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First Localization-Related (Focal, Local, Partial) Epilepsies and Syndroms

1.1 Idiopathic (with altersgebundenem start) 

- Benign epilepsy of childhood with spikes zentrotemporalen 

- Epilepsy of childhood with occipital paroxysms 

- Primary reading epilepsy 

1.2 Symptomatic 

- Chronic progressive epilepsia partialis continua (Kojewnikow syndrome) in childhood 

- Die syndrome, triggered by specific types of seizures are characterized (see Appendix 2) 

Apart from these rare diagnoses symptomatic category contains coarser die die syndrome Individual variability mainly on the types of seizures and other clinical features are based on the anatomical localization and well as the etiology - as far as known this Sindh. 


The seizure types relate to die IKEA. inferences have to die on anatomical localization carefully drawn. If the scalp EEG (interictal and ictal) Can be misleading, and even local morphological findings, as presented by imaging methods are not necessarily identical with an epileptogenic lesion. The attack symptoms, and occasionally other clinical features often provide important clues. The first symptom of a seizure is often the most important information on the origin of seizure discharge, during die following sequence of seizure symptoms your spread through the brain reflect possible. Also, this sequence But can high local Bedeutung of his. Can-It is important to note dass A seizure in a clinically silent area Begin occurs so know that the first clinical symptoms until after the spread of a more or less far away place from the initials of discharge . The following tentative descriptions syndrome die in RELATION to anatomical localization GründeN on data involving Which examination results with depth electrodes. 

- Temporal lobe 

Temporallappensyndrome are characterized by partial seizures Simple and complex partial seizures and generalized seizures secondary or combinations thereof. One often finds a history of febrile seizures and a family history of seizures. occurring memory impairments can. In the metabolic imaging (eg PET) is often a decreased metabolism of view. Unilateral or bilateral spikes over the temporal lobes are usually found in the EEG. 

The onset is often in childhood or young adulthood. The seizures occur in clusters with intervals or in a random distribution. 

General Characteristics 

The following characteristics determine diagnosis very close to dying: 

1st Simple partial seizures with Autonomous and psychological symptoms as well as certain olfactory sensory phenomena and auditory perception (including illusions). It is very often a common rising epigastric FEELING. 

Second complex partial seizures, often die, But Not Forever, starting with one motor paralysis and typically followed by automatisms oroalimentären Sindh. Other automatisms include yourself often. The duration is typically about 1 min Usually it comes to postictal confusion. An amnesia Stay back. The attacks subside gradually. 

Electroencephalographic characteristics 

In the temporal lobe interictal scalp EEG can show the following: 

First no abnormalities. 

2nd Low or pronounced asymmetry of background activity. 

Third temporal spikes, Sharp waves or slow waves, single or double sided, synchronous, asynchronous, but available. This findings are not always limited to temporal region die upgradeable. 

Fourth addition to the scalp-intracranial EEG derivations A better definition of the distribution of interictal intracranial abnormalities Yermogen Konner. 

In temporal lobe epilepsy Different EEG patterns die initial clinical seizure manifestations accompany temporal namely (a) A unilateral or bilateral interruption of the background activity, and (b) or more extended low-amplitude fast activity, rhythmic spikes or rhythmic slow waves can. The beginning of the EEG Needs, in dependence of the Ableitemethode to correlate not with the clinical beginning.derivations can Intracranial Additional Information About die chronological and spatial development of the supply discharges. 

. Epilepsies with amygdalo-hippocampal (mesiobasallimbischen Or rhinenzephalen) seizures are seizures in the hippocampus die common form; your sinus symptoms are occurring as described above, except know that no auditory symptoms. The interictal scalp EEG can be normal, unilateral temporal sharp or slow waves or synchronous bilateral sharp or slow waves or show asynchronously. The intracranial interictal EEG Can front show mesial temporal spikes or sharp-waves. The attacks are characterized by rising epigastric discomfort, nausea, severe vegetative and other symptoms such as stomach rumbling, burping, pallor , bloating of the face, flushing, breathing, dilated pupils, anxiety, panic-gustatory and olfactory hallucinations. 

Epilepsy with lateral temporal seizures. Simple seizures die, by auditory hallucinations or illusions or dreamy states characterized Sindh, visual hallucinations and language disorders in the fall of a focus in the language dominant hemisphere. This can evolve into complex partial seizures, a spread falls to the mesio-temporal or extratemporal structures takes place. The scalp EEG shows unilateral or bilateral temporal spikes or mid back, what most clearly at the lateral Ableitepunkten Sindh. 


- Frontal lobe epilepsy 

Frontal lobe epilepsy are by simple focal, complex focal, secondary generalized seizures or combinations characterized by them. The attacks often occur several times daily and often occur during sleep. Focal seizures of frontal lobe are sometimes called psychogenic seizures misunderstood. Status epilepticus is a frequent complication. 

