8 Epilepsy Syndromes and Management Challenges

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Epilepsy Syndromes and Management Challenges

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INTRODUCTION Having established that a paroxysmal event is epileptic, the diagnosis by the non-specialist is often limited to excluding structural abnormalities of the brain or predisposing medical disease. However, simply diagnos-ing ‘epilepsy’ or ‘seizures’, is insufficient. Symptom / Seizure diagnosis cannot provide guidance on important items such as severity of the disease, prognosis, short and long – term therapeutic decisions and genetics (research and counseling), which are all factors that crucially affect family and social life and the education and career choices of patients. Defining the type of epilepsy should now be considered mandatory as it offers that best guide to both management and prognosis.

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INTRODUCTION (contd…) Syndromic diagnosis of epilepsies provides a firm foundation for short and long term therapeutic decisions and enables natural history, inheritance, treatment efficacy and prognosis of epilepsies to be studied scientifically. The benefits of syndromic diagnosis over seizure/symptom diagnosis or an inclusive diagnosis such as “Epilepsy” far outweigh any morbidity from miscategorization that may arise in difficult cases. Imprecise syndromic diagnosis commonly results in avoidable morbidity and sometimes mortality.

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INTRODUCTION (contd…) The most important milestone in modern epileptology has been the recognition of epileptic syndromes and diseases, most of which are well defined and easy to diagnose. The concept of epilepsies as specific syndromes is old (see for example pyknolepsy=childhood absence epilepsy) and the first attempt to formalise them in an international classification was published in 1970. The current classification originated from a meeting in the Centre Saint Paul, Marseiles, France, in July 1983 and has been the basis of the book Epileptic Syndromes in Infancy, Childhood and Adolescence, also known as Guide Bleu, Which is now Published as an updated third edition.

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ILAE Definitions The classification and definitions from this meeting were adopted in 1985 by the Commission on classification and Terminology of the ILAE and remained essentially the same in the revised proposal of 1989. An epileptic syndrome is an epileptic disorder characterized by a cluster of signs and symptoms customarily concurrent together; these include such items as type of seizure, etiology, anatomy, precipitating factors, age of onset, severity, chronicity, diurnal and circadian cycling, and sometimes prognosis. However, in contradistinction to a disease, a syndrome does not necessarily have a common etiology and prognosis.

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ILAE Definitions Epilepsy syndrome a complex of signs and symptoms that define a unique epilepsy condition. This must involve more than just the seizure type; thus frontal lobe seizures per se, for instance, do not constitute a syndrome.

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The following are the four major classes in this 1989 classification as defined by the ILAE. EPILEPTIC SYNDORMES IN ACCORDANCE WITH CURRENTLY VALID ILAE CLASSIFICATION OF 1989. Localization related (focal, local & partial) epilepsies and syndromes Generalized epilepsies and syndromes. Epilepsies and syndromes undetermined as to whether they are focal or generalized Special syndromes.

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EPILEPTIC SYNDORMES IN ACCORDANCE WITH CURRENTLY VALID ILAE CLASSIFICATION OF 1989. Benign familial neonatal seizures Benign myoclonic epilepsy in infancy Severe Myoclonic epilepsy in infancy (Dravet’s Syndrome) Early myoclonic encephalopathy (Ohtahara syndrome) Migrating partial seizures of infancy West syndrome Benign familial and non-familial infantile seizures Dravet’s syndrome HH syndrome (Hemiconvulsion Hemiplegia Syndrome) SYNDROMES EPILEPSY SYNDROMES AND RELATED CONDITIONS

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Myoclonic status in nonprogressive encephalopathies Benign childhood epilepsy with centrotemporal spikes Early onset benign childhood occipital epilepsy (Panayiotopoulos type) Late onset childhood occipital epilepsy (Gastaut type) Epilepsy with myoclonic absences Epilepsy with myoclonic-astatic seizures Lennox-Gastaut syndrome Landau-Kleffner syndrome

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Epilepsy with continuous spike-and-waves during slow-wave sleep (other than LKS) Childhood absence epilepsy Progressive myoclonus epilepsies Idiopathic generalized epilepsies with variable phenotypes Juvenile absence epilepsy Juvenile myoclonic epilepsy Epilepsy with generalized tonic-clonic seizures only Reflex epilepsies Idiopathic photosensitive occipital lobe epilepsy

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Reflex epilepsies  Idiopathic photosensitive occipital lobe epilepsy  Other visual sensitive epilepsies  Primary reading epilepsy  Startle epilepsy Autosomal dominant nocturnal frontal lobe epilepsy Familial temporal lobe epilepsies Generalized epilepsies with febrile seizures plus Familial focal epilepsy with variable foci

