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Building on the sell-out success of the first edition this thorough revision reflects the lat. A Clinical Guide to Epileptic Syndromes and their Treatment. Authors PDF · Epileptic seizures and their classification. C P Panayiotopoulos. Pages .
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Affecting 4 percent of children and percent of the general population, epilepsy is one of the most common neurological disorders. The 1st edition of this guide proved to be the only one of its kind, covering many important aspects of diagnosis and treatment. Due to the continued advances being made in the subject, and building on the sell-out success of the 1st edition this thorough revision reflects the latest report of the ILAE classification core group and the significant progress made in the diagnosis, classification and treatment of the epilepsies.

There is evidence that early treatment can reduce the risk of seizure recurrence, 1 and its efficacy depends largely on the appropriate drug choice in relation to the particular clinical syndrome.

The current classification of epileptic seizures 4 and epilepsies 5 of the International League Against Epilepsy ILAE and also the new ILAE proposal 6, 7 are essentially organized around two dichotomies in terms of their topography between generalized and localized partial or focal and in terms of their etiology between idiopathic epilepsies genetically determined and symptomatic epilepsies due to cerebral pathology.

The ILAE classification is currently under revision, but is still valid. Localization-related epilepsies also called focal or partial manifest with focal or partial seizures, in which the initial activation of a group of neurons is limited to a part of one hemisphere 5 ; the EEG shows focal abnormalities, but GSW discharges may occur as a result of secondary bilateral synchrony SBS.

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Figure 10—1 Generalized spike-and-wave discharges in patients with idiopathic and symptomatic generalized absence epilepsies. Reprinted with permission from Panayiotopoulos CP. London: Springer; Identification of the seizure type s is the quintessential element of an epileptic syndrome and the first step for its definition. TA in idiopathic generalized epilepsy IGE , for example, may share some principal clinical features with limbic complex partial seizures CPS such as unresponsiveness and automatisms , and one must turn to either their onset presence or absence of aura or immediately post-termination presence or absence of confusion to make an educated clinical hypothesis.

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Interictal EEG studies will assist diagnosis by showing focal epileptiform activity or GSW, but even so the rate of misdiagnosis is high with the generalized epilepsies being erroneously diagnosed as focal rather than the converse. In this chapter, we will discuss primarily the various syndromes of IGE, whose GSW is the defining electrographic trait. From the brief foreword, it follows that GSW discharges do not always indicate IGE or indeed generalized epilepsies, and Table includes all the main epilepsies and syndromes that are associated with 3—4 Hz GSW.

The interictal EEG alone cannot be used for establishing or excluding the diagnosis of epilepsy including IGEs or provide oversimplified clues for reliable syndromic diagnosis; this can only be based on a comprehensive electroclinical synthesis for the individual patient, without which EEG interpretation may be completely meaningless and even misleading.


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EEG background activity is normal. IGE comprise a group of genetically determined epilepsies, unrelated to any structural brain pathology, and associated with normal neurological and neuropsychological status.


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  8. MS start between 4 months to 3 to 4 years and are typically favored by sleep. Two-thirds of the affected children are boys. Differential diagnosis includes nonepileptic conditions, such as hypnagogic jerks and benign nonepileptic myoclonus and epileptic syndromes, including severe myoclonic epilepsy of infancy Dravet syndrome , familial infantile myoclonic epilepsy mapped to 16p13 , infantile spasms West syndrome , epilepsy with myoclonic-astatic seizures MAE , and the myoclonic form of Lennox-Gastaut syndrome LGS. Myoclonic-astatic seizures are the defining seizure symptom and consist of a myoclonic jerk immediately followed by loss of muscle tone.

    A Clinical Guide to Epileptic Syndromes and Their Treatment

    Either component can cause falls, head nodding, or bending of the knees. More than half of patients have brief absences, often with myoclonic jerks, facial myoclonias, and atonia. In two-thirds of patients, febrile and nonfebrile GTCS precede myoclonic astatic seizures by several months. Episodes of absence status may occur, usually introduced by inappropriate treatment such as CBZ; they are frequent in the cryptogenic form.

    MAE shares many clinical features with cryptogenic Lennox-Gastaut syndrome, particularly its myoclonic form.


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    6. There is probable linkage with chromosomes 1p, 8q24, 5q TA are associated with severe impairment of consciousness unresponsiveness and interruption of ongoing activities and are usually multiple per day, hence the term pyknolepsy. They have abrupt onset and termination and last from 4 to 20 sec mainly around 10 sec.