Lennox-Gastaut Syndrome as a Childhood Brain Disorder - Case Study Example

Lennox Gastaut syndrome Background Epilepsy is considered as one of the first brain disorders that were known to man. More than 3,000 years ago, the term was cited in ancient Babylon. In this society, the queer behaviors and actions brought about by seizures added to many superstitions and ostracism. However, in 400 B.C, Hippocrates wrote the first ever book which featured epilepsy entitled “On the Sacred Disease”. In Hippocrates’ time, epilepsy was known to be a curse. In his book, Hippocrates lent evidence suggesting that epilepsy is a brain disorder and not otherwise (Beaumanoir, 1982). In 70 A.D literature, in the Gospel according to Mark (9:14-29), Jesus was said to have casted out an evil spirit from a man with such condition. The man’s father said to Jesus that his son is possessed by a spirit who caused him loss of speech. Furthermore, the father said that whenever the spirit has his son, it throws him to the ground where he later on foams on the mouth and becomes immovable (Beaumanoir, 1982). These were the implicit notions regarding what people now know properly as epilepsy. There are many kinds of epilepsy, but to the layman the image of a person who is having an epileptic seizure cries out, falls to the floor-at most time without consciousness, does not have control over his movements, drools and even loses control over his bladder. This is properly termed as a generalized tonic-clonic seizure which is only one of the many types (Beaumanoir, 1982). This paper will look at the definition, causes, incidence and main characteristics of the Lennox Gaustat Syndrome which Jacinta is suffering from. Moreover, it will also include the health care needs for the first 10 years of life that relate the diagnosis of the disability. However, before moving to these specific areas, the author will first look at the dynamics of epilepsy where Lennox Gaustaf Syndrome is among its kinds. Epilepsy Epilepsy is a malfunctioning of the brain in which groups of nerve cells, or neurons, in the brain sometimes send signals out of the normal. By nature, these neurons work in order to garner and send electrochemical impulses that act on other neurons, glands, and muscles to create human thoughts, feelings and actions (Heiskala 1997). What happens in epilepsy is that the normal pattern of neuronal activity is disrupted. This interruption brings about queer affect and behavior or at times, convulsions, muscle spasms and the loss of consciousness. For the period of a seizure, neurons can or may fire as many as 500 times a second, which is way faster than normal. For some who suffer from epilepsy, a seizure can transpire only occasionally, but to some it may occure more than a hundred times a day (Heiskala 1997). In the case of the United States, it has been reported that about 1 in 100 have experienced an uncalled-for seizure or been diagnosed with epilepsy. Around 80 percent of those diagnosed with epilepsy was said to be controllable with the use of modern medicines and surgical techniques. However, the remaining third of the people will continue to experience seizures even if they are subjected to the most effectual treatments available. This situation is tagged by medical professionals as intractable epilepsy. It does not mean that if a person had a seizure, that this immediately connotes epilepsy. One is considered as epileptic if he had 2 or more seizures (Heiskala, 1997). Epidemiologic studies concluded that the western or the first world countries portrayed that a proportion of patients with LGS appear to be consistent across nations. The prevalence of the disorder is 0.1-0.28 per 1000. Annually, there are about 2 children from 100,000 that suffers from LGS (Heiskala, 1997). It is important to note however, that epilepsy is not contagious and it is not caused by mental illness or mental retardation. There are people with mental retardation that may experience seizures, but then having seizures does not instantly imply that the person will develop mental impairment. Many individuals with epilepsy have normal and even above average intelligence. The great Socrates was noted to have epilepsy along with General Napoleon, Alfred Nobel who established the Nobel Prize Award and inventor of dynamite, and also Dostoyevsky the famous Russian writer (Heiskala 1997). Lennox Gastaut Syndrome (LGS) Lennox-Gastaut syndrome (LGS) or childhood epileptic encephalopathy is an unusual and destructive form of childhood-onset epilepsy. Majority of children usually develop normally when they are first diagnosed, but slowly start to lose skills. At times, this occurs drastically, in relation to uncontrolled seizures (Wakomoto, 2000). These young children with Lennox Gastaut Syndrome may show signs of behavioral issues, personality troubles, mood instability and the slowing of psychomotor