Neurodegenerative Movement Disorders - PowerPoint Presentation

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Neurodegenerative Movement DisordersParkinsonism

Nancy L Diaz-Pechar, MD

Movement Disorder Center Director

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DisclosureDr. Nancy Diaz-Pechar has served as a speaker for Teva pharmaceuticals

There are no financial disclosures that would be a potential conflict of interest with this presentation. Slide3

Goals and objectivesTo understand how to distinguish between atypical parkinsonism and Parkinson’s disease

To review clinical presentation and treatment of progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration

To briefly review the clinical presentation and treatment of Huntington’s DiseaseSlide4

ParkinsonismDiagnosis remains to be clinicalNo imaging, genetic test, biomarkers definitively diagnose

Medical history, time-line, significant clinical signs

Given emerging clinical signs diagnosis can change overtime as it become more clearSlide5

Bradykinesia

An essential clinical sign as it implies an abnormal basal ganglia-cortical neuronal circuit

Slowness of initiation with progressive reduction in speed

Decreased amplitude with repetitive movements.

Exam: finger taps, rapid alternating movement, repetitive hand opening, heel taps, toe tap (?pauses or freezing)

Archimedes spiral, micrographia

Hypomimia

Reduced arm swing

Poor dexterity

HypophoniaSlide6

HypokinesiaSlowed movements without decrement

Extrapyramidal rigidity

Increased resistance independent of velocity

Lead pipe-constant

Cogwheel- inconsistent

Tremor

4-6 Hz rest tremor which ceases or attenuates with action

Asymmetric

Re-emergent tremor- 5-10 secSlide7

Postural instability and gait disturbanceReduced speed

Imbalance

Narrow based, shortened stride

Decreased arm swing

Freezing (initiation, turn, doorway)

En bloc turning

Early sign in atypical parkinsonism

Lurching gait –PSP

Ataxia-MSASlide8

Parkinson’s disease (PD)Clinical Diagnosis

Bradykinesia plus at least one other symptom

Rest tremor

Extrapyramidal rigidity

Postural instability

Shuffling, freezing, festination

Typical parkinsonian features

Unilateral

Levodopa response

DyskinesiaSlide9

Autonomic disturbances: constipation/ gastroparesis, urinary urgency, frequency, nocturia, incontinence, sexual dysfunction, sweating, drooling

Restless leg syndrome

Sleep disorders: REM sleep behavior disorder (RBD), excessive daytime sleepiness, insomnia

Anosmia

Fatigue/ Apathy

Cognitive impairment

Executive dysfunction/ MCI

PDD

Depression/ Anxiety

Hallucinations/ psychosisSlide10

Medications and surgical treatment optionsCarbidopa/levodopa (IR, CR, Parcopa, Rytary, Duopa via JPEG pump)

Dopamine agonist (ropinirole, pramipexol, Neupro, apomorphine)

Monoamine oxidase inhibitors (selegiline, rasagiline, Zelapar)

Catechol-O-Methyltransferase Inhibitors (tolcapone, entacapone)

Amantadine

Anticholinergics (trihexyphenidyl, benztropine)

Deep brain stimulation

Various medications for nonmotor symptomsSlide11

Signs of Atypical Parkinsonism/ Parkinson-plus disordersDiagnosis based on clinical features and are confirmed pathologically

Signs it is not PD but rather an atypical

Early postural instability/ gait disturbance

Rapid progression

Early dysphagia

Dysarthria is different than typical hypokinetic changes seen with PD

Paucity, absent or atypical tremor

Poor or no response to levodopa

Pyramidal tract findings (pseudobulbar palsy, UMN)Slide12

Progressive Supranuclear Palsy (PSP)

Presenting sign is postural instability with early falls (often backward)

Slowed saccades/ supranuclear gaze palsy (paralysis of voluntary vertical and later horizontal gaze but preserve reflexive eye movements)

Parkinsonism which is not usually levodopa responsive and whose onset is typically symmetric

Executive dysfunction/ frontal cognitive deficits

Dysarthria

DysphagiaSlide13

Taupathy (abnormal accumulation of Tau protein in brain)Slightly more common in men

Average age of onset typically in 60’s (can be as early as in 40’s)Slide14

PSP-subtypesPSP-Richardson syndrome

Mean age of onset 65

Early postural instability and falls

Apathy/ personality changes

Visual changes

slowed vertical saccades- hypometric saccades- square wave jerks-supranuclear vertical gaze palsy

