Epilepsy

Changed by Rohit Sharma, 23 May 2022

Updates to Article Attributes

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Epilepsy is a common neurological disorder that hasis characterised by a varied presentation and requirespredisposition to having epileptic seizures.

Epilepsy is defined by the International League Against Epilepsy (ILAE) as 1:

  • at least two or more unprovoked (or reflex) seizures at leastoccurring more than 24 hours apart for diagnosis. MRI is; or
  • one unprovoked (or reflex) seizure and a probability of further seizures similar to the; or general recurrence risk after two unprovoked seizures, occurring over the modalitynext 10 years
  • diagnosis of choice foran epilepsy, most often investigating for an underlying cause, especially in adults.  syndrome

Epidemiology

Epilepsy is very common, with approximately 3% of the population affected at some point in their life 1,52.

Clinical presentation

The hallmark clinical feature of epilepsy is that of epileptic seizures, which may have a variety of semiologies depending on the type of seizure and epilepsy. However, there are other important clinical features which may accompany epileptic seizures 3:

  • psychiatric co-morbidity: e.g. anxiety, depression
  • physical effects/complications of epileptic seizures, e.g. injury, death, neurocognitive deterioration
  • social effects/complications: e.g. lower education levels, lower employment rates with fewer options, limitations on recreational activities, stigma
  • medication side-effects, e.g. neurocognitive deterioration, weight gain, rash, osteoporosis

Pathology

Epilepsy syndromes can be generally divided into two broad categoriesis classified by the International League Against Epilepsy (ILAE) according to a three-level framework 1,54:

  • seizure type 5
    • focal (preferred term to 'partial') onset
      • may have awareness (preferred term to 'simple partial') or impaired awareness (preferred term to 'complex partial' or 'dyscognitive')
      • may be motor onset or non-motor onset (e.g. sensory, autonomic)
      • may progress to focal to bilateral tonic-clonic seizures
    • generalised
      • may be motor seizures, e.g. absence epilepsy, tonic-clonic (preferred term to 'grand mal') seizure, myoclonic clonic seizure, tonic seizure, myoclonic-tonic-clonic seizure, myoclonic-atonic seizure, atonic seizure
      • focal, can progressmay be non-motor or absence (preferred term to 'petit mal') seizure
    • unknown
  • epilepsy type
    • focal
    • generalised
    • combined generalised (focal-to-bilateral)and focal
    • unknown
  • epilepsy syndrome
Aetiology

There is a wide range of causes, however, in adults with new onset of seizures ~50% will not have a determinable cause 1-52. The International League Against Epilepsy (ILAE) have proposed the following classification 64:

Radiographic features

MRI

MRI brain is the radiographic modality of choice2. Please see articles on specific conditions listed above for imaging features. There are a number of MRI protocols that can be used to investigate patients with seizures. 

Nuclear medicine
SPECT perfusion brain

If performed during the crisisan epileptic seizure, it can be useful for localising the epileptogenic focus 6,7,8.

Treatment and prognosis

Management of epilepsy is potentially complex, and may involve 2,3:

  • lifestyle restrictions, e.g. driving restrictions, restrictions on employment, limiting exposure to drugs and alcohol
  • antiseizure medications (antiepileptic drugs), either as monotherapy or in combination, and ketogenic diet
  • epilepsy surgery, e.g. stereo EEG, surgical resection, neuromodulation therapies
  • specific management of psychosocial effects/complications
  • -<p><strong>Epilepsy</strong> is a common neurological disorder that has a varied presentation and requires two or more unprovoked seizures at least 24 hours apart for diagnosis. MRI is the modality of choice for epilepsy, most often investigating for an underlying cause, especially in adults. </p><h4>Epidemiology</h4><p>Epilepsy is very common, with approximately 3% of the population affected at some point in their life <sup>1,5</sup>. </p><h4>Pathology</h4><p>Epilepsy syndromes can be generally divided into two broad categories <sup>1,5</sup>:</p><ul>
  • -<li>generalised, e.g. absence epilepsy, tonic-clonic, myoclonic</li>
  • -<li>focal, can progress to generalised (focal-to-bilateral)<ul><li>e.g. <a href="/articles/temporal-lobe-epilepsy">temporal lobe epilepsy</a>
  • -</li></ul>
  • +<p><strong>Epilepsy</strong> is a common neurological disorder that is characterised by a predisposition to having epileptic seizures.</p><p>Epilepsy is defined by the International League Against Epilepsy (ILAE) as <sup>1</sup>:</p><ul>
  • +<li>at least two or more unprovoked (or reflex) seizures occurring more than 24 hours apart; or</li>
  • +<li>one unprovoked (or reflex) seizure and a probability of further seizures similar to the; or general recurrence risk after two unprovoked seizures, occurring over the next 10 years</li>
  • +<li>diagnosis of an epilepsy syndrome</li>
  • +</ul><h4>Epidemiology</h4><p>Epilepsy is very common, with approximately 3% of the population affected at some point in their life <sup>2</sup>.</p><h4>Clinical presentation</h4><p>The hallmark clinical feature of epilepsy is that of epileptic seizures, which may have a variety of semiologies depending on the type of seizure and epilepsy. However, there are other important clinical features which may accompany epileptic seizures <sup>3</sup>:</p><ul>
  • +<li>psychiatric co-morbidity: e.g. anxiety, depression</li>
  • +<li>physical effects/complications of epileptic seizures, e.g. injury, death, neurocognitive deterioration</li>
  • +<li>social effects/complications: e.g. lower education levels, lower employment rates with fewer options, limitations on recreational activities, stigma</li>
  • +<li>medication side-effects, e.g. neurocognitive deterioration, weight gain, rash, osteoporosis</li>
  • +</ul><h4>Pathology</h4><p>Epilepsy is classified by the International League Against Epilepsy (ILAE) according to a three-level framework <sup>4</sup>:</p><ul>
  • +<li>seizure type <sup>5</sup><ul>
  • +<li>focal (preferred term to 'partial') onset<ul>
  • +<li>may have awareness (preferred term to 'simple partial') or impaired awareness (preferred term to 'complex partial' or 'dyscognitive')</li>
  • +<li>may be motor onset or non-motor onset (e.g. sensory, autonomic)</li>
  • +<li>may progress to focal to bilateral tonic-clonic seizures</li>
  • +</ul>
  • +</li>
  • +<li>generalised<ul>
  • +<li>may be motor seizures, e.g. tonic-clonic (preferred term to 'grand mal') seizure, clonic seizure, tonic seizure, myoclonic-tonic-clonic seizure, myoclonic-atonic seizure, atonic seizure</li>
  • +<li>may be non-motor or absence (preferred term to 'petit mal') seizure</li>
  • +</ul>
  • +</li>
  • +<li>unknown</li>
  • +</ul>
  • -</ul><h5>Aetiology</h5><p>There is a wide range of causes, however, in adults with new onset of seizures ~50% will not have a determinable cause <sup>1-5</sup>. The International League Against Epilepsy (ILAE) have proposed the following classification <sup>6</sup>:</p><ul>
  • -<li>
  • -<strong>idiopathic</strong>: epilepsy due to an underlying single gene condition or complex inheritance (seizures as the only manifestation)</li>
  • -<li>
  • -<strong>cryptogenic</strong>: unknown cause</li>
  • -<li>
  • -<strong>provoked epilepsy</strong>: e.g. secondary to fevers, head trauma, drug-induced</li>
  • -<li>
  • -<strong>predominantly genetic/developmental causation</strong><ul>
  • -<li>childhood epilepsy syndromes</li>
  • -<li><a href="/articles/progressive-myoclonic-epilepsy">progressive myoclonic epilepsies</a></li>
  • -<li>neurocutaneous syndromes, e.g. <a href="/articles/tuberous-sclerosis">tuberous sclerosis</a>
  • +<li>epilepsy type<ul>
  • +<li>focal</li>
  • +<li>generalised</li>
  • +<li>combined generalised and focal</li>
  • +<li>unknown</li>
  • +</ul>
  • -<li>chromosomal disorders, e.g. <a href="/articles/down-syndrome">Down syndrome</a>
  • +<li>epilepsy syndrome<ul><li>e.g. <a href="/articles/dravet-syndrome-1">Dravet syndrome</a>, <a href="/articles/lennox-gastaut-syndrome-1">Lennox-Gastaut syndrome</a>, <a href="/articles/idiopathic-generalised-epilepsy">idiopathic (genetic) generalised epilepsy</a>
