Pathologies of the brain and central nervous system significantly reduce a person’s quality of life and lead to severe disorders. Among the diseases of this group, disorders of the congenital type or those that develop in the first years of life are especially serious. They are accompanied by developmental delays and affect human health in the future. Such pathological processes include West Syndrome.
Vivantes clinics only diagnose this disease in children.
At the Vivantes clinics, diagnosis and treatment of West Syndrome in Germany are carried out by doctors of the highest category. Our clinic’s specialists perform early diagnosis of the disease using modern expert-class equipment in their practice.
Treatment of the disease is carried out according to innovative methods, it includes mainly conservative therapy, but if there are appropriate indications, surgical interventions of varying degrees of complexity are performed.
What is West Syndrome
In neurology, West syndrome is a pathological process of a non-inflammatory nature that affects the structures of the brain. This disease is characterized as infantile epileptic encephalopathy. Its main distinguishing feature is spastic muscle contractions, similar to epileptic seizures.
develops especially often in children in the first years of life. According to statistics, the manifestation of the disease occurs in the first year of a child’s life in approximately 90% of cases. In the remaining 10% of cases, the first signs of the disease appear before 4 years of age. But at the same time, in the vast majority of children, spastic spasms disappear by the age of 3, after which they transform into various forms of epilepsy.
West syndrome belongs to a group of rare neurological disorders. According to statistics, the disease accounts for about 2% of all cases of epilepsy. However, if we talk about infantile forms of epilepsy and epileptic encephalopathies, among them West syndrome accounts for about a quarter of all cases.
The causes of the development of West Syndrome in children are quite numerous, of which the most common in neurology are considered to be:
- consequences of intrauterine infections;
- fetal hypoxia at various stages of intrauterine development;
- premature birth;
- intracranial injuries received during labor;
- respiratory problems in an infant;
- some forms of abnormalities in the structure of the brain.
It is also important to understand that in approximately 13% of cases the true cause of West Syndrome in infants remains unknown.
West syndrome is a seizure disorder (a form of epilepsy) that begins in infancy. The condition is caused by damage to the brain resulting from injury or illness.
A specific type of seizure called infantile spasm is part of this syndrome, as are abnormal brain wave patterns called hypsarrhythmia and developmental delays.
What the future holds for children with West syndrome largely depends on the underlying cause of the disease.
Symptoms of West syndrome in children
West syndrome most often begins in children between 4 and 8 months of age. Symptoms of West syndrome include:
- Infantile spasms
Most often, with this type of seizure, the child suddenly leans forward at the waist, causing the body, arms, and legs to tense. This is sometimes called a "jackknife" attack. Some children may instead arch their back, shake their head, or cross their arms across their body as if hugging themselves.
The spasms last a few seconds and occur in groups of two to 100 spasms at a time; Some children experience dozens of these spasms per day.
Cramps most often occur after the child wakes up in the morning or after a nap.
- Hypsarrhythmia
A child with West syndrome has abnormal, chaotic brain wave patterns called hypsarrhythmia.
- Developmental problems
Developmental problems are associated with the brain injury that causes West syndrome.
Other symptoms may also occur due to the underlying disorder causing West syndrome. Other neurological disorders such as cerebral palsy and autism may also be present.
Infantile spasms usually go away by about 5 years of age, but more than half of children with West syndrome develop other types of seizures.
Many children with West syndrome have long-term cognitive impairment.
Causes of West syndrome
Almost any condition that can cause brain damage can cause West syndrome. Sometimes the original cause of a disease cannot be confirmed, but exists only as a theory. In this case they talk about cryptogenic West syndrome.
Establishing diagnosis
If your baby has infantile spasms, the doctor will likely test him for West syndrome.
The diagnostic process includes a thorough neurological examination to look for possible causes, including:
- Laboratory research;
- Brain scan using computed tomography (CT) or magnetic resonance imaging (MRI);
- Electroencephalogram (EEG): This test detects and records the brain's electrical activity in the form of brain waves, which can identify hypsarrhythmia.
Early diagnosis is important. The sooner a child can begin treatment, the greater the likelihood that a good result will be achieved.
Treatment of West syndrome
The most common medications used to treat West syndrome are:
- Adrenocorticotropic hormone (ACTH);
- Prednisolone;
- Vigabatrin;
- Pyridoxine.
These treatments can be very effective in stopping or slowing down infantile spasms.
Other drugs that are sometimes used:
- Felbatol (felbamate);
- Lamictal (lamotrigine);
- Topamax (topiramate);
- Depakote (valproic acid);
- Zonegran (zonisamide).
If medications are not effective enough, and especially in cases involving a developmental disorder or tuberous sclerosis complex, doctors may recommend surgical removal of the damaged brain tissue. The surgery is usually performed for several types of epilepsy and usually has good results.
Forecast
In a 2015 study looking at long-term outcomes, two-thirds of children with West syndrome continued to have what was considered an acceptable quality of life. Children who were developing normally before they started having infantile spasms, and who have no obvious underlying cause (such as brain damage or neurological disease), have the best outcomes.