Cognitive deficits

Slowed processing

Visuospatial dysfunction

Dysarthria

Dysphagia- aspiration

Dependent on others within 3-4 years, mean duration 7 yearsSlide15

PSP-parkinsonismCan be indistinguishable from PD at onset

Bradykinesia and limb rigidity at onset

Occasional jerky tremor

Axial rigidity

Early responsiveness to levodopa but this typically dissipates over time

Postural instability, cognitive deficits, and supranuclear gaze palsy will occur later in the first few yearsSlide16

PSP-pure akinesia with gait freezingLess common

Bradykinesia mostly affecting gait/ freezing

Hypophonia

Hypomimia

Micrographia

Axial rigidity with no limb rigidity

Supranuclear palsy and blepharospasm occur late

Median duration- >10 yearsSlide17

PSP-corticobasal syndromeKnown only by pathological studies

Presents as corticobasal ganglia degeneration

PSP-frontotemporal dementia

Less common

Known due to pathological studies

Behavioral variant frontotemporal dementia or progressive nonfluent aphasia variant

Typical PSP symptoms develop >5 years after onsetSlide18

PSP TreatmentMedication trial (levodopa, amantadine, vesicare for overactive bladder)

Dystonia/ blepharospasm – consider neurotoxin injections

Diplopic- consider prism glasses

Multidisciplinary care: neurologist, psychiatrist/ psychologist, PT/OT/ Speech, social worker, palliative careSlide19

Physical therapy/ Occupational therapyStrength, aerobic, and balance exercises, eye movement exercises

Fall prevention

Train on wide, staggered stance to improve stability on anterior to posterior weight shift

Big steps, foot clearance, U-turns

Avoid bending low/ standing quickly

Scan area with eyes before walking

Stop and move head and eyes to direction of turn before turning

Heel wedge/ lift

Assistive devices

Rollator with brakes, wheelchair, scooter

Environmental assessments/ home modifications

ADL trainingSlide20

Speech therapyDysphagia

Symptoms: delayed pharyngeal swallow onset, difficulty seeing the whole plate, rapid drinking/ eating, rigidity or hyperextended neck affecting self-feeding, difficulty opening mouth, lack of insight to swallowing issue

Mealtime swallow evaluation, video swallow study, compensatory strategies such as repositioning, keeping plate in line of vision, chin-tuck, mealtime adaptive devices, altered diet, alternating between liquids and solids, discuss feeding tube options with physicianSlide21

CommunicationHypokinetic and spastic dysarthria, stained and slow voice with impaired fluency, palilalia, language and cognitive deficits, emotional lability

Train to speak loudly and slowly (LSVT ), short phrases, use gestures, yes/no questions, communication board or speech generating device, personal portable amplifierSlide22

Multiple System Atrophy (MSA)Prominent autonomic dysfunction precedes motors signs by years

Progressive bladder dysfunction (increased frequency, urgency, incontinence, retention)

Orthostatic hypotension

Erectile dysfunction

Constipation

Difficulty with thermoregulationSlide23

Parkinsonian phenotype

Symmetric

Faster progression than PD

Postural instability , freezing, and falls within 3 years

Stimulus sensitive myoclonus > rest tremor

Bradykinesia, rigidity

Dysarthria and dysphagia

Dystonia

Laryngeal- inspiratory stridor / vocal cord abductor paresis

anterocollis

Cerebellar phenotype

Ataxic gait, limb/ truncal ataxia

Nystagmus, jerky saccadesSlide24

Synucleinopathy (accumulation of alpha synuclein protein) Mean duration- about 7-8 years but actually varies so range is large

Average age of onset is in the 50’s

More common in men

Sub-classified according to prominent symptoms: MSA-C, MSA-PSlide25

MSA Treatment

Levodopa trial (typically poor levodopa response but up to 1/3 have good response)

Anterocollis

Positioning, stretching, bracing

Tilt wheelchair

Treat hypotension

d/c antihypertensive

Increasing salt intake

Compressive stockings

Fludrocortisone, midodrine, Northera

Elevate head of bed

Smaller more frequent meals

Treat urologic issues

Medications/ Botox for bladder spasticity

Self cath/ suprapubic catherization

Multidisciplinary care: neurologist, psychiatrist/ psychologist, PT/OT/ Speech, social worker, palliative careSlide26

Physical and Occupational therapyLarge movements for bradykinesia (BIG)

Gait training, U- turns, wide stance, fall prevention, rising slowly

ADL training/ functional mobility

Avoid overreaching, sitting while dressing

Assistive devices and home modifications

Shower chair, grab bar, hand held shower head, bed rail, etc.

