Dr H. Gharebaghian MD Neurologist Department of Neurology Kermanshah Faculty of Medicine

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1 Dr H. Gharebaghian MD Neurologist Department of Neurology Kermanshah Faculty of Medicine

2 Definitions Seizures are transient events that include symptoms and/or signs of abnormal excessive hypersynchronous activity in the brain Epilepsy as a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition

3 Definitions The traditional definition of epilepsy requires at least two unprovoked seizures The definition proposed by the ILAE in 2005 suggested that one epileptic seizure is sufficient to diagnose epilepsy if there is additional enduring alteration in the brain that increases the likelihood of future seizures The proposed definition has been controversial and has not been widely accepted

4 Definitions Practical clinical definition: Epilepsy was defined as a disease of the brain defined by any of the following conditions: (1) At least two unprovoked seizures occurring >24 hours apart (2) One unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years (3) Diagnosis of an epilepsy syndrome

5 Classification Needs for classification: Variety of seizure types and epilepsies Communication and diagnostic purposes Evaluating drug specificity and prescribing the most appropriate therapy Predict response to therapy and prognosis

6 Classification ILAE diagnostic scheme for the classification of seizures and epilepsy recommend the following axes: Axis 1: Ictal phenomenology Axis 2: Seizure type Axis 3: Syndrome (a syndrome diagnosis may not always be possible) Axis 4: Etiology Axis 5: Impairment

7 Classification Classifications developed by the ILAE continue to be used widely: Clinical and Electroencephalographic Classification of Epileptic Seizures published in 1981 Classification of Epilepsies and Epileptic Syndromes introduced in 1989 New ILAE classification for seizures and epileptic syndromes published in 2010

8 1981 classification of seizures

9 2010 classification of seizures New terminology and concepts that better reflect the current understanding, while also striving for clarity and simplicity Maintained the division of seizures based on generalized or focal onset but has recommended replacing partial with focal Updated the definition of focal seizures as originating within networks limited to one hemisphere, with the possibility of the seizures being discretely localized or more widely distributed, and possibly originating in subcortical structures Abandoned simple, complex, and secondarily generalized

10 2010 classification of seizures Generalized seizures were defined as originating at some point within, and rapidly engaging, bilaterally distributed networks, which do not necessarily include the entire cortex Revised concepts acknowledge that generalized seizures can be asymmetrical and that individual seizures may appear to have a localized onset, but the location and laterality of that onset will vary from seizure to seizure

11 2010 classification of seizures For focal seizures, the distinction between the different types (e.g., complex partial and simple partial) is eliminated It is important, however, to recognize that impairment of consciousness/awareness or other dyscognitive features, localization, and progression of ictal events can be of primary importance in the evaluation of individual

12 2010 classification of seizures Descriptors of focal seizures according to degree of impairment during seizure: Without impairment of consciousness or awareness With impairment of consciousness or awareness (Dyscognitive). This roughly corresponds to the concept of complex partial seizure Evolving to a bilateral, convulsive seizure (involving tonic, clonic, or tonic and clonic components). This expression replaces the term secondarily generalized seizure.

13 2010 classification of seizures

14 1989 classification of epilepsies

15 2010 classification of epilepsies Abandoned Localization-related vs generalized. Syndromes were arranged by age at onset. Instead of the terms idiopathic, symptomatic, and cryptogenic, the following three terms were recommended: 1. Genetic- the epilepsy is the direct result of a known or presumed genetic defect in which seizures are the core symptom of the disorder 2. Structural/metabolic 3. Unknown cause

16 2010 classification of epilepsies syndrome will be restricted to a group of clinical entities that are reliably identified by a cluster of electroclinical characteristics Patients whose epilepsy does not fit the criteria for a specific electroclinical syndrome can be described with respect to a variety of clinically relevant factors (e.g., known etiology and seizure types) This does not, however, provide a precise (syndromic) diagnosis of their epilepsy

17 2010 classification of epilepsies A number of entities that are not exactly electroclinical syndromes but which represent clinically distinctive constellations on the basis of specific lesions or other causes. These are diagnostically meaningful forms of epilepsy and may have implications for clinical treatment, particularly surgery. These include mesial temporal lobe epilepsy (with hippocampal sclerosis), hypothalamic hamartoma with gelastic seizures, epilepsy with hemiconvulsion and hemiplegia, and Rasmussen syndrome. Age at presentation is not a defining feature in these disorders, as we understand them; however, they are sufficiently distinctive to be recognized as relatively specific diagnostic entities

