Epilepsy Epilepsy (from the Ancient Greek ( epilēpsia ): seizure) is a group of chronic neurological disorders characterized by seizures, which are the result of abnormal, excessive or hyper synchronous neuronal activity in the brain.
Epilepsy (sometimes referred to as a seizure disorder) is a common chronic neurological condition that is characterized by recurrent unprovoked epileptic seizures. It affects approximately 50 million people worldwide. It is usually controlled, but not cured.
A chronic disorder characterized by paroxysmal brain dysfunction due to excessive neuronal discharge, and usually associated with some alteration of consciousness. The clinical manifestations of the attack may vary from complex abnormalities of behavior including generalized or focal convulsions to momentary spells of impaired consciousness.
It affects between 2% and 3% of the population , ¾ before adolescence . It can be caused by genetic , structural, metabolic or unknown factors. Among the structural factors, the most common causes in developing countries are infectious and parasitic diseases, perinatal brain damage, vascular disease, and head trauma. The prognosis of epilepsy depends on the etiology of the illness as well as on early and sustained treatment. It is estimated that up to 70% of people with epilepsy can live normal lives if they receive proper care.
The cause of an individual's epilepsy can be divided into two categories: symptomatic and idiopathic. Symptomatic epilepsies originate due to some structural or metabolic abnormality in the brain. The term idiopathic means "a disorder unto itself", and not "cause unknown". No other condition has been implicated as the cause of the epilepsy. Idiopathic epilepsies are often but not exclusively genetic and generalized – for example Juvenile Absence Epilepsy.
Certain circumstances can lead to an increased likelihood of seizures in someone with epilepsy or in certain syndromes. For example: During sleep The transition between sleep and awake Tiredness Illness Constipation Menstruation Stress
They are variously based on: 1. The clinical manifestations of the seizure (motor, sensory, reflex or psychic) 2. The pathological substrate (hereditary, inflammatory, degenerative, neoplastic or traumatic) 3. The location of the epileptogenic lesion ( rolandic , temporal, diencephalic regions), 4. The time of life at which the attacks occur (nocturnal, diurnal, menstrual, etc.)
Seizure types are organized firstly according to whether the source of the seizure within the brain is localized (partial or focal onset seizures) or distributed (generalized seizures). Partial seizures are further divided on the extent to which consciousness is affected. If it is unaffected, then it is a simple partial seizure; otherwise it is a complex partial seizure.
Status epilepticus (SE) Continuous convulsion lasting longer than 30 minutes OR occurrence of serial convulsions between which there is no return of consciousness Idiopathic SE Seizure develops in the absence of an underlying CNS lesion/insult Symptomatic SE Seizure occurs as a result of an underlying neurological disorder or a metabolic abnormality
Seizures Partial Electrical discharges in a relatively small group of dysfunctional neurones in one cerebral hemisphere Aura may reflect site of origin + / - LOC Generalized Diffuse abnormal electrical discharges from both hemi s pheres Symmetrically involved No warning Always LOC
Simple Complex Partial Seizures 1. w/ motor signs 2. w/ somato-sensory symptoms 3. w/ autonomic symptoms 4. w/ psychic symptoms 1. simple partial --> loss of consciousness 2. w/ loss of consciousness at onset Secondary generalized 1. simple partial --> generalized 2. complex partial --> generalized 3. simple partial --> complex partial --> generalized
Simple partial In simple partial seizures, consciousness is not impaired. Patients can present with motor, somatosensory , special sensory, autonomic or psychic symptoms Complex partial A complex partial seizure describes a seizure where consciousness is impaired. A partial seizure may begin with a simple seizure, conversely, its onset may coincide with the impairment of consciousness. It may be presented with or without an aura. 2 nd ary generalized Partial sezure evolve to secondarily generalized seizures . May be gen. Tonic- clonis , tonico or clonic
Simple partial seizures with autonomic symptoms Stiffness in L cheek Difficulty in articulating R side of mouth is dry Salivating on the L side Progresses to tongue and back of throat
