Symptomatic west syndrome is diagnosed when a cause has been determined as responsible. Jan 11, 2019 west syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram eeg pattern termed hypsarrhythmia, and mental retardation, although the diagnosis can be made even if 1 of the 3 elements is missing according to the international classification. Genetic etiologies are also recognized to underlie structural brain. West syndrome is an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia. My baby is suffering from west syndrome according to the neurologists at aiims.
West syndrome is a severe form of encephalopathy that typically affects infants younger than 2 years old. Apr 09, 2019 west s syndrome is also known as infantile epileptic encephalopathy, generalized flexion epilepsy, infantile myoclonic encephalopathy, massive myoclonia, jackknife convulsions and salaam spasms. The original case actually described his own son, james edwin west 18401860. West syndrome happens in about one in 2,5003,000 children. Children with west syndrome have better seizure control and development, if the treatment is started within 1 month of onset of symptoms. West syndrome nord national organization for rare disorders. Feb 15, 2006 infantile spasms and west syndrome version.
Steroids or vigabatrin are used to treat the condition of west syndrome. After an initial response, 1257% of children relapse within 6 months. Oct 16, 2014 west syndrome is rare condition with three distinct characteristics or symptoms, infantile spasms, jackknife seizure, abnormal brain waves, and mental retardation that appear between 3 and 12 months of age. Children with down syndrome and infantile spasms were identified from the comprehensive epilepsy database covering 19912008 of the. Gene abnormalities associated with west syndrome include arx, cdkl5, sptan1, stxbp1. Wests syndromecausessymptomstreatmentlife expectancy. Other names for it are generalized flexion epilepsy, infantile epileptic. Jun 09, 2017 the nord physician guide for west syndrome was developed as a free service of the national organization for rare disorders nord and its medical advisors.
Infantile spasms is is a rare condition that affects around 2500 children each year in the united states, most of them under age 2. Due to the very generous donation by the west syndrome foundation we are able to have a comprehensive center where patients from all over the world can come and receive care for west syndrome and infantile spasms. West syndrome is a seizure disorder a form of epilepsy that begins in infancy. The international league against epilepsy ilae diagnostic manuals goal is to assist clinicians who look after people with epilepsy to diagnose the epilepsy syndrome and if possible the etiology of the epilepsy. Symptomatic infantile spasms west syndrome is a severe form of childhood epilepsy which presents around 36 months of age. If your child is diagnosed with infantile spasms, discuss treatment options with the doctor. The westsyndrome is an infantile epileptic encephalopathy, which was first described by the english physician william james west. Aug 23, 2018 west syndrome is an agerelated epilepsy syndrome that is most common within the first 12 months of life.
Westsyndrome a case of severe vaccination damage and. However, it should be remembered that homeopathy treats each patient individually, and that no sure prognosis about a complete cure restitutio ad integrum can be given in advance. A specific type of seizure called infantile spasm is part of this syndrome, as are abnormal brain wave patterns called hypsarrhythmia and developmental delays. Thus, the primary focus of clinicians should be early identification and prompt initiation of definitive therapy. Treatment of infantile spasms has little class i data, but adrenocorticotropic. West syndrome is characterized by the onset of epileptic spasms, typically in the first year of life. Jun 11, 2012 however, it should be remembered that homeopathy treats each patient individually, and that no sure prognosis about a complete cure restitutio ad integrum can be given in advance. Many children with west syndrome will have moderate to severe learning. Epileptic spasms, is an uncommontorare epileptic disorder in infants, children and adults. Nov 22, 2017 generally, the typical development of the clinical syndrome of west syndrome occurs around 48 months of age national institute of neurological disorders and stroke, 2015. Although is was first described over 160 years ago, its diagnosis, evaluation, and management continue to pose many challenges to health care professionals and affected families. West syndrome center of excellence mcgovern medical school. West syndrome accounts for approximately 30 percent of all cases of epilepsy affecting infants.
