SEIZURES- clinical event in which there is sudden disturbance of neurological fx caused by an abnormal or excessive neuronal discharge
EPILEPSY- recurrent, unprovoked seizures.
25-30% of these children have other neurological disorder (mental handicap, cerebral palsy)
Diagnosed clinically, hence a detailed description of events experienced by patient or an adequate account by a reliable witness is crucial
Epilepsy should be viewed as a symptom of an underlying neurological disorder and not as a single entity
Hx of previous seizures
Recent withdrawal of anti-convulsant medication
Hx of remote neurologic insult ( stroke, intracranial hemorrhage,cerebral palsy, head, trauma,meningitis
Familial hx of seizures
- No obvious underlying pathology
- With underlying pathology
- Appears to be symptomatic but pathology cannot be identified
Beware of non-epileptic event
Syncope - day dreaming
Night terrors - delirium
Migraine - sleep disorder
Onset, duration and time
Pre-ictal and ictal phase
Growth and development hx-weakness, sensory deficits, change in vision, behavior, balance and gait
Pmh- previous hx of seizures or neurologic abnormalities
Recent hx of head trauma
Possibility of ingestion or recent change in antiepileptic medication
CLASSIFICATION OF SEIZURES
Seizures arise from one or part of one hemisphere
Seizures arise from both hemisphere simultaneously
MANIFESTATION OF PARTIAL SEIZURES
Manifestation will depend on the part of the brain where the discharge originates
-the most common
-may result in strange warning feelings or aura with smell and taste abnormalities and distortion of sound and shape.
-lip smacking, plucking at one’s clothing, and walking in non-purposely manner (automatism)
-consciouness can be impaired
Involve motor cortex
May lead to clonic movement, which may travel proximally
Asymmetrical tonic seizures can be seen, which may be bizarre and hyperkinetic and can be mistakenly dismissed as non-epileptic events
-cause distortion of vision
PARIETAL LOBE SEIZURES
- Cause contralateraldysaesthesias (altered sensation), or distorted body image
"I almost enjoy them. The feeling of déja vu, as if I've lived through this moment and I even know what's going to be said next. Everything seems brighter and more alive."
"It is a pressure that starts in my stomach, then rises to my chest and throat. When it reaches my chest, I smell an unpleasant odor of something burnt. At the same time I feel anxious."
SIMPLE PARTIAL SEIZURE
Consciousness not impaired
Clinical symptoms include motor, sensory,psychic or autonomic
Change in muscle activity
Jerking of finger or stiffening of part of the body
Movement may spread (affect one side or extend to both sides)
Weakness affecting speech
Coordinated action such as laughter or automatic hand movements (aware or not)
Change in any of the senses
Smell or taste things that aren’t there
Hear clicking,ringing or person’s voice when there is no actual sounds
Feel sensation of “pins and needle”/numbness
Feel floating or spinnig in space
Changes in the part of the nervous system that automatically controls body fx
Strange or unpleasant sensation in stomach, chest,or head
Change in heart rate
Change in thinking,feeling or experiencing things
Problem with memory
Inability to find right word
Trouble in understanding spoken or written language
Sudden feeling of emotion like fear,depression or happiness
Feeling of dejavu (I’ve been through this)
Feeling of jamais vu (this is new to me)
“Micky's spells begin with a warning; he says he's going to have a seizure and usually sits down. If I ask him how he feels, he just says 'I feel it.' Then he makes a funny face, a mixture of surprise and distress. During the seizure he may look at me when I call his name but he never answers. He just stares and makes odd mouth movements, as if he's tasting something. Sometimes he'll grab the arm of the chair and squeeze it. He may also pull at his shirt as though he's picking lint off of it. After a few minutes, when he's coming out of it, he asks a lot of questions. He never remembers his 'warning' or these questions. The seizures make him tired; if he has two in the same day, he often goes to sleep after the second one."
COMPLEX PARTIAL SEIZURES
COMPLEX PARTIAL SEIZURE
Temporal lobe epilepsy is the most common type
The patient stops what he or she doing and stare blankly, often making rhythmic smacking movements of the lips or displaying other automatism, such as picking at their clothes
After few minutes return consciousness, may initially feel drowsy
Aura may present: alterations of mood, memory
Complex hallucinations of sound, smell, taste,vision,emotional changes (fear,sexual arousal) or visceral sensation (nausea, epigastric discomfort) may also occur.
