Dr Nivedita Bajaj - Basic Facts About Childhood Epilepsy

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Information about Dr Nivedita Bajaj - Basic Facts About Childhood Epilepsy
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Published on March 19, 2018

Author: niveditabajaj

Source: authorstream.com

Epilepsy: the basics: Epilepsy: the basics Dr Nivedita Bajaj Consultant Community Paediatrics GP Training Day 1 Objectives: Objectives An overview of epilepsy List the basic classification of epileptic seizures Getting key facts in the history Being aware of general management 2 Dr Nivedita Bajaj Introduction: Introduction Most common neurological condition Prevalence of 0.5 – 1.0% Annual incidence in childhood is about 60 per 100,000, excluding neonatal seizures Much more common in children with pre-existing neurological disability 12-18% of children with mild learning disability, 21 – 36% of children with severe learning disability and in up to 90% of children with bilateral spastic cerebral palsy (GMFCS V) 3 What is epilepsy?: What is epilepsy? A transient occurrence of signs and / or symptoms due to abnormal excessive or synchronous neuronal activity in the brain Epilepsy is a disorder of the brain characterised by an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological and social consequences of this condition. ILAE definition 4 Dr Nivedita Bajaj Is it Epilepsy?: Is it Epilepsy? In the study of Uldall et al the misdiagnosis rate was of the order of 40%. The commonest reasons for misdiagnosis are: a) Failure to take an accurate history. b) Failure to think of possible differential diagnoses. c) Undue reliance on the EEG. d) Diagnosis may be difficult! 5 Dr Nivedita Bajaj NICE 2012: NICE 2012 “all children, young people and adults with a recent onset suspected seizure should be seen urgently by a specialist”. 6 Classification of the epilepsies : Classification of the epilepsies The International League Against Epilepsy (ILAE) suggests a flexible approach with diagnosis on 5 axes: Axis 1 – description of the seizure. Axis 2 – epileptic seizure type from an accepted list. Axis 3 – syndrome from an accepted list if possible. Axis 4 – aetiological diagnosis if known. Axis 5 – degree of impairment caused by epilepsy. 7 Aetiological classification: Aetiological classification A specific cause for a child’s epilepsy is only found in about 30% of cases Genetic Structural/Metabolic Disorders of brain development (migration disorders, hydrocephalus) Damage to the brain caused by hypoxic-ischaemia, infection, inflammation, etc.; Space-occupying lesions such as hamartomas, arachnoid cysts and tumours. Metabolic disorders ranging from aminoacidopathies to mitochondrial cytopathies Unknown 8 What triggers seizures?: What triggers seizures? No apparent reason Certain triggers make a seizure more likely These are not the cause of epilepsy, but may trigger a seizure Examples: Tiredness, flashing lights, lack of sleep, temperature…. 9 Different types of seizures: Different types of seizures There are over forty different types of seizures. Seizures can be divided into two main types: Generalised seizures Focal seizures 10 Seizure types: Seizure types Generalised seizures These initially involve both hemispheres of the brain. The person will be unconscious Focal seizures These arise from one part of the brain. Symptoms and level of consciousness depend on the area of the brain involved Focal seizures account for around 60% of all cases of epilepsy 11 Categories of seizures: Categories of seizures • Generalised seizures -- Tonic-clonic -- Absence ›› Typical ›› Atypical -- Absence with special features ›› Myoclonic absence ›› Eyelid myoclonia -- Myoclonic ›› Myoclonic ›› Myoclonic atonic ›› Myoclonic tonic -- Clonic -- Tonic -- Atonic 12 Generalised seizures : Generalised seizures Tonic clonic seizures Often preceded by a cry, the person becomes rigid and may fall if standing. The muscles then relax and tighten rhythmically causing the person to convulse. Breathing may stop or become laboured and they may be incontinent. Tonic seizures There is a general stiffening of muscles without rhythmical jerking. There is loss of consciousness causing the person to fall to the ground (usually backwards) with consequent risk of injury. 13 Generalised seizures: Generalised seizures Clonic seizures These are characterised by rhythmic or semi-rhythmic muscle contractions typically involving the upper extremities, neck and face. Atonic seizures There is a sudden loss of muscle tone, with instantaneous collapse (usually forwards) often resulting in facial or other injuries. People normally recover quickly. 