development. Some of the children with this disorder are prone to developing non-convulsive status epilepticus or the so called seizure state that is lined with a change in the child’s level of awareness. This is the stage where medical alternatives are of utmost significance (Wakomoto, 2000). It is inclusive of signs such as frequent seizures and multiple seizure types, mental retardation or regression, and an abnormal EEG with the so called slow spike and wave discharges. This disorder is constituted by 1-4% of pediatric epilepsies and usually manifests between the first to five years of life. At about 30 to 35 percent of the cases, there are still no causes which have been conclusively put forth (Beaumanoir, 1982). The type of seizure that children with Lennox Gastaut Syndrome suffer from varies as they grow. In nearly all cases, the drop is replaced by partial, complex partial, and secondary generalized convulsions. The complex seizures are the most usual among adolescents with LGS (Gastaut, 1982). Some of the children who are suffering from LGS are experiencing acute psychotic episodes, types of psychosis with aggressiveness, and character problems. They also are afflicted by cognitive problems including the slowing of reaction time and information processing. Furthermore, the core traits of mental regression are reported as apathy, memory disorders, impaired vision and motor speed, and perseverance (Glauser, 2004). LGS remains as a disease which devastates not only the lives of the patients but also to their families and caregivers. Children between the ages of 1 and 5 more often than not experience the onset of LGS. Around2 to 7 percent of those who have LGS die within a mean follow-up time of less than 10 years. LGS is said to be difficult to treat because the patients are often taking multiple antiepileptic drugs in their effort to control the seizures (Wakomoto, 2000). The mortality rate of LGS was reported to be no less than 3 percent. The deaths are often associated with accidents. There is also a high level of injuries which are mostly associated with atonic or tonic or both seizures. The common thing that patients do is to wear protective gears such as helmets and face guards so guard the forehead, nose and teeth. The bad side of this is that only a minimal number of patients tolerate the uncomfortable feeling brought by the protective gear and fact that it is “cosmetically unacceptable” (Wakomoto, 2000). There are three notable characteristics of Lennox-Gastaut syndrome; multiple seizures of differing types daily, mental retardation, and a classic EEG rhythm termed as slow spike and wave with slowed background. Jacinta has all the three symptoms. But although LGS is typically marked by a triad of symptoms, there is still contention with regard to the accurate constraints, causes, and diagnosis of the syndrome (Gastaut, 1982). Lennox-Gastaut Syndrome was argued to be marked by tonic seizures. However, these tonic seizures were not found on the onset and the EEG features are not at all pathognomonic of the disorder. On the optimist side of the argument, there are effective treatment options for the multiple seizures and morbidities, but then the long term outlook is poor for most patients. Almost certainly, there were only a limited number of random trials that studies Lennox Gastaut Syndrome mainly because of its complexity and it follows that many of the treatment drugs that are present in the market have a few or no supporting evidence at all based from controlled trials (Shields, 2002). Causes of LGS The causes of LGS according to the experts are not yet fully unified. Nonetheless, they came to a consensual agreement hat LGS can be categorized based on alleged etiology. The first group of patients are those that are considered to have idiopathic LGS if normal psychomotor development happens before the onset of symptoms, and that there are no latent disorders of specific presumptive causes are present and in addition, there should be no neurologic abnormalities found (Heiskala, 2003). On the other hand, the other group is composed of patients who have a symptomatic LGS if a recognizable factor is accountabe for the condition. There are a few investigators and cryptogenic as another variant etiologic category, wherein there are no recognizable cause exists but there is a cause suspected and for that the epilepsy is presumed to be symptomatic (Shields 2002). Diagnosis of LGS In order to diagnose LGS one should perform a number of tests which were originally proposed to diagnose epilepsy in general. The only exception is that the tests should be children friendly since LGS is children- onset epilepsy (Glauser, 2004). An EEG is a device that documents brain waves which are detected by electrodes positioned on the scalp. EEG is the most universally used diagnostic test for epilepsy and it can detect abnormalities in the electrical