Presented during: PSP-MSA-CBD Caregiving Symposium
Symposium Co-Hosts: Stanford Movement Disorder Center and Brain Support Network
Date: June 14, 2025
Jessica Ng, MD
Movement Disorder Specialist, Stanford
REM Sleep Behavior Disorder – Short Video
Brief Summary – “What Caregivers Can Do For PSP-MSA-CBD”
Summary by: Elizabeth Wong, Stanford Parkinson’s Community Outreach
Dr. Jessica Ng, a movement disorder specialist from Stanford, presented information on assessing and treating atypical Parkinsonism, including Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP), and Corticobasal Degeneration (CBD). She emphasized the important partnership between neurologists and caregivers in developing effective treatment plans.
Dr. Ng provided overviews of MSA, PSP, and CBD, categorized under “Parkinson’s Plus” syndromes, highlighting their common motor symptoms like bradykinesia and rigidity, as well as autonomic nervous system involvement in MSA. Dr. Ng discussed the diagnostic challenges, relying primarily on clinical examination, though tests like dopamine transporter (DAT) scans can offer supportive information. Carbidopa/levodopa (Sinemet) is often introduced as an initial treatment option, with its potential benefits and side effects.
Dr. Ng explored both motor (e.g., walking/balance issues, dystonia, bulbar symptoms) and non-motor symptoms (e.g., sleep, orthostatic hypotension, constipation, urination issues), presenting various treatment approaches including physical and speech therapy, Botox®, medications, and lifestyle adjustments.
A significant portion of her talk was dedicated to practical tips for caregivers to optimize neurology visits, stressing the importance of preparing symptom lists, medication updates, and raising safety concerns.
Finally, Dr. Ng talked about the vital need for caregivers to prioritize their own well-being, acknowledging the inherent challenges and encouraging them to seek support for stress, anxiety, and depression from healthcare providers and support groups.
Comprehensive Notes – “What Caregivers Can Do For PSP-MSA-CBD”
Notes by: Elizabeth Wong, Stanford Parkinson’s Community Outreach
OVERVIEW OF ATYPICAL PARKINSONISM (PARKINSON’S PLUS)
Multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD) are categorized under a group of progressive neurodegenerative disorders known as atypical Parkinsonism or Parkinson’s plus.
Parkinsonism refers to specific exam findings, characterized by:
- Bradykinesia: Slowness of movements, specifically a decrease in amplitude and speed with repetitive movements (e.g., finger taps getting slower and smaller).
- Rigidity or Rest Tremors: A common rest tremor is a slow, rhythmic tremor (4-6 Hz), sometimes described as “pill-rolling,” typically present when the limb is at rest and not actively engaged.
Causes of Parkinsonism include:
- Parkinson’s Disease (most common)
- Atypical Parkinsonism (MSA, PSP, CBD)
- Secondary causes (e.g., certain medications that induce Parkinsonian symptoms without the underlying disease).
Atypical Parkinsonism can initially present similarly to Parkinson’s Disease with motor symptoms, but typically shows more advanced symptoms within 1 to 3 years.
- MSA often presents with significant autonomic nervous system involvement, leading to incontinence, fainting (from low blood pressure), dizziness, and issues with balance and coordination.
- PSP commonly features eye movement problems (especially difficulty looking down) and early falls.
- Corticobasal Syndrome (CBS) / CBD frequently shows asymmetric symptoms, apraxia (inability to perform a learned movement despite adequate strength and comprehension), myoclonus (muscle jerks leading to dropping objects), and dystonia (involuntary muscle spasms causing posturing, often affecting one side). An “alien limb phenomenon” (involuntary, seemingly purposeful limb movements) and difficulty recognizing one’s own limb can also occur.
Atypical Parkinsonism typically progresses faster than Parkinson’s Disease.
CHALLENGES IN DIAGNOSIS AND TREATMENT
Diagnosis of atypical Parkinsonism is challenging and often delayed, with many patients initially diagnosed with Parkinson’s Disease.
Clinical examination and history remain the gold standard, as there is no single definitive test.
Supportive tests may include:
- Dopamine Transporter Scan (DaTscan): Shows loss of dopamine neurons but can be abnormal in both Parkinson’s Disease and atypical Parkinsonism, thus not definitive for differentiation.
- Skin Biopsy (for alpha-synuclein): Can be positive in MSA and PD, and rarely in PSP/CBD. While not definitive, a negative result in a suspected Parkinson’s/PSP case might favor PSP.
- Lumbar Puncture (Cerebrospinal Fluid): No specific biomarkers for MSA, PSP, or CBD.
- Brain MRI: May show certain findings supportive of MSA, PSP, or CBD, but a lack of findings does not rule out the diagnosis.
