Epilepsy

1. Epilepsy Samantha Harrison MBBS 3

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. 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. 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. Who is at risk of Epilepsy? Neonatal Period hypoxic ischemic encephalopathy central nervous system (CNS) infections trauma congenital CNS abnormalities Metabolic diseases L ate infancy and Early childhood febrile seizures (fairly common), caused by CNS infections and trauma . Childhood well-defined epilepsy syndromes Adolescence and adulthood CNS lesion I diopathic epilepsy (less comon ) stress , trauma, CNS infections, brain tumors, illicit drug use and alcohol withdrawal. O lder adult cerebrovascular disease (very common cause). CNS tumors head trauma , degenerative diseases e.g. dementia .[15]

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. 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. 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. Seizure Types

10. Typical Seizure Any precipitating Factors

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. International Classification of Epilepsies and Epileptic Syndromes 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. International Classification of Epilepsies and Epileptic Syndromes 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 activation 2.2. 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. International Classification of Epilepsies and Epileptic Syndromes 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. Reflex epilepsy Epilepsy always triggered by a specific stimulus Music Photosensitive epilepsy Reading

16. Precipitating factors Emotional stress S leep deprivation S leep itself H eat stress Alcohol F ebrile illness Menstrual Cycle . Catamenial epilepsy is the term denoting seizures linked to the menstrual cycle

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. 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. Temporal Lobe Epilepsy Automatisms common Lip smacking Fiddling with hands Secondary generalised seizures MRI shows hippocampal sclerosis

20. Status Epilepticus Definition: Epileptic seizure that lasts for over 30 minutes Can be life threatening

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. 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. 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. Differential Diagnoses Psychogenic Cause Malingering Narcolepsy Syncope Other paroxysmal sleep and movement disorders

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. Investigating Epilepsy Examination ECG EEG Video of a seizure MRI Blood Tests: Calcium, LFTs, drug and alcohol screen

27. EEG Electroencephalogram

28. Treatment of epilepsy Carbamazepine – focal Valproate – generalised Cepra – both Lamotrigine Carbamazepine metabolism slowed by clarithromycin

29. Treatments Surgery Vagal stimulation Deep brain stimulation Transcranial magnetic stimulation Ketogenic diet Surgery to disconnect rather than remove brain areas

30. Is Epilepsy Curable? Controllable but not curable. Sometimes surgery can be a curable treatment. 20-40% of patients have AED resistant epilepsy