Simple partial seizure with pyschic symptoms Dysmnesic symptoms “déjà-vu” Affective symptoms fear and panic Cognitive Structured hallucination living through a scene of her former life again
Complex Partial Seizures Simple partial onset followed by impaired consciousness with or without automatism With impairment of consciousness at onset with impairment of consciousness only with automatisms
Simple Partial Seizures followed by Complex Partial Seizures Seizure starts from awake state Impairment of consciousness Automatisms lip-smacking right leg
Complex Partial Seizures with impairment of consciousness at onset Suddenly sit up Roll about with vehement movement
Partial Seizures evolving to Secondarily Generalized Seizures Simple Partial Seizures to Generalized Seizures Complex Partial Seizures to Generalized Seizures Simple Partial Seizures to Complex Partial Seizures to Generalized Seizures
Simple Partial Seizures to Generalized Seizures Turns to his R with upper body and bends his L arm Stretches body Tonic- clonic seizure Relaxation phase Postictal sleep
Simple Partial Seizures to Complex Partial Seizures to Generalized Seizures Initially unable to communicate but understands Automatism Smacking Hand-rubbing Abolished communication Generalized tonic- clonic seizure
Absence seizures Sudden onset Interruption of ongoing activities Blank stare Brief upward rotation of eyes Duration: a few seconds to 1/2 minute Evaporates as rapidly as it started
Absence seizures Stops hyperventilating Mild eyelid clonus Slight loss of neck muscle tone Oral automatisms
Myoclonic seizures Sudden, brief, shock-like Predominantly around the hours of going to or awakening from sleep May be exacerbated by volitional movement (action myoclonus)
Myoclonic seizures Symmetrical myoclonic jerks
Clonic seizures Repetitive biphasic jerky movements Repetitive vocalisation synchronous with clonic movements of the chest (mechanical) Passes urine
Tonic seizures Rigid violent muscle contraction Limbs are fixed in strained position patient stands in one place bends forward with abducted arms deep red face noises - pressing air through a closed mouth
Tonic seizures Elevates both hands Extreme forward bending posture Keeps walking without falling Passes urine
Tonic-clonic seizures (grand mal) Tonic Phase Sudden sharp tonic contraction of respiratory muscle: stridor / moan Falls Respiratory inhibition cyanosis Tongue biting Urinary incontinence Clonic Phase Small gusts of grunting respiration Frothing of saliva Deep respiration Muscle relaxation Remains unconscious Goes into deep sleep Awakens feeling sore, headaches
Tonic-clonic seizures Tonic stretching of arms and legs Twitches in his face and body Purses his lips and growls Clonic phase
Atonic seizures Sudden reduction in muscle tone Atonic head drop
Change in consciousness, so that you can't remember some period of time Change in emotion, like unexplainable fear, panic, joy, or laughter Change in sensation of the skin, usually spreading over the arm, leg, or trunk.
Changes in vision, including flashing lights, or (rarely) hallucinations (seeing things that aren't there) Loss of muscle control and falling, often very suddenly Muscle movement such as twitching that might spread up an armor leg Muscle movement such as twitching that might spread up an arm or leg Muscle tension/tightening that causes twisting of the body, head, arms or leg Tasting a bitter or metallic flavor
Diagnosis in epilepsy Aims: Differentiate between events mimicking epileptic seizures E.g. syncope, vertigo, migraine, psychogenic non-epileptic seizures (PNES) Confirm the diagnosis of seizure (or possibly associated syndrome) and the underlying etiology
Diagnosis in epilepsy Approach: History (from patient and witness) Physical examination Investigations
History Event Localization Temporal relationship Factors Nature Associated features Past medical history Developmental history Drug and immunization history Family history Social history
Physical Examination General esp. syndromal or non-syndromal dysmorphic features, neurocutaneous features Neurological Other system as indicated E.g. Febrile convulsion, infantile spasm
Investigations I. Exclusion of differentials: Bedside: urinalysis Haematological: CBP Biochemical: U&Es, Calcium, glucose, ABGs Radiological: CXR, CT head Toxicological: screen Microbiological: LP (Always used with justification)
