ALS / HD / PD

Question 1

Amyotrophic Lateral Sclerosis (ALS):

• Characterized by a progressive degeneration and ultimately loss of ___ neurons in the __, __, and __.
• Typical age of onset?
• M vs F??
• Etiology?
• Prognosis?
• Disease modifying agent?
• Symptoms to manage and meds?

Answer 1

ALS:

• Characterized by a progressive degeneration and ultimately loss of MOTOR neurons in the SPINAL CORD, BRAIN STEM, and MOTOR CORTEX.
• Typical age of onset? LATE 50s - EARLY 60s
• M vs F?? Males > F (1.56:1)
• Etiology? Unknown, but likely genetic + environmental facotrs
• Prognosis? Poor, but varies widely. Most people die within 2-10 yrs post diagnosis.
• Disease-modifying agent: RILUZOLE: decreases glutamate release and prolongs survival by at least a few months, slows the progress of ALS, allows pt more time in the higher-functioning states
• Symptoms and meds:
• –> Cramps (Dilantin, Valium, baclofen, quinine)
• –> Spasticity (baclofen, valium antrolene)
• —> Excess salivia (aka sialorrhea: elavil, robinul, atropine, scopalamine, botox)
• –> Sleep disturbance (Elavil, valium)
• —> Depression (Elavil, prozac, zoloft)
• —> Excess laughing/crying (Elavil, paxil, prozac)

Question 2

Classical ALS makes up ~___% of total ALS cases. It is more common in [M / F / neither sex]. Involves [ UMN / LMN / both UMN and LMN].
Early signs include: progressive weakness of the ___, reduced ___ of the hands, and muscle __.
–> With additional bulbar involvement, we also see …
—> Person may c/o __ and __.

Later stages of the disease may ultimately see involvement of cranial nerves __, __, and __ as well as the __-__ segments of the spinal cord.

Answer 2

Classical ALS (AKA sporadic ALS) makes up ~66.6% of total ALS cases. It is more common in MALES. Involves BOTH UMN and LMNs.
Early signs include: progressive weakness of the LIMBS, reduced DEXTERITY of the hands, and muscle FASICULATIONS. Tend to see initial spasticity or even increased reflexes, then later in the dz course, see more hypo/areflexia as LMN involvement progresses.
–> With additional bulbar involvement, we also see …WASTING OF THE TONGUE AND MM FOR SPEACH AND SWALLOWING
—> Person may c/o CRAMPS and FATIGUE.

Later stages of the disease may ultimately see involvement of cranial nerves III, IV, and VI (eye movements) as well as S1-S3 (important for autonomic function like bowel/bladder control and sexual fxn)

Question 3

Primary Lateral Sclerosis (PLS) makes up ~___% of total ALS cases. Involves [ UMN / LMN / both UMN and LMN] - specifically, it is a dysfunction of the ___ tracts which lead to these signs and symptoms.

• Key exam findings include: (3)
• Findings on EMG of limb and bulbar mm?
• PLS can progress to ALS, but if no [UMN / LMN / both UMN and LMN] signs are seen after [#] years, prognosis is [ good / bad ] with life expectancy of ____

Answer 3

Primary Lateral Sclerosis (PLS) makes up ~5 % of total ALS cases. Involves ONLY UMN - specifically, it is a dysfunction of the CORTICOSPINAL tracts which lead to these signs and symptoms.

• Key exam findings include: SPASTICITY, UMN PATTERN WEAKNESS, and PSEUDOBULBAR FINDINGS (meaning: excessive laughter or crying; occurs d/t damage to corticobulbar fibers in PLS)
• NORMAL EMG of limb and bulbar mm
• PLS CAN progress to ALS, but if no LMN signs are seen after 4 years, prognosis is GOOD with LIFE EXPECTANCY comparable to NORMAL!!

Question 4

Progressive Spinal Muscular Atrophy (PMA) accounts for __-__% of those who present with ONLY [ UMN / LMN] changes, i.e. (4)
Symptoms typically present [where?] but __ palsy can develop later. May be limited to one region (examples?)
Mainly affects [M / F / both sexes].
Progression varies from slow to very rapid; patients with slowly progressive SMA have a mean survival of __-_ years.

Answer 4

Progressive Spinal Muscular Atrophy (PMA) accounts for 5-10% of those who present with ONLY LMN changes IN SPINAL REGIONS, i.e. FLACCID WEAKNESS, ATROPHY, FASICULATION, AND HYPOREFLEXIA or AREFLEXIA
Symptoms typically present in the LIMBS but BULBAR palsy can develop later. May be limited to one region, e.g. FLAIL ARM SYNDROME (affective cervical regions of the spine) or FLAIL LEG SYNDROME (affecting lumbosacral regions)
Mainly affects MEN (M:F 2:1)
Progression varies from slow to very rapid; patients with slowly progressive SMA have a mean survival of 5-10 years.

Question 5

Bulbar Onset ALS makes up ~___% of total ALS cases. It affects the ___ region, either UMN in ____ (ALS subtype) vs LMN in ___ (ALS subtype). Specifically, it affects ___ nerves __-__, resulting in (symptoms? 2)

If the __ tract is affected, ___ muscle spasticity leads to dysarthria. Psuedobulbar palsy may also occur.

Some patients have bulbar symptoms for an extended period of time (called ____ ALS).

Bulbar onset ALS is more common in [younger / older] [M/F].

Answer 5

Bulbar Onset ALS makes up ~33.3% of total ALS cases. It affects the BRAINSTEM region, either UMN in PSEUDOBULBAR PALSY vs LMN in PROGRESSIVE BULBAR PALSY. Specifically, it affects CRANIAL NERVES IX - XII, resulting in dysarthria AND dysphagia

If the CORTICOBULBAR tract is affected, TONGUE muscle spasticity leads to dysarthria. Psuedobulbar palsy (again, this is the UMN variant! = inappropriate laughter / crying) may also occur.

Some patients have bulbar symptoms for an extended period of time (called ISOLATED BULBAR ALS).

Bulbar onset ALS is more common in OLDER FEMALES (M:F 2:3)

Question 6

ALS with Frontotemporal Dementia (FTD)
- Cognitive symptoms and dementia were once thought to be uncommon ALS symptom, however recent studies suggest that roughly ~__% of patients with ALS demonstrate mild to moderate cog and/or behavioral impairments without dementia and ~__% of individuals with ALS will develop frontotemporal dementia.

• Common deficits involve…(3)
• Can adversely affect compliance, with clinical course usually being [slower / more rapid / about the same] in patients with ALS-FTD.
• Cause?

Answer 6

ALS with Frontotemporal Dementia (FTD)
- Cognitive symptoms and dementia were once thought to be uncommon ALS symptom, however recent studies suggest that roughly ~30% of patients with ALS demonstrate mild to moderate cog and/or behavioral impairments without dementia and ~20% of individuals with ALS will develop frontotemporal dementia.

