Professional Documents
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Status epilepticus:
- series of seizures at intervals too brief to allow the child to
regain consciousness between the time one event ends and the
next begins.
Postictal state:
- appears to relax
- remain semiconscious and difficult to arouse
- may awaken in a few minutes
- remains confused for several hours
- poor coordination
- mild impairment of fine motor movements
- may vomit or complain of severe headache
- when left alone, usually for several hours
- awakening is fully conscious
▪ Absence Seizures (Petit Mal or Lapses)
- onset between 4 and 12 years of age
- common in girls than boys
- usually cease at puberty
- brief loss of consciousness
- no alteration in muscle tone
- abrupt onset, develops 20 or more attacks daily
- mistaken for inattentiveness or daydreaming
- precipitated by hyperventilation, hypoglycaemia, stresses,
fatigue, or sleeplessness.
- brief loss of consciousness
- without warning or aura
- last 5 to 10 seconds
- loss of muscle tone; child drop objects
- maintain postural control; seldom falls
- lip smacking, twitching of eyelids or face, slight hand
movements
- not accompanied by incontinence
- amnesia for episode
- need to reorient self to previous activity
▪ Atonic and Akinetic Seizures (Drop Attacks)
- onset: 2 and 5 years of age
- sudden, momentary loss of muscle tone and postural
control
- events recurring frequently during the day,
particularly in the morning hours and shortly after
awakening
- loss of tone causing child to fall to the floor
violently, unable to break fall by putting out hand, may
incur a serious injury to the face, head, or shoulder
- loss of consciousness
▪ Myoclonic Seizures
- variety of seizures episodes
- isolated as benign essential myoclonus
- sudden, brief contractures of a muscle or group of
muscles
- occur singly or repetitively
- no postictal state
- may or not be symmetric
- may or may not include loss of consciousness
▪ Infantile Spasms
- also called infantile myoclonus, massive spasms, hypsarrhytmia,
salaam episodes, or infantile myoclonic spasms
- occur during the 1st 6 to 8 months of life
- numerous seizures during the day without postictal drowsiness
or sleep
- poor outlook for normal intelligence
- possible series of sudden, brief, symmetric, muscular
contractions
- head flexed, arms extended, and legs drawn up
- eyes sometimes rolling upward or inward
- preceded or followed by a cry or giggling
- may or may not include loss of consciousness
- flushing, pallor, or cyanosis
Diagnostic Evaluation
1. Determining whether epilepsy or seizures exist and not an
alternative diagnosis.
2. Defining the underlying cause, if possible.
3. Complete and accurate history or description of child’s
behaviour during the event, the age of onset, and the
time at which the seizure occurs.
4. Complete physical and neurologic examination –
developmental assessment of language, learning
behaviour, and motor abilities.
5. CT to detect a cerebral haemorrhage, infarctions, and
gross malformations.
6. MRI provides greater anatomic detail and is used to detect
developmental malformations, tumors, and cortical
dysplasias.
7. EEG – most useful tool for evaluating a seizure disorder. It
confirms the presence of abnormal electrical discharges and
provides information on the seizure type and the focus.
Carried out under varying conditions :
- child asleep, awake, awake with provocative
stimulation (flashing lights, noise) and hyperventilating.
- stimulation may elicit abnormal electrical activity,
which is recorded on the EEG.
- high voltage spike discharges – tonic-clonic seizures
with abnormal patterns in the intervals between seizures.
- three-per-second spike and wave pattern is observed in
an absence seizure.
- absence of electrical activity in an area suggest a large
lesion, such as an abscess or subdural collection of fluid.
- normal EEG does not rule out seizures because the EEG
is only a surface recording and only represents
approximately 1 hour of time and , therefore, may show
normal interictal activity.
Therapeutic Management
the goal of treatment :
- to control the seizures
- reduce their frequency and severity
- discover and correct the cause when possible
- help the child live as normal a life as possible
Four treatment options:
- drug therapy
- ketogenic diet
- vagus nerve stimulation
- epilepsy surgery
❖ Drug therapy
- antiepileptic drugs – reducing the responsiveness of
normal neurons to the sudden high-frequency nerve impulses
that arise in the epileptogenic focus. Thus the seizure
activity is suppressed.
- Carbamazepine
- Phenytoin
- Phenobarbital
- Gabapentin
- Clonazepam
- Therapy is begun with a single drug known to be effective
and have the lowest toxicity, that is the safest side effect
profile for the child’s particular type of seizure.
- The dosage is gradually increased until the seizures are
controlled or the child develops side effects.
- If the drug is effective but does not sufficiently control
the seizures, a second drug is added gradually increasing
doses.
