FEBRILE SEIZURE & EPILEPSY

By: SOFEA MUSTAFFA

Seizures
A clinical event in which there is a sudden disturbance of neurological function in association with an abnormal or excessive neuronal discharge.

Febrile Seizures
Convulsions occurring in association with fever in children between 3 months to 6 years of age, in whom there is no evidence of intracranial pathology or metabolic derangement.

Epidemiology
Occurs in about 3% of children between 3 months and 3 years of age (UK) 10-20% of relatives having a seizure disorder including this febrile convulsions. Boys more than girls.

Types of Febrile Seizures

Simple Febrile Convulsions:
Duration < 15 minutes. Generalized seizure Does not recur during febrile episode Does not recur within 24 hours

Complex Febrile Convulsion:

Duration > 15 minutes Focal seizures > 1 seizure during the febrile episode Recurs within 24 hours Residual neurological deficit post- ictally, such as Todds paralysis Indicative of a more serious problem
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Diagnosis
Diagnosis of febrile seizures is based on history.
Also ask about possible ingestions of drug or toxins

Physical examination to rule out of meningitis or encephalitis, if still in doubt, perform a Lumbar Puncture. EEG could be performed but is rarely required
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Investigations
Needed to rule out other causes, per definition Blood Glucose and electrolytes CT Scan rarely required but it is could be useful for complex febrile convulsions EEG or LP seldom required, but could be used to rule out other pathologies.

Management
if in a convulsive state:
Loosen clothing around neck Wipe off vomitus Diazepham (0.5mg/kg)

If not in a convulsive state:

Rapid sponging Anti-pyretic (PCM rectal or syrup- 15 mg/kg/6hourly) Look for cause if initial infection

Prognosis
Benign with excellent prognosis 3-4% of population have febrile convulsions 30% recurrence after the 1st attack 48% recurrence after the 2nd attack 2-7% develop afebrile seizure or epilepsy No evidence of permanent neurological deficit. No deaths reported
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Epilepsy
* A neurological condition characterised by recurrent unprovoked epileptic seizures. An epileptic seizure is the clinical manifestation of an abnormal and excessive discharge of a set of neurons in the brain An epileptic syndrome are complex signs and symptoms that define a unique epilepsy condition.
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Epidemiology
5 per 1000 school aged children
10% are severely affected.

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Causes
Perinatal Conditions:

Infections:
Encephalitis Meningitis Brain abscess

Cerebral malformation Intrauterine infection Hypoxic-ischemic Trauma Hemorrhage

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Causes
Metabolic Conditions
Hypoglycemia Hypocalcaemia Hypomagnesaemia Hyponatremia Hypernatremia Storage diseases Reye syndrome Degenerative disorders Porphyria Pyridoxine dependency and deficiency

Poisoning
Lead Cocaine Drug toxicity Drug withdrawal

Neurocutaneous Syndromes
Tuberous sclerosis Neurofibromatosis Sturge-Weber syndrome Klippel-Trenaunay-Weber syndrome Linear sebaceous nevus Incontinentia pigmenti

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Causes
Systemic Disorders
Vasculitis (CNS or systemic) SLE Hypertensive encephalopathy Renal failure Hepatic encephalopathy

Other
Trauma Tumor Febrile Idiopathic Familial

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Localisation Related Epilepsies

Temporal-lobe epilepsy Frontal lobe epilepsy
Strange feelings in head or abdomen, unusual sensation of taste of smell Psychomotor phenomenon- lip smacking, repetitive stereotyped movements; pulling clothes Dj vu & jamais vuhaving/never having been in a situation

Motor cortex Simple partial seizure with clonic movements which may travel up arm. Post ictal (Todds) paresis may follow.
Distorted vision

Occipital lobe epilepsy Parietal lobe seizures

Contralateral altered sensations, vertigo & distorted body image.

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Classification of Seizures
Seizure Classification Partial
seizure activity starts in one area of the brain

Generalized
seizure involves whole brain & consciousness is affected

Simple
Retains awareness

Complex
Altered awareness and behavior

Secondary generalization
(spreading from one area to the whole brain) "jacksonian" seizure

Tonic Clonic
grand-mal or convulsion Loss of consciousness, stiffening of body then jerking of limbs

Absence
petit mal or starting fit or trance like state

Tonic or Atonic
drop attack Abrupt fall, either with stiffening (tonic) or with loss of muscle tone (atonic or astatic attacks)

Myoclonic
Sudden muscle jerks

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Generalized vs. Partial

Generalized Seizures Always loss of consciousness No warning Symmetrical seizure Bilateral synchronous seizure discharge on EEG

Partial Seizure Begins in a relatively small group of dysfunctional neurons in one of the cerebral hemispheres. May have an aura which reflects site of origin May/not- change in consciousness or more generalized motor jerking

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Partial Seizures
Simple Partial Seizures
The child will retain awareness with consciousness unimpaired

Complex Partial Seizures

Altered conscious state or confusion due to the abnormal electrical discharge spreading from the originating site