• Common deficits involve…IMPAIRED EXECUTIVE FXN (JUDGEMENT, IMPULSIVITY, DECR ABILITY TO HANDLE ROUTINE TASKS), LANGUAGE, OR PERSONALITY CHANGES.
• Can adversely affect compliance, with clinical course usually being MORE RAPID in patients with ALS-FTD.

-Cause: TAR DNA binding protein 43 (DPT-43) positive ubiquinated cytoplasmic inclusions identified in almost all cases of ALS and >50% of pts with frontotemporal dementia

Question 7

Familial ALS affects _-__% of pts with ALS, in which _____ causes familial ALS and contribute to the development of sporadic ALS. If you remember the genes, you get extra credit!

• Pattern of inheritance?
• Age at onest?
• Any hallmarks on neuro exam?

Answer 7

Familial ALS affects 5-10% of pts with ALS, in which MUTATIONS IN SPECIFIC GENES cause familial ALS and contribute to the development of sporadic ALS.
- C9orf72 gene: 30-40% of familial ALS in the US and Europe
- SOD1 gene: 15-20% of familial ALS worldwide; >90 mutations in the SOD enzyme alter the enzyme’s ability to protect against free radical damage to motor neurons
- TARDBP and FUS genes (each accounts for ~5% of familial cases).
Other genes are ANG, ALS2, SETX, and VAPB

• Genetic risk for ALS is probably relateld the combined effects of multiple genes + environmental features
• Pattern of inheritance? VARIES depending on the gene involved; most cases are inherited in an AUTOSOMAL DOMINANT pattern. Less frequently, ALS is inherited in an AUTOSOMAL RECESSIVE or an X-linked dominant pattern.
• Age at onset? Late 40s - early 50s
• Any hallmarks on neuro exam? Nope. No differences between familial and sporadic ALS on neuro exam.

Question 8

Meds for ALS?

Answer 8

Disease-modifying agent: RILUZOLE: decreases glutamate release and prolongs survival by at least a few months, slows the progress of ALS, allows pt more time in the higher-functioning states

Symptom management:

• –> Cramps (Dilantin, Valium, baclofen, quinine)
• –> Spasticity (baclofen, valium antrolene)
• —> Excess salivia (aka sialorrhea: elavil, robinul, atropine, scopalamine, botox)
• –> Sleep disturbance (Elavil, valium)
• —> Depression (Elavil, prozac, zoloft)
• —> Excess laughing/crying (Elavil, paxil, prozac)

Question 9

Early stage ALS

• Common impairments and activity limitations?
• –> Degree of muscle weakness? How widespread?
• –> Difficulty with ADLs?

PT goals?

Answer 9

Early stage ALS

• Common impairments and activity limitations?
• –> MILD to MOD weakness in specific muscle groups
• –> Difficulty with mobility and ADLs toward the end of this early sage

PT goals?

• –> Prevent inactivity
• —> Prevent fear of moving and falling
• –> Maintain or improve physical capacity

Question 10

Middle stage ALS

• Common impairments and activity limitations?
• –> Degree of muscle weakness? How widespread?
• –> Difficulty with ADLs?

PT goals?

Answer 10

Middle stage ALS

• Common impairments and activity limitations?
• –> SEVERE muscle weakness in some groups, vs mild to mod weakness in others
• –> Progressive decline in mobility and ADLs
• –> Increasing pain and fatigue

PT goals?

• —> Prevent falls
• –> Reduce mobility limitations (ie. transfers, posture, reaching/grasping, balance/gait)

Question 11

Late stage ALS

• Common impairments and activity limitations?
• –> Degree of muscle weakness? How widespread?
• –> Difficulty with ADLs?
• –> Other considerations?

PT goals?

Answer 11

Late stage ALS

• Common impairments and activity limitations?
• –> SEVERE weakness of axial and extremity mm
• –> Complete DEPENDENCE w/mobility and ADLs
• –> Dysarthria (and ultimately anarthria where they are unable to speak), dysphagia (-> need for PEG), respiratory compromise, pain

PT goals?

• —> Maintain vital functions
• –> Prevent secondary complications (pressure sores, contractures, pain)

Question 12

ALS Exam:

Body Fxn/Structure Level?

Answer 12

Usual exam things:

• CN screen (especially w/ pts w/bulbar symtpoms)
• Cognitive screen (verbal fluency = give as many words that start with F in 60 seconds, there are norms, verbal fluency test assesses executive cognitive functions of the frontal lobe that are commonly impacted in ALS and has been found to detect even subtle cognitive deficits in people with ALS. It is easy to administer and only takes one minute; Frontal Behavioral Inventory)
• Pain (VAS)
• ROM
• Muscle Strength (MMT, max voluntary isometric contraction [MVIC] using strain gauge tensiometer or dynamometer)
• Reflexes (DTRs, Hoffmann, Babinski)
• Tone (Modified Ashworth Scale) - they tend to show INITIAL HYPERreflexivity, then HYPOreflexive in later stages of the dz as LMNs more invovled
• Respiratory assessment (VC, FVC, aerobic capacity and endurance testing, 6MWT)

Other things:

• Fatigue (Fatigue Severity Scale; high score is more fatigued; really is activity/participation level)
• Psychological screen (Beck Depressison Inventory, Patient Health Questionnaire-2

Question 13

ALS:

Activity-level Outcome Measures?

Answer 13

• ALS Functional Rating Scale (ALSFRS-Revised; VERY often used at ALS clinics, looks at ADLs, life functions, respiratory dysfxn, participation/activity level; high score = best fxn, 0-48)
• Other functional measures (ALS Severity Scale, Norris Scale; LONG to administer)
• General health questions
• Balance (Tinetti Mobility Test, Timed Up and Go, Berg Balance Scale)
• Gait (10MWT)
• 30 sec chair stand test
• Functional Status (Functional Independence Measure)
• Hand/UE Function (Purdue pegboard, 9 hole peg test)

Question 14

Exercise recs in ALS?

Answer 14

5 moderatly strong human studies support benefits (strength, function, and respiratory fxn) MODERATE intensity exercise (aerobic + strength) in ALS without adverse effects

In animal studies, moderate exercise (treadmill/running wheel) may have neuroprotective effect (slower dz progression, increased lifespan) whereas HIGH intensity exercise may be harmful

Guidelines:

• Encourage continued participation in sports or activities they enjoyed prior to dx as LONG as they are able to safely do it
• Start exercise interventions when pts are in early stages of dz so they have sufficient strength, respiratory fxn, and endurance to exercise w/out excess fatigue

Strength:

• Emphasize CONCENTRIC rather than eccentric mm contractions.
• MODERATE resistance/intensity (e.g. 1-2 sets of 8-12 reps, or 3 sets of 5 reps)
• ONLY in muscles that have ANTIGRAVITY (at least 3/5) strength
• AVOID high-resistance exercises that increase the risk of mm damage without any added benefit above moderate exercise

Endurance:

• Emphasize MODERATE intensity activities
• 50-80% peak HR, 11-13 RPE, 3x/wk WITHOUT inducing excessive fatigue
• Rest periods are recommended, especially with continuous activity >15 mins
• Encourage active lifestyle when sport and other exercise activities are no longer feasible
• Advise pts with ALS to build up exercise program slowly and monitor for effects of exercise on their fatigue, pain, and ability to perform daily activities.
• Teach pts to recognize signs of mm overuse such as DOMS (peaks between days 1-5 post exercise), a reduction in max force production that gradually recovers, and/or severe mm cramping after exercise; a feeling of heaviness in extremities; increased mm fasiculations, or prolonged SOB

Question 15

Management of cervical weakness in ALS?

