West syndrome is a seizure disorder a form of epilepsy that begins in infancy. West syndrome with left hemiparesis treatment youtube. Know the causes, symptoms, treatment, life expectancy and prognosis of wests syndrome. It is well known that the incidence of major forms of epilepsy is higher in children with down syndrome than in. In finland, the following guidelines are given guide lines of childhood epilepsy, 2007. In other cases, treatment of west syndrome is difficult and results of therapy dissatisfying. Occurrence, outcome, and prognostic factors of infantile spasms and lennoxgastaut syndrome. 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. The triad of spasms, arrest of psychomotor development, and hypsarrhythmia is known as west syndrome. West syndrome or infantile spasms are a rare form of severe epilepsy, described for the first time by west in 1841. This term denotes the concept that the epileptic activity itself might directly contribute additional cognitive and behavioral impairments. Treatment options in refractory west syndrome springerlink. The guides provide a resource for clinicians about specific rare disorders to facilitate diagnosis and treatment of their patients with this condition. Pdf shortterm nonhormonal and nonsteroid treatment in.
A specific type of seizure called infantile spasm is part of this syndrome, as are abnormal brain wave patterns called hypsarrhythmia and developmental delays. Infantile spasm west syndrome clinical presentation. The condition of west syndrome that occurs in infants is curable. Gene abnormalities associated with west syndrome include arx, cdkl5, sptan1, stxbp1. The combination of the infantile spasms, age of onset and eeg pattern defines the epilepsy syndrome called, west syndrome. West syndrome is generally known as infantile spasm.
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. The west syndrome ws is one of the catastrophic epileptic syndromes in infancy characterised by triad of infantile spasms, psychomotor. In addition, early treatment may only slightly improve longterm outcomes. 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. 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.
The original case actually described his own son, james edwin west 18401860. 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. Wests syndrome is also known as infantile epileptic encephalopathy, generalized flexion epilepsy, infantile myoclonic encephalopathy, massive myoclonia, jackknife convulsions and salaam spasms. Children with west syndrome have better seizure control and development, if the treatment is started within 1 month of onset of symptoms. 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. Outcomes may be more easily predicted when the cause of the disorder is not known. His walking improved after west syndrome with left hemiparesis treatment.
Steroids and antiseizure medication are the most common treatments. It is named after the english physician, william james west 17931848, who first described it in an article published in the lancet in 1841. Spasms, neurodevelopmental regression and hypsarrhythmia on electroencephalogram eeg basically define ws. My baby is suffering from west syndrome according to the neurologists at aiims. West syndrome is an agespecific epileptic disorder that occurs in infancy and early childhood. Infantile spasms information page national institute of. Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant with is. Neonatal abstinence syndrome surveillance in west virginia. 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. Treatment for infantile spasms epilepsy foundation. Westsyndrome a case of severe vaccination damage and its. Despite early diagnosis and rapid initiation of effective treatment, west syndrome is still associated with a poor longterm prognosis.
West syndrome symptoms, causes, treatment, prognosis. After a short trial of pyridoxine, acth or vigabatrin is the firstline drug. Treatment of infantile spasms has little class i data, but adrenocorticotropic. Children with down syndrome and infantile spasms were identified from the comprehensive epilepsy database covering 19912008 of the.
Epileptic spasms, is an uncommontorare epileptic disorder in infants, children and adults. West syndrome is characterized by the onset of epileptic spasms, typically in the first year of life. Note west syndrome is considered an epileptic encephalopathy. Clinical features and diagnosis of infantile spasms. About half of all children can become totally free of attacks with the aid of medication. 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. The japanese scheme of acth therapy in west syndrome. West syndrome is characterized by a specific type of seizure infantile spasms seen in infancy and childhood. 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 severe form of epilepsy of early infancy. West syndrome is a rare neurological syndrome that can affect males and females. Korey department of neurology, albert einstein college of medicine and monte.
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. It is a difficult epilepsy to control and only 25% of children reach normalcy. Adrenocorticotropic hormone for the treatment of west. West syndrome ws, also known as infantile spasms, occurs in infancy with a peak between 4 and 7 months. Aug 23, 2018 west syndrome is an agerelated epilepsy syndrome that is most common within the first 12 months of life. 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. Although seizure control may be initially achieved, children commonly relapse and require alternative medication including the ketogenic diet. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care. 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. These tests help in knowing the cause behind the occurrence of west syndrome.
Always seek the advice of your own physician or other qualified health care professional regarding any medical. Wests syndromecausessymptomstreatmentlife expectancy. The steroid may have adrenocorticotropic hormone acth and be injected into the muscles. West syndrome happens in about one in 2,5003,000 children. 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. A proportion of cases have a genetic etiology and inheritance pattern depends on the gene involved. This syndrome leads to developmental regression and causes a specific pattern, known as hypsarrhythmia chaotic brain waves, on electroencephalography eeg testing. Treatment can also help the child to develop normally or more normally than they would have without treatment. The onset is usually in the first year of life, typically between 48 months. 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.
