What Is Pediatric Myoclonus
Myoclonic twitches happen unexpectedly and will range from small disturbances to severe, jarring movements that affect a childs quality of life .
Myoclonic movements cant be controlled and can occur at any time often happening at night when the body begins to relax at the beginning of sleep. The actions can move from one location of the body to another and may vary in frequency from multiple twitches a minute to a few spasms per hour.
How Is Myoclonus Diagnosed
Following a review of the persons medical history and physical exam, a physician may order additional tests to confirm the diagnosis of myoclonus:
- Electromyography , which measures electrical activity of muscle, is the commonly used method to diagnose myoclonus as well as nerve and muscle dysfunction
- Electroencephalography uses electrodes attached to the scalp to record the electrical activity of the brain that may trigger the myoclonic jerk
- Evoked potential studies capture the electrical activity in the brain, brain stem, and spinal cord evoked by specific stimuli
- Laboratory urine or blood tests for possible causes and to rule out other conditions that may cause symptoms similar to myoclonus
- Magnetic resonance imaging , using computer-generated radio waves and a magnetic field, to produce three-dimensional images of the brain, spinal cord, nerve, and other tissue .
How Is Myoclonic Epilepsy Diagnosed
Correct diagnosis of the seizure origin is of prime importance, because seizures can also occur due to many conditions other than epilepsy. Ascertaining if different types of seizure disorders are co-occurring, and identification of the distinct epilepsy syndrome are essential to formulate the appropriate treatment.
Diagnostic tests include:
- Physical examination, or videotaping by caregivers when direct observation is not possible due to the unpredictable nature of the illness
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What You Need To Know
- The word myoclonic combines the Greek prefix for muscle myo with clonus, which means twitching.
- Myoclonic seizures do not cause any loss of awareness the person is awake and conscious during the seizure.
- Infantile spasms and Lennox-Gastaut syndrome are two of the epilepsy syndromes characterized by myoclonic seizures, among other symptoms.
Understanding The Vicious Cycle
Sleep and epilepsya vicious cycle.
Childrens sleep passes through different sleep stages conventionally divided into rapid eye movement and non-REM sleep. NREM sleep coordinates electrical brain activity into important slow oscillation and faster bursts called spindles. Sleep research suggests that this slow wave synchrony and spindle activity contribute to the rejuvenation of synapses and overnight learning. Unfortunately, it also results in a vulnerable landscape, primed to allow easy and rapid generation of seizure activity.
In this vicious cycle, anything that causes sleep disturbance or fragmentation can further trigger seizures. This includes sleep disorders, antiepileptics or seizures themselves. The association is a powerful one and epilepsy is associated with at least a 12 times higher rate of child-reported and parent-reported sleep disorders, even without nocturnal seizures.
Evidence shows that consolidation of memory during sleep is disrupted by seizures and by nocturnal interictal electroencephalograph discharges.
All these causes of sleep disturbance also leads to daytime sleepiness and worse behaviour, cognitive functioning and quality of life. The impact extends to the whole family parents of these children wake seven times more often than controls and spend an average of 4hours asleep, with adverse effects on marital satisfaction and maternal health.
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Symptoms In The Brain
Even though they’re tiny, thereâs a lot of electrical activity going in your babyâs brain. If itâs abnormal, they might have seizures. Your babyâs doctor may use electroencephalography — EEG for short — to measure their brain activity while they’re awake and asleep. Theyâll put sticky tabs called electrodes on their head, and a machine will record the data they pick up. Babies with infantile spasms often have an abnormal pattern of electrical activity in their brains. It’s called hypsarrhythmia.
The doctor might also want to do scans of their brain. Computed tomography and magnetic resonance imaging scans create pictures that will let them see if there are any parts of it that havenât formed the right way. These pictures can also show lesions, or places where injury or infection may have damaged their brain.
When Is Twitching In Sleep A Concern
Twitching in sleep is usually harmless. However, the primary difference between night myoclonus and other conditions is that it occurs only in sleep. If you notice twitching or stiffening when your baby is awake or if you have developmental concerns, it may be a cause for concern and require a medical evaluation for the probable presence of the following conditions.
- Infantile spasm: It is a type of epilepsy and occurs between two to 12 months of age but becomes prominent when the baby is four to eight months old. The seizures usually last one or two seconds but occur in a series every five to ten seconds. During these infantile spasms, the babys body stiffens, while the arms, legs, and head may bend forward, and the back may arch .
- Benign familial neonatal convulsions: This condition is characterized by recurrent seizures that begin when the newborn is three days old and resolve by the time the baby is one to four months old. The seizures can involve either one or both sides of the brain and affect the entire body. The signs may include convulsions, muscle rigidity, and loss of consciousness .
- These seizures are usually caused by a spike in body temperature or fever along with an infection . Children between the ages of six months and five years may experience febrile seizures. In the majority of the cases, febrile seizures are a form of convulsions. The symptoms may include loss of consciousness, vigorous shaking of arms or legs, rolling of eyes, and rigid limbs .