Partial Seizures with Secondary Generalisation

Focal seizure manifests clinically or on an ictal EEG followed by a generalized Tonic-Clonic Seizure
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Generalised Epilepsies
Absence seizures
Transient loss of consciousness, with an abrupt onset and termination, unaccompanied by motor phenomena except for some flickering of the eyelids and minor alteration in muscle tone. Onset 4-12 years old. Absences may be typical (petit mal) or atypical can often be precepitated by hyperventilation. Occurs for a few seconds (< 30s) Child does not recall seizure. EEG would not a 3Hz Spike and wave activity with frontal accentuation.
A bsence Seizure.flv

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EEG

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Generalised Epilepsies
Myoclonic epilepsy (Juvenile Myoclonic Epilepsy)
Brief often repeatitive, jerking movement of the limbs, neck and trunk. 10-20years old, (Females: Males = 2:1) Myoclonic predominates but absences and tonicclonic seizures also occur

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Generalised Epilepsies
Tonic-Clonic Seizure
Rhythmical contraction of muscle groups following the tonic phase In the rigid tonic phase, children may fall to the ground, sometimes injuring themselves. Do not breath and become cyanosed. Followed by the clonic phase, with jerking of the limbs. Breathing is irregular, cyanosis persists and saliva may accumulate in the mouth May be biting of the tongue and incontinence of urine. Lasts a few seconds to minutes, followed by unconscious or deep sleep for several hours.
Tonic C lonic Seizure.flv

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Generalised Epilepsies
Atonic Seizures
Often combined with a myoclonic jerk followed by a transient loss of muscle tone causing a sudden fall to the floor or drop of the head. 1-2 years old, last 1-4seconds Most frequent during awakening of falling asleep. Often have Mental Retardation or underlying brain abnormalities

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Generalised Epilepsies
Infantile Spasms (West Syndrome)
4-6months Violent flexor spasms of head, trunk and limbs, followed by extension of the arms (Salaam Spasms) Last 1-2s, bursts of 20-30spasms, usually on waking 2/3 children are neurologically abnormal before onset of seizures- development process further arrested EEG- hypsarrthymia, a chaotic background of high voltage dysrhymic slow-wave activity with sharp components. 30-40% respond well to treatment Loss of skills and later learning disability or epilepsy.
Infantile spasms.flv

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Other epilepsies
Lennox-Gaustaut
1-3 years old Myoclonic single jerk, atonic drop attacks or atypical absences. Developmental arrest or regression & behaviour disorder. Poor prognosis

Benign rolandic epilepsy of childhood

Common Sleep: generalized tonic-clonic Day: distortion of face and arm on one side + abnormal feeling of tounge Seizure stop by midteens & may not require treatment
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Differential Diagnosis
Neonates and infants
Jitteryness Benign myoclonus Apnoea Gastro-Oespophageal reflux Shuddering attacks Benign paroxymal torticollis Hyperekplexia

Young children
Breath holding spells Reflex anoxic seizures Parasomnia Benign peroxysmal vertigo Peroxysmal choreoathertosis Tics and ritualistic movements Rage attacks
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Differential Diagnosis
Childhood and Adolescents
Vasovagal syncope Migraine Narcolepsy Panic attacks pseudoseizures

Any Age
Drug-Induced dystonia Cardiac Dysrhythmias

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Investigations
EEG
- if normal, try barbiturate induced sleep, sleep deprivation recording or 24 hour monitoring.

CT or MRI of brain indicated (< 5 years) Blood electrolyte analysis

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Treatment for epilepsy

Medications Surgery Ketogenic Diet Vagus Nerve Stimulation

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Medication
>1 episode Attempt to classify the seizure type(s), monotheraphy is recommended. Increase dose gradually until the desired effect is achieved Add 2nd drug if 1st has failed, optimize second drug and try to withdraw 1st drug. Rational combination (2/3 with different mechanisms) Monitor drug levels to check compliance Withdrawal after 2 years of being seizure free
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Surgical
Lobectomy and Cortical Resection Hemispherectomy

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KETOGENIC DIET
Primarily used in childhood epilepsy. Mechanism- unknown. The high-fat, low-protein, no-carbohydrate diet mimics some effects of starvation that seem to inhibit seizures. The diet is very rigid and carefully controlled and must be supervised by a physician -sometimes in a hospital setting.
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VAGUS NERVE STIMULATION

Approved to treat partial seizures in patients 12 years of age or older stimulator is surgically implanted under the skin in the chest. The device is attached to a wire that is tunneled under the skin and attached to the vagus nerve, which is located in the left side of the neck adjusted to automatically stimulate the vagus nerve from every few seconds to every few minutes.
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Status Epilepticus
Prolonged episode of seizure activity (>30 minutes) or an extended period of recurrent seizures between which the patient does not return to consciousness. Its a medical emergency !!
Can lead to hypoxia, brain damage and death

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Management of Status Epilepticus

ABCs ECG monitoring Oxygen and pulse oximetry IV access Immediate laboratory tests (electrolytes, glucose..) a rapid infusion of 5 mL/kg of 10% dextrose Antiepileptic drug levels Toxicologies
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