Parkinson’s Plus Syndromes & Dystonia

What are Parkinson-Plus Syndromes?

A group of neurodegenerative disorders with the same symptoms as idiopathic PD: neuronal damage to the substantia nigra and additional neuro symptoms that are not characteristic of PD

List some features that are suggestive of Parkinson-Plus Syndrome

• Lack of poor response to anti-Parkinson drugs

• Symmetrical signs at onset

• Lack of or irregular resting tremor

• Rigidity greater in the trunk than the extremities

• Early onset dementia

• Frequent falls

• Autonomic symptoms

• Visual signs

• Cerebellar signs

• Motor apraxia

What is the most common Parkinson-Plus syndrome?

Progressive supranuclear palsy (PSP)

If someone has PSP, is axial or limb rigidity more prominent?

Axial rigidity

Because someone with PSP has axial rigidity, do they more commonly have a backwards or front-facing lean?

Backward

What is the hallmark sign of PSP?

Vertical supranuclear gaze palsy (downward > upward)

What is the median survival rate of PSP?

6.2 yrs

If you are working with someone with PSP, what should the focus of your early training be on?

• Balance activities

• Helping them learn to scan the environment for obstacles to maneuver around via gait training, balance re-ed, rhythmic auditory cueing

When choosing an AD for someone with PSP, what factors do you need to balance? Which AD is most commonly best?

• Gotta balance support from AD AND their ability to see objects

• Rollator walker is the best

What is multi-system atrophy (MSA)?

• Cerebellar dysfunction

  • Gait and limb ataxia

  • Ataxic dysarthria

  • Sustained gaze-evoked nystagmus

  • Early gait impairment

• Autonomic dysfunction

  • Severe OH

  • Urinary and erectile dysfunction

What is the median survival rate for someone with MSA?

9.7 yrs

With what Parkinson-Plus Syndrome should you examine OH because it can be real severe?

MSA

Your pt struggles with OH, what are some things you can tell them to do to help manage their OH?

• have them transition slowly from supine to site and sit to stand

• Have em do ankle pumps and other gastroc exercises before having them stand up

What are the variants of MSA?

• Striatonigral degeneration

• Olivopontocerebellar atrophy

• Shy-Drager syndrome

What are the predominant symptoms in the variant of MSA, striatonigral degeneration?

Parkinsonian symptoms (MSA-P)

What are the predominant symptoms in the variant of MSA, Olivopontocerebellar atrophy?

Cerebellar symptoms (MSA-C)

What are the predominant symptoms in the variant of MSA, Shy-Drager syndrome?

Autonomic symptoms

What are the S/S of cortical basal ganglionic degeneration?

• Coordination real severely impaired

• Limb apraxia

• Dystonia (predominantly in 1 extremity)

• Bradykinesia

• Focal rigidity

• Alien limb phenomenon

• Early cognitive dysfunction with progressive dementia

What is alien limb phenomenon?

Involuntary motor activity of a limb along with the feeling that the limb doesn’t belong to a person

If someone has cortical basal ganglionic degeneration, how many extremities do they commonly have dystonia in?

1 extremity

You are working for someone with cortical basal ganglionic degeneration, what should the focus of your interventions be?

• Eval & train coordination within function (keep an eye out for apraxia)

If someone has dementia with Lewy bodies, do cognitive symptoms or Parkinsonism features appear first?

Cognitive changes

If someone has dementia with Lewy Bodies, do their Parkinsonian motor symptoms appear symmetrically or asymmetrically at onset?

Symmetrically

What is a good tool to track the progression of cognition in someone with Dementia with Lewy Bodies?

MoCA

What Parkinsonism-Plus Syndrome will commonly result in fluctuations in alertness, attention, and cognition?

Dementia with Lewy Bodies

Which Parkinsonism-Plus Syndrome typically progresses slower than idiopathic PD?

Dementia with Lewy Bodies

In the early stages of Dementia with Lewy Bodies, what should the focus of interventions be on?

Safety and problem solving because their cognitive deficits are not very severe yet

Is motor learning more impaired in PD or Dementia with Lewy Bodies?

Dementia with Lewy Bodies

Does someone with Dementia with Lewy Bodies benefit from blocked pracatice or random practice?

Blocked practice

What is the root cause of Parkinson Plus Syndromes?

Loss of DA

What is the main issue for someone with PSP?

Visual scanning

What is the main issue for someone with MSA?

OH management

What is the main problem for someone with corticobasal degeneration?

Ataxia and apraxia

What is the focus of interventions for someone with Dementia with Lewy Body?

• Blocked practice

• Implicit learning

What is dystonia?

Involuntary sustained postures and repetitive movements that result from co-contraction of agonist and antagonist muscles that are patterned, twisting, and will be tremulous

Describe primary dystonia (idiopathic)

When dystonia is their only movement disorder and there’s no secondary etiology

Describe secondary dystonia

Dystonia that is inherited, due to acquired causes, a result of PD, or PD plus syndromes

What is the most frequent presentation of dystonia?

Adult-onset focal dystonia of cervical musculature (69%)

Treatment of dystonia is focused on what?

Symptomatic relief since there ain’t no cure or neuroprotective strategy

What are the different ways you can treat dystonia?

• Meds

  • Dopaminergic replacement, DA blockers, anticholinergics, baclofen, benzodiazepine as muscle relaxant

  • Botulinum toxin injections

  • Surgery (rhizotomies, peripheral denervation, DBS)

There is strongest evidence for what treatment for dystonia?

Botulinum toxin injections

What things can PT do for PT treatment?

• Motor learning exercises

• Passive or active mobs

• Stretching or dystonic muscles

• ROM exercises to prevent or minimize contractures

• Bracing to improve posture and act as a sensory cue

• Relaxation and EMG biofeedback or TENS