What is Exaggerated Startle Response?

Exaggerated startle response is considered as an atypical inherited neurological disorder which may act on the toddlers, kids and even adults. Person, affected by the ailment of exaggerated startle response have sudden and high startle reactions such as body spasms or eye blinking to unexpected or sudden noise, touch or movement.

What is Exaggerated Startle Response?

Hypertonia or severe muscle strain or stiffness is observed in the individuals who are affected with exaggerated startle response. Hypertonia stops voluntary movement of the person's muscles and turns the affected individual stiff just like a piece of wood without losing his or her consciousness.

In addition to hypertonia, hyperreflexia or exaggeration of reflexes and gait or an unbalanced technique of walking may arise in people with exaggerated startle response. Though, exaggerated startle response is an ADT (Autosomal Dominant Trait) by heredity, but it may be autosomal recessive and in rare case, X-linked heritage is found.

In most of the cases, exaggerated startle response is misdiagnosed as one of the types of epilepsy and so it takes a long time to find out the exact diagnosis. The treatment for exaggerated startle response is comparatively uncomplicated and related to the use of the medicines of anti-spastic and anti-anxiety. Cognitive therapy and physical therapy (PT) are considered as additional treating options.

What are Body Spasms?

Twitching of muscle that is also known as muscle fasciculation is seen as small contractions of muscles in the body. Muscles of human beings are built of nerves, tissues and fibers. So, while a nerve is damaged or stimulated, the fibers of the muscle twitch.

Though, individuals experience twitching in their body from time to time and these are very minor, but some kinds of twitching can be a symptom of the condition of nervous system and it is better to check with the doctor.

Causes of Exaggerated Startle Response

Generally, exaggerated startle response is classified as genetic disease, but some other disorders imitate the symptoms of exaggerated startle response.

In majority of the cases, the cause of exaggerated startle response is hereditary, because this disorder is considered as autosomal dominant trait. However, it also can follow X-linked inheritance or autosomal recessive. Mutations of the genes such as GLRA1, GLRB, SLC6A5, X-linked (ARHGEF9), GPHN are related to this condition. Most of the exaggerated startle response affected persons are due to the mutation of SLC6A5, GLRA1 gene or their parents are affected by it.

Genes which are responsible for exaggerated startle response, produces glycine protein and glycine weakens the activities of cells of the nerve in the spinal cord and in the brain. It is identified as "inhibitor transmitter". When the glycine receptors are damaged or hampered the cells of the nerve hang-up and consequently respond to stimuli excessively and very easily.

DGD (Dominant Genetic Disorders) happens when even a single unnatural gene is necessary to cause a particular disease. The affected person may get the unnatural abnormal gene by hereditary from their parent; in addition, it may occur due to gene change (mutation). The possibility of passing the abnormal gene from affected father or mother to their child is 50% during the pregnancy. The risk is the equal for men and women.

Even the physicians are not sure why some people get exaggerated startle response. Just like in most of cases of mental health problems, exaggerated startle response is the result of a complex mixture of the followings:

  • Risks of inborn mental health, such as high risk of depression and anxiety.
  • Negative experiences in life, including the severity and amount of trauma the affected person has passed through since his or her early age or childhood.
  • Inherited characteristics of your personality- which is identified as your temperament.
  • The mode or system of the affected person's brain that regulates the hormones and chemicals in the body of that particular person releases at the time of responding to trauma or stress.

Most common leading causes for developing exaggerated startle response:

  • Exposure to combat
  • Negligence in childhood and physical abuse.
  • Sexual violence
  • Physical assault.

In addition, there are also numbers of traumatic or shocking events that also lead to the problem of exaggerated startle response, for example, natural disaster, terrorist attack, fire accident, plane crash, car accident, robbery, mugging, life-threatening medical diagnosis, kidnapping, torture and numerous severe events.

Most Affected People by Exaggerated Startle Response

Exaggerated startle response which is a rare genetic disorder is found at the time of birth and it affects both men and women. In some cases, the disorder takes time to flourish; it may be belated to the childhood or adulthood. In the United States, about 1 person in 40,000 people is affected by exaggerated startle response.