Answer 15

Often cervical weakness is a source of pain as it progresses

• May use a soft collar for neck pain initially (when strength is 3+ to 4/5), but by mid-stage, will need a semi-rigid or rigid collar to provide adequate support as weakness progresses (i/s/o a “drop head”) Options include:
• -> Philadelphia collar
• –> Miami J collar
• –> Malibu collar
• If the above are not tolerated, the Headmaster collar is a good lightweight option with an open frame to allow air circulation BUT it might not be enough support if rotation and lateral flexion are problematic

Question 16

Interventions for management of respiratory weakness in ALS?

Answer 16

Mid stage:

• Education re: pacing
• Breathing exercises including diaphragmatic breathing, resisted inspiratory exercises with spirometers (10 min sessions, 2-3x/day for 12-16 wks) may help respiratory fxn but evidence is inconclusive
• Body positioning to optimize breathing

Question 17

Bracing for management of dorsiflexor weakness in ALS?

What’s better for mild/moderate vs moderate foot drop?

Answer 17

POSTERIOR LEAF SPRING (PLS): good for mild to moderate foot drop. Has medial and lateral trim lines posterior to malleoli, somewhat flexible

FLOOR REACTION ORTHOSES (FROs) (e.g. ToeOFF Braces); good for mild-to-moderate foot drop WITH quadricep weakness. Brace also helps to compensate for ankle PF weakness d/t design which leverages ground reaction forces to offer a “push” at toe-off to assist w/propulsion and compensate for PF weakness. They also create a knee extension moment to help counteract quad weakness and tendency for knee buckling. They’re nice because they’re light, attach from the shin (so they’re easier to fit into a shoe) and will accommodate progressive girth changes in his calf d/t atrophy

CARBON-FIBER LATERAL or POSTERIOR STRUT DORSIFLEXION ASSIST BRACES: Good for moderate foot drop, also helps w/knee control. Fairly light weight.

HINGED (articulated) AFO: good for moderate foot drop w/ our w/out spasticity. They include an ankle joint, which allows for easier sit to stand transfers than a solid AFO. Antispasticity features (e.g. a PF stop) can be incorporated as needed. These are heavier than carbon fiber options. You need sufficient knee extensor strength for optimal use

Question 18

PT RECS:

Initial visit through early stage ALS

Answer 18

Initial visit through early stage ALS

• AEROBIC exercise (walk, cycle, possibly TM) at MODERATE intensity (65-85% HRMax)
• Fxn’l STRENGTHENING - focus on core and postural mm
• FLEXIBILITY: neck, trunk flexors, shoulder ext/IRs, hamstrings, gastroc/soleus
• RECREATION/FUN: group walking or aquatic programs, dancing, video games
• EDUCATION: re: dz, support groups, energy conservation

Question 19

PT RECS:

Middle stage ALS

Answer 19

Middle Stage ALS

• AEROBIC exercise (walk, cycle, possibly TM) at MODERATE intensity (65-85% HRMax)
• Fxn’l STRENGTHENING - focus on core and postural mm; functional strengthening and balance exercises to minimize deficits d/t muscle disuse
• FLEXIBILITY: neck, trunk flexors, shoulder ext/IRs, hamstrings, gastroc/soleus
• RECREATION/FUN: group walking or aquatic programs, dancing, video games
• EDUCATION: re: dz, support groups, energy conservation; use of assistive devices and orthotics to improve gait efficiency and safety
• PAIN management if needed
• BREATHING EXERCISES to maintain respiratory function
• ADAPTIVE devices to assist w/ADLs
• REFERRALS to approp health care professionals

Question 20

PT RECS:

Late stage ALS

Answer 20

Late stage ALS

• EDUCATION w/family and caregivers re:
• –> Guarding/assist during transfers and gait, fall prevention strategies
• –> Bed positioning and turning
• –> ROM exercises (likely passive at this stage)
• –> Assistive coughing and pulm hygiene
• Wheelchair prescription
• LOTS of psychologic and emotional support to pt, family, and caregivers

Question 21

Huntington’s Dz

Onset age?
Most common in… (ancestry?)
Caused by…

Pathology = severe loss of neurons in the __ and ___, with subsequent ____. This results in shrinking of the ___ and dilation of the ___.

Why do we see the symptoms we do, from a pathophys standpoint?

Answer 21

Huntington’s Dz
- Onset age? 30s-50s. Can also occur <20yo (juvenile HD) and >59yo (late onset)

• More common in European ancestry
• Genetic: Autosomal dominant inheritance (each kid of a person w/HD has a 50% chance of inheriting the fatal gene; everyone w/the gene will develop the dz). Can get genetic test. Caused by mutated Huntington gene on chromosome 4 that contains expanded CAG triplet repeats compared to normal gene <=26 CAG repeats = Normal
  27-35 = Intermediate risk meaning next generation is at risk
  36-39 = Reduced penetrance meaning some but not all individuals in this range will develop HD but next generation will
  >=40 = individual WILL develop HD, kids have 50% change of inheriting the dz
  Normally, the gene produces a protein (huntingtin) which is important for nerve cell functions. Mutant huntington protein is cut into smaller toxic fragments that bind and accumulate in neurons, disrupting their neuronal functions -> neuronal death.

Pathology = severe loss of neurons in the CAUDATE and PUTAMEN, with subsequent ASTROCYTOSIS. This results in shrinking of the CAUDATE and dilation of the ANTERIOR HORNS OF THE LATERAL VENTRICLES. Also see neuronal degeneration within the temporal and frontal lobes of the cerebral cortex, globus pallidus, thalamus, subthalamic nucleus, substantia nigra, and cerebellum.