- One seizures are controlled, the first drug may be tapered
to reduce the potential adverse effects of polytherapy.
- Measurements of the blood levels of the drug is important
id the seizures continue once the child is on a therapeutic
dose of medication, to adjust the dosage, and to assist in
determining which medication may be causing the side
effects if the child is on multiple antiepileptic
medications.
- Some possible causes of low serum blood concentrations
are noncompliance, poor absorption, and drug
interactions.
- The dosage needs to b increased if the child as the child
grows.
- If complete seizure control is maintained on an anticonvulsant
drug for 2 years, it is safe for patients with no risk factors to
discontinue the drug.
- Risk factors: age greater than 12 years at onset, history of
neonatal seizures, numerous seizures before control is
achieved,a nd presence ofa aneurologic dysfunction.
- Children whose medications are discontinued will experience
seizure recurrence.
- Recurrence occurs most frequently within 6 months of
discontinuation.
- When seizure medications are discontinued, the dose is
decreased gradually over several weeks.
- Sudden withdrawal of a drug is not recommended because
it can cause an increase in the number and severity of
seizures.
❖ Ketogenic diet
- high fat, low carbohydrate, and low protein diet.
- consumption of such a diet forces the body to shift from
using glucose as the primary energy source to using fat, and
the individual develops a state of ketosis.
- the diet is deficient in vitamins, vitamins supplements
are necessary.
- the ketogenic diet may be effective in controlling
seizures. A multicentre study of the efficacy of the ketogenic
diet showed that approximately 50% of the patients had a
greater than 50% reduction in seizure frequency.
❖ Vagus nerve stimulation
- uses an implantable devise that reduces seizures in individual who have
not had effective control with drug therapy.
- indicated as adjunctive therapy in patients 12 years and older with partial
onset seizures.
- a programmable signal generator is implanted subcutaneously in the
chest.
- electrodes tunnelled underneath the skin deliver electrical pulses to the
left vagus nerve (CN X)
- the device is programmed noninvasively to deliver a presice pattern of
stimulation to the left vagus nerve.
- the patient or caregiver can activate the device using a magnet at the
onset of a seizure.
❖ Surgical therapy
- when seizures are determined to be caused by a hematoma, tunor or
other cerebral lesion, surgical removal is the treatment.
- in children with epilepsy, surgery ir reserved for those who suffer from
incapacitating, refractory seizures.
- refractory seizures are usually defines as the persistence of seizures
despite adequate trials of three antiepileptic medications, alone or in
combination .
- types of surgical interventions:
o Resective surgery – the focal area of the seizure activity is excised
with the expectation that the surgery will not produce serious
deficits or increase existing deficits.
o Hemespherectomy to remove all or most of one hemisphere is
typically used in patients who have severe epilepsy and who
already have hemiparesis or non-functional hand use. Patient
with Rasmussen syndrome, Sturge-Weber syndrome, or
hemimeganencephaly may benefit from this procedure.
o Colpuscallosotomy involves the separation of the connections
between the two hemispheres of the brain and is used in
some generalized seizures.
o Multiple sub-pial transection, horizontal fibers of the motor
cortex are divided to reduce seizures, whereas the vertical
fibers are spared to allow for function.
Therapeutic Management for Status
Epilepticus
o status epilepticus is a continuous seizure that lasts more than 30
minutes or a series of seizures from which the child does not regain a
premorbid level of consciousness.
o Rectal diazepam is a simple, effective, and safe treatment for home
or prehospital management.
o Available in a prefilled rectal gel syringe for easy administration.
o In-hospital management of status epilepticus – Iv diazepam or
lorazepam (Ativan) is the first-line drug of choice.
o Valproic acid given rectally or IV.
o When diazepam is ineffective, fosphenytoin or phenobarbital is given
IV as the next line of treatment.
Prognosis
Most children who experience a second seizure will experience
additional seizures.
Therefore a history of two seizures is sufficient to diagnose
epilepsy.
Etiology and specific epilepsy syndromes are the most important
factors affecting prognosis.
Children who have mental retardation or cerebral palsy are at
highest risk for developing epilepsy.
Convulsive status epilepticus is convincingly related to serious
morbidity and mortality and is often associated with a severe
neurologic abnormality, uncontrolled seizure disorder, or
concurrent serious illness or infection.
In one study, children who suffered status epilepticus had
neurologic abnormalities.
The highest morbidity was in patients with nonidiopathic ,
nonfebrile cause, whereas children with idiopathic or febrile
status epilepticus had a more favourable outcome.
Nursing Care Management
Important nursing responsibility: to observe the seizure episode and
accurately document the events.