General Characteristics 

The following characteristics determine diagnosis very close to dying: 

1st In general, short seizures. 

Second complex partial seizures beginning in the frontal lobe often have only minimal or no postictal confusion. 

3rd Rapid secondary generalization (frontal attacks more common than in Temporallappenanfällen). 

4th In the foreground stand Motorized phenomena, die or are affecting the tonic posture. 

5th Complex gestural automatisms are frequently at the beginning. 

6th In bilateral discharges, it often comes to the fall. 

A number of seizure types are described below here, BUT can multiple frontal areas are involved quickly, so do not know that specific types of seizures seen Sindh. 

Supplementary motor seizures. die there in supplementary motor seizures, seizure patterns in HaItungsänderungen, focal tonic symptoms with vocalization or speech inhibition and fencing posture. 

Cingular. cingulate complex focal seizure patterns are complex gestures at the start. Vegetative symptoms are common, as are changes of mood and affect. 

Anterior frontopolar region. Anterior frontopolar seizure patterns relate compulsive thinking or initials A loss of contact and tilting movements of the head and eyes to die, To develop any kontraversiven movements and axial clonic jerks and falls, so also available vegetative symptoms. 

Orbitofrontal. orbitofrontal seizures are complex partial seizures with motor and gestural automatisms initials, olfactory hallucinations and illusions, and vegetative symptoms. 

Dorsolateral. Dorsolateral seizure patterns can rarely tonic clonic or perfecting of his eyes and head as well as speech inhibition. 

Operkulär Operkuläre seizures are characterized by mastication, salivation, swallowing, laryngeal symptoms, speech inhibition, epigastric aura, fear and vegetative symptoms. Simple partial seizures, clonic seizures, especially in the face, Sindh often and can ipsilateral his. cases secondary meaningful changes occur, Can deafness One symptom of the hands especially his. Gustatory hallucinations are particularly common in of this region. 

Motor cortex. epilepsies of the motor cortex are mainly characterized by focal seizures Simple, and your location depends on the page, and topography of the involved area. cases die Lower prärolandische region is affected, there may be talk inhibition, vocalization, or dysphasia, tonic-clonic movements in the Or the opposite face to swallow coming. Generalization of the attack is common. rolandic region occur in the focal motor seizures without march to Jackson, or seizures, especially in the dying begin opposite the Upper Extremities. die cases involving seizures paracentral lobule cave, too, can tonic movements of the ipsilateral fusses occur except for the expected contralateral leg movements. postictal Or Todd'sche paralysis is common. 

Kojewnikow syndrome. If two types of Kojewnikow syndrome differed, also available What the One AS-Rasmussen's syndrome is known. The other type is a special form of the Roland focal epilepsy in adults and children constitute and is related to different types of lesions motor cortex. Its main characteristics are a) partial seizures Motorized die always well localized Sindh, b) often late occurrence of myoclonus one same place where die somatomotor seizures, c) An EEG with normal background activity and focal paroxysmal abnormalities (spikes and slow waves), d) occurrence of any age in children and adults, e) is often known etiology (tumor, vascular), f) no progressive development of the syndrome (in clinical, electroencephalographic, or psychological relationship, apart from the potential development of the underlying disease) . This picture Can a mitochondrial encephalopathy (MELAS syndrome) based on couches. 

Note: 

Some of the anatomical origin of epilepsy is sometimes difficult to assign to a lobe. These include epilepsy, with pre-and post-central symptoms (perirolandische seizures). Such an overlap of adjacent anatomical regions also occurs in operkulärer epilepsy. 

In frontal lobe epilepsy, the interictal scalp EEG can show the following: 

First no abnormality; 

Second sometimes asymmetry of background activity, frontal spikes or sharp waves or 

3rd Sharp Waves or slow waves, either unilateral or often bilateral or multiple regions on one side. 

Let differ sometimes unilateral intracranial derivations of bilateral involvement. 

In frontal lobe seizures can Different EEG patterns die initial clinical symptoms accompany. 's rarely die EEG abnormality Dem seizure onset preceded border and then provides important localizing information, such as A) frontal or multilobar, often bilateral low-amplitude fast activity, mixed spikes, rhythmic spikes , Rhythmic spike waves or rhythmic slow waves, or b) two-sided large-amplitude single-Sharp Waves die, followed by flattening diffuser Sindh. 

In dependence of the intracranial methods can provide derivations Additional Information About die chronological and spatial evolution of discharges. The localization can be difficult. 