14 Generalised seizures : Generalised seizures Myoclonic seizures Consist of sudden, brief muscle contractions, either singly or in clusters that can affect any muscle group. Absence seizures Usually last 5-10 seconds. They manifest as a sudden onset of staring and impaired consciousness with or without eye blinking and lip smacking. 15 Focal seizures: Focal seizures Simple partial seizure Often referred to as a ‘warning’ or ‘aura’ No impairment of consciousness Sometimes develop into other seizures These can present with: Rhythmical twitching of one limb, or part of a limb Unusual tastes, smells or sensations such as pins and needles in a distinct part of the body 16 Slide17: Secondarily generalised seizures Focal seizures may spread to involve the whole of the brain and if this happens it is called a secondarily generalised seizure 17 epileptic encephalopathy: epileptic encephalopathy Epileptic activity itself may contribute to disturbances of behavioural and cognitive functioning, above and beyond that expected from the underlying pathology. 18 Epilepsy syndromes: Epilepsy syndromes NICE (2012) gives greater weight to epilepsy syndrome diagnosis in determining treatment choice than seizure type Epilepsy syndrome is formed by the seizure type, EEG characteristics, age at onset, presence of underlying neurologic abnormalities, imaging results and diurnal pattern of seizures 19 Diagnosis of Epilepsy: Diagnosis of Epilepsy Clinical history Eye witness account of the event Medical examination Results of any further investigations should be interpreted with reference to the history of the event 20 History taking: History taking Setting: sleep or awake Stimulus: eg: frustration, fright, breath-holding Aura (warning): precedes a focal seizure Onset - the first event in a seizure Course of seizure Offset Post-ictal condition After effects: such as prolonged confusion, 21 Differential diagnosis:: Differential diagnosis: Reflex anoxic seizures Syncope Sleep disorders Benign sleep myoclonus Non-epileptic attacks Toxins e.g. drug ingestion. Metabolic derangements e.g. hypoglycaemia. Psychological disturbances. Migraine. Tics. Self-stimulation / self-gratification (masturbation). Gastro-oesophageal reflux (Sandifer syndrome). 22 Investigations: Investigations No tests to diagnose epilepsy Diagnosis of epilepsy is clinical Investigations: - may support the diagnosis - help in the classification - guide management - identification of an underlying cause 23 Slide24: Blood glucose at the time of the seizure or early morning Serum calcium 12 lead ECG recording 24 Electroencephalogram (EEG): Electroencephalogram (EEG) There are 4 types Routine Sleep Ambulatory Video telemetry 25 EEG: EEG The EEG may be normal in children with epilepsy and abnormal in children without. An EEG should NOT be done if the clinical diagnosis is probable syncope or non-epileptic because of the possibility of a false positive result. NICE guidance 2012 is clear that the role of the EEG is to provide supporting evidence for the clinical diagnosis of epilepsy and it can help with epilepsy classification or epilepsy syndrome diagnosis. 26 EEG: EEG Ictal EEGs – Recording the EEG during seizures is valuable in defining the epilepsy syndrome and in providing information about site of onset. This may be possible, if seizures are sufficiently frequent, by recording a 24-hour ambulatory EEG or by using video EEG telemetry. Provocative tests – sleep deprivation, hyperventilation, photic stimulation. Invasive recording 27 3D MRI scan overlaid with an EEG : 3D MRI scan overlaid with an EEG 28 Slide29: Magnetic Resonance Imaging (MRI) Investigation of choice Better sensitivity or specificity Useful in identifying structural abnormalities in children Computerised Tomography (CT or CAT) Preferred if patient is acutely unwell Valuable in detecting acute intracranial hematoma, fractures, intracranial calcification, or when there is a contrindicaton to MRI 29 Neuroimaging Neuroimaging : Neuroimaging NICE guidance 2012: children and young people with epilepsies should undergo neuroimaging with MRI if they have epilepsy and: Are under two years of age. Have a suggestion of focal onset on history, examination or EEG (unless clear evidence of benign focal epilepsy). Have seizures that continue despite first line medication. 