Early HD: we see a loss of neurons that project from the striatum (medium-sized, “spiny” neurons) in the indirect (D2) pathway -> disinhibition of the thalamus -> cortical excitation -> hyperkinetic or choreic movements

Late HD: We see loss of not only striatal projection neurons via the D2 (indirect) pathway as in early HD, but ALSO loss of projection neurons via the D1 (direct) pathway as well as cortical neurons -> relatively hypokinetic state

Motor, cogntiive, and psychiatric signs and symptoms progressively worsen over time

Question 22

Huntington’s Pre-manifest stage

Symptoms

Common impairments and activity limitations

Primary PT aim

Answer 22

SYMPTOMS
Motor: NO real motor symptoms, mild gait changes

Cognitive: Difficulty with complex thinking tasks

Psych: Depression, aggression, irritability

IMPAIRMENTS/ ACTIVITY LIMITS

• Mild Cognitive and behavioral changes
• Mild gait and balance changes

PT GOAL
- Advise re: appropriate physical activites

Question 23

Huntington’s Early stage

Symptoms

Common impairments and activity limitations

Primary PT aim

Answer 23

*This is when the disease is diagnosed clinically

SYMPTOMS
Motor: Mild chorea, decreasing RAM, increased muscle stretch reflexes, abnormal extraocular movements (difficulty w/slow pursuit and fast saccades)

Cognitive: Mild problems planning, sequencing, organizing, and prioritizing tasks

Psych: Sadness, depression, irritability

IMPAIRMENTS/ ACTIVITY LIMITS

• Chorea, often limited to hands
• Balance problems (SLS, turning and changing directions quickly)
• Mild visuospatial and cognitive deficits

PT GOALS

• Delay the onset of mobility restriction
• Aerobic: 65-85% max HR, TM cycling, etc.
• Flexiblity: stretch trunk flexors, shoulder ext/IRs, hamstrings, and gastroc/soleus
• Fxn’l strengthening: focus on core and postural mm
• Challenging balance/gait; add cognitive and manual dual task to train dual an dmulti tasking
• Recreational and funactivitie (group walking/aquatic programs, dancing, video games)

Question 24

Huntington’s Middle stage

Symptoms

Common impairments and activity limitations

Primary PT aim

Answer 24

SYMPTOMS
Motor: Chorea, dystonia, rigidity and spasticity, voluntary movement abnormalities, decreased coordination, difficulty holding things, balance deficits/falls, dysphagia/dysarthria

Cognitive: Intellectual decline, memory loss, perceptual problems, lack of insight of self awareness, difficulty with dual tasking

Psych: Apathy, perseveration, impulsivity, antisocial and suicidal behavior, paranoia, delusions or hallucinations

IMPAIRMENTS/ ACTIVITY LIMITS

• Moderate to severe chorea
• Bradykinesia, dystonia
• Gait and balance problems, falls
• Incoordination
• Fatigue
• Difficulties w/ADLs

PT GOALS

• Maintain function, delay further deterioration
• Task-specific practice of functional activities; train dual tasking while still able to rehab it (or compensate if needed)
• Environmental modification (removing/padding sharp edges, clearing obstacles, knee/hip/elbow pads)
• Rollators can increase stability and decrease variability with gait
• Visual & auditory cues to imiprove movement initiation, size, and speed
• Motor imagery can help!
• Train dual task while you can -> compensatory techniques as needed
• Continue w/flexibility, strength (postural mm, extensors), and aerobic (65-85% HRmax)

Question 25

Huntington’s Late stage

Symptoms

Common impairments and activity limitations

Primary PT aim

Answer 25

SYMPTOMS
Motor: bradykinesia, rigidity and spasticity, severe voluntary movement abnormalities, dysarthria, dysphagia, incontinence

Cognitive: Global dementia

Psych: Delirium

IMPAIRMENTS/ ACTIVITY LIMITS

• Bradykinesia
• Hypertonicity
• Decreased eye movements
• Dependence with mobility, self-care, and ADLs
• Dementia
• Dysarthria and dysphagia
• Weight loss

PT GOALS

• Limit impact of complications
• Must weigh benefits/risks of falls while ambulating vs inactivity/atrophy associated with recommending against ambulation (and impact on psyche) - loss of amb is #1 reason associated w/admission to nursing home

Question 26

How is HD diagnosed?

What’s the prognosis?

Answer 26

+ family history of HD, presence of typical motor signs, and symptoms of HD are usually enough for diagnosis, then a +genetic test confirms for individuals with symptoms

PET and fMRI can detect striatal atrophy in affected brains as much as 11 years before symptom onset, may help in dx and tracking dz progression

Duration of illness varies considerably; mean is ~19 years (ranges from 15-25)

Juvenile and late onset patients have the shortest duration (i.e. earlier mortality)

Mortality often d/t complications from falls, malnourishment, infections, and PNA

Question 27

What makes juvenile onset HD unique from adult-onset HD?

Answer 27

• Symptom onset <20yo
• Tend to have the largest #CAG repeats
• Presenting symptoms: rapid decline in school performance, subtle changes in handwritin, bradykinesia, rigidity, tremor, and myoclonus similar to the presentation of PD
• Some have seizures (NOT common in adult onset HD) and mental disability
• The earlier the onset, the greater tendency for a faster disease progression - death often within 10 years

Question 28

HD meds…

• Anti-choreics / antipsychotics?
• Anti depressants?

Any side effect concerns?

Answer 28

ANTI-CHOREIC / ANTIPSYCHOTICS:
Side effects include extrapyramidal symptoms (due to imbalance btwn LOTS of DA and lower amounts of ACh = , drowsiness, akathesia (feeling like you have to move all the time)
- Dopamine-depleting: tatrabenazine (Xenazine)

• Atypical antipsychotics: Olanzapine (Zyprexa), Risperidone (Risperdal), quetiapine (Seroquel), ziprasidone (Geodon), Aripipazole (Abilify)
• Neuroleptics: Halperidol (Haldol), Fluphenazine (Prolixin)

ANTIDEPRESSANTS: side effects include insomnia, GI upset, restlessness, weight loss, dry mouth, anxiety, HA
- SSRIs: Fluoxitine (Prozac), Citalopram (Celexa), Sertraline (Zoloft), paroxitine (paxil)

• Tricyclic antidepressants: Amytriptyline (Elavil), Nortriptyline (Pamelor)
• Other meds: Bupropion (Wellbutrin), Venlafixne (Effexor)

Question 29

Outcome measures to assess change over time?

Answer 29

Unified Huntington’s Disease Rating Scale (UHDRS)

*Of note, the 6MWT, TUG, and 10MWT (though they have good test-retest reliability) often have pts with late stage of HD do better than those in mid-stage! Might not be your best bet. MDCs on all of these exist, but vary based on stage

Tinetti and 4-square step test were highly correlated with spatiotemporal measures of gait in HD! 4-square step test can be very difficult d/t cognitive issues/sequencing - this one is best for early stage HD.

