Chorea

Huntington’s Disease Awareness Month and Clinic

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.

We are running the special Huntington disease clinic every fourth Wednesday of the month. This is a FREE CLINIC for Huntington’s disease patients and families. Dr. Prashanth LK is European Huntington Disease Network Associated member and credentialized investigator of Huntington Study Group.

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.

George Huntington. Copyright – HDSA

Movement disorders

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
  • Tremors or slight involuntary movements
  • Seizures
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Parkinson Disease Treatmentnews

Opicapone, New medication for Parkinson’s Disease got FDA approval

Opicapone, got FDA approval for Parkinson’s Disease Treatment

Parkinson’s disease treatment got a new drug for its management. Opicapone a newer once a day medication helps in better functioning of levodopa/carbidopa (Syndopa / LCD / Madopar / Sinemet). It got Food and Drug Administration (FDA) of USA approval under the brand name of ONGENTYS. It comes with 25mg and 50mg capsules. It helps to treatment and improvement of ON periods in Parkinson’s disease. The company Neurocrine Biosciences plans to launch ONGENTYS later this year.

Opicapone blocks the enzyme catechol-O-methyltransferase (COMT) effectively (>90% at therapeutic doses), selectively and reversibly, and only outside the central nervous system. It dissociates slowly from COMT, resulting in a duration of action longer than 24 hours despite its short blood plasma half-life. As COMT and DOPA decarboxylase are the main enzymes for degrading levodopa, blocking the two effectively increases its concentrations in the bloodstream. More levodopa reaches the brain, where it is activated to dopamine. This helps to improve the symptoms of Parkinson’s disease, such as stiffness and slowness of movement.In June 2016, it was authorised for use in the European Union. It was authorised for use in the United States in April 2020.

This drug is contraindicated in people with cancers that secrete catecholamines (for example epinephrine), such as phaeochromocytoma or paraganglioma, because as a COMT inhibitor it blocks catecholamine degradation. Other contraindications are a history of neuroleptic malignant syndrome (NMS) or non-traumatic rhabdomyolysis, and combination with monoamine oxidase inhibitors that are not used as antiparkinsonians, because of possible drug interactions

People taking opicapone very commonly (18%) experience dyskinesia. Other common side effects (in 1 to 10% of patients) include dizziness, strange dreams, hallucinations, constipation, dry mouth, orthostatic hypotension (low blood pressure), and muscle spasms. Apart from spasms, these side effects are also known from tolcapone and entacapone

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TelegramAwareness

Telegram Channel for the Clinic

Telegram, Online Consultation

Telemedicine and online consultation is current requirement of the the day. Parkinson’s Disease and Movement Disorders Clinic has started exclusive telegram channel for its patients and caregivers. The channel’s primary goal is to provide updates about specific events at the clinic and critical news about research in Movement Disorders world wide.

The channel can be subscribed using the following link :

https://t.me/movementdisordersclinic

To Chat with Doctor

Due to the unforeseen circumstances of COVID 19 Pandemic, everyone is encouraged not go out of the houses or attend clinics for regular visits. However, some of the patients/caregivers, may require to consult the doctor for clarifications or for repetition of prescriptions. In such cases, the telegram consult app is available to chat with doctor. Please use it only for minor clarifications. This is available for previously consulted patients only. The turnaround time for responses vary depending upon the time of the day and other resources. If anything urgent, please call up to the hospital or nearest emergency room.

Link : https://t.me/DrPrashanthLK

Online Consultation

Telemedicine / Online consultations are available for select cases and scenarios. You can read about it further using this link.

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DBS for dystoniaDeep Brain Stimulation

Deep Brain Stimulation (DBS) in a 5 year old child – Youngest to undergo surgery in India

DBS for dystonia

A 5 yr child who is affected by a rare type of dystonia called DYT16 was managed in Vikram hospital, Bengaluru. Only about 10-12 cases have been described worldwide with this rare disease. The child was completely bed bound and dependent for all the activities. The child underwent Deep Brain Stimulation surgery (DBS), the youngest person to undergo this procedure in India till date. Its been 3 months now and the child is making great improvements. Kudos to all involved. Looking forward to hear more about the recovery curve about this rare disease

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Apomorphine Therapy

Apomorphine Launched in India

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Deep Brain Stimulation

NATIONAL GEOGRAPHIC CHANNEL TO SHOW LIVE DBS SURGERY

As a live TV audience watches, a neurosurgery team at UH Case Medical Center in Cleveland will perform an operation that has helped many Parkinson’s disease patients get significant relief from their debilitating symptoms—tremors, rigidity, stiffness, slowed movements and difficulty walking—and also enabled them to reduce their amount of medication.

Deep Brain Stimulation, or DBS, was pioneered as a Parkinson’s treatment by Dr. Alim-Louis Benabid, a neurosurgeon who also possessed a Ph.D. in physics. In the 1980s, patients were treated by surgically destroying parts of their brains where tremors originated, or using medication with unpleasant side-effects. In 1987, Benabid was performing a surgery using the then-standard method of burning away brain tissue with an electrode. As he tested various regions with electrical pulses, Benabid wondered what would happen if he used different frequencies. To his surprise, he found one that suppressed the patient’s tremor. “I thought, aha, this might be the solution,” he later recalled in a 2010 Lancet article.

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