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The Role Of Cyclic Alternating Pattern
CAP is defined as an alternation of transient activations over a more stable background pattern within periods of NREM sleep .
The different A phases express the arousability-reactivity of the sleeping brain at different ages and sleep stages A1 being prevalent in younger subjects during N3, whereas A2 and A3 express more sleep disruption in adults. Both nocturnal seizures in SHE, as well as SRMD and parasomnias, occur during the A phases, once again confirming the arousal mechanism as the prompting disinhibiting factor initiating the motor rhythm pattern in seizures as well as in normal sleep.
Sleep Myoclonus In Children
Myoclonus is a condition that may worry parents when it occurs in children as it may seem like a seizure or infantile spasms. The important difference is that sleep myoclonus occurs only in sleep.
Infants who experience sleep myoclonus should have a neurologic examination and electroencephalogram , a test that measures electrical activity in the brain. If these movements occur during wakefulness, further evaluation by your child’s doctor may be needed to rule out seizures and other causes.
Sleep myoclonus is common during the first week of a newborn’s life and usually resolves within a year. Commonly referred to as “benign neonatal sleep myoclonus,” there are no inherent consequences to the condition or reasons for concern.
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What Causes A Seizure In A Child
A seizure can be caused by many things. These can include:
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An imbalance of nerve-signaling brain chemicals
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Genetics
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Breathing problems or stopping breathing
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Loss of bowel or bladder control
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Falling suddenly for no apparent reason, especially when associated with loss of consciousness
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Not responding to noise or words for brief periods
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Appearing confused or in a haze
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Nodding head rhythmically, when associated with loss of awareness or consciousness
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Periods of rapid eye blinking and staring
During the seizure, your childs lips may become tinted blue and his or her breathing may not be normal. After the seizure, your child may be sleepy or confused.
The symptoms of a seizure may be like those of other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.
Is Twitching A Sign Of Seizure
Suspect a seizure when jerking occurs while the baby is awake, and it lasts more than 10 to 20 seconds. Also the movements that occur in seizures are involuntary and are not associated with voluntary or play movements. However, it is recommended to consult a doctor for the right diagnosis and rule out other conditions.
Twitching is usually not a cause of concern and more often related to your babys sensorimotor development. Most babies twitch in sleep during infancy. However, if your baby has been experiencing certain seizure-like symptoms recurrently when awake, it is recommended to seek expert advice for the right diagnosis.
References:
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Key Points About Epilepsy And Seizures In Children
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A seizure occurs when one or more parts of the brain has a burst of abnormal electrical signals that interrupt normal signals
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There are many types of seizures. Each can cause different kinds of symptoms. These range from slight body movements to loss of consciousness and convulsions.
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Epilepsy is when a person has 2 or more seizures with no known cause.
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Epilepsy is treated with medicine. In some cases, it may be treated with VNS or surgery.
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Its important to avoid anything that triggers seizures. This includes lack of sleep.
Children and Epilepsy: Everything a Family Needs to Know
Pediatric epilepsy specialists Dr. Dody Robinson and Dr. Sarah Kelley recently hosted a webinar for patients and families. Hear from them about the latest in surgical and non-surgical treatment options for this neurological condition.
What Is Epilepsy In Children
Epilepsy is a brain condition that causes a child to have seizures. Itis one of the most common disorders of the nervous system. It affectschildren and adults of all races and ethnic backgrounds.
The brain consists of nerve cells that communicate with each otherthrough electrical activity. A seizure occurs when one or more parts ofthe brain has a burst of abnormal electrical signals that interruptnormal brain signals. Anything that interrupts the normal connectionsbetween nerve cells in the brain can cause a seizure. This includes ahigh fever, high or low blood sugar, alcohol or drug withdrawal, or abrain concussion. But when a child has 2 or more seizures with no knowncause, this is diagnosed as epilepsy.
There are different types of seizures. The type of seizure depends onwhich part and how much of the brain is affected and what happensduring the seizure. The 2 main categories of epileptic seizures arefocal seizure and generalized seizure.
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What Are The Symptoms Of A Seizure In A Child
Your childs symptoms depend on the type of seizure. General symptomsor warning signs of a seizure can include:
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Staring
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Breathing problems or stopping breathing
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Loss of bowel or bladder control
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Falling suddenly for no apparent reason, especially when associated with loss of consciousness
-
Not responding to noise or words for brief periods
-
Appearing confused or in a haze
-
Nodding head rhythmically, when associated with loss of awareness or consciousness
-
Periods of rapid eye blinking and staring
During the seizure, your childs lips may become tinted blue and his orher breathing may not be normal. After the seizure, your child may besleepy or confused.
The symptoms of a seizure may be like those of other health conditions.Make sure your child sees his or her healthcare provider for adiagnosis.
What Research Is Being Done
The mission of the National Institute of Neurological Disorders and Stroke is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. NINDS is a component of the National Institutes of Health, the leading federal supporter of biomedical research in the world. As part of its mission, the NINDS supports research on myoclonus at its laboratories in Bethesda, Maryland and through grants to major research institutions across the country.