ABC scale is not wonderfully reliable or valid in HD

Question 30

Unified Huntington’s Disease Rating Scale (UHDRS)

Answer 30

Used to monitor dz progressions and effects of therapy

Scored 0 (normal) - 4 (severe) = HiGHER score is MORE IMPAIRED

Assesses motor, cognition, behavior, and function
MOTOR subscale (impairment level) = each item 0-4, max 124 is most impaired
- Occular pursuit, saccade initiation and velocity
- Dysarthria, tongue protrusion (pts have difficulty maintaining force to keep tongue out)
- Maximal dystonia (trunk and extremities)
- Maximal chorea (face, mouth, trunk, extremities)
- Retropulsion pull test (reactive balance when pulled backwards)
- Finger taps (looks at bradykinesia; normal = 15 in 5 sec)
- Pronate/supinate hands
- Luria (looks at motor sequence of fist-hand-palm test)
- Rigidity-arms (R and L)
- Bradykinesia (body)
- Gait (looks at speed, BOS)
- Tandem walking

Cognitive battery includes:

• Verbal fluency test (max words in 60 sec for F or P - shows frontostriatal fxn)
• Symbol Digit modalities Test (pairing #s with geometric figures in 90 sec; looks at complex scanning and visual processing) - highly associated with falls!
• Stroop Test (color naming, word reading, intereference test; looks at executive functions (selective attention, cognitive flexibility, processing speed)

Functional assessment (yes/no):
- Looks at Activity and Participation level (e.g. can pt manage finances? handle money? engage in employment? prepare meals/laundry? Get in/out of ed? Walk w/out falling? Use toilet without help?)

Total functional capacity scale
Used to divide HD into early, (TFC stage I), middle (TFC stage II and III) and late (TFC stages IV and V) stages
- Able to live at home through stage III (TFC 3-6), home vs SNF at stage IV, full time SNF at stage V (unable to do job, finances, chores, or ADLs)

Question 31

HD PT eval

Answer 31

Body Function/Structure
- ROM and posture (flexed posture, may loose back extension and strength d/t dz + disuse)
- Examine for motor impersistence
- CNs: often have impaired saccades and smooth pursuit that begin early in dz, may impact balance/walking
- Chorea: not typically impacting function, unless severe. Look at extremities, trun, and face.
—-> Describe as absent, intermittent, or constant
—-> Mild (small movements, don’t impede function); moderate (small to mod size movements, may impede function), or marked (medium to large motions, DOES impede fxn)
- Coordination: especially eye-hand coordination (know what their job is and what is required of them!)
- Balance: static (SLS often hard and is impaired early; easy to quantify) and anticipatory, reactive balance (Pull test: can use UHDRS ratings!)
- Dual-Task cost (e.g. Walking while talking test)
Calculate % detriment by:
Dual task score - single task score) / single task score)

Question 32

Big PT concepts in HD management

Answer 32

• Enriched environments (lots of physical, mental, and emotional stimulation) helped!
• —> in animal/mice models, those mice maintain motor function longer and delayed loss of peristriatal cerebral volume!
• Exercise may be NEUROPROTECTIVE in people with HD and should be started early!
• —> Stimulation and wheel running started early can delay the onset of some motor deficits!
• Gym-based supervised aerobic exercise program is feasible and safe; no training effect at lower intensity (1x/wk + walking 2x/wk for 12 wks) but mental scale of SF-36 improved, but IMPROVED VO2 max with higher intensity (3x/wk 50 min sessions for 2 wks) w/good adherence/retention
  (COMMET-HD = low intensity
  EXeRT-HD = higher intensity)
• Home-based DVD exercise program (Move to Exercise) showed significant improvements in UHDRS motor, Berg, and PPT! Good especially if pt has transportation barriers/fatigue.
• Home based task specific training (e.g. walking, sit to stand) 2x/wk for 8 weeks was feasible, acceptable, and exceeded goals (TRAIN-HD)
• Clinic-based PT supervised exercise (1hr, 2x/wk for 6 wks focused on transfers, walking, balance, coordination, postural stability) -> significant changes in %double support, stride length, gait velocity, TUG, and Berg (Bohlen et al)

Frequency/Duration:
Go for short bouts of exercise, frequently t/o week so it is less fatiguing. Can use pedometer (early stages)!

Mode: go for FUNCTIONAL skills that pt wants to improve

Work on strengthening postural muscles, trunk/neck/hip extensors

Question 33

Motor learning and HD?

Answer 33

• Striatum is involved in all stages of motor learning, particularly learned skills that consist of a sequence of movements.
• Like individuals w/PD, HD pts show deficits in motor sequence learning and speed-accuracy motor skill learning (e.g. typing, drawing, handwriting, shooting basketball)

Early stages:
- Still have cognitive ability to learn new tasks (e.g. stair negotiation) BUT maybe avoid activities that are highly demanding of RESPONSE INHIBITION, DIVIDED ATTENTION, and WORKING MEMORY (e.g. walking while talking on phone, driving on busy city street)

Mid to later stages:

• May need more REPETITION to learn new motor tasks d/t impaired short term memory and cognitive deficits.
• Use direct practice of the skill, BLOCKED PRACTICE

External cueing:

• Auditory cue w/walking: does help them modify cadence/gait velocity, but not perfectly synchronized w/sound, and definitely not synchronized w/dual task.
• Visual targets (e.g. perpendicular line on path, tied theraband between back legs of walker to step to target)

Motor imagery is LESS affected in individuals with HD than in Parkinsons, might help w/learning movements!

Dual tasking:

• Train this during the EARLY to MID stage, then when they are no longer responding to the training, teach them to limit multitasking and break tasks down into discrete steps when possible
• Dual task training in PD does work, no direct research in HD.
• TUG cog vs walking while talking (depending on severity) can guide whether to train dual task vs just educate to avoid it.
• –> Can always try a rehabilitative approach for 2-3 weeks, then change to compensation if ineffective

Question 34

Parkinson’s disease affects the ___-containing ___ neurons in the ___ of the brainstem (midbrain). The pathologic hallmarks of PD are round intracytoplasmic inclusions called “___ ___” which consist mainly of misfolded __-___ protein, which results in [increased / decreased ] levels of __ in other parts of the basal ganglia.

Answer 34

Parkinson’s disease affects the MELANIN-containing DOPAMINE neurons in the SUBSTANTIA NIGRA PARS COMPACTA of the brainstem (midbrain). The pathologic hallmarks of PD are round intracytoplasmic inclusions called “LEWY BODIES” which consist mainly of misfolded ALPHA-SYNUCLEIN protein, which results in INCREASED levels of DOPAMINE in other parts of the basal ganglia.

Question 35

How is PD diagnosed?