- Parietallappenepilepsien 

. Epilepsy syndromes of the parietal lobe are usually by single-focal and generalized seizures characterized secondary Most of the parietal lobe resulting seizures remain single-focal, Over But during propagation can cave parietal addition, complex partial seizures develop from them. seizures of parietal lobe have the following properties: They are predominantly sensory characteristic with many features.A positive phenomena find yourself tingling and feelings described die of Electrifyingly or demonstrated according to a Jackson March spread Konner. There may be a desire to move a body part, or a feeling that moves Whether such a will. Can go The muscle lost. The most commonly affected are parts die with the greatest cortical representation of how the hand, arm and face. occur in the tongue can feel the tingling, the stiffness or coldness, meaningful symptoms in the face both sides of his can. Occasionally a Abdominal FEELING of sinking, choking or nausea occur, especially if the lower and lateral parietal lobe is involved Konner. Rarely Occurs pain, possibly in the form of a superficial burning dysesthesia or a vague, very violent Painful discomfort. Visual symptoms of the parietal lobe may safely AS show formed hallucinations, AS metamorphopsia with distortions, shortening and elongation. They are more common in discharges in the non-dominant hemisphere. 

The negative phenomena include numbness, the feeling of lack of a body part and an AS-known Asomatognosie loss of awareness of a body part or one side of the body. This is especially true IF die non-dominant hemisphere is affected. 

Pronounced drehschwindel or spatial disorientation speaking for seizures of lower parietal lobe. seizures in the dominant parietal lobe lead to a range of receptive and connective language disorders. occur in paracentral location can significantly lateralized genital sensations. rotatory conscience or attitude concerned Motor symptoms occur can. paroxysms of lobule paracentral are particularly prone to secondary generalization. 

- Okzipitallappenepilepsien 

Epilepsy syndromes of the occipital lobe are usually by single-focal well as by secondary generalized seizures characterized. occur Complex partial seizures can in spreading over the occipital lobe addition.The meeting of Common Okzipitallappenanfällen and migraine is complex and controversial judges WILL. Among the clinical seizure symptoms usually, but Not necessarily, visual phenomena. Are Elementary visual seizures characterized by fleeting visual perceptions, negative die his Konner (scotoma, hemianopia, amaurosis), or more frequently positive (sparks or flashes, phosphenes). appear Such perceptions in the visual field opposite the discharging lesion in the . specific visual cortex can, but spread over the entire field of illusory perceptions, with those dying objects appear distorted occur Konner. The following versions of Sindh distinguished: changes in size (macropsia Or micropsia), change in the distance, unbalanced look, twist and shape changes of objects (metamorphopsia). Occasionally visual hallucinatory seizures are characterized by complex visual perceptions, such as colored scenes Variable complexity. Sometimes death scene appears distorted or smaller, and in rare fallen Does the patient his own image (Heautoskopie). Such illusory or hallucinatory visual seizures say, know that the temporo-parietooccipital border area is included. The initial symptoms can also search a tonic, clonic or tonic-clonic counterpoint version of eye and head, or involve only the eyes (oculoklonische Or oculogyrische deviation), Lidkloni and forced eyelid closure. The perception of one oscillation Or the eyes of the whole body can occur. 

The discharge may spread to the temporal lobe through, resulting in seizure symptoms as with lateralposterior-temporal, hippocampal, or amygdaloid epilepsy. IF the primary focus localized in the vicinity supracalcarina is possible to discharge die forward to suprasylvischen convexity or the mesial surface-spread and die mimic symptoms of epilepsy of the parietal lobe or frontal lobe. Occasionally there is a tendency for secondary generalization. 

1.3 cryptogenic 

Cryptogenic epilepsies are presumed symptomatic, but of unknown etiology. THUS This category differs from the previous one, know that no etiology is found (see Preface). 

Generalized epilepsy AND SYNDROME 


First Idiopathic (with altersgebundenem beginning, for THE age of onset ordered) 

Benign familial neonatal convulsions 

Benign neonatal convulsions 

Benign myoclonic epilepsy of infancy 

Epilepsy with absences pyknoleptischen (pyknolepsy, absence epilepsy of infancy) 

Juvenile absence epilepsy 

Impulsive petit-mal epilepsy juvenile myoclonic epilepsy) 

Grand mal epilepsy on awakening Other idiopathic generalized epilepsies 

With specific epilepsy seizure triggers (see Appendix 2) 


First cryptogenic or symptomatic (in order of THE age of onset) 

Epilepsy with lightning-Nick-Salaam spasms (West syndrome) 

Lennox-Gastaut syndrome 

Epilepsy with myoclonic-astatic seizures 

Epilepsy with myoclonic absences 


First symptomatic 

First Unspezfische etiology 

Early myoclonic encephalopathy 

Early infantile epileptic encephalopathy with suppression-burst 

Other symptomatic generalized epilepsies 


1st Specific Syndromes 

Epileptic seizures can complicate MANY disease states. One of this site is about those diseases in which seizures Leading Or A dominant symptom Sindh. They are listed in APPENDIX 1. 

