Huntington Disease Awareness day is celebrated on 15th May every year. It is a genetic disorder that has no cure. It causes a progressive breakdown of nerve cells in the brain leading to the deterioration of a person’s physical, mental, and emotional abilities.
How is Huntington’s disease awareness done?
Blue, purple lights on municipal headquarters, Mumbai, India
During May, buildings, monuments, and statues are usually lit up in blue and purple, blue to raise awareness of Huntington’s disease, and purple for juvenile Huntington’s. Patients, caregivers, families, and friends nationwide are organizing fundraisers, planting virtual flowers, lighting landmarks blue and purple, taking hikes, flooding social media, and sharing stories about H.D. It is all intended to heighten awareness among the general public and draw the attention of policymakers, public authorities, scientists, health professionals, and industry representatives.
What are the symptoms of Huntington’s Disease?
The symptoms of HD can vary and your doctor can help you to understand or either direct to specialized HD clinics
Huntington’s disease typically starts between ages 30 and 50, but it can begin when you are younger.
HD affects your: • Movement • Behavior • Thinking, understanding, learning, remembering • Personality
The most common symptom is movement you cannot control, called chorea. Chorea causes dance-like movements. Other movement problems can include trouble with speech and walking.
You may also experience these symptoms: • Memory loss, poor concentration, trouble doing tasks, impulse control problems • Depression and lack of interest • Sleep changes • Sexual problems • Difficulty swallowing • Falling
In the early years, some slight mental, emotional, and behavioral changes may come before the more obvious physical symptoms.
What is the cure for Huntington’s Disease?
Currently there is no treatment that can slow down or reverse the disease. HD is not curable now, but there are medications that can reduce some symptoms. These medications may help improve movement, depression, and behaviors. Ask your neurologist about your medication options
When we decide or think to undergo Deep Brain Stimulation (DBS) surgery for Parkinson’s disease, the most common thought comes is HOW IS THE SURGERY DONE?. There are various steps before the surgery and you would have gone through these process like Levodopa challenge, cogntive assessment, decision making ability, speaking to people who have underwent surgery. BUT, still the question of how the surgery is done remains an intriguing enigma. In this blog we go through the process of SURGICAL DAY EVENTS along with the video and images to give an insight to all the people to understand the same. A brief visual overview of the events can be found on youtube : https://youtu.be/4QAugdLKYjg (This video link is available for above 18+ years only due to the descriptive videos)
One Day Prior to DBS Surgery
Most of the people planned for Deep Brain Stimulation (DBS) surgery are admitted a day prior to the surgery. Usually this day Patient and family gets settled after hospital admission process are finished. No medication changes are done on that day (If any changes are usually done atleast a week before – either stopping or adding). Surgical site preparation instructions are given by the surgeon (like head shaving / Part preparation etc). You would have your usual meal and medications before going to sleep.
On the Day of DBS Surgery
Your day begins around 6 to 7am in the morning, when the treating nurse checks and provides instructions. You would not be given any Parkinson’s disease medications (unless specifically indicated by the doctor) or food (you will be fasting overnight till the surgery is finished). You can take your other medications like for blood pressure etc. These would have been instructed to you a day before itself by the treating team and the Neuroanesthetist. Once your pre-surgical instructions are carried out by the nursing team (like part preparation, supervising medications), you would be shifted for the Frame fixation. From this point onwards your surgical day starts. We will go through them as step by step below.
Step-1 : Frame Fixation
DBS Frame being fixed by Dr. Kiran Khanapure and Dr. Harish PN
You would be taken for Frame fixation, where in a box type frame is fixed on your head with local anesthesia. This box will act like a landmarks for various brain structures. (In simple way it like graph computation using X, Y and Z co-ordinates to locate different brain structure)
Step – 2 : Imaging of the Brain with Frame
DBS Patient undergoing CT Brain after Frame being fixed
Following the fixation of the frame, you will undergo imaging (Commonly CT Brain is done. MRI can also be done). This will help to get the images of brain with the frame, which will act as anchor points to locate different distances in the brain.