Answer 35

No tests, but looking at samples from e.g. CSF, blood, and salivary gland/fluids, as well as biomarkers including alpha synuclein protein (seems to play a role in synaptic plasticity and degeneration), tau, DJ-1 protein, uric acid and others

Imaging techniques do not presently assist in the PRE-clinical diagnosis fo PD or in differentiating different Parkinsonian syndromes. Imaging biomarkers can be used to:

• ID presymptomatic individuals (probably yes)
• In a limited fashion, measure PD severity (but frequently is done poorly)
• Investigate pathyphysiology of PDs (if done carefully)
• Assist in differential dx of parkinsoniasim (in a limited fashion)
• In conjunction w/postmortem data, can assist in investigating pathology underlying cognitive impairment associated w/PD

In reality: Diagnosed clinically + with response to dopaminergic interventions

Question 36

Differentiate between:

• Primary Parkinsonism: idiopathic, makes up ~___% of cases
• Secondary Parkinsonism: which can occur following…(examples of precipitating events/ injury)
• Parkinson-plus syndromes including… (4)

Answer 36

Differentiate between:

• Primary Parkinsonism: idiopathic, makes up ~78% of cases
• –> SLOW variable rate of progression, insidious onset, then lasts 20-30 years post onset
• –> Usually one side of the body then progresses to bilateral
• –> How to assess disability?
• Hoehn and Yahr classification of Disability (5 stages: I= min dz involvement, V = severe involvement)
• Unified Parkinson Disease Rating Scale (UPDRS): contains mentation/behavior/mood, ADLs, and Motor Scales (looks at rigidity/impairments, activity, and some participation!)
• Secondary Parkinsonism: which can occur following…
• –> Brain injury from stroke
• –> Toxins (e.g. pesticides [MPTP, once laced on heroin and wipes out BG] and industrial chemicals - e.g. manganese which is a hazard for miners)
• –> Trauma (e.g. boxing)
• –> Infections (e.g. encephalitis, HIV)
• –> Metabolic abnormalitite (hypo or hyper thyroidism or para thyroidism, liver failure)
• –> Drug-induced (particularly in the elderly!) Withdrawal of the drugs typically reduces symptoms within a few weeks. Drug Induced PD largely from…
• Neuroleptic drugs (Chlorpromazine/Thorazine, Halperidol/Haldol, Thioridazine/Mellaril, Thithixiene/Navene)
• Antidepressants (Amytriptyline/Triavil, Amoxapine/Asendin, Trazodone/Desyrel)
• Antihypertensives (Methyldopa/Aldomet, Reseripine)
• Parkinson-plus syndromes including… (4)
• –> Progressive supranuclear palsy
• —> Multiple system atrophy
• –> Corticobasalganglionic degeneration
• –> Lewy body dementia

Question 37

Differentiate idopathic vs vascular PD in terms of…

• Age at onset?
• Disease duration?
• Response to Levodopa?
• MRI findings?
• Disease severity measures
• Postural tremor, gait disorders, and LE pyramidal signs?

Answer 37

Idiopathic PD:

• Significantly YOUNGER age at onset
• Longer duration
• GOOD response to L-dopa
• 93% have normal findings on MRI

Vascular PD

• SHORTER dz duration
• POOR response (only ~29% respond) to L-dopa
• ALL had cerebral vascular lesions visible on MRI
• UPDRS scores higher at baseline/onset, but more rapid decline compared to idiopathic PD
• More frequent to see postural tremor, gait disorders, and LE pyramidal signs compared to in idiopathic PD

Question 38

Parkinson-Plus Syndromes are neurodegenerative conditions OTHER than idiopathic PD that have Parkinsonism (clinically).

Clinical features suggestive of Parkinson-plus syndrome include:

• Lack of or diminished response to __
• Early onset __ or __ (clinical findings)
• Marked [asymmetry / symmetry ] of signs in the early stages of the dz
• [Truncal / limb] symptoms are more prominent
• Prominent motor ___
• Ocular signs such as impaired __ and __
• Autonomic symptoms such as __ and __ [early / late] in the course of the dz

Answer 38

Parkinson-Plus Syndromes are neurodegenerative conditions OTHER than idiopathic PD that have Parkinsonism (clinically).

Clinical features suggestive of Parkinson-plus syndrome include:

• Lack of or diminished response to LEVODOPA
• Early onset DEMENTIA or POSTURAL INSTABILITY
• Marked SYMMETRY of signs in the early stages of the dz
• TRUNCAL symptoms are more prominent than limb symptoms
• Prominent motor APRAXIA
• Ocular signs such as impaired VERTICAL GAZE and NYSTAGMUS
• Autonomic symptoms such as POSTURAL HYPOTENSION and INCONTINENCE EARLY in the course of the dz

Question 39

Primary or idiopathic Parkinsonism:
Makes up ~__ % of cases

[Fast / slow] rate of progression, [etiology?] onset, then lasts 20-30 years post onset

Usually one [unilateral / bilateral] initially, begins in [trunk / extremities}
—> Scales used to assess disability?

Answer 39

Primary or idiopathic Parkinsonism:

• –> Makes up ~78% of cases
• –> SLOW variable rate of progression, insidious onset, then lasts 20-30 years post onset
• –> Usually one side of the body then progresses to bilateral, initially observed in extremities
• –> How to assess disability?
• Hoehn and Yahr classification of Disability (5 stages: I= min dz involvement, V = severe involvement)
• Unified Parkinson Disease Rating Scale (UPDRS): contains mentation/behavior/mood, ADLs, and Motor Scales (looks at rigidity/impairments, activity, and some participation!)

Question 40

Progressive Supranuclear Palsy
- One of the [ rarest / most common] variants of the Parkinson-plus syndromes

• Age of onset?
• Caused by damage to the…
• Response to levadopa?
• Rate of progression relative to idiopathic PD? Duration of symptoms?
• Clinical features? (highlighting those that are key to diagnosis of PSP and differentiating it from PD)

Answer 40

Progressive Supranuclear Palsy
- One of the MOST COMMON variants of the Parkinson-plus syndromes

• Age of onset? 50s-60s
• Caused by damage to the… BASAL GANGLIA, BRAINSTEM, THALAMUS, and FRONTAL CORTEX
• Response to levodopa? About 20-30% respond initially to levodopa
• More RAPID progression than PD, with median survival of 5-6 years following symptom onest
• Clinical features? (highlighting those that are key to diagnosis of PSP and differentiating it from PD)
• Prominent EARLY postural instability and falls
• Limitation of VERTICAL GAZE especially downward (due to the degeneration of the rostral internucleus of Cajal in the midbrain can abolish ability to look up/down)
• Also have bradykinesia, truncal rigidity more common than rigidity, tremor (though this is rare in PSP, and very common in idiopathic PD), dysarthria/dysphagia, dementia, emotional and personality changes

Question 41

Multiple System Atrophy is a degenerative neurological disorder affecting autonomic failure

Corticospinal signs include

• [a / hypo / hyper] reflexia
• [+ / -] Babinski

Signs and symptoms

• Age at onset? Prognosis?
• Response to levodopa or dopamine agonists?
• Autonomic findings?
• Cognitive status?

Clinical features include varying degrees of…

• Autonomic changes?
• Movement/tone changes?
• Vision changes?