Disorders Which Mimic Exaggerated Startle Response

The symptoms of some disorders are alike to the symptoms of exaggerated startle response. So, it is necessary for the doctor to compare the different diagnosis reports before starting the treatment.

  • Jumping Frenchmen of Maine Disorder: Different symptoms of neuropsychiatric startle, like psychiatric or behavioral symptoms are also observed besides startling such as in Jumping Frenchmen of Maine- It is a disorder where acute startle reactions like raising hands, jumping, hitting, yelling, obeying the commands given suddenly, repeating sentences involuntarily. These specific startle reactions like obeying commands which are given suddenly or repeating sentences are not observed in exaggerated startle response.
  • Startle Epilepsy: In startle epilepsy the muscles of the affected person contracts briefly and the body responses to sudden movement or noise. The patients fall down frequently being startled and other symptoms of spasm also are expressed.
  • Tic Disorder or General Anxiety: Disorders such as tic or general anxiety and other conditions which are related to startle are considered as having related symptoms, such as SPS (Stiff Person Syndrome) is noticed in aged persons (normally between 50 years and 60 years) and it is accompanied with progressive and prolonged firmness and periodic body spasms.

Signs and Symptoms of Exaggerated Startle Response

There are severe and mild types of exaggerated startle response. Unusually severe startle response to unexpected or sudden touch, noise and movement are characterized as severe type of exaggerated startle response. HRR or exaggerated head-reaction response, myoclonic jerks or spastic jerking movements, falling stiffly on the floor and still being awake/conscious and these tendencies occur when the individual has exaggerated startle response.

Below mentioned are the signs and symptoms of exaggerated startle response:

  • A patient of exaggerated startle response may experience jerking movement when he is trying to sleep.
  • High muscle strain or hypertonia (firmness) is very much common in those infants who are suffering from exaggerated startle response, particularly at the time of birth. Babies, affected by exaggerated startle response cannot move much, and if they want to do so, they usually move leisurely which is called hypokinesia.
  • Excess body reflexes/ hyperreflexia, intermittent apnea or intervallic breathing or/and gait- unbalanced walking with slight wide-based posture are the other symptoms of those infants and also the adults suffering from exaggerated startle response. A few infants have dislocated hip at the time of their birth. Additionally, inguinal hernias or hernias in the lower abdomen are also common.
  • In the case of minor or mild type exaggerated startle response, the affected individuals experience irregular or changeable startle response with none or a few other symptoms. In infants, fever can bring this type of exaggerated startle response and in the adults; the intensity of the responses of startle may be influenced by anxiety or stress.
  • Usually, the severe and mild types of exaggerated startle response start from the time of birth, though some patients experience it at the period of their adulthood. Intellectual weakness in mild form is also noticed.

Risk Factors and Complications in Exaggerated Startle Response

There are many risk factors for exaggerated startle response which can lead to complications such as depression, eating disorders etc… Exaggerated startle response that is prominent with aggressive jerking to touch and noise shows a continuous stiffness of limbs and trunk, tightened fists, as well as the attacks of high frequency trembling.

The violent exaggerated startle response body jerk affects the heart rate immediately, goes up the anxiety level and finally trembling occurs. Any type of the exaggerated startle response episode whether minor or major, brings embarrassing situation for the sufferers and leads them to seclusion and public isolation.

Once attacked with exaggerated startle response, it can shatter your life: you will find yourself unfit in your job, relationships, health and everyday activities unfit. With exaggerated startle response, you may prone to be attacked with different kinds of psychological disorders. The most important of them are:

  • Anxiety and depression.
  • Strong desire to consume alcohol or drugs.
  • Eating disorders like consuming a small amount of food or over eating.

Tests to Diagnose Exaggerated Startle Response

The family history of the patient or the genetic linkage is very important to diagnose exaggerated startle response. The disease of exaggerated startle response is a possibility when an infant is having seizures. The most important features of the disorder are:

  • Extreme startle reflex or response.
  • Stiffness from birth.
  • Inability to move for a time period following the shock.