The seizures start with a tingling in my right thumb. In seconds, my thumb starts jerking. Soon, my whole right hand is jerking. I have learned that by rubbing and scratching my forearm I can sometimes stop the seizure. Other times the jerking spreads up my arm. When it reaches my shoulder, I pass out and people tell me that my whole body starts to jerk.
SECONDARILY GENERALIZED ( “JACKSONIAN” SEIZURES)
When partial seizures spread to involve the whole brain and produce generalized tonic-clonic seizures.
focal seizure followed by generalised tonic-clonic seizure
-generalised brain involvement
Junsu, a 7-year-old boy, often "blanks out" anywhere from a few seconds to 20 seconds at a time. During a seizure, Junsu doesn't seem to hear his teacher call his name, he usually blinks repetitively, and his eyes may roll up a bit. During shorter seizures, he just stares. Then he continues on as if nothing happened. Some days Junsu has more than 50 of these spells
TYPICAL ABSENCE (4-6 years of age)
Brief loss of environmental awareness ( LOC), with an abrupt onset and termination, unaccompanied by motor phenomenona except for some eye fluttering and minor alteration in muscle tone or simple automatism such as head bobbing and lip smacking
Can often be precipitated by hyperventilation and lasted for few seconds
primary generalised absence/petit mal
Stoppage of activity, staring and alteration of concsiousness and may include automatism
Sustained contraction or stiffness
Generalised increase in muscle tone
- Rythmic jerking of one limb, one side or all of the body
"These seizures frighten me. They only last a minute or two but it seems like an eternity. I can often tell Jessica's going to have one because she acts cranky and out of sorts. It begins with an unnatural shriek. Then she falls, and every muscle seems to be activated. Her teeth clench. She's pale, and later she turns slightly bluish. Shortly after she falls, her arms and upper body start to jerk, while her legs remain more or less stiff. This is the longest part of the seizure. Finally it stops and she falls into a deep sleep."
Rhythmical contraction of muscle groups following the tonic phase
In the rigid tonic phase, children may fall to the ground, sometimes injuring themselves. They do not breathe and become cyanosed.
this is followed by clonic phase, with jerking of the limbs. Breathing is irregular, cyanosis persists and saliva may accummulate in the mouth.
There may be biting of tongue and incontinence of urine
Lasts from a few second to minutes
followed by unconsciousness or deep sleep up to several hours
post-ictal confusion or headache and a period of subsequent malaise is common
"In the morning, I get these 'jumps.' My arms fly up for a second, and I often spill my coffee or drop what I'm holding. Now and then my mouth may shut for a split second. Sometimes I get a few jumps in a row. Once I've been up for a few hours, the jumps stop."
shock/lightning like jerk of part of the body
Caused by rapid contraction and relaxation of muscles
May involve neck,shoulders and upper arms
Usually associated with multiple seizure type
non-epileptic myoclonus movement are also seen physiologically in hiccoughs (myoclonus of diaphragm) or on passing through stage ii sleep (sleep myoclonus)
"When Minho has a 'drop' seizure, he falls to the ground and often hits his head and bruises his body. Even if I'm right next to him and prepared, I may not catch him. Even with carpet in the bedroom and mats in the bathroom, he gets hurt."
ATONIC SEIZURES @ DROP SEIZURES
Often combined with myoclonic jerk followed by transient loss of muscle tone causing a sudden fall to the floor or drop of the head
Consciousness is impaired during fall and regain alertness immediately upon striking the floor
they only occur in context of epilepsy syndromes with other forms of seizure
Always a/w with LOC
Preceded by aura according to different lobe
Laboratory and diagnostic evaluation of seizures
Complete lab evaluation of child with new onset of seizures:
Measurement of blood chemistry: glucose,calcium,sodium,potassium,chloride,bicarbonate,BUN, creatinine,magnesium,phosphorus
Blood or urine toxicology
Csf analysis-LP is not necessarily if the patient is afebrile and has no other neurologic signs or if the history does not suggest meningeal infection or subarachnoid hemorrhage.
Brain imaging with CT or MRI
INDICATION OF BRAIN IMAGING IN AFEBRILE SEIZURES
First afebrile seizure episode- to exclude any intracranial pathology ie:hematoma,space-occupying lesion which may lead to seizure
Complex seizure disorder
EPILEPTIC SYNDROMES- complex of signs and symptoms that define a unique epilepsy condition.