Answer 41

Multiple System Atrophy is a degenerative neurological disorder affecting autonomic failure

Corticospinal signs include

• HYPERreflexia
• UPGOING (abnormal) Babinski

Signs and symptoms

• Mean age of onset is ~54 years (range 33-78 years), with median survival of 9-10 years
• POOR response to levodopa or dopamine agonists
• Autonomic findings? Orthostatic HoTN in 68% of patients
• Cognitive dysfunction is LESS COMMON than in other Parkinson-plus syndromes (e.g. PSP and CBGD)

Clinical features include varying degrees of…

• Autonomic changes?
• –> Autonomic failure (41%)
• –> Severe orthostatic HoTN
• –> Urinary dysfxn (again, we tend to see these only LATE in idiopathic PD)
• –> Erectile dysfunction or impotence in men
• Movement/tone changes?
• –> Parkinsonism (46%)
• –> Asymmetric tremor (tends to be POSTURAL tremor, irregular, and jerky)
• –> Bradykinesia (hypokinetic dysarthria)
• –> Rigidity
• –> Postural instability
• –> Cerebellar dysfxn
• –> Gait and limb ataxia
• –> Ataxic dysarthria
• Vision changes?
• –> Sustained gaze-evoked nystagmus

Question 42

Multiple System Atrophy (MSA) is subdivided into two categories based on the neurosystem predominantly involved:

• MSA-P exhibits predominant parkinsonian symptoms secondary to ___ degeneration
• MSA-C exhibits prominent cerebellar symptoms secondary to ___ atrophy

When autonomic failure predominates, MSA is sometimes is called __-__ Syndrome

Answer 42

Multiple System Atrophy (MSA) is subdivided into two categories based on the neurosystem predominantly involved:

• MSA-P exhibits predominant parkinsonian symptoms secondary to STRIATONIGRAL degeneration (SND)
• MSA-C exhibits prominent cerebellar symptoms secondary to OLIVOPONTOCEREBELLAR atrophy

When autonomic failure predominates, MSA is sometimes is called SHY-DRAGER Syndrome

Question 43

Cortical Basal Ganglionic Degeneration CBGD) is another parkinsonian syndrome which involves atrophy of the __ and __ lobes and loss of __ in the substantia nigra.

Initial symptoms may first appear [ unilaterally / bilaterally] but eventually affect both sides of the body as the dz progresses

Onset: mean age is ___ years.
Prognosis?

Clinical features?

Answer 43

Cortical Basal Ganglionic Degeneration CBGD) is another parkinsonian syndrome which involves atrophy of the FRONTAL and PARIETAL lobes and loss of DOPAMINE in the substantia nigra.

Initial symptoms may first appear UNILATERALLY but eventually affect both sides of the body as the dz progresses (similar to idiopathic PD)

Onset: mean age is 63 (range 60-80 years) years, which is about the same as idiopathic PD), with severe disability and death within 6-8 years

Clinical features:

• Progressive dementia
• Limb apraxia (usually in one arm)
• Parkinsonism (rigidity, bradykinesia, NO resting tremor, marked DYSTONIA usually affecting 1 arm, myoclonus usually in one hand)
• Prominent SENSORY symptoms
• –> Visual or tactile spatial neglect
• –> Loss of graphesthesia
• –> Alien limb (“my hand/leg has a mind of its own”)
• Apraxia of speech, or nonfluent aphasia
• Impaired EYE movements (horizontal and upgaze), with downgaze usually not affected (vs in Progressive Supranuclear Palsy, DOWNGAZE is the one more affected)

Question 44

Lewy Body Dementia

Clinical features include early presentation of ___ symptoms. Additional clinical signs:

• Visual signs?
• Motor signs?
• Arousal/Cognitive signs?
• Symmetrical/Asymmetrical onset?

Age of onset? Prognosis?

Answer 44

Lewy Body Dementia

Clinical features include early presentation of COGNITIVE symptoms (many presentst o memory clinic and are dx with Alzheimer’s first). Additional clinical signs:

• Visual HALLUCINATIONS
• Motor signs?
• –> Rigidity (may be MORE severe than in idiopathic PD)
• –> Bradykinesia
• –> Neuroleptic sensitivity (may be fatal)
• –> Extrapyramidal signs of resting tremor (but less severe)
• Arousal/Cognitive signs?
• –> Fluctuations in cognition (>=5 point changes on MMSE over 3 tests in a 6 month period)
• –> Pronounced fluctuations in alertness and attention
• If motor changes are SYMMETRICAL, favors Lewy Body Dementia over PD

SLOW onset between ages 50-85
Median survival: 8 years after diagnosis

Question 45

Describe unique features of the following variants of idiopathic PD:

• Postural Instability Gait Disturbed (PIGD)
• Tremor Predominant
• Early Frontal Dementia with Bradykinesia and rigidity

Answer 45

Describe unique features of the following variants of idiopathic PD:

• Postural Instability Gait Disturbed (PIGD): dominant symptoms of postural instability and gait disturbance
• Tremor Predominant: tremor is main symptom; very FEW problems with bradykinesia or postural instability
• Early Frontal Dementia with Bradykinesia and rigidity

Question 46

PT INTERVENTIONS specific to Parkinson-Plus Syndromes:

Progressive Supranuclear palsy

Answer 46

Progressive Supranuclear palsy

• Big problem: EYE MOVEMENTS
• Work on EYE MOVEMENTS early in disease
• Later in dz, work on compensatory strategies for gait and transfers (e.g. cue to move head to look down to see obstacles and enhance safety)

Question 47

PT INTERVENTIONS specific to Parkinson-Plus Syndromes:

Cortical Basal Ganglionic Degeneration

Answer 47

Cortical Basal Ganglionic Degeneration

• Big problem: SENSORY issues
• Teach COMPENSATION. Work on using VISION when possible to compensate for apraxia
• Need LOTS of and MULTIPLE CUES (e.g. tools, objects, and visual models and feedback) (e.g. cue for tying sues by showing pictures of each step of the task)
• May not be able to use assistive devices due to apraxia if too severe

Question 48

PT INTERVENTIONS specific to Parkinson-Plus Syndromes:

Lewy Body Dementia

Answer 48

Lewy Body Dementia

• Big problem: COGNITION
• Consider using blocked practice and other strategies used for motor learning in individuals w/dementia
• For motor problems, treat as you would for PD

Question 49

PT INTERVENTIONS specific to Parkinson-Plus Syndromes:

Multiple System Atrophy

Answer 49

Multiple System Atrophy

• Big problem: AUTONOMIC dysfxn
• Screen for ORTHOSTATIC HoTN, teach strategies for dealing with it
• If pt has Cb features, work on COORDINATION exercises

Question 50

Hoehn and Yahr classification of Disability

Answer 50

I = min dz involvement

• –> Early on, may see ceiling effect on some standardized measures (MoCA, FGA, Mini BESTest)
• –> 5x Sit<>stand, 6MWT, and 9hole peg test will allow comparison to other adults pt’s age, lets you set better goals
• –> TUG cog and Fatigue scale may be helpful here
• –> Can do PDQ-8 (or 39) to look at QoL across all stages of dz

Mid-stage:
III = bilateral involvement, +falls
- Likely to demonstrate deficits on FGA, MiniBest, and MoCA which could be addressed w/therapy
—> 5x Sit<>stand, 6MWT, and 9hole peg test continue to provide good info re: ability to transfer, walk in community, and hand fxn

Late Stage dz (Stages IV - V)

• Outcome measures are trickier d/t may have floor effects on Mini BESTest, FGA, and MoCA (these are typically valid through stage IV, but too high level for H&Y 5)
• May need to change to 2MWT, but 9 hole peg test and 5x STS still useful until pt unable to use hands or a device, at which point you can no longer compare it to prior tests (so document it)

V = severe involvement

Question 51

How to quantify freezing of gait?