  • +</li></ul>
  • -<li>single gene disorders</li>
  • -<li>developmental cortical abnormalities, e.g. <a href="/articles/focal-cortical-dysplasia">focal cortical dysplasia</a>, <a href="/articles/grey-matter-heterotopia">grey matter heterotopia</a>
  • +</ul><h5>Aetiology</h5><p>There is a wide range of causes, however, in adults with new onset of seizures ~50% will not have a determinable cause <sup>2</sup>. The International League Against Epilepsy (ILAE) have proposed the following classification <sup>4</sup>:</p><ul>
  • +<li>structural aetiology<ul>
  • +<li>acquired, e.g. <a href="/articles/stroke">stroke</a>, <a href="/articles/cerebral-palsy">cerebral palsy</a>, post-<a href="/articles/leptomeningitis">meningitis</a>, <a href="/articles/traumatic-brain-injury">traumatic brain injury</a>, <a href="/articles/cerebrovascular-malformations">vascular malformations</a>, <a href="/articles/mesial-temporal-sclerosis">mesial temporal sclerosis</a>, <a href="/articles/meningoencephalocoeles">meningoencephaloceles</a>, tumours (e.g. <a href="/articles/ganglioglioma">gangliogliomas</a>, <a href="/articles/dysembryoplastic-neuroepithelial-tumour">DNET</a>, <a href="/articles/pleomorphic-xanthoastrocytoma">PXA</a>, low-grade gliomas)</li>
  • +<li>genetic, e.g. <a href="/articles/focal-cortical-dysplasia">focal cortical dysplasia</a>, <a href="/articles/grey-matter-heterotopia">grey matter heterotopia</a>
  • -<li>
  • -<strong>predominantly acquired</strong><ul>
  • -<li>
  • -<a href="/articles/stroke">stroke</a>, <a href="/articles/cerebral-palsy">cerebral palsy</a>, bacteria/viral <a href="/articles/leptomeningitis">meningitis</a>, <a href="/articles/traumatic-brain-injury">traumatic brain injury</a>, <a href="/articles/cerebrovascular-malformations">vascular malformations</a>
  • +<li>genetic aetiology<ul><li>e.g. channelopathies (e.g. <a href="/articles/dravet-syndrome-1">Dravet syndrome</a>), neurocutaneous syndromes (e.g. <a href="/articles/tuberous-sclerosis">tuberous sclerosis</a>)</li></ul>
  • -<li>
  • -<a href="/articles/mesial-temporal-sclerosis">mesial temporal sclerosis</a>, <a href="/articles/meningoencephalocoeles">meningoencephaloceles</a>
  • +<li>infectious aetiology<ul>
  • +<li>e.g. <a href="/articles/neurocysticercosis">neurocysticercosis</a>, <a href="/articles/tuberculosis-intracranial-manifestations">tuberculosis</a>, <a href="/articles/neurotoxoplasmosis">toxoplasmosis</a>, <a href="/articles/cerebral-malaria-2">cerebral malaria</a>, <a href="/articles/subacute-sclerosing-panencephalitis-1">subacute sclerosing panencephalitis</a>, <a href="/articles/congenital-cytomegalovirus-infection">congenital CMV</a>
  • -<li>tumours, e.g. <a href="/articles/ganglioglioma">gangliogliomas</a>, <a href="/articles/dysembryoplastic-neuroepithelial-tumour">DNET</a>, <a href="/articles/pleomorphic-xanthoastrocytoma">PXA</a>, low-grade gliomas</li>
  • +<li>not referring to seizures occurring in the setting of an acute CNS infection</li>
  • -</ul><h4>Radiographic features</h4><h5>MRI</h5><p>MRI is the modality of choice. Please see articles on specific conditions listed above for imaging features. There are a number of <a href="/articles/epilepsy-protocol-mri">MRI protocols</a> that can be used to investigate patients with seizures. </p><h5>Nuclear medicine</h5><h6>SPECT perfusion brain</h6><p>If performed during the crisis, it can be useful for localising the epileptogenic focus <sup>7,8</sup>.</p>
  • +<li>metabolic aetiology<ul><li>e.g. <a href="/articles/porphyria-1">porphyria</a>
  • +</li></ul>
  • +</li>
  • +<li>immune aetiology<ul><li>e.g. <a href="/articles/autoimmune-encephalitis">autoimmune encephalitis</a>
  • +</li></ul>
  • +</li>
  • +<li>unknown aetiology</li>
  • +</ul><h4>
  • +<strong>​</strong>Radiographic features</h4><h5>MRI</h5><p>MRI brain is the radiographic modality of choice <sup>2</sup>. Please see articles on specific conditions listed above for imaging features. There are a number of <a href="/articles/epilepsy-protocol-mri">MRI protocols</a> that can be used to investigate patients with seizures. </p><h5>Nuclear medicine</h5><h6>SPECT perfusion brain</h6><p>If performed during an epileptic seizure, it can be useful for localising the epileptogenic focus <sup>6,</sup><sup>7</sup>.</p><h4>Treatment and prognosis</h4><p>Management of epilepsy is potentially complex, and may involve <sup>2,3</sup>:</p><ul>
  • +<li>lifestyle restrictions, e.g. driving restrictions, restrictions on employment, limiting exposure to drugs and alcohol</li>
  • +<li>antiseizure medications (antiepileptic drugs), either as monotherapy or in combination, and ketogenic diet</li>
  • +<li>epilepsy surgery, e.g. stereo EEG, surgical resection, neuromodulation therapies</li>
  • +<li>specific management of psychosocial effects/complications</li>
  • +</ul>