It is often caused by brain damage due to trauma or illness. West syndrome nord national organization for rare disorders. Symptomatic infantile spasms west syndrome is a severe form of childhood epilepsy which presents around 36 months of age. West syndrome is an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia. The brain scan includes magnetic resonance imaging. 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 symptomsinfantile spasms, life expectancy. If your child is diagnosed with infantile spasms, discuss treatment options with the doctor. 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. Feb 15, 2006 infantile spasms and west syndrome version. West syndrome symptoms, causes, treatment, prognosis, life.
West syndrome center of excellence mcgovern medical school. Know the causes, symptoms, treatment, life expectancy and prognosis of west s syndrome. West syndrome is a type of epilepsy that affects babies. The west syndrome is an infantile epileptic encephalopathy, which was first described by the english physician william james west. Ws is commonly associated with poor longterm outcome, especially in symptomatic cases, with development of other seizure types, impaired cognitive and. Prognosis the long term prognosis is poor in the majority, however early treatment can improve prognosis. The age of that baby is 11 months, weighing about 10 kg. Shortterm nonhormonal and nonsteroid treatment in west syndrome. What the future holds for children with west syndrome largely depends on the underlying causes. Symptomatic west syndrome is diagnosed when a cause has been determined as responsible. The infantile spasms usually begin in the first year of life, typically between 48 months. West syndrome treatment both the incidence and the prevalence vary significantly depending on the geographical area, however, different statistical studies have shown. The life expectancy for west syndrome depends upon the cause and severity of the symptoms. The westsyndrome is an infantile epileptic encephalopathy, which was first described by the english physician william james west.
Other names for it are generalized flexion epilepsy, infantile epileptic. The majority of patients with west syndrome have structural brain abnormalities. Thus, the primary focus of clinicians should be early identification and prompt initiation of definitive therapy. Children presenting with infantile spasms and hypsarrhythmia have a high risk of developing cognitive deterioration the so called west syndrome. Treatment and cure for west syndrome doctor answers on. West syndrome genetic and rare diseases information center. It is characterized by the triad of infantile spasms, neurodevelopmental regression or delay, and hypsarrhythmia on electroencephalogram eeg. West syndrome, or infantile spasms syndrome is a frequently catastrophic infantile. After an initial response, 1257% of children relapse within 6 months. 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. 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.
Steroids or vigabatrin are used to treat the condition of west syndrome. 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. 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. 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. Many children with west syndrome will have moderate to severe learning. West syndrome accounts for approximately 30 percent of all cases of epilepsy affecting infants. West syndrome is an agerelated epilepsy syndrome that is most common within the first 12 months of life. Epileptic spasms must be associated with a psychomotor regression and electroencephalography eeg hypsarythmia to make the diagnosis of west syndrome.
Genetic etiologies are also recognized to underlie structural brain. This term denotes the concept that the epileptic activity itself might directly contribute. Thereafter, spasms tend to disappear before 5 years of age, but relapses are possible, as in our patient. Treatment outcomes of west syndrome in infants with down syndrome.
West syndrome is a severe form of encephalopathy that typically affects infants younger than 2 years old. The international league against epilepsy commission classifies the aetiologies of ws into genetic, structural, metabolic and unknown. West syndrome genetic and rare diseases information. With early treatment the seizures can be controlled. The xlinked form of west syndrome affects males more often than females. Sometimes called west syndrome, infantile spasms demands early identification, diagnosis, and treatment to help limit lasting effects. West syndromeis is characterized by epileptic spasms, developmental problems, and a specific brain wave pattern on electroencephalography eeg testing called hypsarrhythmia. The other names of this condition are generalized flexion epilepsy, infantile epileptic encephalopathy, infantile myoclonic encephalopathy, jerk knife convulsion, massive myoclonic, and salaam spasm. Treatment outcomes of west syndrome in infants with down. Educating pediatricians and general pediatric health care providers about is may be especially.
It is called this after dr william west, who first described the condition in his 4monthold son in 1841. On april 1st, 2014 we began the process of opening our brand new west syndrome center of excellence. Global developmental impairment with or without regression is typically seen. Guidelines for imaging infants and children with recentonset epilepsy. The condition can be corrected by proper medication.
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. Infantile spasms is is a rare condition that affects around 2500 children each year in the united states, most of them under age 2. Westsyndrome a case of severe vaccination damage and. Due to the poor prognosis associated with the condition, treatment is usually initiated in.