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Symptomatic (or probably symptomatic) focal epilepsies Limbic epilepsies Mesial temporal lobe epilepsy with hippocampal sclerosis Mesial temporal lobe epilepsy defined by specific etiologies Other types defined by location and etiology Neocortical epilepsies Rasmussen syndrome Other types defined by location and etiology Syndromes in development

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CONDITIONS WITH EPILEPTIC SEIZURES THAT DO NOT REQUIRE, A DIAGNOSIS OF EPILEPSY Benign neonatal seizures Febrile seizures Reflex seizures Alcohol withdrawal seizures Drug or other chemically-induced seizures Immediate and early post traumatic seizures Single seizures or isolated clusters of seizures Rarely repeated seizures (oligo-epilepsy)

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Myoclonic epilepsy - Benign - Severe West Syndrome Syndromes in Neonatal period : Benign Neonatal Convulsions (5 th day fits) Benign Familial neonatal convulsions Myoclonic epilepsy – severe (malignant) Syndromes in Infancy :

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Age of occurrence 1 to 2 years of life. In previously normal children characterised by brief episodes of generalized myoclonic seizures of duration of 1-3 seconds with no loss of consciousness, affects axis of body and limbs and does not occur in sleep. Benign myoclonic epilepsy in infancy EEG in the inter ictal period is normal and generalized spike and wave discharges seen ictally. Seizures are easily controlled with sodium valproate, and may persist if untreated and later develop generalized tonic clonic seizures.

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In a previously normal child, usually starts with unilateral or bilateral clonic seizures usually with fever. Later myoclonic jerks supervene associated with partial seizures with autonomic or atonic features and automatisms may occur. EEG shows generalised spike wave and polyskike activity, photosensitivity and focal abnormalities. Refractory to treatment and developmental arrest are common. Severe myoclonic epilepsy in infancy (Dravet’s Syndrome)

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Ohtahara Syndrome (OS) also known as Early infantile Epileptic Encephalopathy with Burst Suppression (EIEE) is a progressive epileptic encephalopathy. It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe mental retardation. OHTAHARA SYNDROME No single cause has been identified, although in many cases structural brain damage is present.

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Ohtahara syndrome is rare and the earliest-appearing age-related epileptic encephalophy, with seizure onset occurring within the first three months of life, and often in the first ten days. Many, but not all, cases of OS evolve into other seizure disorders, namely West syndrome and Lennox-Gastaut syndrome . Clinically, OS is characterized by a “burst suppression” pattern on an EEG. This pattern involves high voltage spike wave discharge followed by little brain wave activity. Characteristics

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Treatment outlook is poor. Anticonvulsant drugs and glucocorticoid steroids may be used to try to control the seizures, but their effectiveness is limited. Most therapies are related to symptoms and day-to-day living. Causes and treatment

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Age of onset 3 to 12 months (peak 3-7) West Syndrome The full syndrome comprises : Infantile spasms (flexor, extensor, clonic or myoclonic seizures) occurring singly or in clusters more on awakening. Hypsarrythmia or modified hypsarrythmia on EEG Mental deterioration.

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West syndrome or West's Syndrome is an uncommon to rare epileptic disorder in infants. It is named after the English physician, (1793-1848), who first described it in an article published in The Lancet in 1841. The original case actually described his own son, James Edwin West (1820 - 1840) Other names for it are "Generalized Flexion Epilepsy", "Infantile Epileptic Encephalopathy", "Infantile Myoclonic Encephalopathy", "jackknife convulsions", "Massive Myoclonia" and "Salaam spasms". The term "infantile spasms" can be used to describe the specific seizure manifestation in the syndrome, but is also used as a synonym for the syndrome itself. West syndrome is the triad of infantile spasms, West syndrome

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Infantile spasms 2. Mental deterioration 3. Hyps arrhythmia or Modifed Hyps arrhythmia on EEG West syndrome, Classical Triad & Prevalence The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. There are various causes (""). The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal , perinatal (caused during birth) or postnatal . Prevalence is around 1:3200 to 1:3500 of live births. Statistically, boys are more likely to be affected than girls at a ratio of around 56:44. In 45 out of every 50 children affected, the spasms appear for the first time between the third and the twelfth month of age. In rarer cases, spasms may occur in the first two months or during the second to fourth year of age.