Treatment outcomes of west syndrome in infants with down syndrome. The onset is usually in the first year of life, typically between 48 months. West syndrome or infantile spasms are a rare form of severe epilepsy, described for the first time by west in 1841. Adrenocorticotropic hormone for the treatment of west. In addition, early treatment may only slightly improve longterm outcomes. After a short trial of pyridoxine, acth or vigabatrin is the firstline drug. Infantile spasms west syndrome content highlights an infantile spasm is a seizure with sudden stiffening of the body and brief bending forward or backward of the arms, legs and head. It is important to weigh the possible benefits and risks of any given treatment. West syndrome symptoms, causes, treatment, prognosis.
Children presenting with infantile spasms and hypsarrhythmia have a high risk of developing cognitive deterioration the so called west syndrome. The triad of spasms, arrest of psychomotor development, and hypsarrhythmia is known as west syndrome. Pdf shortterm nonhormonal and nonsteroid treatment in. Wests syndrome is also known as infantile epileptic encephalopathy, generalized flexion epilepsy, infantile myoclonic encephalopathy, massive myoclonia, jackknife convulsions and salaam spasms. It is characterized by the triad of infantile spasms, neurodevelopmental regression or delay, and hypsarrhythmia on electroencephalogram eeg. Generally, the typical development of the clinical syndrome of west syndrome occurs around 48 months of age national institute of neurological disorders and stroke, 2015. Global developmental impairment with or without regression is typically seen. On april 1st, 2014 we began the process of opening our brand new west syndrome center of excellence. Treatment for infantile spasms epilepsy foundation. A proportion of cases have a genetic etiology and inheritance pattern depends on the gene involved. In the picture on the right the arms have been lifted up and slightly outwards, the eyes have opened and the head has bent slightly forwards. Guidelines for imaging infants and children with recentonset epilepsy.
Clinical features and diagnosis of infantile spasms. Due to the poor prognosis associated with the condition, treatment is usually initiated in. Steroids and antiseizure medication are the most common treatments. The age of that baby is 11 months, weighing about 10 kg. Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant with is. The west syndrome ws is one of the catastrophic epileptic syndromes in infancy characterised by triad of infantile spasms, psychomotor. Thereafter, spasms tend to disappear before 5 years of age, but relapses are possible, as in our patient. The international league against epilepsy commission classifies the aetiologies of ws into genetic, structural, metabolic and unknown. Currently, the most important modifiable risk factor responsible for poor response in west syndrome is the long treatment lag which is even more pronounced in developing countries. Infantile spasms information page national institute of. West syndrome genetic and rare diseases information. The west syndrome is an infantile epileptic encephalopathy, which was first described by the english physician william james west.
West syndrome is a type of epilepsy that affects babies. What the future holds for children with west syndrome largely depends on the underlying causes. Always seek the advice of your own physician or other qualified health care professional regarding any medical. The japanese scheme of acth therapy in west syndrome. West syndrome is an agerelated epilepsy syndrome that is most common within the first 12 months of life. Infantile spasm west syndrome clinical presentation.
Drugs used to treat west syndrome the following list of medications are in some way related to, or used in the treatment of this condition. This syndrome leads to developmental regression and causes a specific pattern, known as hypsarrhythmia chaotic brain waves, on electroencephalography eeg testing. Outcomes may be more easily predicted when the cause of the disorder is not known. In september 2014, west virginia neonatologists and pediatricians met with coders and members of the perinatal partnership to develop a standardized definition for neonatal withdrawal and guidance on documenting exposure and withdrawal in newborns. These tests help in knowing the cause behind the occurrence of west syndrome.
Prognosis the long term prognosis is poor in the majority, however early treatment can improve prognosis. West syndromeis is characterized by epileptic spasms, developmental problems, and a specific brain wave pattern on electroencephalography eeg testing called hypsarrhythmia. West syndrome is generally known as infantile spasm. In other cases, treatment of west syndrome is difficult and results of therapy dissatisfying. It is often caused by brain damage due to trauma or illness. It is a difficult epilepsy to control and only 25% of children reach normalcy. West syndrome is a rare neurological syndrome that can affect males and females. Ws is commonly associated with poor longterm outcome, especially in symptomatic cases, with development of other seizure types, impaired cognitive and. West syndrome is one of the most refractory epileptic syndromes in infancy, and many researchers have made great effort to find optimal treatment modalities for this syndrome. Development and psychokinetic therapy of children suffering from. Korey department of neurology, albert einstein college of medicine and monte.