A trial of carbidopa/levodopa (Sinemet®) is often considered even if atypical Parkinsonism is suspected, as some patients (though fewer than in Parkinson’s Disease) may show symptomatic improvement.
- Purpose: Primarily to improve tremors, stiffness, and bradykinesia.
- Effectiveness: Improvement is generally less pronounced, shorter-lived, or absent compared to Parkinson’s Disease.
- Potential Side Effects: ○ Nausea: Common, often mitigated by slow dose escalation or additional medications.
○ Lightheadedness/Dizziness (Orthostatic Hypotension): A concern due to blood pressure lowering effects, especially in patients with existing autonomic symptoms.
- Hallucinations: Can worsen existing hallucinations or induce new ones.
- Dyskinesia: Involuntary wriggling movements.
- These considerations warrant discussion with the neurologist.
WALKING AND BALANCE ISSUES (MOTOR SYMPTOMS AND TREATMENTS)
Parkinsonian Gait: Slow, shuffling, hunched posture with reduced arm swing.
Postural Instability: Tendency to fall backward, common in MSA and PSP, sometimes leading to spontaneous falls.
Freezing of Gait: Sensation of feet “stuck to the floor,” often occurring at initiation, turning, or in narrow spaces/doorways.
Festination: Taking smaller, faster steps with difficulty stopping.
Treatments:
- Physical Therapy, Balance and Exercise Training are the mainstays.
- Stanford Neuroscience Wellness Classes offer free online/livestream resources (chair yoga, Tai Chi, mindful meditation) for those without easy access to PT or needing supplemental exercise.
- Visual or Musical Cues: Aids like laser canes (projecting a line to step over) can help with freezing of gait.
- Assistive Devices: Canes, walkers, and head protection for frequent fallers.
- Home Safety Evaluation: Recommended (by occupational therapists, ordered by neurologists or PCPs) to identify and mitigate hazards.
- Medications for Freezing of Gait: Donepezil, Rivastigmine (available in patch form for swallowing difficulties), Amantadine (can cause sleepiness, leg swelling).
DYSTONIA (MOTOR SYMPTOMS AND TREATMENTS) Involuntary muscle spasms or contractions leading to sustained abnormal posturing of the body (e.g., facial grimacing, head tilting, hand/foot posturing, spinal curvature, neck bending backward -retrocollis in PSP).
Primary Treatment: Botulinum toxin injections (Botox®).
- Mechanism: Relaxes the injected muscles, reducing involuntary contractions.
- Benefits: Localized effect, minimal systemic side effects.
- Drawbacks: Effects wear off after approximately three months, requiring repeat injections.
- Technique: EMG (electromyography) may be used to guide injections into the correct muscles.
- Injection Sites: Can be in feet (for toe curling), neck (for head tilting, though can cause swallowing issues), or around eyes (for involuntary eye closing).
Other Medications: Muscle relaxants (baclofen), clonazepam, trihexyphenidyl. These are generally less preferred due to systemic side effects like sleepiness and disorientation, which can worsen existing cognitive issues.
BULBAR SYMPTOMS AND TREATMENTS
Affecting speech and swallowing.
Treatment Approach (Minimalist Philosophy): Prioritize non-pharmacological interventions and treatments with the fewest side effects.
Dysarthria: Difficulty with speech articulation.
Treatment: Early involvement of speech therapy and a comprehensive swallowing study. Diet modifications based on study results.
Dysphagia: Difficulty with coordinated swallowing.
Treatment: Early involvement of speech therapy and a comprehensive swallowing study. Diet modifications based on study results.
Sialorrhea: Drooling (due to reduced swallowing, not excess saliva production).
Treatment: Botox® injections into salivary glands, or medications like scopolamine drops or glycopyrrolate (oral). Chewing candy can also help stimulate swallowing.
Stridor: A high-pitched sound, usually on inspiration, specific to MSA, which can indicate respiratory compromise and is an emergency.
Treatment: An emergency; often requires immediate referral to ENT for consideration of tracheostomy, which can be life-saving.
SLEEP ISSUES
Insomnia: Difficulty falling or staying asleep, often due to motor symptoms (stiffness, dystonia pain), frequent urination, anxiety, or rumination.
Treatments for Insomnia:
- Start with sleep hygiene (non-pharmacological).
- Cognitive Behavioral Therapy for Insomnia (CBT-I).
- Sleep study to rule out other causes like sleep apnea or restless leg syndrome.
- Medications: Melatonin, trazodone, mirtazapine (selected based on specific targets).
REM Sleep Behavior Disorder (RBD): Common in Parkinson’s and MSA; involves acting out vivid dreams, potentially leading to self-injury or injury to a bed partner (e.g., falling out of bed, hitting objects).