Syndromes are classified on the basis of seizure type(s), clinical context, EEG features and neuroimaging.
Paediatric Protocol for Malaysian Hospital
epilepsies that can grouped together according to age, sex, hx, seizure semiology and EEG pattern.
For details, can refer Tom Illustrated and Paeds Protocol
childhood absence epilepsy
Juvenile myoclonic epilepsy
Benign rolandic epilepsy
Diagnosing epilepsy is a multi-step process, usually involving the following evaluations:
Confirmation through patient history, neurological exam, and supporting blood and other clinical tests that the patient has epileptic seizures and not some other type of episode such as fainting, breath-holding (in children), transient ischemic attacks, hypoglycemia, or non-epileptic seizures.
Identification of the type of seizure involved.
Determination of whether the seizure disorder falls within a recognized syndrome.
A clinical evaluation in search of the cause of the epilepsy.
Based on all previous findings, selection of the most appropriate therapy.
Junho could feel it. He was in the middle of an exam and didn't want to make a scene, so he tried to control it. But it was no use. The stress of the exam was getting to him, and the longer he held in his tic, the more he could feel it building up inside him. Finally he had no choice but to let it out. It wasn't as bad as he anticipated — his shoulders jerked slightly and no one seemed to notice.
Usually a temporary condition (transient tic)
Affect 25% of people before the age of 18
Sudden, repetitive movement or sound that can be difficult to control.
Motor and vocal tics (simple or complex)
Shoulder shrugging is one of the most common simple motor tics (nose wrinkling,headtwitching,eyeblinking,lipbiting,kicking,jumping)
Causes: no definite causes (emotional factors like stress)
Diagnosis: accidental,no specific test,run test to rule out other condition with similar presentation
My two-year-old son continually holds his breath, either after hurting himself or when he doesn't get his own way.
He went blue today and actually appeared to faint – this only lasted a short time.
Following this his lips looked blue for a while and he then slept for half an hour.
An episode in which the child stop breathing and loses consciousness for a short period immediately after a frightening or emotionally upsetting event or a painful experience
Usually triggered by physically or emotionally upsetting events
Usually begin in the first year of life and peak at the age of 2
Cyanotic/blue type or pallid type
Further diagnostic and evaluation needed if the spells occur often
May resolve in later year
Emily Stevens* is afraid to do the ordinary things most of us take for granted. For years, she has been afraid to drive a car, be active in sports or even walk to the grocery store. Ever since Emily was diagnosed with epilepsy as a child, the constant threat of seizures - with loss of body control and consciousness - has made this 41-year-old woman a prisoner in her own home.
As a child, Emily experienced sexual abuse and extreme emotional trauma. As an adult, she again became a victim, but this time of rape. To deal with the horror in her life, Emily learned a long time ago to keep her emotions and feelings to herself.
When even large doses of epilepsy medications couldn't stop all her seizures, Emily went to the University of Michigan Epilepsy Clinic. It was not long before Linda Selwa, M.D., medical director of the Adult Neurology Outpatient Clinic, discovered that Emily's problem was not only epilepsy, but a recently discovered condition called pseudoseizures.
PSYCHOGENIC NONEPILEPTIC SEIZURES/PSEUDOSEIZURE
Paroxysmal episodes that resemble and often misdiagnosed as epileptic seizure
PNES are psychological (emotional,stress-related) in origin
Do not result from an abnormal electrical discharge from the brain
PNES-commonly misdiagnosed as epilepsy in patient with previous diagnosis of epilepsy that does not respond to drug
The diagnosis requires an inpatient admission where the patient is continuously monitored by both an electroencephalogram (EEG) and video camera
Physicians believe pseudoseizures are psychological defense mechanisms induced by stress or episodes of severe emotional trauma.
The seizures happen when patients try to avoid or forget the trauma.
term used to describe the loss of consciousness from temporary disruption of cerebral oxygenation
due to the interruption of blood flow to the brain, and the loss of consciousness usually lasts for less than 30 seconds
Patients who complain of dizziness, light-headedness, passing out, and/or blacking out must be questioned carefully.
a careful history often can clarify the condition and guide further evaluation and care
Syncope is a symptom of either cardiac or hemodynamic dysfunction and not a disease
an unintentional, somewhat rhythmic, muscle movement involving to-and-fro movements (oscillations) of one or more parts of the body
most common of all involuntary movements and can affect the hands, arms, head, face, vocal cords, trunk, and legs.
