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  1. 1. Epilepsy Samantha Harrison MBBS 3
  2. 2. What is Epilepsy? • Etymology: From the greek word Epilepsia. Epi meaning upon and Lepsis meaning seizure. In ancient times epilepsy was known as the sacred disease. • Definition: • Epilepsy is a disorder of the brain characterized by an enduring predisposition to epileptic seizures [Fisher et al, 2005] • WHO: Two or more unprovoked seizures. A chronic noncommunicable disorder of the brain that affects people of all ages. • Epilepsy is a medical condition that produces seizures affecting a variety of mental and physical functions. It is also called a seizure disorder. When a person has two or more unprovoked seizures, they are considered to have epilepsy (Epilepsy Foundation)
  3. 3. What is a seizure? • Etymology: Seiz + ure meaning sudden attack of illness • Definition • An epileptic seizure is the transient occurrence of signs or symptoms due to abnormal electrical activity in the brain [Fisher et al, 2005]. This manifests itself as a disturbance of consciousness, behaviour, emotion, motor function, or sensation. • Seizures happen when clusters of nerve cells in the brain signal abnormally, which may briefly alter a person's consciousness, movements or actions.
  4. 4. Epilepsy Epidemiology • 30 million people worldwide are epilepsy sufferers (WHO) • Prevalence: 4-6 people per 1000; 0.5% • Epilepsy has been estimated to affect between 260,000 and 416,000 people in England and Wales. • Incidence: 40-70 new cases per 100000 annually; 0.05% . Twice as high in developing countries. • 80% of the world’s epilepsy is found in the developing countries • Why?
  5. 5. Who is at risk of Epilepsy? • Neonatal Period • hypoxic ischemic encephalopathy • central nervous system (CNS) infections • trauma • congenital CNS abnormalities • Metabolic diseases • Late infancy and Early childhood • febrile seizures (fairly common), caused by CNS infections and trauma. • Childhood • well-defined epilepsy syndromes • Adolescence and adulthood • CNS lesion • Idiopathic epilepsy (less comon)stress, trauma, CNS infections, brain tumors, illicit drug use and alcohol withdrawal. • Older adult • cerebrovascular disease (very common cause). • CNS tumors • head trauma, • degenerative diseases e.g. dementia.[15]
  6. 6. Epilepsy Classification • Epilepsy is a symptom – not a diagnosis • Epilepsies are classified in five ways: • By their first cause (or etiology). • By the observable manifestations of the seizures, known as semiology. • By the location in the brain where the seizures originate. • As a part of discrete, identifiable medical syndromes. • By the event that triggers the seizures, such as reading or music • Or more simply • Symptomatic – definite lesion as the cause • Idiopathic – what it says on the tin • Cryptogenic – not idiopathic but no definite origin, high prevalence of associated learning disabilities
  7. 7. Seizure Types - Definitions • Simple – no impairment of consciousness • Complex – impairment of consciousness • Generalised (primary and secondary): abnormal electrical activity is over the entire cortex immediately (primary) or spreads over the entire cortex from a single point (secondary). • Focal/Partial – Abnormal electrical activity is restricted to a region of the brain
  8. 8. Seizure Types (cont.) • Absence – seizures characterised by an abrupt, transient loss or impairment of consciousness (which is not subsequently remembered) • Tonic-clonic – type of generalised seizure that involves a tonic – stiffening phase and a clonic – jerking phase • Atonic -seizures characterised by abrupt brief loss of muscle tone. AKA drop seizures • Jacksonian March – type of simple partial seizure, characterised by jerking. Spreads from arm > face > leg (ipsilaterally) • Tonic seizures characterised by a stiffening of all the muscles • Febrile seizures characterised by convulsions brought on by a significant rise in body temperature.
