Blog Archives

Dr. Aparna Wagle Shukla weighs in on the "Top 5" things you need to know about COVID-19 and dystonia care

4/14/2020

COVID-19 and Dystonia Care: What do you need to know?

While we patiently wait for the COVID-19 crisis to resolve, we approached leading dystonia experts for tips and advice on caring for patients. We were fortunate to sit down with one of the leading clinical and research experts, neurologist Dr. Aparna Wagle Shukla. She shared with us her “top 5” tips for dystonia care during the COVID crisis.

Who is Dr. Aparna Wagle Shukla?

Aparna Wagle Shukla MD is a physician-scientist who provides clinical care for patients with dystonia and she has focused her laboratory on identifying new treatments for focal and generalized forms of dystonia. Dr. Wagle Shukla is the clinical director for Tyler’s Hope Foundation for Dystonia Cure.
Dr. Wagle Shukla is an expert on dystonia treatments including pharmacological therapies (pills), botulinum toxin injections, surgical management (DBS), and also on rehabilitation based therapies.
Her lab develops novel treatment approaches including exercise. She has performed many studies involving the use of repetitive transcranial magnetic stimulation (TMS); a brain stimulation treatment currently approved by the FDA for treating medication refractory depression.
She has been funded by various dystonia organizations and also by the National Institutes of Health. She has published over 75 research articles in prestigious journals such as Brain, Neurology, Journal of Neurology Neurosurgery and Psychiatry (JNNP), Parkinsonism and Related Disorders and Movement Disorders.

What should every dystonia patient know about the Corona virus?

COVID-19 is a viral infection caused by a Coronavirus. The infection first emerged in December 2019 in Wuhan, China.
The World Health Organization named the infection COVID-19 (Coronavirus disease of 2019) and declared it a pandemic in March 2020. The infection has been spreading rapidly across the world….and some have compared it to a raging wildfire.
COVID-19 presents with symptoms of fever, common cold symptoms, cough, headaches, muscle aches, difficulty breathing, and in< 20% of patients pneumonia. The pneumonia may requiring hospital support.
Signs and symptoms of COVID-19 commonly appear two to 14 days after exposure to the Coronavirus.
There is currently no cure and there is no vaccine.
Those who believe they may have the illness should seek medical treatment immediately. We can help others and also help ourselves by taking many precautions.

THERE IS NO VACCINE FOR COVID AT THE CURRENT TIME WHICH MEANS PREVENTION IS THE KEY.

We can “flatten the curve” of rising infection numbers.
The curve refers to how many patients will present with the Coronavirus and how fast they will seek medical attention. If the curve is steep and goes straight up quickly, all of the infections will come at once and this will quickly overcome the healthcare system since we don’t have enough beds, ventilators, protective gear, etc. By socially distancing and isolating we can prevent a rapid peak, save lives and not overcome the capacity of hospitals.

What are important precautions every dystonia patient should take at home?

Wash your hands. Wash them often with soap and water for at least “20 seconds.” Be sure to wash your hands with soap, scrubbing all the surfaces between and around your fingers for at least 20 seconds and dry them thoroughly. When you cannot wash your hands, use a hand sanitizer that contains at least 60% alcohol.

Carry a small bottle of hand sanitizer. When in public spaces, use a hand sanitizer that contains at least 60% alcohol.
Clean the surfaces around you that you may contact. Stay as healthy as possible and use disinfectant wipes especially in public areas.
Avoid touching your eyes, nose, and mouth with unwashed hands.

How important is social distancing for dystonia patients? What is the risk of COVID-19 in dystonia?

If there is a COVID-19 outbreak in your community, stay home as much as possible as this will reduce your risk of being exposed.

There is not an increased risk of getting COVID-19 if you have dystonia.
There is however an increased risk for complications and you may have a more difficult recovery.
Social distancing means avoiding gatherings of people and trying to limit public interactions to only the absolute essentials such as groceries and gasoline purchases. Though note that even groceries can in many zip codes be delivered.

While in public settings such as grocery stores or when visiting a hospital, one should keep at least 6 feet distance between other people. This will help to avoid droplets containing the virus to be spread .
Avoid cruises and all non-essential air travel.
The CDC recommends that if in public wear a mask or something to cover your nose and mouth.

What should a dystonia patients know about medication management during the COVID-19 crisis? What about the need to see a doctor during the COVID crisis?