Step – 3 : Planning of the Surgical co-ordinates
DBS plan on a stealth station
Once the imaging is done, you are shifted to the operating theatre room. Here you would be set in a comfortable position (so that you can stay like that for next few hours). In the mean time, the treating team will merge your images on a specialized software. From this software, they will choose the brain target (in PD mostly it would be subthalamic nucleus. Some time GPI is considered) and would be able to get co-ordinates based upon there distances from the frame anchor points. This will act as X, Y, Z co-ordinates of a 3d graph to target the brain.
Step – 4 : Positioning, Burr hole
Dr. Kiran Khanapure and Dr. Harish PN in the surgical process
Once the planning and co-ordinates for the surgery are finalized. The surgical team will set up a frame at the operating table. With this frame, then can mark the distances the electrodes to travel to reach the intended location. You would be awake or mildly sedated during this whole process. Once the frame is fixed a small hole is done in the skull (a size of a 25 paisa coin) to target one side of the brain. Later similar process with another hole is done to target other brain site. Once this is done, the scene is set up for assessment and target finalization.
Once the burr hole is done a microdrive is fixed and fine recording electrodes are pushed in the planned trajectory. Each part of the brain has different electrical activity pattern and based upon which we can assume where the electrodes are going get placed. This is a method of re-affirmation of Imaging planning done previously and gives an confirmation of right position. Based upon these recordings the position of electorde is considered.
Step – 6 : Macro Stimulation and Clinical assessment
Once the microelectrode recordings are done, then the most important clinical assessment will start. In this step, small currents are given in the intended target region and clinical assessment for benefits would be done. This is point where patient has to be very alert and respond to various questions and tasks given by Dr. Prashanth LK. The benefits of stimulation and at voltages sides effects / excess effects are noted and recorded. This clinical assessment gives an final confirmation of the electrode placements.
Step – 7 : Electrode fixation
X-Ray imaging during the process of electrode fixation
Following the electrode placement, it is fixed to the position and locked by a cap.
Step – 8 : Repeat procedure for the second site
After the electrode placement on one side, Step 4,5,6,7 are repeated on opposite side to fix the second electrode.
Step – 9 : Check Imaging
CT imaging showing Reconfirmation of trajectories after the electrodes are fixed
Once both the electrodes are fixed, a check imaging is done preferabbly and then these images are merged with the actual intended trajectories. The goal is to cross confirm the initial planned trajectories with the final fixed trajectories.
Following the check imaging, now you would be planned for battery placement. This point onwards you would be in general anaesthesia and deep sleep. A small pocket is made in your chest, just below the collar bone. After this an tunnel is created from the battery area to the head (Near the burr holes) just beneath the skin and both the parts are connected by the wire. Once all the electrical systems are connected, they are checked for integrity of the circuit, before closing the surgical sites. With this the surgical process is finished.
Step – 11 : Immediate Post Surgical care
Once the surgical process is finished you are weaned from the anesthesia and then shifted to the ICU. You would be awake (probably exahusted /sleepy). No oral feeds are given for another few hours at the discretion of the anesthesia team. Following that your regular Parkinson’s disease and other medications are started. You would be in the Intensive care unit overnight. There would some pain at the surgical wound points, but usually less as you would be given pain killer.
Step – 12 : Point to Remember
Please remember, surgical process is to fix the DBS stimulation system in the brain. The system is not yet assessed and started. It would be done subsequently at a later date. The whole surgical process lasts for 8-10 hours. In some patients the process may be staggered on different days, based upon the comfort levels of the patient and the surgical teams. There would be some deviations based upon each case to case basis, which would be discussed with the patient and family before and during the procedure (if required)
Day After the DBS Surgery
The next day following the Deep Brain Stimulation (DBS) surgery, you would be assessed. Most of the people would be back to normal status, with possible some sense of improvements, due to microlesional benefits. If you are deemed fit, then you would shifted to the ward, where you would stay for next few days.
Your whole stay in the hospital will last between 5-7 days on average.