One of the effective tests of exaggerated startle response is electromyography and the other is Electroencephalography. The former records the electrical muscle impulse and the latter measure the brain electrical activity.

The primary step for testing the molecular genetics for those who already detected the diseases is mutation test in SLC6A5 genes and GLRA1. The analysis of the sequence of genetic materials ARHGEF9 can be believed in men without identifying the mutations of SLC6A5 or GLRA1, especially in the case of cognitive injury and the presence of epilepsy. If transformations or mutations cannot be identified, the genetic testing of the molecule for the mutations in the genetic material in the GPHN and GLRB can be granted for consideration.

Treatment for Exaggerated Startle Response

The Standard Therapies for Exaggerated Startle Response

Hypereflexia due to exaggerated startle response in case of the adults and infants can be treated most successfully with anti-spastic and anti-anxiety drug Clonazepam. There are some other drugs, that can be helpful for the same. The most remarkable are phenobarbital, carbamazepine, 5-hydroxytryptophan, phenytoin, sodium valproate, diazepam, piracetam. Sometimes genetic counseling proves beneficial for the sufferers and the person of their families. The physical and cognitive healing is also helpful to decrease anxiety can be supportive.

Psychotherapy for Treating Exaggerated Startle Response

Different kinds of Psychotherapy or talk treatment for exaggerated startle response like counseling are used to treat the adults and the children suffering from exaggerated startle response. Some of them are:

Cognitive Therapy for Treating Exaggerated Startle Response

Cognitive therapy for exaggerated startle response is a kind of therapy helps to identify the process of thinking that is cognitive patterns. That keeps you fixed, such as inaccurate, negative ways of comprehending usual situations. For curing PTSD (Posttraumatic stress disorder), the cognitive remedy often is used along with the contact with the therapy.

Exposure Therapy for Treating Exaggerated Startle Response

Exposure therapy for treating exaggerated startle response helps to inspire you to face the situation safely so that the mind can enhance the power of tolerance effectively. With the help of virtual reality programs, you can come in with the setting of the previously experienced background of trauma.

Only the health experts can confirm which kind of therapy goes well with your problem.

The Individual Therapy and the Group Therapy or both can offer a means to link to the other people suffering from the same ailment of exaggerated startle response.

Treatment for Children with Exaggerated Startle Response

It is frequently seen that the exercises for children with exaggerated startle response are more fruitful than the customary physiotherapy, resolution, or requiring training. Wide ranging of outdoor activities are very effective. Intrusion against mockery, incomprehension, or pressure from unapprised teachers, friends and relatives may be essential.

Changes of Lifestyle and Coping with Exaggerated Startle Response

If stress and other problems caused by the exaggerated startle response trouble you, see your health care professional. Below mentioned are some of the lifestyle changes and coping techniques for exaggerated startle response:

  • Follow the given plan of treatment. Though it takes some time to enjoy the benefits of the healing process, it may be effective and most of the people do get well. Tell yourself that it is a long term process and not one or 2 week treatment like in fever or cold.
  • Research more about exaggerated startle response. This knowledge can help you understand what you are feeling, and then you can develop coping strategies to help you respond effectively.
  • Be careful about yourself. Avoid taking nicotine and caffeine that can aggravate the condition. Take sufficient rest, consume a healthy diet, do yoga every single day.
  • Avoid self-medication. Taking drugs or alcohol can numb your feelings. Despite the fact, it may be an alluring way to deal with. Proceeding through this road of alcoholism might bring greater problems for you and put up resistance to real treatment.
  • Break the series. When you experience anxiety, take some light walk or jump into your favorite hobby to restart or refocus.
  • Talk to anyone whom you like. Stay linked to the supportive and caring people, friends, and the family. You need not talk about your problem if it is not necessary. Just sharing time with loved ones can offer healing and comfort.
  • Take a group support. Inquire the health expert to help finding a support group. Or, you can contact with the veteran people in the organization or the community for the support. Presently, online search can inform you about the online support groups in your area.
  • Talk to someone. Stay connected with supportive and caring people- family, friends, faith leaders or others.