In some people, tremor is a symptom of another neurological disorder.
The most common form of tremor, however, occurs in otherwise healthy people
Causes and diagnosis
caused by problems in parts of the brain that control muscles throughout the body or in particular areas, such as the hands
Neurological disorder ie multiple sclerosis,neurodegenerative disease
Diagnosis: detailed hx,physicalexamination,brain imaging
Management and treatment of epilepsy
Khiddir bin Nasharuddin
Treatment recommended > 2 episodes (recurrence risk up to 80%
Attempt to classify the seizure and epileptic syndrome
Add on the 2nd drug if first drug failed
Rational combination therapy (usually 2 / max 3 drugs ) i.e combine drug with different MOA and consider their spectrum of efficacy, drug interactions and adverse effects
Monitor drug levels
Give consideration when planned for medication withdrawal
Principle of anticonvulsants therapy for Epilepsy
Depends on the probability of recurrence of seizures if the drugs are discontinued and the risk of subsequent epilepsy
This can be determined by considering the neonatal neurological examination, cause of the seizure and the EEG.
If neonatal neurological examination become normal, discontinue therapy
If neonatal neurological examination is persistently abnormal
Consider aetiology and obtain EEG
In most cases – continue phenobarbitone, discontinue phenytoin and reevaluate in a month
Duration of anticonvulsant therapy
One month after discharge
If neurological examination has become normal, discontinue phenobarbitone over 2 weeks
If neurological examination is persistently abnormal, obtain EEG
If no seizures activity or not overtly paroxysmal on EEG, discontinue phenobarbitone over 2 weeks
If seizure activity is overtly paroxysmal continue phenobarbitone until 3 months of age and reaccess in the same manner
A severe bullous form of erythemamultiforme also involving mucous membrane. Eye involvement may include conjunctivitis, corneal ulceration and uveitis. Caused by drug sensitivity / infection with morbidity and mortality.
Inc. in frequency of channel opening produced by GABA
Binding of GABA to its receptor triggers an opening of a chloride channel which leads to an increase in chloride conductance
Influx of chloride ion causes a small hyperpolarization that moves the postsynaptic potential away from its firing threshold and thus inhibiting the formation of action potential
Mechanism of Action of Anticonvulsants Drug
Combination of sodium valproate and valproic acid which reduced to valproate when reaches GI tract
Sodium channel blocker, block GABA transaminase and action at T-type Ca+ channel
Valproate is bound to albumin
Beware of teratogenecity
Blocks voltage gated sodium channels by selectively bind to the channel in the inactive state and slowing its rate of recovery
Bound 90 percent to plasma albumin
Blocks voltage dependant sodium channels
Increase frequency of chloride channel opening by binding to GABA receptor
Reduced high voltage calcium current
Carbonic anhydrase inhibitor
Induced by phenytoin and carbamezipine
Reduces the propagation of abnormal impulses in the brain by blocking sodium channels thereby inhibiting the generation of repetitive action potentials in epileptic focus and preventing
Do not prescribed in patient with absence seizures as it may cause an increase in seizures
Two or more sequential seizures without full recovery of consciousness between seizures or more than 30 minutes of continuous seizure activity
Epilepsy Foundation of America
Any seizure lasting > 30 minutes or
intermittent seizures, without regaining full consciousness in between for 30 minutes.
Paediatric Protocol for Malaysian Hospital
However, parents and public are advised to call for emergency assistance when a convulsion continues for more than 5 minutes without signs of stopping.