Treatments for RBD:
- Bed Safety: Lowering the bed, adding soft padding, removing hazardous objects, using bed rails.
- Consider removing bed partner if there’s a risk of injury.
- Medications: Melatonin (often at higher doses like 3-15mg) preferred over clonazepam due to fewer side effects, though clonazepam has stronger evidence.
Daytime Sleepiness: Can be profound and significantly impact quality of life.
Treatments for Daytime Sleepiness:
- Review and potentially adjust current medications (e.g., reducing carbidopa/levodopa or dopamine agonists if motor benefit is minimal).
- Adding caffeine (coffee/tea).
- Light therapy (e.g., 30 minutes daily or outdoor sun exposure).
AUTONOMIC NERVOUS SYSTEM ISSUES
This system controls involuntary bodily functions like breathing, digestion, and sweating.
Orthostatic Hypotension (OH): A significant drop in blood pressure (systolic >20 mmHg or diastolic >10 mmHg) upon standing, which can be delayed (up to 5-10 minutes).
Symptoms: Lightheadedness, dizziness (especially with position changes or prolonged standing), “coat hanger pain” (shoulder/neck pain), leg weakness, syncope (fainting).
Treatment:
- Rule out other causes (e.g., blood pressure medications, cardiac issues).
- Non-pharmacological measures (first-line):
- Hydration: Half body weight in pounds in ounces of water daily (e.g., 60oz for 120lb).
- Increased Salt Intake: Salty snacks mid-morning/afternoon.
- Avoiding Large Meals: Can lower blood pressure.
- Elevating Head of Bed: 20-30 degrees (wedge pillow if no hospital bed).
- Abdominal Binders and Compression Socks (thigh-high are best): Apply binder while lying down before standing.
Medications: Midodrine, fludrocortisone (can cause supine hypertension, i.e., increased blood pressure while lying down).
Constipation: Can manifest as hard/small stools, straining, bloating, nausea, loss of appetite, or alternating constipation/diarrhea (overflow diarrhea due to severe constipation). Can lead to malnutrition and reduced quality of life.
Management:
- Hydration (essential for fiber effectiveness).
- High-fiber diet.
- Prune juice (warm glass in the morning).
- Probiotics.
- Medications: Senna (stimulant), MiraLAX® (stool softener), or a combination.
- For nausea from medications, increasing carbidopa/levodopa can sometimes help.
Urination Issues:
Overactive Bladder: Urgency, difficulty holding urine, leakage, incontinence, frequent nighttime urination.
Retention: Difficulty with bladder emptying, feeling residual urine.
Management:
- Rule out structural causes (especially in men, by a urologist).
- Medications (e.g., Tamsulosin for retention; avoid oxybutynin if possible due to side effects).
- More advanced options (managed by urology): Bladder injections, nerve stimulations.
OPTIMIZING NEUROLOGY VISITS
Caregivers are crucial during neurology visits.
Before the Visit:
- Maintain a Symptom/Question List: Keep an easily accessible list (e.g., on a phone) to add symptoms or questions as they arise. Before the visit, review this list to prioritize the most important items for discussion, placing urgent concerns at the top.
- Update Medication List: Ensure the medication list is current, including the name, dose, and timing of each medication.
- Discuss Caregiver Role with Patient and Providers: Have a conversation with the person being cared for about the caregiver’s role, ensuring alignment with the patient’s desires. This discussion should extend to healthcare providers to establish:
- Access to the patient’s healthcare portal.
- Documentation of the caregiver’s authorization to discuss the patient’s care with providers.
- Formal healthcare power of attorney documentation, if needed.
- Research Clinical Trials: Caregivers are encouraged to explore clinical trials via clinicaltrials.gov. This resource allows filtering for open and enrolling studies, and caregivers may have more up-to-date information on specific trials for their loved one’s diagnosis than the neurologist.
During the Visit:
- Prioritize Concerns: Bring up the most pressing concerns and symptoms at the beginning of the visit. Avoid saving critical issues for the last few minutes.
- Report Safety Concerns: If the neurologist doesn’t ask, proactively mention any safety concerns: ○ Balance or imbalance issues.
- Falls (frequency and circumstances).
- Swallowing difficulties (medications are less effective for these; prompt referral to speech or physical therapists is crucial to prevent falls or aspiration).
- Dangers in the house (e.g., firearms, unsafe stairs, rugs).
- Unsafe behaviors (e.g., leaving the stove on, impulsive actions, unsafe driving). Neurologists rely heavily on caregiver feedback for these observations, as they are not present in the home environment.
- Ask What to Watch Out For: Inquire about specific symptoms or progressions to expect given the individual’s unique case, so you know what to monitor before the next appointment.