Post Discharge
Post discharge, you would be guided on dates for surgical suture removals and planning for DBS programming.
The understanding of Parkinson’s disease has been dramatically changing over the years. The gastrointestinal symptoms of Parkinson disease are well recognized and keeps on adding up to the number of symptoms of Non Motor Symptoms (NMS). Even further the question of whether Parkinson’s disease arises from Gut perse has become a promienent question among the scientists across the world. The has given raise to concept of GUT-BRAIN axis and further postulation of Gut first and Brain first theories. In this blog, we are trying to highlight the awareness about various gastrointestinal symptoms noted in patient with Parkinson’s disease.
The Gastrointestinal symptoms in Parkinson disease is one of the common NMS in PD, even early in the disease course. Several factors such as loss of dopaminergic activity, presence of Lewy bodies, gut exposure to neurotoxins, gut dysbiosis, cytokine-induced toxicity, inflammation-derived oxidative damage and aging have been associated with the pathogenesis and progression of PD although robust evidence is lacking.
Gastrointestinal Symptoms of in Parkinson Disease
The symptoms of Gut in Parkinson’s disease can be noted all the way from the mouth and salivary glands. the following list helps to understand the possible symptoms which could be associated with Parkinson’s disease.
Salivary Glands symptoms
Reduced Saliva Production
Low swallowing frequency leading to drooling
Mouth related symptoms
Pooling of saliva and problems with movements needed to brush teeth can cause dental dysfunction
Jaw tremors related discomfort
Pharynx related symptoms
Oropharyngeal dysphagia increases risk of aspiration
The Elephant in the Room art by Banksy at Barely legal exhibition in 2006
The expression “Elephant in the room” is an metaphorical idiom used to describe something which everyone knows, but no one wants to mention or discuss it for various reasons. The above image is a part of “controversial art” by famous British artist Banksy. This art named “Elephant in the room” was featured as a part of US exhibition – “Barely legal” in 2006. It had it’s own set of controversies. However, the primary objective of this art was to highlight that billions of people lived below the poverty line. This art perse was a metaphor to speaking about elephant in the room and controversies there off.
Parkinson’s Disease is well known disorder where in people know issues about tremors, slowness, stiffness and walking related issues. However, there are many more other issues, which is well know to the patients, caregivers, and doctors, but most of the time, these symptoms are either brushed under the carpet or not enquired upon at consultation. Currently these features are becoming more important in the management of Parkinson’s Disease and more efforts are being put into the assessment and treatment of these issues. These are broadly classified under the category of Non Motor Symptoms (NMS) under the management of Parkinson’s Disease. However, these for all of us is the Elephant in the room which needs to be more outspoken and appropriate management needs to be done.
The International Parkinson Disease and Movement disorders society (IPMDS), has come up with exclusive scales on non motor symptoms to address this concern of elephant in the room.
“Elephant in the Room” – Parkinson’s Disease
The Motor and the Non Motor symptoms of Parkinson’s disease. Image source: Parkinsonnsw
There are various symptoms which qualify for elephant in the room for Parkinson’s disease and prominent among them are
Mood : All Parkinson’s disease patients might have some form of mood/behavioral changes, which is mostly under reported and only symptoms which become much bothersome are spoken about. Depression is one of the well know and needs to be tackled properly.
Sleep : Sleep disturbances are common and sometimes predate the onset of motor symptoms by many years. These symptoms might include vivid dreams, nightmares, shouting/yelling/enacting in dreams (aka RBD), restless leg syndrome (RLS).
Bladder issues : Urinary urgency, increased frequency, night time increased urinary frequency/volume are common bladder issues noted.
Constipation : Constipation is probably one of well accepted and acknowledged symptom, which is noted well before the onset of motor symptoms
Sexual dysfunction : Probably this is one of the least discussed non motor symptom, and mostly not addressed. The issue has to be discussed promptly by the patient or the doctor as its fairly treatable symptom
Excessive sweating / drooling / skin oilyness : These changes are noted in patients with Parkinson’s disease and skin changes can be noted.