Second epilepsies and syndromes, AS is not determinable focal or generalized ARE 

First focal With both generalized seizures and 

Neonatal convulsions 

Severe myoclonic epilepsy of infancy 

Epilepsy with continuous spike-wave discharges in synchronized sleep 

Aphasia-epilepsy syndrome (Landau-Kleffner syndrome) 

Other undetermined epilepsies 

First generalized or focal Without Obvious signs 

This subheading covers all cases with generalized tonic-clonic seizures, those with clinical and EEG findings a clear classification as generalized or localization-related non-permit, how many cases of sleep-grand mal. 

2nd SPECIAL SYNDROME 

Opportunity attacks 

Febrile seizures 

Isolated seizures or isolated status epilepticus 

Seizures die, exclusively in acute metabolic or toxic events occur with factors such as alcohol, drugs, eclampsia, non-ketotic hyperglycemia 


Definitions 


LOCATION SPECIFIC (focal, LOCAL, partial) epilepsies and syndromes. 

Are these epileptic disorders in those dying recognize seizure symptoms or examination findings a localized start of the attacks leave. They include patients with small localized, constant epileptogenic lesions (anatomic or functional), ie genuine focal epilepsy, also available, but patients with less well-defined lesions, whose seizures can go out-of variable types. For the most symptomatic localization-related epilepsies epileptogenic lesion in one part of a dying brain hemisphere to find, while Konner idiopathic epilepsies with focal seizures Comparable age-related regions in both hemispheres included functional. 

According to "bilateral seizures, those dying in the First Clinical changes include an initial view of both hemispheres. The ictal EEG patterns are beginning." International Classification of Epileptic Seizures, generalized epilepsies and epileptic disorders Sindh syndromes with generalized seizures, ie 

EPILEPSY SYNDROME AND THAT AS A non-focal or generalized ARE determinable. 

Is this for various reasons his undecidable: 

a) the patient had both partial seizures, generalized AS coexistence or succession (eg, partial seizures and absence seizures) Also, too, and according to both focal THAN generalized EEG discharges (eg, a temporal spike focus and independently Bilateral Spike-Waves) ; 

b) On a positive notes either focal or generalized seizure onset are lacking. The most common reason for this is, dass die seizures occur during sleep, the patient remembers no aura, and additional investigations including EEG inconspicuous Sindh. 

Localization-related idiopathic epilepsies 

This is epilepsy of childhood with partial seizures and EEG findings. They occur age-related, with no demonstrable anatomic lesion and care to remit spontaneously. Clinically Did die patient Neither Neurologic even intellectual loss and no history of relevant previous diseases, often but A family history of benign epilepsy. The seizures are usually brief and rare, can occur, but often at first. The attack sequence can from case to case his, but remains in the same child in the regular constant. The EEG is characterized by normal background activity and repeated localized high-amplitude spikes, sometimes with several independent sites. Short happen generalized spike-wave bursts can. The focal findings to take in his sleep, with your form does not change. 

Benign epilepsy of childhood with zentrotemporalen spikes. This is a syndrome of short, simple focal, hemifacial motor seizures, often associated with sensitive symptoms associated with a tendency to secondary generalized tonic-clonic seizures. You start with 3 to 13 with no age restriction (peak incidence 9 ~ 10 years), and healing before the 15th-1ß. year. There is often a genetic predisposition, the male sex predominates. The EEG shows large-amplitude zentrotemporale spikes often followed by a slow wave die by sleep activated, and a tendency to spread to Or the other side to change sides have. 

Epilepsy of childhood with occipital paroxysms. Of this syndrome is similar in general previous Dem. The seizures start with visual symptoms (amaurosis, PhotoME, illusions or hallucinations), often with subsequent hemiklonischen seizures or automatisms. go in 25% of cases die in attacks migraine-like headaches about. The EEG shows paroxysms of high-amplitude spike waves or sharp waves over which the occipital and temporal regions of the rear of one or both hemispheres repeat rhythmically, but only with closed eyes. Konner During the attacks yourself die occipital discharges for Central expand or temporal region. At present, no final decision about prognosis is possible to die. 

. Primary reading epilepsy . In the case of this syndrome all or quickly released all attacks by reading (especially aloud), regardless of the content of the text , the attacks are simply focal motor with involvement of the masticatory muscles or visually, IF the stimulus generalized non-interrupted, will be tonic Konner develop-clonic seizures. The syndrome can be inherited. It typically begins in adolescence and the late course is benign with little tendency to development of spontaneous seizures. The neurological findings and die results of imaging studies are normal, the EEG shows spikes or spike -Waves in the dominant temporoparietal region. Generalized spikes and waves can also occur. 

Idiopathic generalized epilepsies (age-related) 

Here are a series of generalized epilepsies, in which all attacks from the beginning a generalized Sindh, where the EEG generalized Bilateral shows synchronous and symmetrical discharges (see description of the types of seizure classification). The patients are between the seizures usually unremarkable, without neurological or neuroradiological findings. In general, shows the interictal EEG A normal background activity and generalized discharges as spikes, Polyspikes, spike-waves and poly-spike waves of 3/sec. Or Faster. Take the discharges to the synchronized sleep. The various syndromes of idiopathic generalized epilepsies differ mainly by Various age of onset. 

