the disease n n Adapted from Olanow 2001 & Baker 2003


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Case Histories •  The Electrician •  The Opera Singer •  The Psychiatrist & Musician •  The Journalist •  The Wool Trader


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The Electrician - History •  46 y/o left handed father of 4; Telstra electrician •  6 y h/o slowly progressive right upper limb tremor

•  Left side unaffected •  Attributed to work stress / dissatisfaction with Telstra •  Changed jobs back to work as an electrician

• Symptoms worse with stress; unchanged after 8 months; •  Unable to do things fast; tasks easier if he thinks rather than ‘auto-

pilot’ • Wife and workmates have noticed his tremor • History of mild anxiety • Denies other motor / non motor symptoms

•  Parasomnia, restless legs syndrome, constipation, difficulty with gait, balance, difficulty moving in bed, bulbar symptoms, cognition


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The Electrician - Examination •  Reactive affect mild hypomimia •  Normal cognition •  Mild right upper limb abnormal posturing and slowness •  Decreased arm swing on the right •  Minor right upper limb tremor, decreased dexterity and fine

movements •  mild cog-wheeling without rigidity right worse than left, •  No retropulsion. •  The gait balance, Romberg’s test and tandem gait intact. •  Right palmomental reflex •  Diagnosis?

•  Mild asymmetric extrapyramidal syndrome. •  Probable Parkinson’s disease


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The Electrician - Progress •  Diagnosis

•  Mild asymmetric extrapyramidal syndrome. •  Probable Parkinson’s disease

•  Progress •  Involvement of Parkinson’s disease nurse

•  Had a fall at home •  Work

•  Last consultation – still working •  PD more obvious motor problems - slowing (bradykinesia), êdexterity •  Initially declined regular medication

•  When not under stress or working to any kind of deadline not particularly affected by the symptoms

•  Later accepted medication •  Time pressures at work

•  Not safe up ladders and working at heights


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The Opera Singer - History •  64 year old left handed acclaimed Australian Opera Singer. •  18 m h/o impaired dexterity, balance, posture, sinus, bladder Sxs •  2 y h/o urinary problems

•  Urgency, enuresis, nocturia, poor flow, terminal dribbling, low volume, •  Previous few months, symptoms affecting movement, on left side.

•  Truncal stiffness; difficulty straightening up during rehearsals •  Balance problems; stooped posture during Tosca performances; aware that he

might topple into the orchestra when he moved suddenly or changed position. •  Occasional left hand and forearm tremor •  Trouble alighting from a deep settee and rolling in bed. •  Mild sialorrhea •  Longstanding hyposmia.

•  Voice husky •  Friends and family told him he is not moving well.


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The Opera Singer - Examination •  Sense of smell at least partially intact (coffee granules) •  Asymmetric left > right mild cogwheeling; augmentation +ve •  Slight increase in truncal tone

•  Stiffness and slight neck and trunk flexion.

•  Hint of tremor of the outstretched left upper limb. •  Glabellar tap +ve; other atavistic reflexes –ve. •  Slight overall bradykinesia, paucity of facial expression,

diminution of blinking •  Power, coordination, manual dexterity – normal.

•  Gait - mildly reduced arm swing; very slightly stooped posture •  Romberg’s test, tandem gait, retropulsion – normal

•  Able to stand on the heels and toes. •  Diagnosis?


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The Opera Singer - Progress •  Sinemet (Carbidopa-Levodopa)

•  Small improvement in posture and stiffness. No side effects. •  Physiotherapy for core muscle strength •  Osteopath for back and right shoulder stiffness •  Difficulty with voice particularly when speaking; partially

overcome by singing in operatic mode, but unable to reach peak performance levels in rehearsal.

•  Upcoming studio recording session at the end of the month •  Due to perform in a physically demanding role in Europe •  Advised

•  Unrealistic to perform in two months; he should inform the promoters. •  Singing performance might benefit from additional medication but

uncertain whether he will regain his former vocal ability.