18 2010 classification of epilepsies

19 2010 classification of epilepsies

20 Major symptomatic epilepsies

21 Genetic/congenital epilepsies

22 Acquired epilepsies

23 Brain malformations In children, developmental brain malformations are an important cause of epilepsy These can be generalized, hemispheric, or focal They are also classified as related to: Abnormal cell proliferation or differentiation including tuberous sclerosis, focal cortical dysplasia, and hemimegalencephaly Abnormal neuronal migration, including lissencephaly, subcortical band heterotopias, and periventricular nodular heterotopias Abnormal cortical organization, including polymicrogyria and schizencephaly

24 Brain malformations Some of these malformations are genetically determined Many of these malformations have other associated neurological disorders or physical findings Focal malformations associated with epilepsy are less likely to have other neurological manifestations than hemispheric or generalized malformations The severity of epilepsy and its response to therapy can be quite variable, but it is commonly drug-resistant, prompting evaluation for surgical treatment

25 Brain malformations Scizencephaly Lissencephaly Tuberous sclerosis

26 Head trauma Head trauma is an important risk factor for epilepsy, with the greatest risk seen in association with penetrating head injury, depressed skull fracture, and severe head trauma Mild traumatic brain injury (characterized by absence of fracture and a loss of consciousness or post-traumatic amnesia for less than 30 min) is associated with only a 1.5-fold increase in risk Moderate head injury (defined as loss of consciousness or posttraumatic amnesia for 30 minutes to 24 hours or a skull fracture), is associated with a 2.9-fold increase in risk Severe head injury (including brain contusion or intracranial hematoma or loss of consciousness or post-traumatic amnesia for more than 24 hours), is associated with 17-fold increased risk

27 Head trauma The risk was highest in the first year after the injury but remained increased thereafter for a duration that varied with severity of the injury For those with moderate brain injuries, the risk was markedly increased for up to 10 years only; for those with severe traumatic brain injury, the risk continued to be increased Early seizures appeared to be a strong risk factor for late seizures, but early seizures were usually related to the severity of the head injury and intracranial lesions Phenytoin and Levetiracetam are effective in preventing seizures in the first week

28 Vascular Malformations The two vascular malformations most commonly associated with epilepsy are arteriovenous malformations and cavernous malformations Arteriovenous malformations (AVMs) are high-pressure vascular malformations with arteriovenous shunting. They are a tangle of feeding arteries and draining veins without intervening capillary bed They may come to attention during evaluation for seizures or after they bleed; or may be incidental finding on imaging Because of the high pressure, they are susceptible to bleed at a rate of 1% to 3% per year, which is the main reason they require therapy Treatment options include: Surgical resection, Endovascular treatment with embolization and radiosurgery

29 Vascular Malformations Cavernous malformations consist of blood-filled epithelium lined caverns with no discrete arteries or veins On MRI they have a characteristic popcorn appearance with mixed signal within the lesion and a rim of decreased signal, reflecting hemosiderin They may be multiple in approximately a third of cases Cavernous malformations are low-pressure lesions with a much smaller risk of bleeding than AVMs They are strongly associated with epilepsy If seizures are controlled with medical therapy, there is no clear indication for surgical resection when epilepsy is drug resistant, resection of the cavernous malformation is associated with excellent results

30 Vascular Malformations Cavernous malformation Arteriovenous malformation

31 Brain Tumors Brain tumors are a common cause of epilepsy, particularly drugresistant epilepsy. Most drug-resistant epilepsy occurs with low-grade tumors, especially those in the temporal lobe Benign tumors associated with epilepsy are gangliogliomas, dysembryoblastic neuroepithelial tumors (DNET), and low grade gliomas Excellent seizure control most often occurs after removal of gangliogliomas and DNET tumors Seizures contribute to the morbidity of malignant brain tumors in approximately a quarter of patients Grade 3 anaplastic astrocytomas are more likely to present with seizures at onset than glioblastoma multiforme