  9. 9. Seizure Types
  10. 10. • Typical Seizure • Any precipitating Factors
  11. 11. Epilepsy Syndromes • Epilepsy syndromes are a list of diseases that cause epilepsy • Epilepsy syndrome as a term – means a syndrome where recurrent seizures are the predominant feature • 29 Epilepsy syndromes (ILAE)
  12. 12. InternationalClassificationof Epilepsiesand EpilepticSyndromes • 1. Localization-related (focal, local, partial) epilepsies and syndromes • 1.1. Idiopathic with age-related onset • A. Benign childhood epilepsy with centrotemporal spikes • B. Childhood epilepsy with occipital paroxysms • 1.2. Symptomatic • A. Chronic progressive epilepsia partialis continua of childhood • B. Syndromes characterized by seizures with specific modes of precipitation • C. Temporal lobe epilepsies • D. Frontal lobe epilepsies • E. Parietal lobe epilepsies • F. Occipital lobe epilepsies • 1.3 Crytopgenic
  13. 13. InternationalClassificationof Epilepsiesand EpilepticSyndromes • 2. Generalized epilepsies and syndromes • 2.1. Idiopathic, with age-related onset (listed in order of age) • A. Benign neonatal familial convulsions • B. Benign neonatal convulsions • C. Benign myoclonic epilepsy in infancy • D. Childhood absence epilepsy (pyknolepsy) • E. Juvenile absence epilepsy • F. Juvenile myoclonic epilepsy (impulsive petit mal) • G. Epilepsy with grand mal seizures on awakening • H. Other generalized idiopathic epilepsies not defined above • I. Epilepsies with seizures precipitated by specific modes of activation2.2. Idiopathic and/or symptomatic (listed in order of age) • A. West syndrome (infantile spasms) • B. Lennox-Gastaut syndrome • C. Epilepsy with myoclonic-astatic seizures • D. Epilepsy with myoclonic absences • 2.3. Symptomatic • A. Nonspecific etiology • a. Early myoclonic encephalopathy • b. Early infantile epileptic encephalopathy with suppression burst • c. Other symptomatic generalized epilepsies not defined above • B. Specific etiology • a. Epileptic seizures may complicate many disease states
  14. 14. InternationalClassificationof Epilepsiesand EpilepticSyndromes • 3. Epilepsies and syndromes undetermined as to whether they are focal or generalized • 3.1. With both generalized and focal seizures • A. Neonatal seizures • B. Severe myoclonic epilepsy in infancy • C. Epilepsy with continuous spike waves during slow-wave sleep • D. Acquired epileptic aphasia (Landau-Kleffner syndrome) • E. Other undetermined epilepsies not defined above • 3.2. Without unequivocal generalized or focal features • 4. Special syndromes • 4.1. Situation-related seizures • A. Febrile convulsions • B. Isolated, apparently unprovoked epileptic events • C. Seizures related to other identifiable situations such as stress, hormonal changes, drugs, alcohol, or sleep deprivation
  15. 15. Reflex epilepsy • Epilepsy always triggered by a specific stimulus • Music • Photosensitive epilepsy • Reading
  16. 16. Precipitating factors • Emotional stress • Sleep deprivation • Sleep itself • Heat stress • Alcohol • Febrile illness • Menstrual Cycle. • Catamenial epilepsy is the term denoting seizures linked to the menstrual cycle
  17. 17. What happens during the Ictal phase? • Dependent on the seizure • Faecal/urinary incontinence • Tongue biting – lateral vs anterior • Observer – collateral history – jerking • Loss of consciousness
  18. 18. What happens post-ictally? • Confusion • Extreme fatigue • Memory Loss • Drowsiness • Nausea • Headache/migraine • psychosis • Poor attention and concentration • Depression • Todd’s paresis • Post ictal bliss
  19. 19. Temporal Lobe Epilepsy • Automatisms common • Lip smacking • Fiddling with hands • Secondary generalised seizures • MRI shows hippocampal sclerosis
  20. 20. Status Epilepticus • Definition: Epileptic seizure that lasts for over 30 minutes • Can be life threatening
  21. 21. Psychogenic Seizures • More common in women • More common in people with personality disorders • Commonly a history of physical or sexual abuse as a child • Can also suffer epileptic attacks as well as psychogenic non- epileptic psychogenic attacks
  22. 22. Pathophysiology of Epilepsy • PET Scanning has shown that areas of the brain that induce seixures are less active than other areas of the brain between seizures (is the brain suppressing the overactive region?) • PET scanning also revealed abnormal receptor functioning in these regions with reduced GABAa density.
  23. 23. Complications of Epilepsy • The risk of premature death in people with epilepsy is two to three times higher than it is for the general population. • Driving Restrictions – must have had no awake seizures for 6 months or only had sleep seizures for the previous three years to retain licence in a first seizure scenario. Recurrent sseizure profile revoke licence – 12 months seizure free
  24. 24. Differential Diagnoses • Psychogenic Cause • Malingering • Narcolepsy • Syncope • Other paroxysmal sleep and movement disorders
  25. 25. Taking a History of a seizure • What you want to know? • Are these really epileptic seizures? • How you are going to find out? • Ask the right questions • Did anything bring on the attack? • Before the attack was there any lightheadedness or blurring/fogging of vision? • Did anyone else comment that you had become pale? • Did this happen during or following exercise? • Did you make an abnormal noise at the start of the attack? • How did you fall? Stiff vs Floppy • Did you hurt yourself when you fell? • Tongue Biting – Lateral vs Anterior • Faecal incontinence • How long was the attack? • Were you able to recognise where you were, who you were with after the attack? If so how long did this take to resolve? • Were you lethargic for hours after the attack?
  26. 26. Investigating Epilepsy • Examination • ECG • EEG • Video of a seizure • MRI • Blood Tests: Calcium, LFTs, drug and alcohol screen
  27. 27. EEG • Electroencephalogram
  28. 28. Treatment of epilepsy • Carbamazepine – focal • Valproate – generalised • Cepra – both • Lamotrigine • Carbamazepine metabolism slowed by clarithromycin
  29. 29. Treatments • Surgery • Vagal stimulation • Deep brain stimulation • Transcranial magnetic stimulation • Ketogenic diet • Surgery to disconnect rather than remove brain areas
  30. 30. Is Epilepsy Curable? • Controllable but not curable. Sometimes surgery can be a curable treatment. • 20-40% of patients have AED resistant epilepsy