Postural dizziness / fatigue : Patients with Parkinson’s disease tend to have
In the subsequent weeks, we will try to bring out exclusive write ups, including their symptoms, management, both on video and web write up. Effort will be put to get Kannada language audio/video files for the patients and care givers. Keep a lookout on the facebook page and youtube channel.
Prof. Elena Moro, Secretary General of European Academy of Neurology, had interview with Dr. Prashanth LK on COVID19 and its impact on medical practice at ground level. This is published in the the European Academy of Neurology (www.ean.org) EAN pages section
Brief excerpt of the interview with Dr. Prashanth LK is below and the rest can be accessed at the EAN Pages section (https://www.eanpages.org/2020/11/01/covid19-around-the-world-clinician-interviews-on-experience-and-impact-of-the-virus/).
1. How has COVID19 and national measures to control it affected routine and emergency neurology service delivery in your country? How have you adapted to deal with the impact?
The speed and rampage of COVID19 pandemic have paralysed every country in 2020. No matter what, the amount of measures taken at every level, appears to be miniscule in front of its wrath. The neurology community across India was gearing up for the COVID19 breakout but, the impact of COVID19 was beyond anyone’s perception until it hit upon us. The Annals of Indian Academy of Neurology (Official journal of Indian Academy of Neurology), came up with a special supplement of COVID-19 in early April 2020, which addressed various issues in relation to management of COVID-19 and neurological disorders. The special issue included consensus statements on management of various neurological disorders and care related points. At ground level in routine neurology care the scene was dramatically changing minute by minute. The emergency departments were filling up with COVID19 patients and it appeared as if all the neurological emergencies have ‘vanished’ – which ended up being like a calmness of ‘eye of the hurricane’, before it hit us. The pattern and ‘ease’ of neurological practice has significantly changed with masks, face shields, personal protective equipment (PPE) kits and whole attire changing from a formal dressing to “Scrub suit” / “PPE suits” taking the forefront. This has definitely drained down a significant energy and amount of work output, but it appears like this will become a new norm for near future. Another interesting change has been the raise in tele consults / video consults. The medical practicing laws were quickly amended to accommodate the requirement of telemedicine. COVID19 has been a blessing in disguise for the telemedicine and this has become a main stay for most of the stable follow-up patients, albeit everyone does miss the personal/social touch with in-person consults.
2. Have you seen many people whom you were concerned had neurological complications of COVID19 infection? What are the most common neurological manifestations of COVID19 in your region?
COVID19 pandemic has made every speciality to gear up for its care. Most of us are working in regular COVID19 care which caters beyond neurological issues. The neurological manifestations of COVID19, which I have seen can be considered as those primarily related to COVID-19 infection, secondly to worsening of pre-existing neurological disorders due to COVID19 and finally those due to psychological effects of COVID19 breakout. The core neurological issues related to COVID19 included primarily thrombotic events in the form of acute ischemic events, cerebral venous sinus thrombosis in people, in whom no other risk / aetiologies could be pointed out. In addition, encephalitis / encephalopathy presentations were also seen in patients with COVID19. Many patients with severe COVID19 infections were reviewed for neurological consults and considered to have encephalopathies secondary to systemic COVID19 infection. The second set of problems was people with pre-existing neurological disorders like myasthenia gravis, multiple sclerosis, parkinsonism coming with acute worsening on a background of COVID19 infections. The third set of people were those who were not directly infected with COVID19, but with trauma of separation, quarantine, fear of not able to reach out to their doctors, limitation of social life leading to worsening of neurological symptoms especially with those of Parkinsonism disorders and dementia.
3. What are your main concerns for the future in regard to COVID19? What do you think are the key measures and contingency plans necessary to ensure high quality care for people living with neurological diseases during this pandemic? (read further)
#PDchallenge was initiated on July 22nd 2020 on the occasion of World Brain Day to create awareness about Parkinson’s Disease. The challenge included, showing various activities with a theme to win over Parkinson’s Disease. It included videos of patients, caregivers, friends, relatives, movement disorder specialists, neurologists, neurosurgeons, therapists, researchers from across the globe.