What to do When Someone You Love has Exaggerated Startle Response?

Exaggerated startle response can considerably damage the mental and emotional health of the people whom you love. It is not possible for you to change anybody, but you can:

  • Find out the facts of exaggerated startle response to know the condition that your loved ones are going through. This can help you understand what your loved one is living through.
  • Know it that detachment or social isolation is the part of the disorder. If the person you love does not allow you to help, give that person some space or let him or her feel that you are never away from the person, rather you are available always.
  • Help the person to take him/her to attend medical appointments. If you succeed in taking the person to attend the appointment, assist the sufferers in getting treatment.
  • Always listen to the problem with eagerness. Let the sufferer know that you are very keen in listening to their problems.
  • Encourage involvement in any opportunities that allow activities that celebrate good events.
  • Along with encouraging the loved ones, take care of your health, eating habit, take adequate rest. Spend some time with your friends intimately that will help refresh.
  • Try to find help when you are in difficulty to cope, consult with the doctor. The victim might refer your name to the counselor who will help you serve with your emotion.
  • Protect yourself and your family if your loved ones get violent or rude.

Recovery Period for Exaggerated Startle Response

Startling to unforeseen stimulation like loud noises, touch or movement lingers for lifelong. Some of the patients may suffer phobic anxiety, such as, crossing any open areas, hesitant gait disorder etc… If the patient sticks to the treatment plan then the extent or the intensity of the disorder from a major state of exaggerated startle response can be brought to a minor exaggerated startle response.

Yoga for Exaggerated Startle Response

Yoga is very supportive for the sufferers of exaggerated startle response in helping them stay relaxed and cool in their daily activities. It gives them enough strength to face adversities. Yoga includes an inclusive package of different body postures or Asanas, breathing techniques or pranayam, meditation or dhyana, and also the Yoga Philosophy of ancient time. These are all helpful for recovering the positivity to the patients.

Yoga Poses/Asanas for Exaggerated Startle Response

The following yoga asanas or poses are helpful for the victims of exaggerated startle response:

  • The Bow Pose or Dhanurasana
  • The Fish Pose or Matsyasana
  • The One-Legged Forward Bend or JanuShirsasana
  • The Bridge Pose or Setubandhasana
  • The Cat Stretch or Marjariasana
  • The Two-Legged Forward Bend Pose or Paschimottanasana
  • The Standing Forward Bend or Hastapadasana
  • The Downward Facing Dog Pose or Adhomukha Shwanasana
  • The Headstand or Shirshasana
  • The Corpse Pose or Shavasana.

Pranayama or Breathing Techniques for Exaggerated Startle Response

It is the method of concentrating your entire focus on the breath. This is a process to clean your mind from confusing thoughts. Here are the breathing techniques that you must follow.

  • Skull Shinning Techniques of Breathing (Kapal Bhati Pranayama)
  • Bhastrika Pranayama
  • Alternative Nostril Breathing (Nadi Shodhan Pranayama)
  • Bee Breath (Bhramari Pranayama)

Meditation for Exaggerated Startle Response

Meditation for exaggerated startle response is a great way to calm down the agitated mind. It gives a sense of peace. It keeps you engaged with your work with cool disposition. You will be able to do the pretty things in a very tidy manner. It will get rid of you from the unnecessary anxiety of the future.

During the exaggerated startle response episode, there happens 'adrenalin rush' where the adrenalin hormone becomes higher. This condition leads to fast heartbeat, sweating and muscle tension. The researches show that, doing meditation regularly, help reducing the adrenalin level significantly.

Prevention of Exaggerated Startle Response

The timely support during the episodes of exaggerated startle response might help preventing the stress level to be higher and getting worse. Others support also may help to put off your health from turning to harmful method of coping, for example taking drugs or alcohol.

Support from others may also help prevent the sufferers if exaggerated startle response from turning to unhealthy coping methods, such as misuse of alcohol or drugs.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: May 3, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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