West syndrome is characterized by a specific type of seizure infantile spasms seen in infancy and childhood. Although seizure control may be initially achieved, children commonly relapse and require alternative medication including the ketogenic diet. The guides provide a resource for clinicians about specific rare disorders to facilitate diagnosis and treatment of their patients with this condition. The life expectancy for west syndrome depends upon the cause and severity of the symptoms. Educating pediatricians and general pediatric health care providers about is may be especially. Know the causes, symptoms, treatment, life expectancy and prognosis of west s syndrome.
Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care. Know the causes, symptoms, treatment, life expectancy and prognosis of wests syndrome. Neonatal abstinence syndrome surveillance in west virginia. West syndrome with left hemiparesis treatment youtube. West syndrome is a severe form of epilepsy of early infancy.
Shortterm nonhormonal and nonsteroid treatment in west syndrome. The condition of west syndrome that occurs in infants is curable. Treatment can also help the child to develop normally or more normally than they would have without treatment. It is named after the english physician, william james west 17931848, who first described it in an article published in the lancet in 1841. West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram eeg pattern termed hypsarrhythmia, and mental retardation, although the diagnosis can be made even if 1 of the 3 elements is missing according to the international classification. This term denotes the concept that the epileptic activity itself might directly contribute. It is called this after dr william west, who first described the condition in his 4monthold son in 1841. Up to 70% of affected females develop infantile spasms is, often in conjunction with hypsarrythmia, and in some cases, they are diagnosed with west syndrome. With early treatment the seizures can be controlled. West syndrome genetic and rare diseases information center.
The majority of patients with west syndrome have structural brain abnormalities. His walking improved after west syndrome with left hemiparesis treatment. Sometimes called west syndrome, infantile spasms demands early identification, diagnosis, and treatment to help limit lasting effects. West syndrome, or infantile spasms syndrome is a frequently catastrophic infantile. The infantile spasms usually begin in the first year of life, typically between 48 months. About half of all children can become totally free of attacks with the aid of medication. Westsyndrome a case of severe vaccination damage and its. The brain scan includes magnetic resonance imaging. West syndrome ws, also known as infantile spasms, occurs in infancy with a peak between 4 and 7 months. West syndrome ws is one of the catastrophic epileptic syndromes in infancy characterized by a triad of infantile spasms, psychomotor deterioration and hypsarrhythmic eeg pattern. West syndrome treatment both the incidence and the prevalence vary significantly depending on the geographical area, however, different statistical studies have shown.
Treatment is satisfactory in about 3 out of 10 cases with 1 in every 25 showing motoric and cognitive development evolving more or less normally. Note west syndrome is considered an epileptic encephalopathy. Epileptic spasms must be associated with a psychomotor regression and electroencephalography eeg hypsarythmia to make the diagnosis of west syndrome. The other names of this condition are generalized flexion epilepsy, infantile epileptic encephalopathy, infantile myoclonic encephalopathy, jerk knife convulsion, massive myoclonic, and salaam spasm. The sooner your little one starts treatment for infantile spasms or west syndrome, the better, because this condition can affect how his mind and body develop. A syndrome is a group of signs and symptoms which, if they occur together, can suggest a particular condition. Treatment outcomes of west syndrome in infants with down.
In finland, the following guidelines are given guide lines of childhood epilepsy, 2007. Occurrence, outcome, and prognostic factors of infantile spasms and lennoxgastaut syndrome. Original article treatment outcomes of west syndrome in infants with down syndrome oranee sanmaneechai mda, yoshimi sogawa mda,b,c, wendy silver mda,b, karen ballabangil mda,b,c, solomon l. The combination of the infantile spasms, age of onset and eeg pattern defines the epilepsy syndrome called, west syndrome. It is well known that the incidence of major forms of epilepsy is higher in children with down syndrome than in. Treatment and cure for west syndrome doctor answers on. West syndrome symptoms, causes, treatment, prognosis, life. Treatment options in refractory west syndrome springerlink. The steroid may have adrenocorticotropic hormone acth and be injected into the muscles. West syndrome is rare condition with three distinct characteristics or symptoms, infantile spasms, jackknife seizure, abnormal brain waves, and mental retardation that appear between 3 and 12 months of age. West syndrome symptoms, treatment, life expectancy. The condition can be corrected by proper medication.
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