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The Opera Singer - Progress •  Improvement in motor function including voice with

medication • Some constipation • Gets tired towards the end of the day but in the mornings

his function is best. • Able to complete his recording of Tosca but can only

maintain singing voice for 2 – 3 hours before losing the low end of the scale

• Concentration flags; 12 hours to recover.


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The Opera Singer - Summary •  Diagnosis •  Early Parkinson’s disease affecting gait, posture, manual dexterity

and voice (glottal incompetence consistent with Parkinson’s disease) •  Symptoms responded to Sinemet and Sifrol (pramipexole),

physiotherapy / rehabilitation and speech pathology. •  Can sing for 15 minutes; limited by fatigue •  Mild difficulty with dexterity; does not affect ADL •  No falls; can jog at a slow pace •  Bladder problems - prostate. No other symptoms of autonomic

dysfunction nor mood or cognitive problems. •  Occupational Therapy home visit in the future. •  Work

•  Nearly completed the studio recording sessions •  Cancelled performance in Europe next month •  All but retired from Opera.


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The Psychiatrist & Musician - History • One year history

•  Subtle appreciation of right hand tremulousness •  Occasional twitches •  Difficulty writing - “becoming more cramped and more illegible and

difficult to complete medical records as easily”. •  Difficulty cello playing - executing quick alternating movements •  More recently gait difficulty

•  Decrease in the arm swing on the right •  A sense that the right leg is ‘dragging’ •  Gait changes noticed by others – unsurprising considering her job

•  Initially unsure as to the presence of these symptoms •  Attributed to recently diagnosed hypertension.

•  Father had Parkinson’s disease diagnosed at 60.


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The Psychiatrist & Musician - Examn • Mild decrease in arm swing, slight right shoulder drooping

•  Otherwise normal narrow based gait; no unsteadiness; turns well

• Mildly impaired right upper limb rapid movements and mildly decreased dexterity.

• Rt leg resting, tremor é tone; cogwheeling, reflexes ++ •  Right arm was free of tremor.

• Hint of cogwheeling tone on the left hand side • No bradykinesia or rigidity in the left hand side.


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The Psychiatrist & Musician - Diagnosis • Diagnosis: • Asymmetrical extra pyramidal syndrome • Most likely diagnosis early Parkinson’s disease • Some atypical features • Declined drug therapy • Discussed treatment plan and use of drugs when needed • Non-pharmacological therapy

•  Generalised physiotherapist directed fitness program •  Type and frequency of exercises •  OT advice directed at difficulties writing and cello playing.


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The Psychiatrist & Musician - Work •  Work issues

•  Right upper limb in activities which require dexterity affected •  Handwriting and cello playing •  Effects of stress and arousal on the tremor & handwriting esp. in ED •  Practical solutions to this problem discussed

•  Declined L Dopa therapy •  Strong views on early use of L-dopa; other drugs side effects profile

unfavourable •  “Any consideration of the implications of uncertain long term

complications of therapy needs to considered in the light of the effects of PD on function and quality of life at the present moment and the potential for immediate benefits from effective therapy now.”

•  PD MED The Game Changer - In 2014 concluded •  Start any drug •  Don't be afraid of using L-dopa if needed


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The Psychiatrist & Musician - Progress • Motor symptoms stable for some time when last seen • Occasional problems with dexterity • Medication, speech therapy, physiotherapy – all helping • Coping well albeit with increased effort at work, talking to

small groups, treating patients and writing. • Minor cognitive problems, work block when speaking or

when writing •  Uses mental tricks or devices to help overcome these problems.

• Occasionally forgets the date. • Concerned possibility of cognitive impairment due to

Parkinson’s Disease or medication effects


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The Journalist - History •  33 F Journalist; 1 month painful reduction in vision left eye • Arrived with MR scan

•  Results consistent with optic neuritis and white matter demyelination (did not quite reach criteria for MS diagnosis).