Write down or print a list of all your medications. Include the medication name, strength, times taken and dosages.
Stock up on supplies. When you have the option, you may consider switching prescriptions to 90-day supplies. This can reduces trips to the pharmacy.
Check all your medications. Take inventory of your list and re-order any that are running low.
Make sure you have made a list of your doctors with recent contact information and take it with you in the event of a hospitalization.
Call your doctor when you have your very first symptom.
Your doctor will likely want to monitor you including your dystonia symptoms and COVID symptoms and your doctor will make the decision as to whether you will need COVID testing.
If you have to attend the clinic, emergency room or hospital environment call before you go so arrangements can be made to keep you in a non-crowded waiting room.
Many doctors can manage you by telephone or telemedicine for your routine dystonia care as well as for possible COVID symptoms.

What do you tell dystonia patients with deep brain stimulation (DBS) devices during the COVID-19 outbreak?

We advise checking with your DBS provider on options for optimal care of the DBS system while in your home. Some health care providers or DBS centers provide dystonia patients with multiple possible “groups or programs.” This is useful as the patient can cycle through the settings without actually having to come in person to the clinic.
Some clinicians offer patients the option to change other settings at home (voltage, how wide are pulses, how many pulses)
During the early stages of programming following surgery, some patients will have worsening of their dystonia symptoms after a setting change. The worsening can in some cases be a consequence of “a part of the brain fighting the “positive changes.” In other words, the DBS can flare the dystonia in some cases, but if you wait, it could get better. Calling your doctor for advice if you worsen is a good idea.
One unique aspect to dystonia DBS is that clinical benefits may not manifest for weeks to months.
We recommend that dystonia patients do not turn off the DBS device especially at night. This could worsen symptoms in many cases. Additionally, re-activation of the device may result in an uncomfortable side effect.
If your DBS has been in place a long time it may be possible to be managed at home through the COVID crisis.
In some dystonia patients running out of battery can result in a serious flare in symptoms. You and your doctor should monitor battery life and plan for replacement to prevent rebound of symptoms. If your hospital deems the DBS battery an elective procedure you and your doctor may need to manage with medications until a replacement surgery is available.
Many centers consider DBS battery replacement as an urgent procedure.

Dr. Vaillancourt's "Top 5" updates in imaging and drug/device development in dystonia

4/5/2020

There are so many exciting projects going on in dystonia research that it has become hard to keep up with all of the advances. We sat down with one of the worlds top dystonia and Parkinson imaging researchers (Dr. David Vaillancourt) to talk about the “top 5” updates in therapies and in dystonia imaging research.

Who is David Vaillancourt?

David Vaillancourt is Chair and Professor in the Department of Applied Physiology and Kinesiology at the University of Florida.
He is funded by Tyler’s Hope for a Dystonia Cure to do cutting edge imaging research and he collaborates with UF researchers Yuquing Li and Marcelo Febo.

Yuquing LI, PHD.,
University of Florida

Marcelo Febo, PHD.,
University of Florida

Vaillancourt’s research focuses on how the brain regulates voluntary and involuntary movement with a specific focus on motor disorders.
He uses advanced neuroimaging techniques to study the functional and structural changes in the brain of humans and animal models.
He has used rehabilitative, surgical, and pharmacological interventions, and published his work in journals including Brain, Journal of Neuroscience, JAMA Neurology, Neurology, Human Brain Mapping, Neuroimage, Cerebral Cortex, and Neurobiology of Aging.
He has been continuously funded by NIH since 1999, and now directs several grants from NIH.
In dystonia he studies the pharmacological and neuroimaging in mouse and human models.

1) What is imaging and how is it used in dystonia?

Neuroimaging is a broad term. It encompasses imaging using positron emission tomography, magnetic resonance imaging, and other techniques. In positron emission tomography, the approach is to inject a drug or tracer into the blood stream. This drug or tracer can cross into the brain. The tracer is then imaged in an effort to measure how the neural receptors respond. This procedure can be useful in many ways because the tracer can be designed to be specific to the neural receptors in the brain.
In dystonia, the types of tracers that are mostly used are dopamine related tracers and cholinergic related tracers.
Other forms of imaging include functional magnetic resonance imaging and structural magnetic resonance imaging. These techniques examine how protons in water are affected by blood flow and tissue specific changes. These methods can help to pinpoint where in the brain dystonia can manifest and where in the brain specific new therapies should be targeted

2) What is a specific example in humans for how imaging is being used in dystonia?

