Epileptiform disorders Classification.pptx

Seizures convulsion and their classification

1. EPILEPSY & SEIZURE Classification Part 1 Mentor : Presentor : Dr.Rahul Gaikwad Sir Dr.karuna Tayade. DR. AKSHAY MOHNANI SIR

2. Introduction Seizure is a transient occurrence of signs or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Epilepsy describes a condition in which a person has a risk of recurrent seizures due to a chronic , underlying process. Using the definition of Epilepsy as two to more unprovoked seizures

3. Difference b/w Seizure & Epilepsy

4. Classification of Seizures The international league against Epilepsy (ILAE) Commission on Classification and Terminology uptades their uproach to Classification of seizures in 2017.

5. Focal seizures are often associated with structural abnormalities of the brain . In contrast , Generalised seizures may result from cellular , biochemical , or structural abnormalities that have a more widespread distribution

6. Focal Onset Seizures Focal seizures arise from neuronal network that is localised within one brain region or more broadly distributed but within a cerebral hemisphere. Focal Seizures with intact Awareness : Focal seizures can have motor manifestation ( such as tonic, Clonic, myoclonic movements) or non motor manifestations ( such as sensory, autonomic or emotional symptoms) without impairment of awareness. EEG recorded with scalp electrodes during the seizure( ie an ictal EEG) may show abnormal discharged in a limited region.. Focal Seizures may manifest as change in somatic sensation (eg , paresthesias ) C hange in vision (flashing lights or formed hallucinations) Equilibrium (sensation of falling or vertigo) A utonomic function (flushing , sweating , piloerection)

7. Focal Onset seizures Focal Seizures with impaired Awareness: The patient is unable to respond appropriately to visual or verbal commands during the seizure and has impaired recollection or Awareness of the ictal phase. The seizure frequently begin with an aura ( ie a focal seizures without cognitive disturbance) The start of the ictal phase is often a motionless state . The impaired awareness is usually accompanied by automatisms . Automatisms may consist of basic behaviour such as : Chewing. Lip smacking. Picking movements of hands Patient is confused following the seizure, & the transition to full recovery of consciousness may range from seconds to hour.

8. FOCAL EPILEPSY: Focal epilepsy ➡️ Features M edial temporal lobe epilepsy ➡️automatisms, epigastric aura, amnesia. Frontal lobe epilepsy ➡️ hypermotor seizures , nocturnal seizures. Occipital lobe epilepsy ➡️ visual aura. Occpito- parieto- temporal lobe epilepsy ➡️ complex aura. Focal onset seizure

9. Primary Generalised Epilepsy Also known as Idiopathic generalised epilepsy.. Most common generalised epilepsy syndromes. Most common generalised epilepsy in childhood. Normal development/ normal neurological examination/ normal imaging. Provocating Factors: Sleep deprivation. Alcohol P hoto sensitivity. Worsens along time of awakening. EEG: Normal background. Photoparoxysmal response. Generalised spike wave discharge.

10. Primary Generalised Epilepsy Types: Myoclonic epilepsy of infancy ( 6 month to 3 yrs) Epilepsy with myoclonic absense( 1- 12 years) Childhood absence ( 4- 10 years) Juvenile absence epilepsy ( 9 – 13years ) Juvenile myoclonic epilepsy( 6-25 yrs) Epilepsy with generalised tonic clonic seizures( 6-47yrs )

11. Generalised Tonic Clonic Seizures The seizure usually begins abruptly without warning , although some patients describe vague symptoms The initial phase of the seizure is usually is usually tonic contraction of muscles throughout the body. Tonic contraction of the muscles expiration and larynx will produce a load moan or “ ictal cry” Respiration are impaired , secretion pool in the oropharynx and Cyanosis develops. A marked enhancement of sympathetic tone leads to increase in heart rate, blood pressure, pupillary size. After 10 – 20 sec; the tonic phase of seizure evolves into the Clonic phase produced by the superimposition of periods of muscle relaxation on the tonic muscle contraction. The period of relaxation progressively increase but lasts not more than 1 min The post ictal phase is characterised by unresponsivness, muscular facility and excessive salivation that can cause stridorous breathing .