The following collage video shows the various Movement Disorders specialists, Neurologists, Neurosurgeons, researchers taking part in the #PDchallenge, released on August 15, 2020. This matches with the essence of ‘Mile Sur Mera Tumhara‘ theme of Unity in diversity theme song launched on August 15th 1988.
Every year July 22nd is commemorated as WORLD BRAIN DAY. Annually one disorder will be highlighted to increase the awareness of the disorder. World Brain Day 2020 is dedicated to raising awareness for Parkinson’s Disease, a neurodegenerative brain disorder affecting more than 70 lakh people of all ages worldwide. This year, World Federation of Neurology and International Parkinson and Movement Disorder Society have joined together to end #parkinsons.
In this context, here in India, we are doing an active Parkinson’s Disease awareness campaign in the form of #PDchallenge. The challenge is open to all patients, caregivers, friends, health care workers and everyone across. The primary goal is to create awareness about Parkinson’s Disease. Please do a brief video about your active movements or agility and challenge your family members, friends, colleagues to show their agility. Come lets create awareness about Parkinson’s disease and remove the myths about it. Let’s create awareness by challenging our friends and relatives
10 things about Parkinson’s Disease for this World Brain Day:
Parkinson’s Disease is neurodegenerative disease affecting the brain
More than 70 Lakh people of all age groups affected by Parkinson’s Disease Worldwide.
Parkinson’s Disease classically causes Slowness, Stiffness, Tremors and Balance problems.
Mood changes, Anxiety, Depression, reduced smelling ability, Sleep disturbances, Urinary issues, Constipation, can also be affected.
The Scare of the name “PARKINSON’S DISEASE”, make many people to loose hope in life.
Parkinson’s disease is treatable
People affected with Parkinson’s disease can lead a near normal quality of life.
Good treatment options are available including mediations, injections and surgeries.
Active life style and Exercises form the most important part of management of Parkinson’s Disease.
Come on the World Brain Day, “Lets Shake the Parkinson’s”
The COVID-19 breakout has lead to a significant limitations in mobility of people across the world. This is primary concern for People with Parkinson disease and Parkinsonism disorders. In these disorders people need to do workouts on daily basis to have a good quality of life. Many people require supervised care and some require a group to motivate the exercise schedules. This has been significantly hindered by the current #pandemic outbreak and #Lockdown. To over come this modern day problem, people have adapted and improvised to bridge the current issues. In this context “Hirshikesh’s Center for Contemporary Dance” in Pune, has been successfully working on online dance programs for Parkinson’s Disease and Parkinsonism Disorder patients. The dance program for Parkinson’s disease facilitators integrate movement from Indian classical and modern dance, and choreographic repertory. This initiative has been helping many Parkinson’s Disease people from across India.
Difficulties with gait and balance are common among individuals with Parkinson disease (PD), contributing to an increased incidence of falls. Gait changes include slowness of walking with short, shuffling steps and a flexed posture, and may also include festination and/or freezing of gait. Aspects of walking that appear to be particularly impaired include dual tasking, turning and walking backward
Given the potential benefits of exercise for those with PD, recommendations have been made regarding key components of an exercise program designed for those with PD. The recommendations include four key areas: 1) cueing strategies to improve gait, 2) cognitive movement strategies to improve transfers, 3) exercises to improve balance, and 4) training of joint mobility and muscle power to improve physical capacity. Emerging evidence also suggests that aerobic training, such as walking on a treadmill, may result in improved quality of life, reduced disease severity as reflected by lowering of UPDRS-III scores, and improved aerobic capacity
Dance as a group-based treatment for Parkinson’s disease (PD) incorporates physical exercise, cognitive tasks, sensory experience (music), emotional expression, and social interaction. As such a multidimensional activity, dance has the potential to address many of the challenges faced by patients. Indeed, in addition to significant motor and cognitive impairment, patients are troubled by mood changes and social isolation. Therefore, dance may highly impact the quality of life in patients with PD.