Benign familial neonatal convulsions. This is a rare, dominantly inherited disease, usually dying on second and third day of life with clonic or apnoeic attacks, but without a specific manifested EEG criteria.Background investigations and no ceiling on etiologic factors. About 14% of One patient later develop epilepsy. 

Benign neonatal convulsions. These are very often repeated clonic or apnoeic attacks, die by the 5th day of life occur without a known etiology or accompanying metabolic disorder. The EEG often shows interictal alternating sharp theta waves. The attacks recur later not, and psychomotor development is undisturbed. 

Benign myoclonic epilepsy in infancy. This epilepsy is characterized by brief bursts of generalized myoclonus in the course of the first or 2 Year of life occur in otherwise healthy children who often have a family history of seizures or epilepsy. EEG studies show generalized spike-waves that occur in short bursts in the early stages of sleep. The seizures are easily controlled by appropriate treatment. They are not accompanied by other types of seizures, however, in adolescence, generalized tonic-clonic seizures occur. A relative delay of intellectual development and light 

Personality disorders may be added. 

Pyknoleptischen epilepsy with absence seizures (pyknolepsy, absence epilepsy of infancy). This syndrome occurs in otherwise normal children with a strong genetic predisposition in the school age (main age of onset 6 ~ 7 years). It is more common in girls than in boys and is characterized by very frequent absence seizures (several to many per day). The EEG shows bilateral, synchronous and symmetrical spike-waves of mostly 3/sec. with normal background activity. In adolescence often develop generalized tonic-clonic seizures. Moreover, the absence seizures stop or, more rarely, persist as a single seizure type. 

Juvenile Absencenepilepsie. The absences of this syndrome are the same as in epilepsy with absences pyknoleptischen, but with absence seizures are rare retropulsiven movements. The Age of onset is around puberty. The seizure frequency is lower than in the pyknolepsy (less than a day, mostly intermittently). Addition of a generalized tonic-clonic seizures are frequent, this will require more frequent than in the absences of pyknolepsy, where they are often tied to the Aufwachsituation. Not infrequently, patients have myoclonic seizures. The gender distribution is equal. The spike-waves are often faster than 3/sec. The response to therapy is very good. 

lmpulsiv-Petit mal epilepsy (juvenile myoclonic epilepsy). This syndrome manifests itself at puberty and is characterized by seizures with bilateral, single or repetitive arrhythmic, irregular myoclonic jerks predominantly in arms. Some patients may fall through the contractions. A lack of awareness is not apparent. The disease can be inherited, the gender distribution is uniform. Often there are generalized tonic-clonic seizures, absence seizures less frequently. The seizures usually occur shortly after awakening and are often triggered by sleep deprivation. The interictal and ictal EEG shows rapid, generalized, often irregular spike-waves and poly-spike-waves, between the spikes in the EEG and the twitching is no close phase relationship. Often the patients are photo sensitive. You speak in an appropriate medication to good. 

Grand mal epilepsy on awakening. This syndrome usually begins in the second decade of life. The generalized tonic-clonic seizures occur exclusively or predominantly (over 90%) shortly after waking up, regardless of time of day or in a second peak incidence in the evening. If any other seizures, absence seizures or myoclonic then mostly impulsive petit mal type. The attacks can be triggered by sleep deprivation or other external factors. Quite often, there is a genetic predisposition. The EEG shows a pattern of idiopathic generalized epilepsy. There is a significant correlation with photosensitivity. 

Cryptogenic or symptomatic generalized epilepsies (age-related) 

Epilepsy with lightning-Nick-Salaam spasms (West syndrome). Usually, the West's syndrome is a characteristic triad: Lightning-Nick-Salaam convulsions and, stalling of psychomotor development and hypsarrhythmia in the EEG, although one of the elements may be lacking. The spasms can be reflector or extensor, lightning or pitching fits, but are most often mixed. The beginning is always in the first year of life, usually at age 4-7 months. Boys are affected more frequently. The prognosis is generally poor. The West syndrome can be divided into two groups. The symptomatic group is characterized by previous signs of brain damage (mental retardation, neurological symptoms, radiological findings, other seizure types) or by a known etiology. The smaller cryptogenic group is characterized by the absence of preceding symptoms of brain damage and a known etiology. The forecast is partly determined by early treatment with ACTH or oral steroids. 

Lennox-Gastaut syndrome. This syndrome manifests itself in children aged 1-8 years, but mainly of preschool age. Tonic-atonic seizures and absence seizures and axial are the most common types of seizures, but others, such as myoclonic, generalized tonic-clonic and partial seizures to occur frequently. The seizure frequency is high, status epilepticus (stuporous states with myoclonus, tonic and atonic seizures) are common. The EEG usually shows an abnormal background activity, slow spike-waves slower than 3/sec. and often multifocal abnormalities. .. Sleeping in bursts of fast rhythms appear (to 10/sec> In general, mental retardation, the seizures are difficult to treat and development is usually unfavorable in 60% of cases, the syndrome affects children with a pre-existing brain disease,.. At the others it occurs primarily. 