In addition to NINDS, other NIH institutes and centers support research on movement disorders that include myoclonus. More information is available through the NIH RePORTER, a searchable database of current and previously funded research, as well as research results and publications.
Many neurological disorders do not have effective treatment options. Clinical studies offer hope for many people and an opportunity to help researchers find better ways to safely detect, treat, or prevent disease. For more information about finding and participating in a clinical study, visit Clinicaltrials.gov. Use the search term myoclonus to find trials on this disorder.
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Central Pattern Generators : The Carillon Theory
An Italian neurologist, C.A. Tassinari, claimed that genetically determined motor neural networks embedded in the spinal cord and brainstem of primates, first described by T.G. Brown in 1911 , can generate coordinated rhythmic movement patterns. These networks named Central Pattern Generators are able to promote a spectrum of species-specific self-sustained patterns of archetypical behaviors. The latter include locomotion, self-defense, feeding and reproductive behaviors critical for survival.
According to the Triune brain theory by MacLean , they are the expression of the disinhibition of more primitive layers of the human brain, the limbic or paleopallium, and the midbrain or reptilian brain, which under certain conditions may escape the control of our most elaborate brain, the neopallium . An arousal is an event that triggers a spectrum of behaviors that look the same whether they are part of a seizure or of a parasomnia/sleep motor event.
Fig. 1
The Triune Brain . The immature paleomammalian neonatal brain is unable to efficiently control motor output, due to incomplete myelination. Thus, fundamental and protective motor reflexes, like grasping and avoidance, are carried out by subcortical CPGs. The mature neopallium controls and inhibits during conscious wakefulness most of these reflexes which may reemerge under special conditions, due to transient loss of neocortical control, as in c , or in d . Modified with permission from Tassinari et al., 2009
What Is A Myoclonic Seizure
Myoclonic seizures are brief, shock-like jerks of a muscle or a group of muscles. “Myo” means muscle and “clonus” means rapidly alternating contraction and relaxationjerking or twitchingof a muscle. Usually they don’t last more than a second or two. There can be just one, but sometimes many will occur within a short time.
Even people without epilepsy can experience myoclonus in hiccups or in a sudden jerk that may wake you up as you’re just falling asleep. These things are normal.
In epilepsy, myoclonic seizures usually cause abnormal movements on both sides of the body at the same time. They occur in a variety of epilepsy syndromes that have different characteristics:
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Baby Twitching In Sleep: Is This Normal
Youre lovingly watching your baby peacefully snooze when suddenly you notice theyre twitching. You might think that the tiny twitches you see in your baby are a response to a dream. You might worry that theyre some kind of seizure.
But hold on, because researchers now believe that many of those twitches actually contribute to your babys motor skills development.
Benign Sleep Myoclonus Of Infancy
Benign sleep myoclonus of infancy is characterized by repetitive myoclonic jerks that occur during sleep in neonates and infants.3 Its diagnostic features include the observation of repetitive myoclonic jerks involving limbs, trunk, or whole body. This movements occur only during sleep and stop abruptly and consistently when the infant is aroused. BSMI occurs in early infancy, typically from birth to 6 months of age.
Standardized scoring criteria for BSMI are lacking, but the muscle jerks last between 0.04 to 0.3 second and occur usually in clusters of 4 to 5 jerks per second.3,136 These clusters may repeat in irregular series for 1 to 15 minutes, and in rare cases up to 60 minutes. The jerks often are bilateral and typically involve large muscle groups. BSMI is benign and relatively rare, but it has been included in the ICSD3 as a sleep-related movement disorder because it often is confused with epilepsy.3
Kenneth F. Swaiman MD, inSwaiman’s Pediatric Neurology, 2017
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Reduces The Burden Of Care
Extensive clinical studies have proven that NightWatch can detect 85% of all severe nocturnal seizures and as many as 96% of the most dangerous seizures. The wireless system reduces the burden of care on you, the parent/carer, by alerting you in the event of a nocturnal epileptic seizure. As well as helping to increase confidence and reduce stress, it is also reassuring to parents. You can go back to sleeping in your own bed, both you and your child will have more privacy and you will have more restful nights. After all, everyone needs a good night’s sleep in order to function properly.
What Is This Sleep Twitching
While it may not seem like it when you wake up in the middle of the night for the fourth time, babies actually sleep a lot. You can figure on about 14 to 17 hours per 24-hours.
Youll spend some of that time wishing those hours were consecutive and some of that time just watching your baby sleep. For the pure pleasure of it.
Thats when youre likely to notice that your baby is twitching in their sleep. Heres the correct term for the twitches: sleep myoclonus .
Generally, these twitches are perfectly normal. In fact, now believe that these myoclonic twitches help babies transform their rudimentary movements into coordinated movements.
There are two types of sleep twitches:
- Myoclonic twitches caused by sudden muscle contractions. This is known as positive myoclonus.
- Myoclonic twitches caused by muscle relaxation. This is negative myoclonus.
If youve ever been just on the verge of sleep and suddenly felt your body jerk, startling you, youve experienced a form of myoclonus a hypnagogic jerk. Another type of myoclonus youre likely familiar with? Hiccups.
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