•  Three days IV methylprednisolone • Vision worse after a hot bath. Denies Lhermitte’s • Other symptoms

•  Left foot paraesthesia present for a few days, then resolved •  Mild unsteadiness on and off for the last couple of years •  Two years fatigue,

• PMH - anxiety, PTSD (stress as a Police reporter), tension-type headaches


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The Journalist - Examination •  Neurological examination normal •  Mild visual blurring on testing visual acuity in the left eye. •  Mild blurring of the nasal margin of the left optic disc. •  Reflexes and coordination gait were normal •  Diagnosis

•  Presentation is consistent with optic neuritis •  Imaging consistent with more extensive but fairly limited demyelination

with small plaques evident in the subcortical frontal areas. Treatment •  Disease modifying treatment deferred (this was 2002) •  Lifestyle - reduction in caffeine and analgesia to reduce

headache frequency •  Advise to cease tobacco, consider diet, exercise - deferred


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The Journalist - Progress •  Fatigue and low grade mood dysfunction

•  Fluctuating course after commencing IFN treatment

• Work •  Cut down to 4 days a week

• Ceased strenuous exercise for a time • MS in stable remission for several years – no relapses • Mood & fatigue improved with

•  Psychological counseling and antidepressant drugs

• Returned to work full time • When last seen was considering having a baby


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The Wool Trader – History & Examn • Presented aged 34; 5y history of non-specific symptoms

•  Visual disturbance, paraesthesia, fatigue, unsteady gait.

• Significantly exacerbated by exercise •  Ceased serious exercise two years previously

•  Intermittent symptoms; ?? periods of recovery •  No definite acute relapses

• Mildly anxious, slightly fatuous affect, thin body habitus • Mild left arm incoordination • Signs of mild paraparesis; possible sensory level at T10 • Mild left optic disc pallor, mildly decreased vision and

hearing on the left


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The Wool Trader – Diagnosis • MRI brain fairly extensive changes consistent with MS • Multiple cord plaques consistent with MS

•  No enhancing lesions. • Diagnosis – MS ? RRMS ? Primary Progressive MS

•  Significant spinal cord and cognitive features • Commenced on Betaferon (Interferon beta-1b)

•  Side effects flu like symptoms • OTC medications

•  Selenium, Vitamin E, Primrose Oil, Fish oil and Beta Carotene. • Made lifestyle changes – mainly diet • Also diagnosed Depression

•  Difficulty adjusting to diagnosis and work issues; improved on therapy


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The Wool Trader - Work •  Work

•  Ceased because of symptoms and diagnosis •  Unable to return to his previous job due to fatigue •  Unsure whether he would be capable of alternative employment •  Receiving income protection •  Initially questionable motivation and decision not to RTW

•  Relatively mild initial neurological impairment; unusual affect; ongoing activities - home decorating, share trading and ‘dabbling’ in wool trading

•  Progress •  Disability slowly progressed over several years •  Spinal cord disease, fatigue, difficulty concentrating •  L>R spastic paraparesis; mild étone êdexterity in the left upper limb •  Progressive gait deterioration; walking aid; bladder urgency •  Less optimistic re success in vocational rehabilitation •  Recommended stopping Betaferon (Interferon beta-1b)


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Summary •  Two examples of relatively common NDD have been presented

•  MS and Parkinson’s disease •  Both affect people of working age •  Both lack objective and unique biomarkers •  Both require clinical diagnosis •  Highly variable symptoms; PD more consistent •  MS is usually relapsing and remitting at first •  Parkinson’s disease is slowly progressive from the onset •  Individual prognosis very difficult to predict

•  Knowledge of prognosis & disease STAGES helps plan RTW / Rehab •  MS – effective disease modifying therapy alters natural history •  PD - symptomatic therapy only •  Understand time course of different neurodegenerative diseases


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Conclusion - The Importance of Individualisation Drug treatment, Rehabilitation, Return to work


Manageable expectations, rational rehabilitation and return to work decisions require a broad understanding of factors that determine outcome in neurodegenerative diseases especially TEMPO

ABriefOverviewofMul'pleSclerosis&Parkinson’sDiseaseReturntoWork&Rehabilita'onChallenges

QUESTIONS?DrDavidRosen|PrinceofWalesPrivateHospital

degenerave neurological diseases · 2018-07-09 · degenerave neurological diseases a brief...

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