Epilepsy with myoclonic-astatic seizures. The Age of onset is between 7 months and 6 years, mostly between 2 and 5 years. Boys are (at the beginning of the first year> twice as likely to frequent. There is often a hereditary predisposition, and usually precedes a normal development. Is in the attacks, there are myoclonic, astatic, myoclonic-astatic, absence seizures with clonic and tonic components and tonic -clonic seizures. Often it comes to status. Tonic seizures develop late in adverse gradients. The EEG is often initially normal, except for 4-7/sec. rhythms, but irregular fast spike-waves or poly-spike waves have . The course and prognosis are variable. 

Epilepsy with myoclonic absences. This syndrome is clinically characterized by absence seizures with severe bilateral rhythmical clonic jerks, often associated with a tonic contraction. In the EEG there are always bilateral synchronous and symmetrical spike discharges of rhythmic waves of 3/sec. Similar to the pyknoleptischen epilepsy with absences. The seizures occur more frequently every day. The jerks may be perceived subjectively. Rarely occur to other attacks. The Age of onset is 7 years, boys predominate. The prognosis is less good than in the pyknolepsy because of resistance to therapy of seizures, intellectual deterioration and possible evolution to other types of epilepsy such as Lennox-Gastaut syndrome. 

Symptomatic generalized epilepsies and syndromes 

Symptomatic generalized epilepsy are most common in infancy and are characterized by generalized seizures, where they differ clinically and EEG of the idiopathic generalized epilepsies. It can be a single seizure type, but more often occur in various types, including myoclonic jerks, tonic seizures, atonic seizures and atypical absences. Their EEG patterns are bilateral, but less rhythmical than in idiopathic generalized epilepsy, and more or less asymmetrical. The interictal EEG findings differ from those of idiopathic generalized epilepsies, with suppression-bursts are, hypsarrhythmia, slow spike-waves or generalized fast rhythms. Focal abnormalities may be added to each of these patterns. There are clinical, neuropsychological and neuroradiological signs of diffuse normally, specific or nonspecific brain disease. 

Generalized epilepsies of nonspecific symptomatic etiology (age-related) 

Early myoclonic encephalopathy. The main characteristic of this syndrome are onset in the first 3 months of life, initially fragmentary myoclonus, then erratic partial seizures, massive myoclonia or tonic spasms. The EEG is characterized by suppression-burst activity, which may evolve into a hypsarrhythmia. The course is severe, are the psychomotor development remains, it may come in the first year of life or death. Familial occurrence is common, and suggests the influence of one or more congenital metabolic disorders, but there is no constant pattern of inheritance. 

Early infantile epileptic encephalopathy with suppression-burst. This is by Ohtahara et al (5) described syndrome defined by a very early start in the first months of life, frequent tonic spasms and suppression-burst pattern in the waking and sleep EEG. Focal seizures can occur. Myoclonic seizures are rare. The etiology and underlying pathology is unclear. The prognosis is poor with severe psychomotor retardation and resistance to therapy of seizures, often it comes at the age of 4-6 months to transition into a West syndrome. 

EPILEPSY SYNDROME AND NOT AS A focal or generalized determinable 

Neonatal seizures. The attacks of newborns differ from those of older children and adults. The most common neonatal seizures are described as discrete, because the clinical symptoms are often overlooked.These include tonic horizontal eye movements with and without flinching, blinking or fluttering of the eyelids, sucking, lip smacking or other movements of the mouth, tongue and cheeks, swimming or cycling movements, occasional apnoeic attacks. Others show a similar neonatal seizures as tonic extension of the limbs or Dekortikationshaltung decerebration. These are particularly seen in premature infants. It can also happen multifocal clonic seizures with clonic movements of a limb, which migrate to other body parts or other members may, or very much more localized focal clonic seizures. In the latter, the child is usually not unconscious. Rare myoclonic seizures may occur. The EEG pattern often corresponds to a suppression-burst activity. The tonic seizures have a poor prognosis because they are often a symptom of an intraventricular. Also, the myoclonic seizures have a poor prognosis because they often show the presence of the syndrome of early myoclonic encephalopathy. 

Severe myoclonic epilepsy of infancy. This is a recently described syndrome. Its characteristics include a family history of epilepsy or febrile seizures, normal development until the onset of the disease, first seizures in their first year in the form of generalized or unilateral clonic febrile seizures, myoclonic jerks and often occurs after focal seizures. The EEG shows generalized spike waves and poly-spike waves, early photosensitivity and focal abnormalities. The psychomotor development is from the 2nd Age is delayed, and it comes to the appearance of ataxia, pyramidal signs, and interictal myoclonus. This form of epilepsy is very resistant to all forms of treatment. 