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CAUSES & SYMPTOMS It is still unknown which bio-chemical mechanisms lead to the occurrence of West syndrome. It is conjectured that it is a malfunction of neurotransmitter function, or more precisely, a malfunction in the regulation of the GABA transmission process. Another possibility being researched is a hyper-production of the Corticotropin-releasing hormone (CRH). It is possible that more than one factor is involved. Both hypotheses are supported by the effect of certain medications used to treat West syndrome. If a cause presents itself, the syndrome is referred to as symptomatic West syndrome, as the attacks manifest as a symptom of another problem. Almost any cause of brain damage could be associated, and these are divided into prenatal, perinatal, and post-natal.

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Clinical Presentation The epileptic seizures which can be observed in infants with West syndrome fall into three categories, collectively known as infantile spasms. Typically, the following triad of attack types appears; while the three types usually appear simultaneously, they also can occur independently of each other: Lightning attacks : Sudden, severe myoclonic convulsions of the entire body or several parts of the body in split seconds, and the legs in particular are bent ( flexor muscle convulsions here are generally more severe than extensor ones).

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Clinical Presentation (Contd..) Nodding attacks : Convulsions of the throat and neck flexor muscles, during which the chin is fitfully jerked towards the breast or the head is drawn inward. Salaam or jackknife attacks : a flexor spasm with rapid bending of the head and torso forward and simultaneous raising and bending of the arms while partially drawing the hands together in front of the chest and/or flailing. If one imagined this act in slow motion, it would appear similar to the oriental ceremonial greeting (Salaam), from which this type of attack derives its name.

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Therapy Compared with other forms of epilepsy, West syndrome is difficult to treat. To raise the chance of successful treatment and keep down the risk of longer-lasting effects, it is very important that the condition is diagnosed as early as possible and that treatment begins straight away. However, there is no guarantee that therapy will work even in this case. Insufficient research has yet been carried out into whether the form of treatment has an effect upon the long-term prognosis. Based on what is known today, the prognosis depends mainly on the cause of the attacks and the length of time that hypsarrhythmia lasts.

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Therapy In general it can be said that the prognosis is worse when the patient does not react as well to therapy and the epileptic over-activity in the brain continues. Treatment differs in each individual case and depends on the cause of the West syndrome (etiological classification) and the state of brain development at the time of the damage. Due to their side-effects, two drugs are currently being used as the first-line treatment. ACTH - Use primarily in United States Side effects are: Weight gain, especially in the trunk and face, hypertension , metabolic abnormalities, severe irritability, osteoporosis , sepsis , and congestive heart failure . Vigabatrin (Sabril) - Approved in several countries, like most Europe, Canada and Mexico.

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Therapy Side effects are: Somnolence, headache, dizziness, fatigue, weight gain, decreased vision or other vision changes Vigabatrin is known for being effective, especially in children with tuberous sclerosis , with few and benign side effects. But due to some recent studies[3] showing visual field constriction (loss of peripheral vision), it was not approved in the United States until mid-2009. It is currently debated that a short use (6 months or less) of Vigabatrin will not affect vision. Also, considering the effect of frequent seizures on day to day life and mental development, some parents prefer to take the risk of some vision loss.

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Therapy When those two are proving ineffective, other drugs may be used in conjunction or alone. From those, corticosteroids (prednisone) are often used. In Japan, there is a good experience with pyridoxine therapy. Further, topiramate (Topamax), lamotrigine (Lamictal), levetiracetam (Keppra) and zonisamide (Zonegran) are amongst those drugs most widely used. The ketogenic diet has been shown to be effective in treating infantile spams,[4], up to 70% of children having a 50% or more reduction in seizure.

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Classical hypsarrhythmia on EEG.

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West Syndrome EEG : Slow waves of high voltage intermixed with diffuse asynchronous spikes over both hemispheres, basal rhythm not discernibe, REM sleep causes dimunition or disappearance of the abnormality. Neuroimaging has brought a decrease in number of cryptogenic cases. Prognosis is poorer in symptomatic spasms and also if diagnosis is delayed. Steroids (ACTH, prednisone), sodium valproate, benzodiazepines and more recently vigabatrin are the treatment of choice.

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Early childhood Febrile Seizures : Age 3 Months – 5 yrs ( 1-2yrs) Lennox – Gastaut Syndrome : 3 % of child hood seizures Age : 2 to 8 yrs. (3 to 5 yrs) seen more in males with no family history. Facial injuries due to frequent atonic falls in a child with Lennox-Gastaut syndrome.

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Lennox–Gastaut syndrome Lennox–Gastaut syndrome (LGS), also known as Lennox syndrome , is a difficult-to-treat form of childhood-onset epilepsy that most often appears between the second and sixth year of life, and is characterized by frequent seizures and different seizure types; it is often accompanied by mental retardation and behavior problems .