Infantile spasms usually resolve by mid-childhood. However, more than half of children with West syndrome eventually develop other types of seizures, with some of these patients developing a severe form of epilepsy known as Lennox-Gastaut syndrome.
Research shows that the most important thing you can do for a child with West syndrome is to get a diagnosis quickly, start treatment, and stick to it.
Symptoms and diagnosis of West Syndrome
As mentioned earlier, in most cases the pathological process debuts in the first year of a child’s life. At the same time, the clinical picture becomes more diverse as West Syndrome develops , and the progression of the pathology begins mainly with a delay in psychomotor development. Among the most characteristic clinical signs, experts include:
- loss of the infant’s grasping reflex;
- disorders of fixation, gaze focusing;
- the child cannot follow a specific object with his eyes;
- generalized epileptic seizures of varying duration.
It is important to understand that intervals between spastic muscle contractions can be less than 1 minute. Moreover, the frequency of seizures varies significantly, from several dozen per day to hundreds. In this case, the head bends and twitches, the arms are extended, the body bends into an arc (the attack ends with complete relaxation of the body).
During the diagnostic process, our doctors pay special attention to the symptoms of West Syndrome , record every parental complaint, and find out the frequency, frequency, and nature of attacks. A detailed examination of the medical history, examination of the child is also carried out, and the specifics of psychomotor developmental delay are assessed. After this, a series of laboratory and hardware tests are carried out:
- general clinical blood and urine tests;
- biochemical blood test;
- electroencephalography;
- polysomnography;
- computed tomography or magnetic resonance imaging of the brain.
A mandatory stage of diagnosis is consultation with specialized specialists, including a pediatrician, pediatric neurologist, and epileptologist.
Treatment of West syndrome in Berlin – impressions of the patient’s parents
Anton’s parents had heard a lot about the level of German medicine even before their trip to Germany, and yet, the child’s treatment in Berlin made a very strong impression on them. Here are just a few moments when they saw striking differences in the approaches of the German and domestic doctors they met. At the Center for the Treatment of Epilepsy in Children in Berlin they were pleasantly surprised:
- excellent organization and consistency of diagnostic and treatment processes, despite the fact that many specialists from different fields simultaneously take part in them;
- very dynamic selection of the drug and its dose. Thus, from several proposed drugs, the most effective one was chosen over the course of several weeks and in the optimal dose, while at home the child took the same anticonvulsants for several months, despite the lack of results;
- assessing the effectiveness of treatment using laboratory tests;
- warmth, responsiveness, friendliness of the staff, willingness to discuss in detail all the patient’s problems, desire to explain the need for each manipulation and prescription;
- refund of part of the prepaid funds after summing up the results of treatment.
Remembering today how their child suffered from West syndrome for almost a whole year, Anton’s parents are happy that he got rid of infantile spasms. They are eternally grateful to the doctors from Berlin who found effective treatment for their son.
Conservative
As part of conservative therapy, our specialists use innovative techniques, use the latest generation drugs, the duration of administration and dosage of which are calculated individually. The main groups of drugs aimed at treating West epilepsy syndrome include:
- muscle relaxants;
- glucocorticosteroids;
- valproate group agents;
- adrenocorticotropic hormone;
- vitamins of group B6;
- immunomodulators.
Diagnostics
The diagnosis is made by a neurologist (the child may need consultation with other specialists, such as a geneticist, epileptologist, endocrinologist) based on the clinical manifestations of the disease and data obtained during instrumental studies.
To make a diagnosis, a child must undergo:
- EEG to determine brain activity during sleep and wakefulness.
- CT to detect structural changes in the organ.
- MRI to determine the extent of brain damage and identify the cause of the disease.
- PET to determine metabolic disorders in the brain.
- Angiography to evaluate the condition of blood vessels in the brain area.
- Cranioscopy to identify abnormalities in the structure of the skull.
Treatment
West syndrome is a dangerous disease in which complete cure of patients is impossible. Since the pathology rapidly and steadily progresses, it is necessary to prescribe adequate therapy as early as possible.
Convulsions that last 5-6 minutes, repeat every half hour and are accompanied by heavy, intermittent and uneven breathing are a reason to immediately call emergency services.
Drug therapy
Patients are prescribed:
- adrenocorticotropic hormone - “Corticotropin”,
- valproates - “Depakine”, “Konvulex”, “Konvulsofin”,
- glucocorticosteroids - Prednisolone, Dexamethasone, Hydrocortisone,
- central muscle relaxants - “Phenazepam”, “Clonazepam”, “Nordiazepam”,
- immunomodulators - “Octagam”, “Gamunex”, “Imbioglobulin”,
- vitamin B6.
Sick children can only be treated in a hospital setting under the constant and strict supervision of a neurologist with periodic electroencephalography.
Other treatments
- Surgery. If the cause of the attacks is tumors or tuberous sclerosis, surgical treatment is performed - removal of the lesion. The adhesions of the meninges are dissected, neoplasms and aneurysms are removed. To treat sick children, only gentle surgical techniques are used. Recovery after surgery is carried out in specialized neurorehabilitation centers.
