The guides provide a resource for clinicians about specific rare disorders to facilitate. Infantile spasms is are a type of seizure that is linked to an epileptic condition known as west syndrome. In such disorders, if a person is watched and cared. My son was diagnosed with west syndrome at 5 months on my 30th birthday. What is the life expectancy of someone with west syndrome. Know the causes, symptoms, treatment, life expectancy and prognosis of west s syndrome. The condition of west syndrome generally arises due to the combination of three different conditions of infantile spasms, an interictal electroencephalogram eeg pattern termed hypoarrhythmia, and mental retardation. Seven deceased subjects were not included in these groups.
West syndrome is a constellation of symptoms characterized by epilepticinfantile spasms, abnormal brain wave patterns called hypsarrhythmia and intellectual disability. If your child is diagnosed with an epilepsy syndrome, it means that their epilepsy has some specific signs and symptoms. Other names for it are generalized flexion epilepsy, infantile epileptic. One website reported a 3%7% mortality in patients over a 9 year period that sounds like 12 % per year. West syndrome is a type of epilepsy that develops within the first year of life and typically between four to eight months old.
This means that west syndrome, or a subtype of west syndrome, affects less than 200,000 people in the us population. The eeg is always abnormal in children with west syndrome but sometimes the abnormality is seen only during sleep. 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. Due to the various causes of west syndrome, each individual will have a somewhat different prognosis. Usually, the spasms stop by the time a child is 4 years old.
Despite the heterogeneous nature of causes of infantile spasms, a careful diagnostic evaluation can lead to diagnosis in many patients and may guide treatment choices. Medical marijuana for west syndrome marijuana doctors. Review article eissn 23500204 west syndrome and its. The diagnosis of west syndrome is made on a combination of the clinical features together with a typical eeg. A syndrome is a group of signs or symptoms that happen together and help to identify a unique medical condition. West syndrome genetic and rare diseases information. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care. According to the literature, 2, 3 the mortality varied widely between 3% and 30%, largely driven by the duration of follow. Epileptic seizures were under control by aeds in 50 patients 45. Babies with west syndrome usually have mental disabilities later in life, but up to 1 in 5 will have normal mental skills or only mild mental disabilities. Whereas children with uncomplicated epilepsy are not at higher risk of death than the general population, the mortality risk is many. Answers from specialists on west syndrome life expectancy.
Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant with is. Pdf autistic spectrum in west syndrome original article. It is often caused by brain damage due to trauma or illness. Learn more about west syndrome, its symptoms and how medical marijuana and west syndrome treatment can help ease the effects of this condition. However, children that can become free of attacks with appropriate medication about 50% of all individuals with west syndrome have a fair prognosis. West syndrome or west s syndrome is an uncommon to rare epileptic disorder in infants. West syndrome is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Children with west syndrome experience seizures, the child will suddenly jerk and then their muscles become stiff resulting in them bending forward with elevated arms or legs. West syndrome ws is the most frequent epileptic encephalopathy in the first year of life, with an incidence ranging between 2 and 3. There is no single cause for west syndrome but instead many different causes. Osbornea proposal for case definitions and outcome measures in studies of infantile spasms and west syndrome. West syndrome also called infantile spasms should go away by the time your child is 4 years old. The most common symptom of infantile spasms is stiffening of the body.
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. But most people who had it will get another kind of epilepsy or seizure condition in childhood or as an adult. About 1 in 5 will have lennoxgastaut syndrome, a severe form of epilepsy. I saw his first seizure and i knew right away that it was a seizure, which helped out a lot with early detection. West s syndrome is also known as infantile epileptic encephalopathy, generalized flexion epilepsy, infantile myoclonic encephalopathy, massive myoclonia, jackknife convulsions and salaam spasms.
It is named after the english physician, william james west 17931848, who first described it in an article published in the lancet in 1841. The guides provide a resource for clinicians about specific rare disorders to facilitate diagnosis and treatment of their patients with this 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. West syndrome is a rare epileptic encephalopathy involving infantile spasms, altered electroencephalographic pattern with hypsarrhythmia, and psychomotor development delay. The original case actually described his own son, james edwin west. What the future holds for children with west syndrome largely depends on the underlying causes. Infantile spasms are a rare epileptic syndrome presenting most commonly in infancy. They are diagnosed by recognising seizure patterns, assessing for delay in developmental milestones and a characteristic eeg termed hypsarrhythmia. He was rushed to the doctors, then to the emergency room, got an emergency eeg and he was diagnosed with west syndrome. Wests syndromecausessymptomstreatmentlife expectancy. Tell a friend about us, add a link to this page, or visit the webmasters page for free fun content. West syndrome is a rare epileptic disease having the triad of infantile spasms, a pathognomonic.
West syndrome center of excellence mcgovern medical school. The eeg shows a very disorganised pattern, called hypsarrhythmia. West syndrome an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarhythmia. West syndrome, or infantile spasms syndrome is a frequently catastrophic infantile epileptic encephalopathy with a variety of etiologies. Maybe nobody has collected and published that information. The condition is called west syndrome after a dr west who was the first to describe the condition in his own son. A specific type of seizure called infantile spasm is part of this syndrome, as are abnormal brain wave patterns called hypsarrhythmia and developmental delays. It arises in paediatric patients, generally within the first year of life, in symptomatic or idiopathic form. The spasms that occur may range from violent jackknife or salaam movements where the whole body bends in half, or they may be no more than a mild twitching of the shoulder or eye changes. Please feel free to email me with any questions or just a hello. 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. West syndrome is a severe form of encephalopathy that typically affects infants younger than 2 years old.
West syndrome infantile spasms triad of infantile spasms, developmental delay and hypsarrhythmia on eeg. West syndrome life expectancy, symptoms, treatment. West syndrome life expectancy answers on healthtap. West syndrome is a rare and severe infant epileptic encephalopathy, covering 2530% of infantile epilepsies. Cluster seizures west syndrome epilepsy foundation. On april 1st, 2014 we began the process of opening our brand new west syndrome center of excellence. West syndrome is an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia. West syndrome is an epileptic encephalopathy accounting for onefourth of epilepsies occurring in the first year of life and is highly associated with cognitive impairment. 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. He was first treated with vitamin b6 shots, and they did nothing. Babies with west syndrome usually have mental disabilities later in life, but up to 1 in 5 will have normal. But most people who had it will have other kinds of epilepsy or seizure conditions as children and adults.
West syndrome is a seizure disorder a form of epilepsy that begins in infancy. West syndrome definition of west syndrome by medical. Havent seen any numbers on life expectancy of lennoxgastaut patients. The original case actually described his own son, james edwin west 18401860. But since he was six months of age, he started having frequent seizures. How childhood epilepsy syndromes are diagnosed, details of some specific syndromes and sources of further support.
The condition of west syndrome is more prevent in male child as compare to the female child. We consulted a doctor who suggested an eeg and a mri of his brain. West syndrome is a severe epileptic encephalopathy occurring in infancy that comprises specific seizure types consisting of spasms and interictal eeg pattern known as hypsarrhythmia, together with psychomotor regression. Normally the movement of a childs arms and legs are controlled by tiny electrical signals in special parts of the brain. West syndrome treatment both the incidence and the prevalence vary significantly depending on the geographical area, however, different statistical studies have shown greater occurrence in males arce.
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