activity of the brain. People with epilepsy most of the time show substantial changes in the pattern of their brain waves. EEG is a very useful diagnostic tool in the diagnosis of epilepsy. However, it is crucial to note that it is not fully prcecise. Some individuals still show a normal brain wave pattern on their EEG even if they have just experience a seizure. If at all possible, an EEG should be performed within 24 hours after the patient’s first seizure (Glauser, 2004). Magnetic resonance imaging (MRI) in general there is chosen over this test for patients with LGS. It is an important part of the research on the underlying etiology in a patient with LGS. It reveals abnormalities which are related to LGS such as tuberous sclerosis, brain malformations, hypoxia-ischemia injury and ever frontal lobe lesions (Hancock and Cross, 2003). Treatment of LGS The aim of LGS treatment is almost similar for all patients with epilepsy which encompass the best value of life having the least occurrence of seizure, the least unpleasant treatment effects and lastly the least medicine. Antiepileptic medications are the foundation of therapy for patients with LGS. It is unfortunate to note that there is no single medical treatment that can offer sufficient relief for all or even most patients with LGS. Typically, a combination of medical treatments and medicines is imperative (Arzimanoglou, 2009). It is argued that there is still no cure of LGS. The syndrome is one of the hardest possible diseases to treat and most of the time it does not respond to the common seizure medications. Some of the treatments of LGS include ketagonic diet, vagus nerve stimulation therapy, IVIG, or seldom corpus callosotomy surgery (Freeman 1999). The debate surrounding LGS is a very sensitive one. The conservative medical community is sure that there is still no known cause of Jacinta’s disease. However, the counter argument points to the uncertainty of not having a cure. When Jacinta had her first treatment, the aim was to relieve her from the symptom. However, seizures are merely a symptom of a latent problem. It is said that the symptoms are still uncontrollable such that even undergoing one medication and another does not seem effectual. At such a stage, the disorder becomes incurable (Arzimanoglou, 2009). One of the things that give hope to these patients and also to Jacinta is the Fetal Stem Cell therapy. However, the problem with this method is that there are still minimal data on its application on patients with LGS. Epilepsy patients in general should not just be taken care of if they are undergoing rare medical treatments in hospitals. There should be the aim to totally care for them so as to make certain that the best possible management for each patient is given. The basis of treatment in epilepsy is pharmacotherapy for the suppression of seizures, though this is the case, social and environmental problems should not just be pushed to the background because they are just likely to agonize patients as ongoing seizures. Hospital administrators should not neglect the provision of adequate information to patients and relatives in relation to the different aspects of the seizure disorder. A more superior and unbiased education towards epilepsy is needed in order to remove the many misconceptions and prejudices that are still in the society. It is very substantial for hospital administrators and relatives as well to encourage self-confidence and to avoid over protection as well. There should also be restrictions on the lifestyle of the patient if it is deemed necessary, such as the avoidance of driving or some employment or jobs (Hancock and Cross, 2003). References Arzimanoglou, A. (2009). Epilepsy Therapy. Biotech Business Week. Atlanta. Pg 134. From http://proquest.umi.com/pqdweb?did=1626429451&sid=1&Fmt=3&clientId=20863&RQT=309&VName=PQD Beaumanoir, A.(1982) The Lennox-Gastaut syndrome: a personal study. Electroencephalogr Clin Neurophysiol Suppl. 85-99 Hancock E, Cross H.(2003) Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev.  Freeman, J.M. (1999).  Seizures decrease rapidly after fasting: preliminary studies of the ketogenic diet. Arch Pediatr Adolesc Med.  946-9.  Gastaut H. (1982)The Lennox-Gastaut syndrome: comments on the syndromes terminology and nosological position amongst the secondary generalized epilepsies of childhood. Electroencephalogr Clin Neurophysiol Suppl. 71-84. Glauser, T.A. (2004). Following Catastrophic Patients from Childhood to Adulthood. Pg. 23-26 from http://proquest.umi.com/pqdweb?did=1626429451&sid= Heiskala H. (1997). Community-based study of Lennox-Gastaut syndrome. Epilepsia. 526-31. Shields WD. (2002) Diagnosis of infantile spasms, Lennox-Gastaut syndrome, and progressive myoclonic epilepsy. Epilepsia.  Wakomoto, H. (2000). Longterm Medical, Educational, and Social Prognoses of Childhood Onset Epilepsy. Japan p. 246-255 Read More