30 Treatment of epilepsy: Treatment of epilepsy Why should epilepsy be treated? Mortality Morbidity Injuries and accidents SUDEP Learning Behaviour Status epilepticus Quality of life issues 31 Treatment of epilepsy: Treatment of epilepsy The main objectives of treatment: To enhance quality of life by stopping all seizures with minimal side effects Where seizures cannot be stopped without side effects: Minimize the number of seizures, especially tonic clonic, tonic and atonic seizures Minimise adverse effects of treatment 32 Anti epileptic drugs: Anti epileptic drugs Drug should be selected with reference to seizure type/syndrome, age and sex of the individual Started at a low dose and increased slowly to aid tolerance Regime kept simple with once or twice daily dosing Drugs which might increase some seizures should be avoided 33 Epilepsy Surgery : Epilepsy Surgery Neurosurgery should be considered if: The drug treatment has been ineffective Diagnostic investigations point to focal onset Evidence of medical, social and educational disability due to the seizures, with the child’s quality of life likely to improve after surgery Acceptable risk-benefit ratio for the proposed surgery 34 Slide35: Vagus nerve stimulation Pacemaker-like pulse generator Bipolar lead with two stimulating electrodes Battery life of 5-10 years 35 Slide36: How does it work? Mild electrical pulses applied to the left vagus nerve in the neck for transmission to the brain Intermittent stimulation Typically 30 sec on/5 min off 24 hours a day, 7 days a week Magnetic empowerment On-demand stimulation Acute stimulation related side effects control Simple out-patient programming (dosing) by treating physician 36 Ketogenic diet: Ketogenic diet The ketogenic diet is a high-fat, low-protein and very low carbohydrate diet that can be effective in children with drug resistant epilepsy. 37 Slide38: Ketogenic diet High fat diet designed to mimic starvation Production of ‘ketone bodies’ ‘Classical diet’ ‘MCT diet’ 38 Non-pharmacological approaches to seizure control: Non-pharmacological approaches to seizure control Behaviour therapy Avoidance of seizure triggers Counter measures to provoking situations; e.g. relaxation strategies for anxiety induced Sz Life-style focused approaches - Exercise - Diet - Compliance 39 Status Epilepticus: Status Epilepticus Generalised convulsive (tonic clonic) status epilepticus is a life-threatening medical emergency characterised by: A generalised convulsion lasting for 30 minutes or longer or Repeated tonic-clonic convulsions occurring over a 30 minute period without recovery of consciousness between each convulsion. 40 SUDEP: SUDEP The sudden unexpected, witnessed or unwitnessed, non-traumatic, non-drowning death with or without evidence for seizure. 500 deaths in the UK per year Post mortem- no structural or toxicological cause suspected causes include hypoxia due to a seizure or cardiac arrhythmia. 41 Non-epileptic attack disorder (NEAD) : Non-epileptic attack disorder (NEAD) also referred to as psychogenic non-epileptic seizures (PNES). Indicators : If the nature of the events change over time There are multiple unexplained physical symptoms There are unusually prolonged events Refer for neurological assessment if NEAD is suspected Information for patient with NEAD can be helpful for the person suffering these attacks and their family to take away and reflect on 42 Comorbidities: Comorbidities Learning disabilities: strong association ADHD Obsessive compulsive disorder Autism Behaviour difficulties: such as depression, isolation, anxiety, fear, The psychological effects Motor disorders such as dyspraxia, dystonia, ataxia, 43 Epilepsy and education: Epilepsy and education Some degree of educational problem is present in 50% Psychiatric disorders are common and under diagnosed Common problems: slow information processing, attention deficit, memory impairment, language deficit (comprehension, word finding), poor motor planning, executive dysfunction, etc. Early identification and a multidisciplinary approach (schools, educational psychologist, CAMHS) Epilepsy and education: Epilepsy and education Factors for poor educational progress Early age of onset Longer seizure history Poor seizure control Involvement of dominant hemisphere Nocturnal attacks High level of medications & poly-therapy Absences from school Low self-esteem & anxiety Associated co-morbidities 45 Managing epilepsy in adolescent girls : Managing epilepsy in adolescent girls Effect on oral contraceptive (may need a higher strength one). Valproate can cause ovarian cysts. Some drugs, especially valproate, are antifolate and may therefore contribute to causing spinal cord defects in the foetus. • Several drugs are known to be teratogenic. Valproate can cause weight gain. 46 References: : References: International league against epilepsy: website www.ilae.org.uk Epilepsy action: website www.epilepsy.org.uk National Institute for Clinical Excellence (2004) The epilepsies diagnosis and management of the epilepsies in children and young people in primary and secondary care. London Oaktree Press LTD Epilepsy.com website 47 More From Dr Nivedita Bajaj: More From Dr Nivedita Bajaj https://drniveditabajaj.blogspot.co.uk/ https://drniveditabajaj.tumblr.com/ https://drniveditabajaj.blogspot.co.uk/2018/03/dr-nivedita-bajaj-mbbs-nivedita.html 48

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