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Speech therapyDysphagia

tendency toward bolus holding in oral cavity and discoordinated bolus propulsion, difficulty sitting upright, increased pharyngeal secretions, vocal fold motion impairment may compromise airway protection

Moist, soft food, chin-tuck, restrict bolus volumes, alternate food and liquid, smaller more frequent meals, mealtime adaptive devices

Communication

Hypokinetic dysarthria, hypophonia or ataxic or spastic dysarthria

LSVT, reduce rate of speech to improve coordination and accuracy, communication board or speech generating device, personal portable amplifier, yes/ no questions. Slide28

Corticobasal Degeneration/ Syndrome

Affects the fronto-parietal cortex and the basal ganglia

Signs and symptoms include

Asymmetric ideomotor apraxia

Asymmetric rigidity and bradykinesia

Myoclonus, atypical tremor

Dystonia

Alien-limb phenomenon

Corticosensory deficits

Postural instability/ progressive gait changes until bedbound

Progressive aphasia

Mild cognitive or behavioral issues

Dysphagia

DysarthriaSlide29

Corticobasal Degeneration (CBD)Tauopathy

Average age of onset in the 60’s but can be as early as in 40’s

Various phenotypes (overlapping features with other disorders such as PSP, FTD, PPA, posterior cortical atrophy, Alzheimer’s, etc..)

Definitive diagnosis only by autopsy

Mean duration 5-10 years from onset of symptoms Slide30

CBD treatmentSupportive to optimize functionPatient and caregiver education

Not responsive to dopaminergics

Treat myoclonus with antiepileptic medications

Treat dystonia with neurotoxin injections with the goal of reducing pain and improving hygieneSlide31

Physical and Occupational therapyStretching or bracing for anterocollis/ dystonia

Encourage use affected limb (hand/ wrist extension to maintain an open hand)

Large movements for bradykinesia

Gait training, U- turns, wide stance, Fall prevention

ADL training

Assistive devices and home modifications

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Speech therapyDysphagia

Symptoms: slow or incomplete chewing and swallowing, difficulty self feeding, oral and swallowing apraxia

Treated as in PSP

Communication:

Symptoms: hypokinetic and spastic dysarthria, progressive apraxia, may have non fluent aphasia

Encourage to use short phrases and simpler language, write, yes/no questions, communication boardSlide33

Huntington Disease Progressive, inherited, neurodegenerative disorder with no cure

Autosomal dominant, trinucleotide repeat of CAG coding for glutamate, gene on short arm of chromosome 4

AnticipationSlide34

Chorea- involuntary, irregular, rapid and unsustained movements which flow between body parts

Motor impersistence (milkmaid grip, tongue protrusion)

Dystonia

Ataxic gait

Cognitive decline: impaired judgement, forgetfulness

Dysarthria, eventually may be unable to speak but will comprehend and retain awareness

Dysphagia

Psychiatric manifestations: personality changes, mood swings and depressionSlide35

Huntington’s disease TreatmentNothing alters course. No cure.

Multidisciplinary care: neurologist, psychiatrist/ psychologist, PT/OT/ Speech, social worker, palliative care

Medications to lessen chorea

Tetrabenazine, other antipsychotic medications such as risperidone, olanzapine, haloperidol, etc..

Medications for psychiatric disorders

Antidepressants

citalopram, fluoxetine, sertraline

Antipsychotics

Mood stabilizers

valproate, carbamezapine, lamotrigineSlide36

Physical and occupational therapy

Strengthening and coordination exercises

Balance and gait , fall prevention

Posture

Respiratory exercises

Assistive devices: walker (add weights for chorea), wheelchair, adaptive utensils, tub bench, foam grip, lift chair, etc.

Adaptive improvement of ADL’s, caregiver training, improve transfers

Goal is to maintain cognitive, motor, and functional performance

Speech therapy

Impaired control of muscles of mouth and throat

Mealtime swallow evaluation, video swallow study, compensatory strategies such as repositioning, mealtime adaptive devices, altered diet

Speech and cognitive effects variableSlide37

SummaryDiagnosis of the type of parkinsonism is complicated as they are separate by subtle changes in signs and symptoms. Careful evaluation is required.

Neurodegenerative movement disorders discussed are progressive, and have no cure, but diagnosis helps with patient and family education, prognosis and guide specific treatment.

Multidisciplinary care is essential in caring for patients with neurodegenerative movement disorders. Slide38

True or falseDifferent subtypes of progressive supranuclear palsy have been described, one of which shows early responsiveness to levodopa which dissipates over time.