32 Brain Tumors GBM Meinigioma Metastasis

33 Stroke Stroke increases the risk of seizures and epilepsy at any age It is the most common cause of seizures in the elderly Early seizures that occur within 2 weeks of the stroke The risk of chronic epilepsy is highest in the first year after stroke, with a 17-fold increase in the risk Compared to individuals who did not have early seizures, approximately 30% of individuals who have early poststroke seizures develop later epilepsy. This is a 16-fold increase in risk

34 Stroke Seizures and even status epilepticus can be a presenting symptom of acute stroke. Strokes involving the cortex are more likely to produce epilepsy than deep white-matter strokes The incidence of seizures is also higher in patients with intracerebral hemorrhage Prophylactic AED therapy is contraversial

35 Autoimmune Disorders Immune disorders increase the risk of epilepsy and seizures In SLE, the risk of seizures is 12% to 20% and is more likely with anticardiolipin and anti-sm antibodies Between 2% and 6% of patients with multiple sclerosis have seizures. Those who do tend to have more extensive cortical involvement. Seizures are more likely to occur in the context of acute relapse, but some patients develop chronic epilepsy Seizures are a more common acute manifestation of acute disseminated encephalomyelitis, (approximately 50%). However, chronic epilepsy is much less likely (only about 5% )

36 Autoimmune Disorders Limbic encephalitis is an increasingly recognized cause of epilepsy Suggested diagnostic criteria include one of disturbance of episodic memory, temporal lobe seizures, or affective disturbance plus the presence of either well-characterized antibodies or unexplained increased signal in the mesial temporal structures, or histopathology of mesial temporal encephalitis It can be paraneoplastic or nonparaneoplastic Paraneoplstic cases are associated with small cell lung cancer, testicular cancer, thymoma, breast cancer, or teratoma

37 Autoimmune Disorders An immune basis of epilepsy should be suspected in individuals who have other autoimmune disease, abrupt or recent onset of seizures (particularly if resistant to AEDs and progressive in frequency and severity), associated manifestations such as behavioral changes and psychosis, severe memory disturbances, and abnormal signal on MRI in the hippocampi An immune origin is suspected in several well-described epileptic syndromes. These include West syndrome, Lennox-Gastaut syndrome, LKS, and Rasmussen syndrome

38 Acute Symptomatic Seizures Acute symptomatic seizures occur in close temporal relationship with an acute CNS insult metabolic, toxic, structural, infectious, or inflammatory Metabolic disturbances known to cause seizures include hypoglycemia, hyperglycemia, hyponatremia, hypocalcemia, hypomagnesemia, and uremia Withdrawal from chronic alcohol abuse, benzodiazepines and barbiturates (more commonly with short acting agents) may be associated with generalized tonic-clonic seizures

39 Acute Symptomatic Seizures Several illicit drugs produce acute symptomatic seizures, particularly cocaine and other stimulants Several therapeutic medications can trigger acute symptomatic seizures at high doses or in susceptible individuals These same medications, as well as metabolic derangements, can also trigger seizures in people with epilepsy, at lower thresholds than for people without epilepsy Metabolic and toxic causes of acute symptomatic seizures do not usually cause chronic epilepsy

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41 Provoked epilepsy The category of provoked epilepsy is differentiated from both idiopathic and symptomatic epilepsy Provoked epilepsy is defined as: An epilepsy in which a systemic or environmental factor is the predominant cause of the seizures and in which there are neuropathological or neuroanatomical changes Many patients with provoked seizures have an increased susceptibility to epilepsy (either genetic or acquired) and the term provoked epilepsy should be applied where the overwhelming influence is the provoking factor and not the underlying susceptibility Provokative factors: Fever; menstrual cycle and catamenial epilepsy; sleep; startle

42 Self assessment questions

43 The diagnosis of epilepsy can be made after : A.Two unprovoked seizures >24 hours apart B.Two unprovoked seizures <24 hours apart C.One unprovoked seizure if there is a coexisting brain lesion D.One provoked seizure and a second unprovoked seizure

44 Thank you

45 References 1. Neurology in clinical practice, Bradley New concepts in classification of the epilepsies: Entering the 21st century 3. Seizures and Epilepsy: Pathophysiology and Principles of Diagnosis 4. The causes of epilepsy 5. Harrison 2015