Investigations II. Confirmation of epilepsy: Dynamic investigations : result changes with attacks E.g. EEG Static investigations : result same between and during attacks E.g. Brain scan
Electroencephalography (EEG) EEG indicated whenever epilepsy suspected Uses of EEG in epilepsy Diagnostic: support diagnosis, classify seizure, localize focus, quantify Prognostic: adjust anti-epileptic treatment
Electroencephalography (EEG) EEG interpretation in epilepsy Hemispheric or lobar asymmetries Periodic (regular, recurring) Background activity: Slow or fast Focal or generalized Paroxysmal activity: Epileptiform features – spikes, sharp waves Interictal or ictal Spontaneous or triggered
Electroencephalography (EEG) Certain epilepsy syndromes have characteristic or suggestive features E.g. Infantile spasms Hypsarrhythmia Childhood absence epilepsy Generalized 3-Hz spike-wave Juvenile myoclonic epilepsy Generalized/ multifocal 4-5 Hz spike-wave and polyphasic-wave Benign occipital epilepsy Unilateral/ bilateral occipital sharp/ sharp-slow activity that attenuates on eye opening Lennox-Gastaut syndrome Generalized/ bianterior spike-wave activity at <2.5 Hz
Electroencephalography (EEG) E.g. Brief absence seizure in an 18-year-old patient with primary generalized epilepsy
Electroencephalography (EEG) Note: Normal in 10-20% of epileptic patients Background slowed by: AED, diffuse cerebral process, postictal state Artifact from: Eye rolling, tremor, other movement, electrodes Interpreted in the light of proximity to seizure
Structural Neuroimaging Who should have a structural neuroimaging? Status epilepticus or acute, severe epilepsy Develop seizures when > 20 years old Focal epilepsy (unless typical of benign focal epilepsy syndrome) Refractory epilepsy Evidence of neurocutaneous syndrome
Structural Neuroimaging Modalities available: Magnetic Resonance Imaging (MRI) Computerized Tomography (CT) What sort of structural scan? MRI better than CT CT usually adequate if to exclude large tumor MRI not involve ionizing radiation I.e. not affect fetus in pregnant women (but nevertheless avoided if possible)
Functional Neuroimaging Principles in diagnosis of epilepsy: When a region of brain generates seizure, its regional blood flow, metabolic rate and glucose utilization increase After seizure, there is a decline to below the level of other brain regions throughout the interictal period
Functional Neuroimaging Modalities available: Positron Emission Tomography (PET) Single Photon Emission Computerized Tomography (SPECT) Functional Magnetic Resonance Imaging (fMRI) Mostly used in: Planning epilepsy surgery Identifying epileptogenic region Localizing brain function
Venn Diagram
Seizure Therapy Anticonvulsant Surgery Specific Treatments Reassurance and Education General Treatment Seizure
Education & Support Information leaflets and information about support group Avoidance of hazardous physical activities Management of prolonged fits Recovery position Rectal diazepam Side effects of anticonvulsants
Anticonvulsants Suppress repetitive action potentials in epileptic foci in the brain Sodium channel blockade GABA-related targets Calcium channel blockade Others: neuronal membrane hyperpolarisation
Anticonvulsants Cabamazepine Phenytoin Valproic acid Tonic-clonic and partial Ethosuximide Valproic acid Clonazepam Absence seizures Valproic acid Clonazepam Myoclonic seizures Diazepam Lorazepam Short term control Phenytoin Phenobarbital Prolonged therapy Status Epilepticus Corticotropin Corticosteroids Infantile Spasms Drugs used in seizure disorders
Medical Intractability No known universal definition Risk factors High seizure frequency Early seizure onset Organic brain damage Established after adequate drug trials Operability
Surgery Curative Catastrophic unilateral or secondary generalised epilepsies of infants and young children Sturge-Weber syndrome Large unilateral developmental abnormalities Palliative Vagal nerve stimulation
Surgical Outcome Medical Intractability A well-localised epileptogenic zone EEG, MRI Low risk of new post-operative deficits
References Stedman’s Medical Dictionary. MDConsult : Nelson’s textbook. Illustrated Textbook of Pediatrics . Video atlas of epileptic seizures – Classical examples, International League against epilepsy. Guberman AH, Bruni J, 1999, Essentials of Clinical Epilepsy, 2 nd edn . Butterworth Heinemann. Manford M, 2003, Practical Guide to Epilepsy, Butterworth Heinemann.