Answer 51

Freezing of Gait Questionnaire!
Assists with assessment of how freezing of gait impacts individual’s daily life

Scored 0 (freezing never happens with X example) to 4 (always happens with X activity)

Question 52

Motor Learning in PD - differentiate between PT intervention approaches in the early vs mid/later stages of dz

Answer 52

Early PD:
- Pt should retain (at least to some extent) the ability to motor learn! Can use interventions (e.g. TM training) and transfer any learning to overground walking
- Video games: you have to choose the RIGHT game. Ability to learn and transfer performance depends on COGNITIVE demands of the games - greater deficits in ability to transfer noted in games that need decision making, response inhibition, divided attention, and working memory.
Clients with PD WERE able to transfer motor ability trained on the game to a similar untrained task, as long as the cognitive demands weren’t too great (in H&Y stage I and II)
—> Similar considerations when making decisions re choosing a sport to improve motor function (e.g. basketball requires response inhibition when choosing who to guard on defense)
—> Train dual tasking in EARLY to mid stages
—> Start training ECCENTRIC training (hip/knee extensors, PFs, trunk extensors) early on to prevent decline!

Mid and Late stage dz: Interventions may require DIRECT practice of a skill in a BLOCKED practice (i.e. approach used with LSVT BIG training)

• PD pts are STIMULUS RELIANT. By mid stage, may need to adopt more compensatory strategy for dealing with initiation problems, e.g. auditory cue or visual cue. Downside is this limits pt’s independence and ability to function in natural environments (can’t say “big step” at work)
• Cueing is a strategy that bypasses the BG and uses cortical areas to initiate and control movements. Downside: motor control is kept COGNITIVE rather than AUTOMATIC in order to maintain performance. If pt tries to revert to automatic control, it is run through BG and movement degrades
• A self-given auditory cue OUT LOUD is MOST effective than an external cue (in a reaching study)!
• A MOVING visual target will better cue a movement than a stationary target. Visual targets for stride length should be perpendicular to walking path, at about normal step length.
• Micrographia (tiny handwriting) also responds to visual cues (e.g. dots on paper, connect letters to top and bottom dots)
• Mental rehearsal and visualization can also help to prepare for initiation challenges. Can augment this with external cue to engage premotor cortex. OR can cue pt to focus on a critical aspect of movement (e.g. LIFT your foot over the object)
• Aim for EXTERNAL focus of control, but avoid confounding instructions , make instructions specific enough for pt to understand what exactly they’re being asked to concentrate on
• Unclear if pts in midstage will respond to dual task training, so try it! But if no response after 2-3 wks of training (3-4 PT visits), switch to compensatory methods: limit multitasking, break activities down into discrete steps when able - use measures of TUG vs TUG cog to assess dual task cost (dual task score - single task) divided by Single task score.
• Eccentric training improves strength, 6MWT scores, and stair climbing (ie improves fxn’l abilities!) - focus on hip/knee extensors + PFs to improve stair climbing, maybe also some eccentric strengthening to back extensors

Question 53

TM trainign in PD?

Answer 53

• Pace-controlled TM training (~10% greater than pt’s self-selected OG walking speed) improves gait parameters including velocity and stride length
• Goal initially is to get him to normal walking speed for his age, then use maintenence program to keep this up
• NO BWS, but can use harness for safety if needed
• Need to steadily increase speed
• To improve walking, work on WALKING (no running)
• TM training leads to neural plasticity in the brain!

Question 54

Dance and PD?

Answer 54

Trials support partnered dance (e.g tango), but both partnered and non-partnered dance show equal benefits on motor fxn (but partners are fun!)

Question 55

Cycling and PD?

Answer 55

Pace setting is KEY, best done on tandem bike w/high consistent pedaling cadence (set by an experienced cyclist) ->
Improved scores on UPDRS

Question 56

LSVT BIG in PD?

Answer 56

Improves movement amplitude and speed, and retains this overtime.

Needs certification through LSVT BIG program

LSVT Speech: Many strong studies show significant improvement in speech volume, enunciation, and rate!

Can improve brady and hypokinesia at mid-stage disease, then continue w/home program

LSVT covered by insurance ONCE pt is showing deficits (ie around mid stage)

Question 57

Boxing and PD?

Answer 57

NOT actually taking punches or punching someone else

Boxing (including stretching and strengthening) leads to the same improvements as stretching, strengthening, and balance exercise programs

So, if pt is interested in it, go for it! No RCTs done yet.

Question 58

Tai Chi and PD?

Answer 58

Key to know the type of Tai Chi being used - want to use a form of Tai Chi that encourages BIG movements, feel weight shifts and focus on that (not any forms of Tai Chi that focus on small movements)

Tai chi works as well as standard exercise, particularly if pt enjoys it! Often has a group/social benefit

Question 59

Yoga and PD?

Answer 59

Higher-speed yoga specifically designed for PD (compared to PWR! training) was equally effective

But not a lot of research to support recommending yoga to address motor issues r/t PD, but can improve mode and QoL

Question 60

PWR! and PD?

Answer 60

No studies

Program incorporates evidence into the educational programming, instructors use that knowledge when designing classes

If PWR! were available

Question 61

Summary exercise recommendations for PD at:

• Early stage
• Mid stage
• Late stage

Answer 61

Early stage:

• Exercise that incorporates BIG movements, lots of reps of “normal” movements
• ECCENTRIC strengthening, focus on extensor mm groups
• Include some walking at a pace FASTER than his usual and equivalent to normal walking speed on a treadmill
• Fun group activities (e.g. dance, boxing) or group exercise programs focused on PD (e.g. PWR! and appropriate/large-movement Tai Chi)

MID STAGE

• All of the above plus…
• Use self-cue to initiate movement
• Use auditory and visual cues to improve movement speed and initiation
• EXTERNAL focus when learning new movement/activity or when practicing a skill
• Train dual task!
• Will likely need an assistive device eventually - rollator walkers are beneficial. If freezing/forward weight shift are an issue, consider having a bike shop reverse the brakes
• Might benefit from a bout of LSVT Big or tandem cycling at fixed/forced pace (set by an experienced cyclist)

LATE STAGE

• Continue exercise program for strengthening and big/normal movements, with modifications for balance challenges as needed
• Continue fun group activities (e.g. boxing, dance, PD-focused group exercise and Tai Chi as feasible)
• Encourage use of self-cues, auditory, and visual cues
• LSVT big therapy may be beneficial early in this stage
• Teach compensatory strategies for dual tasking and initiation and freezing issues