Epilepsy with continuous spike-wave discharges in synchronized sleep. To this syndrome different focal or generalized seizure types are atypical absences during sleep and waking. Tonic seizures do not occur. The characteristic EEG pattern consists of continuous diffuse spike-waves during sleep with slow waves, which turn out after the onset of seizures. The duration varies from months to years. The forecast is to be assessed cautiously, because in spite of the usually benign evolution of seizures can adjust neuropsychological disturbances. 

Aphasia-epilepsy syndrome (Landau-Kleffner syndrome). The Landau-Kleffner syndrome is a disorder of childhood with the development of aphasia, multifocal spikes and spike-wave discharges. Epileptic seizures, psychomotor and behavioral problems occur in two thirds of patients. There is an auditory Wortagnosie and rapid reduction in spontaneous speech. The seizures are usually generalized tonic-clonic or focal motor. They are rare and hear as well as EEG abnormalities before 15 Years on. 

SPECIAL SYNDROMES 

Febrile convulsions. Febrile seizures are an age-related disorder that is characterized by generalized seizures almost always during an acute febrile illness. The majority of febrile seizures are brief and straightforward, but a minority may be prolonged in temporary or permanent neurological symptoms pass, as in hemiplegia - hemiatrophy - epilepsy (HHE) - syndrome. In approximately one third of cases there is a tendency for repetition of febrile convulsions. The controversy about the risk of developing epilepsy is later, through some new comprehensive studies done largely, it seems that the overall risk does not exceed 4%. The indications for prolonged drug prophylaxis against the recurrence of febrile seizures are now well defined, in most cases it is not necessary. In principle, there is a relatively benign disorder of early childhood. 

Annex 1 

Symptomatic generalized epilepsies of specific etiology 

(6 in line with Roger et al) 

Here only the diseases are listed, in which epileptic seizures, the main symptom or a major symptom. These diseases often lead to epileptic state images, which are similar to the symptomatic generalized epilepsies of nonspecific etiology and occur in similar age groups. 

MALFORMATIONS 

Aicardi's syndrome occurs in females. It consists of Netzhautlakunen callosum, absence of the corpus, Blitz-Nick-Salaam convulsions with early onset 

and often asymmetric, diffuse, usually asynchronous EEG abnormalities with suppression-burst patterns or atypical hypsarrhythmia. 

Lissencephaly-pachygyria characterized by facial malformations and specific characters in computed tomography, axial hypotonia and lightning-Nick-Salaam convulsions. The EEG shows large-amplitude fast "alpha-like" pattern with no difference in waking and sleeping. 

For each phacomatoses there are no typical clinical and electroencephalographic patterns. Note is the frequency of seizures with lightning-Nick-Salaam spasms in tuberous sclerosis, with generalized and focal seizures may be added during the course of the otherwise typical BNS-convulsions. The Sturge-Weber syndrome is a common cause of simple partial seizures followed by hemiparesis. 

Hypothalamic hamartomas can be in fits of laughter, precocious puberty and retardation manifest. 

Proven or suspected congenital metabolic ANOMALIES 

Newborn child 

At the age of the newborn metabolic defects include non-ketotic hyperglycinemia and the D-Glycerizazidämiemit manifestation as early myoclonic encephalopathy with erratic myoclonus, partial seizures, and suppression-burst patterns in the EEG. 

Toddler 

The classical phenylketonuria can manifest as West syndrome. A variant of phenylketonuria with seizures starting in the second Biopterinmangelverursacht Six months of life in children who are hypotonic since birth. The seizures are generalized motor combined with erratic myoclonus and seizures oculogyrischen. 

The Tay-Sachs disease and Sandhoff disease horror show together on an acoustic stimulus and myoclonus in the first months of life without EEG changes. Myoclonic jerks occur in the second year and erratic focal seizures with a marked slowing of background activity in the EEG. 

At the early infantile type of ceroid-lipofuscinosis (Santavuori-Haltia disease-Hagberg) between the start of the fifth and 18 Months of massive myoclonus with a very characteristic EEG pattern ("vanishing" EEG). 

The Pvridoxin dependence is manifested by seizures without any special characteristics, but one must always think for therapeutic reasons to this situation. 

Child 

The late infantile ceroid lipofuscinosis (Jansky-Bielschowski disease) is characterized by onset between the 2nd and 4 Age with massive myoclonic jerks, atonic or astatic seizures. The EEG shows a slow background activity, multifocal spikes and a characteristic response to photic stimulation slower rate. 

An infantile type of Huntington's disease occurs after the third Followed by slowing of age on the mental development of dystonia, generalized tonic-clonic 

Seizures, atypical absences and myoclonic seizures. The EEG shows generalized spike-wave and poly-spike-wave discharges during photic stimulation in the normal range. 