References changed:

  • 1. Fisher R, Acevedo C, Arzimanoglou A et al. ILAE Official Report: A Practical Clinical Definition of Epilepsy. Epilepsia. 2014;55(4):475-82. <a href="https://doi.org/10.1111/epi.12550">doi:10.1111/epi.12550</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/24730690">Pubmed</a>
  • 2. Chang BS, Lowenstein DH. Epilepsy. N. Engl. J. Med. 2003;349 (13): 1257-66. <a href="http://dx.doi.org/10.1056/NEJMra022308">doi:10.1056/NEJMra022308</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/14507951">Pubmed citation</a><span class="auto"></span>
  • 3. Lin J, Mula M, Hermann B. Uncovering the Neurobehavioural Comorbidities of Epilepsy over the Lifespan. Lancet. 2012;380(9848):1180-92. <a href="https://doi.org/10.1016/S0140-6736(12)61455-X">doi:10.1016/S0140-6736(12)61455-X</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23021287">Pubmed</a>
  • 4. Scheffer I, Berkovic S, Capovilla G et al. ILAE Classification of the Epilepsies: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017;58(4):512-21. <a href="https://doi.org/10.1111/epi.13709">doi:10.1111/epi.13709</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28276062">Pubmed</a>
  • 5. Fisher R, Cross J, French J et al. Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017;58(4):522-30. <a href="https://doi.org/10.1111/epi.13670">doi:10.1111/epi.13670</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28276060">Pubmed</a>
  • 6. von Oertzen TJ. PET and ictal SPECT can be helpful for localizing epileptic foci. (2018) Current opinion in neurology. 31 (2): 184-191. <a href="https://doi.org/10.1097/WCO.0000000000000527">doi:10.1097/WCO.0000000000000527</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29303866">Pubmed</a> <span class="ref_v4"></span>
  • 7. Catafau AM. Brain SPECT in clinical practice. Part I: perfusion. (2001) Journal of nuclear medicine : official publication, Society of Nuclear Medicine. 42 (2): 259-71. <a href="https://www.ncbi.nlm.nih.gov/pubmed/11216525">Pubmed</a> <span class="ref_v4"></span>
  • 1. Bronen RA. Epilepsy: the role of MR imaging. AJR Am J Roentgenol. 1992;159 (6): 1165-74. <a href="http://dx.doi.org/10.2214/ajr.159.6.1442376">doi:10.2214/ajr.159.6.1442376</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/1442376">Pubmed citation</a><span class="auto"></span>
  • 2. Cook M, Lhatoo S. Oxford Textbook of Epilepsy and Epileptic Seizures (Oxford Textbooks in Clinical Neurology). OUP Oxford. ISBN:B009ZAGIDO. <a href="http://books.google.com/books?vid=ISBNB009ZAGIDO">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/B009ZAGIDO">Find it at Amazon</a><span class="auto"></span>
  • 3. Bronen RA. Epilepsy: the role of MR imaging. AJR Am J Roentgenol. 1992;159 (6): 1165-74. <a href="http://dx.doi.org/10.2214/ajr.159.6.1442376">doi:10.2214/ajr.159.6.1442376</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/1442376">Pubmed citation</a><span class="auto"></span>
  • 4. Friedman E. Epilepsy imaging in adults: getting it right. AJR Am J Roentgenol. 2014;203 (5): 1093-103. <a href="http://dx.doi.org/10.2214/AJR.13.12035">doi:10.2214/AJR.13.12035</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/25341150">Pubmed citation</a><span class="auto"></span>
  • 5. Chang BS, Lowenstein DH. Epilepsy. N. Engl. J. Med. 2003;349 (13): 1257-66. <a href="http://dx.doi.org/10.1056/NEJMra022308">doi:10.1056/NEJMra022308</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/14507951">Pubmed citation</a><span class="auto"></span>
  • 6. Shorvon SD. The etiologic classification of epilepsy. Epilepsia. 2011;52 (6): 1052-7. <a href="http://dx.doi.org/10.1111/j.1528-1167.2011.03041.x">doi:10.1111/j.1528-1167.2011.03041.x</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/21449936">Pubmed citation</a><span class="auto"></span>
  • 7. von Oertzen TJ. PET and ictal SPECT can be helpful for localizing epileptic foci. (2018) Current opinion in neurology. 31 (2): 184-191. <a href="https://doi.org/10.1097/WCO.0000000000000527">doi:10.1097/WCO.0000000000000527</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29303866">Pubmed</a> <span class="ref_v4"></span>
  • 8. Catafau AM. Brain SPECT in clinical practice. Part I: perfusion. (2001) Journal of nuclear medicine : official publication, Society of Nuclear Medicine. 42 (2): 259-71. <a href="https://www.ncbi.nlm.nih.gov/pubmed/11216525">Pubmed</a> <span class="ref_v4"></span>

Systems changed:

  • Paediatrics

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