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Management of Lennox – Gastaut Syndrome : Resistant to treatment; the following treatment has been tried : Sodium valproate, nitrazepam, Clonazpam, Vigabatrin, felbamate. ACTH, IV gammaglobulin Ketogenic diet Corpus Callosotomy. Prognosis : For seizure control and mental development is poor.

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MYOCLONIC ASTATIC Incidence – 1-2% of all epilepsies. Age 2-5 yrs Characterised by high genetic pedisposition Normal development and no neurological deficits before onset. EEG : Irregular spike and wave activity in sleep Bilateral synchronous irregular spike and wave activity Paroxysms of irregular spike, wave and polyspikes.

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Myoclonic astatic, Ictal EEG showing generalized bisynchronous spike and wave discharge followed by polymorphous delta and rhythmic theta activity.

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Myoclonic-astatic epilepsy usually begins in previously normal children, and there is a high incidence of familial antecedents of idiopathic generalized epilepsy. Familial occurrence of seizure disorders can be detected in about one third of those cases. The main disorders to be differentiated from epilepsy with myoclonic-astatic seizures include late-onset infantile spasms, Lennox-Gastaut syndrome, and continuous spike waves in slow sleep. ETIOLOGY & DIFFERENTIAL DIAGNOSIS

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The EEG may initially show only an abnormal 4- to 7-Hz rhythm. Later, regular or irregular bilaterally synchronous 2- to 3-Hz spike-waves and/or polyspike-waves will be superimposed on the background activity. Sleep can facilitate the appearance of spike-wave discharges DIAGNOSTIC WORKUP

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Therapy for epilepsy with myoclonic-astatic seizures in early childhood remains empirical. Some compounds should never be given because they contribute to worsen the condition: carbamazepine, phenytoin, and vigabatrin. In addition, phenobarbital should be avoided because of its metabolic interaction with valproate. Indeed, valproate, ethosuximide and benzodiazepine, and clobazam more than clonazepam, have been used successfully. However, the most efficient combination seems to be valproate with lamotrigine, since these two compounds exhibit beneficial potentiation. In practice, the best therapy seems to begin with valproate followed by the addition of lamotrigine in case of resistance to valproate, whereas benzodiazepines should be restricted to episodes of status epilepticus. It remains to be determined whether there is any benefit in the use of steroids. MANAGEMENT

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Typical absence epilepsy Benign partial epilepsy with rolandic (centro-temporal) spikes Benign partial epilepsy with occipital spikes/paroxysms Landau – Kleffner syndrome. Epilepsy Syndromes Later Childhood (5-10 yrs)

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Childhood absence Onset : Before puberty (5-10 yrs) Previously normal children, more often in females.

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Childhood absence EEG : Shows a bilateral, symmetrical, synchronous discharges of spike and wave at 3c/Second, with a normal background activity and precipitated by hyperventilation, with an abrupt onset and termination of spike and wave complexes. The response to ethosuzimide, valproate or clonazepam is complete.

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Landau Kleffner : The Acquiped aphasia with epilepsy syndrome Age of onset : 3 yrs – 9 yrs. Abrupt or gradual onset. Defect in language function and verbal auditory agnosia with an inability to comprehend language and reduction in spontaneous oral expression. Behavioural and intellectual difficulties are present. Seizures are partial or generalised seen in two thirds f cases and not very severe. They remit by 15 yrs.

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Landau Kleffner (Contd..) EEG : multifocal spikes and spike and wave discharges. Long term recovery of language function is not very good especially in those with early age of onset before age of 5 yrs. Early diagnosis is essential as treatment with ACTH or steroids can reverse or arrest the disorder. Valproate or carbamazepine are used for seizures.

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Syndromes in Adolescence Juvenile absence epilepsy. Juvenile myoclonic epilepsy (Janz syndrome) Grand mal seizures on awakening. Photosensitive epilepsy Age of occurrence is around puberty with equal male – female rating. EEG : Shows generalised spike and wave discharges with frontal predominalnce which are more than 3c/sec. Precipitated by hyper ventilation and sleep deprivation. Response to treatment is seen not as readily as childhood absence. Juvenile absence

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Syndromes in Adolescence Juvenile myoclonic epilepsy (Janz syndrome) Incidence 5-10% of epilepsy Age of occurrence : 8-26 yrs (more between 12-18 yrs), more in females 2:1 ratio Treatment : with appropriate medication (Valproate) patients are seizure free. There is a high relapse rate if medication is withdrawn even if seizure free for several years.