Childhood and adolescence 

A juvenile form of Gaucher disease is characterized by onset between the ages of 6-8 years with epileptic seizures of various types, most often tonic-clonic or focal motor seizures from. The EEG shows a progressive deterioration of background activity, an abnormal response to photic stimulation, diffuse paroxysmal abnormalities and multifocal abnormalities with distinct occipital emphasis. 

The juvenile form of ceroid lipofuscinosis (Spielmeyer-Vogt's disease Sjogren) is characterized by onset at 6-8 years, decreased visual acuity, slowing of psychomotor development and appearance of cerebellar and extrapyramidal symptoms. After one to four years appear to generalized tonic-clonic seizures and fragmentary, and massive segmental myoclonus. The EEG shows bursts of slow waves and slow spike-waves. 

The Lafora disease begins with 6-19 years (mean 11.5 years) and is characterized by generalized clonic and tonic-clonic seizures, partial seizures often also with visual symptoms, a constant myoclonic syndrome (fragmentary, and massive segmental myoclonus), and rapidly progressive dementia. The EEG shows occipital spike-waves discharges faster and poly-spike waves, photosensitivity, deterioration of background activity and development of multifocal abnormalities, mainly. The disease leads to an average of 5 ½ years for death. 

The so-called progressive degenerative Myoklonusepilepsie type Lundborg) falls into this category. The only significant group of well-demarcated border is the Finnish type, which was described by Koskiniemi et al (4). Start with 8-13 years with myoclonus (segmental, fragmentary, and solid) and generalized seizures tonischklonischen, also cerebellar ataxia and slowly progressive, generally mild dementia. The EEG shows slow abnormalities (theta rhythms, and later delta rhythms) with generalized spike-waves, especially in the frontal region and photosensitivity. The patients survive 15 years or more. 

The cerebellar Dyssynergia myoclonica with epilepsy (Ramsay-Hunt syndrome) begins at the age of 6-20 years (average age 11) with myoclonus or generalized tonic-clonic seizures. The myoclonic syndrome is mainly characterized by action and Intentionsmyoklonien. The generalized seizures are rare and well treated. If dementia occurs, it develops slowly. The neurological symptoms are usually restricted to cerebellar signs. The EEG shows normal background activity, generalized paroxysms of spikes, spike-waves and poly-spike waves, and photo sensitivity. During REM sleep occur fast poly-spikes in the central and vertex regions. 

The clinical picture of the cherry-red spot-myoclonus syndrome (sialidosis with isolated deficiency of neuraminidase) is that of Ramsay-Hunt syndrome are very similar with myoclonus, photosensitivity, and a cerebellar syndrome. Other characteristics are the most frequently encountered amblyopia and a cherry-red spot at the back of the eye. 

The EEG is similar to that of the Ramsay-Hunt syndrome with the following characteristics: The poly-spike-wave discharges always correspond with massive myoclonus, and there is no photosensitivity. 

A Ramsay-Hunt-like syndrome can occur in combination with a mitochondrial myopathy with abnormalities of lactate and pyruvate metabolism (Fukuhara et al, 3). 

Adult 

The Kufs disease (adult ceroid lipofuscinosis) is a relatively slowly progressive storage disease with frequent generalized seizures, which can be very resistant to therapy. In contrast to the juvenile storage diseases, the fundus may be normal. Main characteristic is an extreme photosensitivity at low photic stimulation. 

A large number of epileptic seizures, concomitant diseases in childhood, younger and later adulthood are not listed here, because the attacks are different from other types of seizures and not for the diagnosis because they are not of vital importance. 

Annex II 

CAUSED SEIZURES are those preceded by external or internal factors in a constant manner the attacks, which distinguishes them from spontaneous epileptic seizures that do not trigger factors can be identified. Certain non-specific factors (eg lack of sleep, alcohol or drug withdrawal or hyperventilation) are common triggers as opposed to specific seizure triggers. With certain epilepsy syndromes is clear that the seizures respond more to non-specific triggering factors, but rarely contributes to the classification of epileptic syndromes. 

In contrast, in cases of epilepsy with specific seizure triggers a constant relationship be established between one or more definable non-ictal events and the subsequent occurrence of a specific, uniform attack. Some epilepsies (reflex epilepsies), the attacks by specific sensations or perceptions are triggered in response to well-defined or specific stimuli. Usually, these stimuli limit for the individual patient to a single specific stimulus or a limited number of closely related stimuli. The belonging here are generalized epilepsies, and usually idiopathic, in certain cases but also in acquired focal lesions, where it is usually tactile or proprioceptive stimuli. 

Epileptic seizures can be triggered by a sudden fright, if it is an unexpected stimulus is (startle epilepsy). The seizures are usually generalized tonic, but can also focally Being, the cause is usually symptomatic. 

Seizures that are triggered by higher cerebral benefits, such as memories and pattern recognition are most commonly associated with complex partial seizures, but are occasionally observed and generalized epilepsies. In most of these patients are also spontaneous attacks.