- Unconventional therapy. Currently, the process of restoring the affected area of the brain using stem cells is quite popular and very expensive. This is an unconventional method, not yet recognized by official medicine.
- Exercise therapy. Complex therapy for the syndrome includes physical therapy. It helps to recover from a serious illness and develop new motor skills. All exercises must be performed under the strict supervision of a physical therapy specialist. But without properly selected medications, physical therapy will not bring the expected result.
- Diet therapy. The diet of patients should contain a lot of fat and a minimum of carbohydrate and protein foods. This contributes to changes in metabolism and the production of large amounts of ketones, which reduce the severity of convulsive syndrome and reduce the frequency of attacks by 2 or more times. Diet therapy is indicated for children over two years of age.
- Sick children also need consultation with a defectologist, psychologist and speech therapist.
Parents need to be explained how to care for sick children and provide them with the most comfortable life possible. Regression occurs when the child turns 3 years old. At the same time, spasms disappear. In most patients, the pathology smoothly develops into another form of epilepsy.
Surgical
Surgical interventions in the treatment of West Syndrome occur in rare cases when attacks are caused, for example, by a brain tumor or tuberous sclerosis. In such cases, surgeons of the highest category are involved, who perform operations to remove lesions and tumor processes. The maximum accuracy and efficiency of neurosurgical methods is due to both the qualifications of doctors and the use of the latest equipment, for example, the Da-Vinci robotic system.
Signs and symptoms
West syndrome has the following symptoms:
- frequent, particularly untreatable epileptic seizures;
- hypsarrhythmia – EEG changes standard for the syndrome;
- psychomotor development disorder.
In 90% of children with this disorder, seizures begin in the first year of life, most often between 3 and 8 months.
Seizures when affected are initially short and rare, and have a single character. Therefore, the disease is often not diagnosed immediately.
First, doctors determine colic, because The signs and cries that a baby makes during and after a seizure are very similar to colic.
Standard features are:
- bending the body forward;
- cramps of the body, arms and legs;
- arms and legs can be extended to the sides to the same extent for the right and left sides of the body.
Most often, each attack lasts one or two seconds, then there is a short pause, after which the next spasm occurs. In the video you can see how West syndrome manifests itself in a small child.
Despite the fact that children may experience single spasms, infantile attacks with the syndrome, as a rule, occur in a sequence of several spasms in a row.
Children with this disorder are characterized by irritability, developmental inhibition and even degradation, which do not disappear without treatment. These infants may also act as if they cannot see.
The health status returns to normal when the disease begins to be treated and the EEG data improves.
Prof. Dr. Bettina Schmitz
Neurology – Epilepsy
Head of the Department of Neurology
Specialization
- Epilepsy
- Differential diagnosis of non-epileptic paroxysms
- Long-term EEG - video monitoring
- Medical and non-medical care for paroxysms and complications associated with epilepsy
- Member of the British Medical Association
- Instructor and member of the European Academy for the Study of Epilepsy (EUREPA)
- Member of the Commission on Psychobiology and the International League Against Epilepsy (ILAE)
- Psychoorganic syndrome, World Federation of Societies of Biological Psychiatry (WFSBP)
- Video consultation
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Prof. Dr. Jörg Müller
Neurology – Parkinson's, MS
Head of the Department of Neurology
Specialization
- Recognized international expert in Parkinson's disease, dystonia and tremor
- Neuromuscular diseases
- Multiple sclerosis
- Treatment using butolotoxin (dystonia, spasticity)
- Deep brain stimulation treatment
- Author of more than 70 scientific publications, is a member of international expert councils
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Prof. Dr. Bruno-Marcel Mackert
Neurology
Head of the Department of Neurology
Specialization
- Internationally recognized specialist in stroke, peripheral nervous system diseases and clinical electrophysiology
- Author of over 40 original publications
- Board of Directors of the Berlin Stroke Society (BSA)
- Member of the Board of Directors of the Berlin Center for Stroke Research (CSB)
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Prof. Dr. Jörg Wissel, MD, FRCP
Neurological rehabilitation
Head of the Department of Neurological Rehabilitation and Physiotherapy, Department of Neurology and Rehabilitation Center
Specialization
- Neurological rehabilitation after stroke, traumatic brain injury and spinal cord injury
- Rehabilitation therapy for Parkinson's disease and dystonia
- Botulinum therapy
- Treatment of spasticity
- Neurological rehabilitation using intrathecal baclofen therapy and deep brain stimulation
- Author of more than 90 scientific publications, member of several expert commissions
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Clinical manifestations of West syndrome
As mentioned earlier, West syndrome is characterized by three main symptoms.
- Infantile spasms – the child develops spasms that often involve symmetrical muscle groups and can last from a few seconds to a minute. Sometimes these attacks can pose a threat to the child's life. It should also be mentioned that attacks often occur at the same time of day.
- Changes in the electroencephalogram that are specific.
- The child has a developmental delay. This manifestation is difficult to detect in the early stages. The fact is that at first children look absolutely normal, but in the first year of life, and even after, a slight delay is acceptable. Plus, the child is practically no different from a healthy baby.