12. Epilepsy with Generalised Tonic Clonic Seizures Occurs in 6 to 47 yrs ( peak 16 to 20 yrs). Bladder or bowel incontinence may occur in post ictal phase. EEG: During the tonic Clonic phase of seizure shows a progressive increased in generalised low voltage fast activity , followed by generalised high amplitude, polyspike discharged. In the Clonic phasev, the high amplitude activity is interrupted by slow wave to create a spike and slow wave pattern. Brief < 3sec .but burst of 2.5 to 4 Hz spike / wave .

13. Juvenile Myoclonic Epilepsy JME is a generalised seizure disorder of unknown cause that appears in early adolescence and is usually characterised by bilateral myoclonus jerks that may be single or repetitive.. The myoclonis seizures are most frequent in the morning after awakening and can br Provoked by sleep deprivation. Consciousness is preserved .

14. Juvenile Myoclonic Epilepsy Occurs in 6 to 28 yrs old ( peak 6 to 25 yrs). Myoclonus jerk on awakening Photosensitivity is common. EEG : Polyspike occurring in descending limb without waves. 3 to 6 Hz , 1 to 4 sec. Frontal dominant, shifting asymmetry can occur. Typical 3 Hz spike and wave can seen. Treatment : Valproate Lamotrigine. Phenobarbitone. Isolated Myoclonus : Clonazepam

15. Myoclonic Epilepsy of Infancy Earliest Idiopathic Generalised Epilepsy ( 6 months – 3 yrs) Present with myoclonus on awakening: Yell because of diaphragm contraction. Consciousness is usually preserved. Multiple episodes happening per day. Provoked by auditors or tactile has better prognosis. Remission: 6 month to 5 yrs of onset. Treatment : Valproate (DOC) , Clonazepam , levetiracetam. DOC in reproductive age group : levetiracetam. Alternative drug in reproductive age group : Lamotrigine.

16. Epilepsy with myoclonic absence Onset: 1 to 12 yrs ( peak 7 yrs) Myoclonic absence are the defining symptoms. Eyelid twitching is absent. Personal myoclonus are frequent. May have intellectual disability before seizure onset. Some may show decline over time. Treatment : Resistance to monotherapy - 1. Valproate. 2 . Ethosuximide. 3. Valproate/ Ethosuximide+ lamotrigine .

17. Childhood Absence Epilepsy Also known as pyknolepsy. Occurs in 4 to 10 years of age ( peak 6 to 7 yrs) Girls > boys. Typical Absence: 4 to 20 sec. Severe impairment of consciousness. Multiple times per day.(> 10per day) Abrupt onset and sudden stoppage. Will not be associated with eyelid myoclonia / perioral myoclonia. PPR / sensory activation never occurs. EEG : 3 Hz discharge .

18. 2. Atypical Absence: Myoclonic jerks, perioral myoclonia. GTCS before or during state of absence. Mild or no impairment of consciousness. EEG : 3 to 4Hz paroxysms of <4<= or >3 spikes. PPR is not a feature. TREATMENT : Valproate. Lamotrigine : 50 to 60% used in reproductive age group. Combination of Valproate+ Lamotrigine. PROGNOSIS : Usually stop by 12 yrs of age , taper over 2 to 3 yrs

19. Juvenile Absence Epilepsy Occurs in 7 to 16 yrs old ( peak 10 to 12 years). Typical Absence in all cases. Less frequent and last longer. GTCS in 80 % cases. Myoclonus can occur in around < 20 % cases. Absence status is common in the childhood absence epilepsy. Mild Myocloniv elements of the eyelids are common during the absence. EEG : 3.5 to 4 Hz , last >4 sec., PPR never occurs. TREATMENT: Valproate. Lamotrigine. Combination of Valproate & Levetiracetam. PROGNOSIS: Life long treatment required.