Destination Unknown – My Journey with Parkinson’s, is an autobiography of Mr. Rajeev K Gupta, who is diagnosed with Young Onset Parkinson’s Disease. The book fills a long overdue void about personal experiences about Parkinson’s Disease, especially from Indian Context. It goes through the various phases from, ‘Overcoming the Shock’ attached with diagnosis of Parkinson’s disease in a young active professional life. The story leads on to explain the “Million Dollar Decision” on how and when to reveal the diagnosis to the family members and on professional front. It also gives insights about how to cope with the diagnosis, adapt and overcome, to win over this ‘Mental Game’. Mr. Gupta, further goes into the depth of various treatments he received, their benefits / limitations. Later, he continues on his decision on ‘Deep Brain Stimulation’ surgery and his whole experience from the point of considering it to the post-surgery outcomes. The book also gives a Q&A section, which will be helpful for all Parkinson’s disease patients, to understand about Parkinson’s disease and its treatment. Overall, a must read for every Young Onset Parkinson’s Disease Patients and their families. The Book is currently available on various online sellers including Amazon, Kobo, Google Play books and on publishers site (Notion Press).
Huntington’s disease patients and families require special attention and care. Month of ‘May’ is #HDawareness month. There are various activities going on world wide during this #Lockdown time to create more awareness about #Huntingtonsdisease.
Huntington disease is named after George Huntington (April 9, 1850 – March 3, 1916) was an American physician from Long Island, New York who contributed the clinical description of the disease. Dr. Huntington wrote his paper “On Chorea”when he was 22 years old, and was first published in the Medical and Surgical Reporter of Philadelphia on April 13, 1872.
Huntington’s disease usually causes movement, cognitive and psychiatric disturbances with a wide spectrum of signs and symptoms. Which symptoms appear first varies greatly among affected people. During the course of the disease, some disorders appear to be more dominant or have a greater effect on functional ability.
The movement disorders associated with Huntington’s disease can include both involuntary movement problems and impairments in voluntary movements, such as:
Involuntary jerking or writhing movements (chorea)
Muscle problems, such as rigidity or muscle contracture (dystonia)
Slow or abnormal eye movements
Impaired gait, posture and balance
Difficulty with the physical production of speech or swallowing
Impairments in voluntary movements — rather than the involuntary movements — may have a greater impact on a person’s ability to work, perform daily activities, communicate and remain independent.
Cognitive Symptoms (Memory related)
Cognitive impairments often associated with Huntington’s disease include:
Difficulty organizing, prioritizing or focusing on tasks
Lack of flexibility or the tendency to get stuck on a thought, behavior or action (perseveration)
Lack of impulse control that can result in outbursts, acting without thinking and sexual promiscuity
Lack of awareness of one’s own behaviors and abilities
Slowness in processing thoughts or ”finding” words
Difficulty in learning new information
Behavioral Symptoms:
The most common psychiatric disorder associated with Huntington’s disease is depression. This isn’t simply a reaction to receiving a diagnosis of Huntington’s disease. Instead, depression appears to occur because of injury to the brain and subsequent changes in brain function. Signs and symptoms may include:
Feelings of irritability, sadness or apathy
Social withdrawal
Insomnia
Fatigue and loss of energy
Frequent thoughts of death, dying or suicide
Other common psychiatric disorders include:
Obsessive-compulsive disorder — a condition marked by recurrent, intrusive thoughts and repetitive behaviors
Mania, which can cause elevated mood, overactivity, impulsive behavior and inflated self-esteem
Bipolar disorder — a condition with alternating episodes of depression and mania
In addition to the above symptoms, weight loss is common in people with Huntington’s disease, especially as the disease progresses.
Symptoms of juvenile Huntington’s disease
The start and progression of Huntington’s disease in younger people may be slightly different from that in adults. Problems that often present themselves early in the course of the disease include:
Behavioral changes
Loss of previously learned academic or physical skills
Rapid, significant drop in overall school performance
Behavioral problems
Physical changes
Contracted and rigid muscles that affect gait (especially in young children)
Changes in fine motor skills that might be noticeable in skills such as handwriting