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Syndromes in Adolescence Photosensitive epilepsy

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Photoconvulsive disorder Age of onset : 12-14 yrs (more in females) 40% are pure photosensitive, 60% have spontaneous along with precipitation by photic stimulaion. EEG : Intermittent photic stimulation at 15-18 Hz flash frequency elicit photoconvulsive response. Treatment : is to view TV under bright light from a distance of 2m, cover one eye (monocular vision), use of polaroid lens, dark glasses and sodium valproate.

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PAIRING ANTI EPILEPTIC DRUGS WITH SYNDROMES SYNDROMES ANTI EPILEPTIC DRUGS Benign neonatal seizures (5 th day fits) Remit on their own – need counseling Benign familial neonatal seizures (first week of life and remit within few months) Remit within a few months-need counceling Early Myoclonic encephalopathy (early neonatal period) (Ohtahara Syndrome) Associated with severe neurological abnormalities and early death – Resistant to treatment Benign myoclonic epilepsy of infants (1 to 2 yrs of life) Seizures controlled with sodium valproate,

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PAIRING ANTI EPILEPTIC DRUGS WITH SYNDROMES SYNDROMES ANTI EPILEPTIC DRUGS Severe myoclonic epilepsy in infants (within one year of life) (Dravet’s Syndrome) Refractory to treatment and developmental arrest are common. Valproate & Benzodiazepines (clonazepam, Lorazepam) Topiramate Carbamazepine & Lamotrigine often aggravate. Ketogenic diet needs to be proved West syndrome (3 – 10 months – peak 3-7 yrs) Steroids (ACTH, prednisone), sodium valproate, Topiramate, benzodiazepines and more recently vigabatrin (sabril). Febrile seizures (age 3 m – 5 yr) (1-2 yrs) Diazepam given orally or rectally effective in reducing further febrile seizures. Recently oral clobazam used effectively for febrile convulsions.)

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PAIRING ANTI EPILEPTIC DRUGS WITH SYNDROMES SYNDROMES ANTI EPILEPTIC DRUGS Lennox – Gastaut syndrome (age 2-8 yrs) (3-5 yrs) Resistant to treatment. Sodum valproate, nitrazepam, clonazpam, vigabatrin, felbamate. ACTH, IV gammaglobulin. Ketogenic diet. Myoclonic Astatic (2-5 yrs) Valproate, ethosuximide & benzodiazepine have been use successfully. However, the most efficient combination would be valproate with lamotrigine , since these compounds exhibit beneficial potentiation. Carbamazepine, phenytoin & vigabatrin worsen the condition.

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PAIRING ANTI EPILEPTIC DRUGS WITH SYNDROMES SYNDROMES ANTI EPILEPTIC DRUGS Typical childhood absence (5-10 yrs) Ethosuximide, Valproate or clonazepam is complete. Benign partial epilepsy with centrotemporal spikes (BECT - Rolandic) (age 3-10 yrs) Prognosis is excellent and recovery is the rule without recurrence after discontinuation of medication. Benign epilepsy with occipital paroxysms(BEOP) (age 15 months – 17 yrs) (Mean 7.5 yr) Prognosis is usually good but not as good as BECT. Seizures cease in adult life. Landau Kleffner Syndrome : the acquired aphasia with epilepsy syndrome (age 3 yrs – 9 yrs) Early diagnosis is essential as treatment with ACTH or steroids can reverse or arrest the disorder. Valproate or carbamazepine are used for seizures.

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PAIRING ANTI EPILEPTIC DRUGS WITH SYNDROMES SYNDROMES ANTI EPILEPTIC DRUGS Juvenile Absence epilepsy (Age : around puberty with equal male female rating) Ethosuximide, sodium valproate, lamotrigine, clonozapam. Response to treatment to seen not as readily as childhood absence. Juvenile Myoclonic Epilepsy of Janz (age 8-26 yrs) (more between 12-18 yrs & more in females 2:1 ratio) Sodium valproate Grand mal seizures on awakening (age 9-25 yrs) Peak at puberty) Controlled with medication in 65% but high relapse rate if medication is tapered.

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PAIRING ANTI EPILEPTIC DRUGS WITH SYNDROMES SYNDROMES ANTI EPILEPTIC DRUGS Chronic Progressive Epilepsia partialis Continua (EPC) Rasmussen’s Syndrome (mean age 6.8 yrs, 85% before 10 yrs) Anti epileptic drugs not effective. High dose steroids, IV gamma, Interferon, ? Gancyclovir, Functional hemispherectomy Photoconvulsive epilepsy (age : 12-14 yrs, More common in females). Treatment is view TV under bright light from a distance of 2m, cover one eye (monocular vision), use of polaroid lens, dark glasses and sodium valproate.

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