20. Mesial Temporal Lobe Epilepsy Syndrome

21. Dravet Syndrome It has a trial: “ Febrile MAC” Early infantile febrile Seizures(<1 year) Myoclonic jerks, Atypical absence , Partial seizures. Cognitive & Neurological deficit ( 2- 6 th year) Boys>> Girls Atypical < 1 yr, onset ; Clonic component is predominant > 15min lasting. PRESESSIMIC PERIOD- Febrile Seizures. Early onset < 1 yr. EEG – May be Normal. Prolonged > 15min . Unilateral. Mainly Clonic. Associated with non Febrile Seizures.

22. DRAVET SYNDROME 2. SEISMIC PERIOD- Myclonus , atypical absence & progressive Neuropsychiatric issues. Aggressive seizures. PPR may be seen. 3. POST SEISMIC PERIOD – Seizures decreases by 11- 12 yrs . Myoclonus & absence decreases. Neurological deficit persist. TREATMENT : Myoclonus – Valproate/ clonazepam/ leviteracetam . Atypical absence- Ethiosuximide. Stipentol + BZD + Valproate in Refractory cases. Ketogenic Diet.

23. LANDAU KLEFFNER SYNDROME The focus of epilepsy is present in dominant temporal area. Functional ablation of cortex . They present with language abnormalities. 2 – 8 yrs onset. Auditory Agnosia – complete word deafness. Remit by 15 yrs. Neuropsychiatric abnormalities like ADHD, Aggression and behaviour problems. TREATMENT- 1 st line - Valproate Absence – Ethosuximide. If no response after 3 or 4 months then go with 2 nd line therapy. 2 nd line – Oral / high dose Steroid for 3 months. Behavioural - Amphetamine . Phenytoin Phenobarbitone are contraindicated as they worsen the Neuropsychiatric component.

24. Kozhevnikov Rasmussen Syndrome Idiopathic. Onset : 1 to 14 yrs. Severe acquired sub acute Encephalopathy. It starts with focal 3 months later develop hemiplegia / hemiparesis may improve later. ON MRI : T2 hyper density or oedema can be seen in initial phases. Hemi spherical atrophy is seen in later stages. Progressive hemi – atrophy. Intractable focal seizures / EPS ( 60 %) Progressive neurocognitive deterioration.

25. WEST Syndrome Triad : infantile spams, arrest of psychomotor development, Hypsarrhythmia.2 1. INFANTILE SPASM – Classically seen in west syndrome. Clusters of 1- 30% day, each with 20 – 150 attacks. Flexor- extensor> flexor >extensor. Treatable causes of infantile spasm: Theophylline. Hi receptor antagonist : ketotifen. Pyridoxine deficiency. 2. ARREST OF PSYCHOMOTOR DEVELOPMENT. HYPSARRHYTHMIA : Disorganised , giant , slow waves . Multifocal spikes. Onset – 3 – 12 months .

26. Lennox Gastaut Syndrome TRIAD : Mental retardation or regression. Intractable Epilepsy. Multiple seizure type. FEATURES( Mnemonic: TADAPS) Tonic seizure.( 80 %) Atonic seizure. Drop attacks . Atypical absence Seizure. EEG : Paroxysmal fast activity & Slow Spike wave . Myoclonus can occur but never the prominent seizure type.

27. Status epilepticus Status epilepticus refers to continuous seizures or repetitive , discrete seizures with impaired consciousness in the interictal period.. It has numerous subtypes, including Generalised Convulsive status epilepticus( (GCSE) and non convulsive status epilepticus . The duration of seizure activity is around 25 – 20 min GCSE is an emergency because cardiorespiratoty dysfunction, hyperthermia and metabolic dearangenents can lead to irreversible neuronal injury. The most common causes of GCSE are anticonvulsant withdrawal or non Compliance, metabolic disturbances , drug toxicity , cns infection , cnc tumors , refractory epilepsy and head trauma. After 30 – 45 min of uninterrupted seizures, the signs may become increasingly subtle. Patients may havemild Clonic movement s of only fingers , rapid movement of eyes

28. STATUS EPILEPTICUS REFRACTORY STATUS EPILEPTICUS: Not controlled by BZD and 2 nd line drugs. 12 – 43 % cases progress to refractory status. SUPER REFRACTORY STATUS EPILEPTICUS: Continues for > 24 hours or recur even after anaesthetics. 10 – 15 % progress to super refractory status.

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