In a recent study published in the journal Brain (Corp et al. 2019; PMID: 31099831) the authors used a clever approach. They studied people with cervical dystonia who had lesions in the brain (e.g. strokes, tumors) causing the dystonia. Whereas most types of dystonia do not have a visual lesion on the MRI, these authors focused on rare patients with visible lesions on their brain scan. Next, they used a technique called resting state functional MRI to map a “connectome” type network that presumably links all of these brain regions together.
The authors observed that two regions of the brain were connected to all of the brain lesions across all of the patients with cervical dystonia. Thus there were common nodes of dysfunction possibly underpinning the cause(s) of the dystonia. The two regions— somatosensory cortex and the cerebellum— were found to link to all of the lesioned regions across all of the cervical dystonia patients. This new finding tells us two things:
1) It tells us that these two regions may be critical for therapies targeting dystonia; and
2) It tells us that studies in dystonia may consider targeting therapies directly to these parts of the brain or to their connections.

3) What is a specific example in animals for how imaging is being used in dystonia?

The Calakos Laboratory at Duke University is working on new therapies to potentially help people with dystonia through a large alignment grant funded by Tyler’s Hope for a Dystonia Cure. Their work is focused on therapies that can target specific cellular pathways. They are focused on the eIF2alpha signaling pathway in DYT1 dystonia. In a recent paper (Rittiner et al. 2016; PMID: 27939583) the authors found that patient-derived cells and a mouse model both supported a pathogenic role for this pathway (e.g. it was likely involved in dystonia). The authors also found that this pathway had therapeutic potential and may help to develop a “drug-able” target.
Dr. Calakos is now working with Dr. Vaillancourt to test if a medication can be safely delivered to the brains of mice which are genetically similar to human DYT1 dystonia. We are using a high-field MRI that goes up to 17 Tesla (a typical MRI is 1.5-3 Tesla). The high-field MRI will provide enhanced signal for the tiny mouse brain so we can see the brain and the drug of interest.
The experiments will determine if the medication is affecting the brain tissue of the mouse. This type of experiment will help move us closer to a therapeutic for dystonia.

4) How can imaging be used in humans to assess a new therapeutic (e.g. drug, device)?

If a therapeutic (e.g. drug, device) is deemed safe for humans, it can be further studied
The safety is typically assessed in a Phase 1 study, which will examine different dose levels of a medication and will determine which levels are safe. If a Phase 1 study is successful, the drug can move to a Phase 2 study where safety and other data readouts will be assessed (e.g. how well it works, what pathways does it affect). In the Phase 2 study, imaging methods can be used to determine where in the brain a medication is influencing the system.
In moving from a Phase 2 to a Phase 3 study, the investment for a device or pharmaceutical company can be significant, and thus the company will typically want to see several “readouts” that show the medication could will have the potential to work on the disease (e.g. dystonia).

5) What does this all mean for people with dystonia?

I believe that these issues means several things and here is my interpretation:
1) People care about you and care about dystonia. There are labs across the world working on dystonia. They are conducting studies in cells, live animals, and in human clinical trials.
2) Treatments take time to develop. The studies that I have mentioned, took years to complete. There are many other studies in the field of dystonia and even though this is a big effort, it takes time--it is 100% worth it.
3) Stay the course. In research, sometimes research does not have a positive outcome. It can take several years to find out you are “going down the wrong path.” However, sometimes research yields a breakthrough that can change how we treat a patient and improve their quality of life. This is what we hope for and strive for…so stay the course! We are optimistic we are making progress in imaging and dystonia drug/device research!

Dystonia Tips and Research Blog

Dr. Aparna Wagle Shukla weighs in on the "Top 5" things you need to know about COVID-19 and dystonia care

Dr. Vaillancourt's "Top 5" updates in imaging and drug/device development in dystonia

Archives

Categories

Location:
13301 US Hwy 441
Alachua, FL 32615
(Located inside The InterMed Group's Corporate Office)

Mission Statement

Contact Us

​​Dystonia Tips and Research Blog

Dr. Aparna Wagle Shukla weighs in on the "Top 5" things you need to know about COVID-19 and dystonia care

Dr. Vaillancourt's "Top 5" updates in imaging and drug/device development in dystonia

Archives

Categories

Location:13301 US Hwy 441Alachua, FL 32615​(Located inside The InterMed Group's Corporate Office)

Mission Statement

Contact Us

Dystonia Tips and Research Blog

Location:
13301 US Hwy 441
Alachua, FL 32615
(Located inside The InterMed Group's Corporate Office)