Precipitant of status epilepticus in children
fever/febrile seizure – leading cause of SE
About 25% of children presenting with status epilepticus have an acute brain injury such as:
Purulent or aseptic meningitis
20% have hx of brain injury or cengenital malformation
50% no definable etiology- 50% of them associated with fever
Other frequent causes-cessation of anticonvulsant medication
Any type of epileptic seizures can progress to status epilepticus
- Continuous absence seizures and partial status epilepticus
Generalized tonic-clonic seizures
Has greatest potential for long-term damage
Death or brain damage from status seizures (as opposed to death from the underlying cause) is most likely to result from:
Direct damage to the brain caused by the injury that causes the seizures
Stress on the system from repeated generalized tonic clonic seizures
Injury from repeated electrical discharge in the brain
Convulsive status epilepticus is a medical emergency
Algorithm for Status Epilepticus
Child with seizures
Seizures > 5 min (impending status epilepticus)
At home / in the ambulance
PR diazepam (0.2 – 0.5 mg/kg) – max 10 mg
Perfusion adequate (ABC’s)
In the hospital
Obtain IV access
IV diazepam (0.2 mg/kg slow bolus)
Seizures 5 – 30 mins (established status epilepticus)
IV Diazepam (0.2mg/kg slow bolus)
IV phenytoin 20 mg/kg (max 1.25gm)
If <2 yr old – consider IV pyridoxine 100 mg bolus
Child not on maintenance phenytoin
Monitor blood sugar, electrolytes, blood counts, liver function, blood gases
Consider blood culture, toxicology, neuroimaging, antiepileptic drug levels
Early Refractory Status Epilepticus (seizures >10 min after phenytoin)
IV Midazolam (0.2 mg/kg bolus)
IV Phenobarbitone (20mg/kg)
Monitor BP, respiration
Start inotropic support, especially if Midazolam/phenobarbitone is given
Arrange for ICU
Secure airway, prepare to use mechanical ventilation
Titrate phenobarbitone to achieve burst suppression pattern on EEG
If seizures persist despite 30mg/kg loading dose of phenobarbitone, contact higher up for discussion
By : Adam Safin bin Abdul Mutti
“Raju was just 14 months old. He caught a bad cold from one of the children in the playgroup. He had a fever and runny nose. He was taking a nap when I heard this strange banging sound. I ran into his room, and his whole body was stiff and shaking. The whole thing probably lasted less than 10 minutes. They were the longest 10 minutes of my life. He has never had another one, and doesn't need any seizure medication."
“ A seizure associated with fever in the absence of another cause and not due to intracranial infection from meningitis or encephalitis” [ Illustrated Textbook of Paediatrics, 3rd edition].
Occur in children between 3 months old and 6 years old.
Occur in 3% of children between the ages of 6 months and 5 years.
Has a genetic predisposition; 10% risk if the child has a first-degree relative with febrile seizures.
Why it is common in children???
Seizure threshold is low in children.
Viral infection (predominant causes) rapid increase in temperature can’t tolerate well seizures.
How to differentiate meningitis and febrile seizure in children???
Sign and symptom of meningitis :
Positive Kernig and Brudzinski sign (> than 12 months old child).
There are sign of increase intracranial pressure in meningitis, not in febrile seizure :
Sixth nerve palsy.
Recurrent Febrile Seizures
30-40% of children will have recurrent febrile fits.
Risk factors :
Early age of onset (less than 15 months old).
Epilepsy in first degree relatives.
Febrile fits in first degree relatives.
Low degree of fever (less than 40°C) during first febrile fits.
Brief duration between onset of fever and initial fits.
Why Children Need Admission???
Main reason :
To exclude intracranial pathology.
Fear of recurrent fits.
To treat or investigate the cause of fever besides meningitis or encephalitis.
To allay parental anxiety, especially those who staying far from hospital.
Children who developmentally normal at the time of febrile fits will continue to develop normally [ Nelson&Ellenberg, Verity et al]
There was no difference in children between those experiencing simple seizures and complex seizures.
Management and treatment of febrile convulsion
Call for help
Evaluate and Resuscitate
Glasgow coma scale
Admit to ward
Not all children are admitted to ward
Investigation (lumbar puncture)
Must be done if
Sign of intracranial infection
Prior antibiotic therapy
Persistent lethargy and not fully interactive 6 hours after seizures
Strongly recommended if
Age < 12 months
First complex febrile convulsion
In district hospital without paediatrician
Patient have a problem bringing the child in again if deterioration at home
Take of cloth and tepid sponging
Monitor the patient condition
Counseled parents regarding the benign nature of the disease
Advised parents on first aid measures during a convulsion
Do not panic
Loosen child cloth especially around neck
Place child on left lateral position with head lower than body
Wipe out any vomitus
Don’t insert any object or drug
Stay near the child
For parents whose children have high risk of recurrence febrile convulsion
Convulsion last more than 5 minutes
Prevention of recurrent febrile convulsions
Anticonvulsants are not recommended for prevention of recurrent febrile convulsions