After the Visit:
- Ensure Contact Method: Confirm a way to contact the doctor or support staff for follow-up questions or updates before the next scheduled visit.
- Access Notes: Secure a way to access the assessment notes, which can be shared with other involved providers (e.g., primary care doctor, urologist) for coordinated care.
CAREGIVER SUPPORT AND WELL-BEING
Being a caregiver is incredibly challenging, often leading to feelings of resentment, regret, anxiety, depression, fatigue, and isolation. These feelings are common, and caregivers are not alone.
Caregivers are urged to prioritize their own self-care and openly communicate their struggles to healthcare providers.
Seeking help through support groups or therapists is strongly encouraged.
Question and Answer
Question: Do you have any tips for helping loved ones with postural instability or gait festination outside of physical therapy?
Answer: Working with a physical therapist knowledgeable in atypical Parkinsonism is vital. They can suggest additional cueing techniques (e.g., visual or musical cues) and methods like taking wider turns. Physical therapists can ensure proper use of assistive devices (e.g., holding a cane correctly, using a multi-point cane for better stability). A physical therapist can assess and recommend appropriate devices (e.g., a higher walker for individuals with a hunched posture to encourage uprightness). It’s crucial not to dismiss physical therapy but to ensure it’s utilized effectively. ——-
Question: Any idea on how to support someone with apathy to do their exercises?
Answer: Start with small, achievable goals. Instead of committing to an hour, aim for 10 minutes, perhaps multiple times a day. The exercise doesn’t need to be formal (e.g., a gym class); even a 10-minute walk outside before lunch and after dinner can be a good start. Gradually adding more can help, and seeing progress, even in small increments, can be motivating.
Question: What can be done for dysphagia and maintaining hydration?
Answer: If not already done, a swallowing study and consultation with a speech therapist are the first steps. This is crucial for safely ensuring hydration and preventing aspiration.
Question: A family member got a G-tube inserted 1.5 years ago. What could we expect to end their life?
Answer: This is a complex question with many variables, and it’s difficult to provide a general answer. (The speaker moves to the next question, indicating it’s beyond a brief scope).
Question: How do you tell the difference between stridor and snoring?
Answer: Recording the sound (audio or video) and playing it for the neurologist is the best approach. If needed, a sleep study can provide further diagnostic information.
Question: How to balance fluid restriction for heart failure with hydration needs for neurogenic orthostatic hypotension?
Answer: For patients with heart issues requiring fluid restrictions, prioritize non-fluid measures to manage orthostatic hypotension, such as compression socks, abdominal binders, and elevating the head of the bed. Specific medications for OH would be considered, but always require a consultation with the cardiologist to avoid adverse interactions with existing heart medications.
Question: A family member has one bowel movement per week. Is this safe? Is daily lactulose safe?
Answer: The safety and appropriate management depend on food intake and the patient’s symptoms. Lactulose can generally be used safely on a regular basis. Consistent, daily use of bowel regimens is key, and sometimes more than one medication might be required.
Question: How often should a swallowing study be done?
Answer: There’s no strict guideline. A swallowing study is usually done as a baseline for new Parkinsonism presentations. It should be repeated if new concerns about swallowing arise, but there’s no set annual frequency.
Question: Is respiratory therapy helpful for these conditions?
Answer: Respiratory therapy can be involved, especially for conditions like sleep apnea (where devices like CPAP/BiPAP are used) or stridor in MSA.
Question: For CBD patients with dystonia, will Botox® stop working? Are there other options if it seems less effective?
Answer: Botox® is unlikely to stop working entirely. If it seems less effective, it might be due to disease progression being faster than the relief Botox® provides. Options to discuss with the neurologist include:
- Increasing the Botox dose.
- Injecting different muscles.
- Shortening the interval between injections (e.g., every 10 weeks instead of 3 months).
Question: Do you recommend acupuncture for symptomatic treatment?
Answer: No, I don’t have personal experience and there is not a strong evidence base. However, generally, it does not contradict other medical treatments, so I would not advise against it.
Question: Any there benefits to a feeding tube? Should I discuss with my family members what they want?
Answer: Yes, definitely have this conversation with your family member to understand their wishes. The primary benefit of a G-tube is often to minimize the risk of aspiration.
Question: Is there anything to help with the alien limb phenomenon?
Answer: Physical therapy can be helpful. There is no specific medication primarily used to treat alien limb phenomenon.
Question: When a caregiver shares concerns and is obviously distressed, what do you, as the neurologist, do?
Answer: The neurologist views the relationship with the caregiver as a partnership and aims to ensure the caregiver is also well, as this contributes to the best care for the patient. Common actions include:
- Referring caregivers to support groups for connection with others facing similar challenges.
- Recommending they talk to their own therapists or healthcare providers for personal support.