Observations during seizure
o Describe
- order of events (before, during, and after)
- duration of seizure
* tonic-clonic – from first signs of event until jerking stops
* Absence – from loss of consciousness until consciousness is re-
gained
* complete partial – from first sign of unresponsiveness, motor
activity, automatisms until there are signs of responsiveness to
environment.
o Onset
- time of onset
- significant precipitating events – missed medication dosage,
illness, stress, sleep deprivation, menses.
o Behavior
- change in facial expression
- cry or other sound
- stereotypic or autonomous movements
- random activity (wandering)
- position of eyes, head, body, extremities
- unilateral or bilateral posturing of one or more extremities.
o Movement
- change of position
- site of commencement – hand, thumb, mouth, generalized
- tonic phase – length, parts of body involved
- clonic phase – twitching or jerking movements, parts of body
involved, sequence of parts involved, generalized, change in character
of movements
- lack of movements or muscle tone of body part or entire body
o Face
- Color change – pallor, cyanosis, flushing
- Perspiration
- Mouth – position, deviating to one side, teeth clenched,
tongue bitten, frothing at mouth, flecks of blood or
bleeding
- Lack of expression
- Asymmetric expression
o Eyes
- position – straight ahead, deviation upward or outward,
conjugate or divergent gaze
- pupils – change in size, equality, reaction to light
o Respiratory effort
- presence and length of apnea
o Other
- incontinence
Postictal observations
- duration of postictal period (After the tonic–clonic period, the
child falls into a sound sleep, called the postictal period)
- state of consciousness
- orientation
- arousability
- motor ability
*any change in motor
*ability to move all extremities
*paresis or weakness
- speech
- sensations
*complaint of discomfort or pain
*any sensory impairment
*recollection of preseizure sensations aura
The child must be protected from injury during the seizure;
nursing observations made during the event provide valuable
information for diagnosis and management of the disorder.
The nurse must remain calm, stay with the child, and
prevent the child from sustaining any harm during seizure.
Provide privacy.
Placing the child o the side facilitates drainage and helps
maintain a patent airway.
Suctioning may be necessary.
Monitor vital signs.
MYASTHENIA GRAVIS
MYASTHENIA GRAVIS
Uncommon in childhood.
Juvenile MG appears to be identical to that seen in adults
and usually ha sits onset after age 10 years, but may
appear as early as age 2 years.
Juvenile and adult forms of the disease are autoimmune
disorders associated with the attack of circulating
antibodies on the acetylcholine receptors on the muscle
end plate, which blocks their function, causing muscle
weakness.
NEONATAL MYASTHENIA GRAVIS
Transientform of MG occurs to 10% to 20% of infants born to
mothers with MG, who may not know they have the disease.
Themuscular weakness results from transplacentally acquired
maternal acetylcholine receptor antibodies.
Infants display generalized muscle weakness and hypotonia at
birth with depressed Moro reflex, ptosis, ineffective sucking
and swallowing reflexes, and weak cry.
Symptoms may be evident within a few hours after birth,
following a period of normal appearance after delivery.
Cholinesteraseinhibitors may be given o a short-term basis to
improve feeding ability.
Symptoms usually disappear within 2 to 3 weeks.
Infantswith transient neonatal MG regain strength once
maternal antibodies clear the system, and they are not at
increased risk of MG later in life.
CONGENITAL MYASTHENIA GRAVIS
Familial
abnormality of neuromuscular transmission that is
not immunologically mediated.
Appears as transient form, but the mother usually does not
have the disease.
Persiststhroughout life, and more than one sibling may be
affected, which suggests a genetic etiology.
Resistant to drug therapy.
Eyelidand extraocular muscles seem to be the muscles most
severely affected.
Clinical Manifestations
General paralysis of the optic muscles with ptosis and
diplopia (common symptoms).
Difficulty in swallowing, chewing, and speaking
accompanied by weakness and paralysis of all skeletal
muscles.
The signs and symptoms are more pronounced in the late
afternoon and evening.
Relieve by rest and made worse by exercise and stress.
Diagnostic Evaluation
Made on the basis of the characteristic distribution of muscle
weakness and the progressive weakness on repeated r sustained
muscular contraction.
EMG - definitive diagnosis, which demonstrates a decrease in
muscle potentials with repetitive nerve stimulation.
Clinical diagnosis may be established by observation of the
response to the anticholinesterase drugs.
IV administration of a small test dose of edrophonium (Tensilon)
produces a beneficial effect in 1 minute, but the effect lasts less
than 5 minutes.
Electrophysiologic studies – help document transmission
failure at the neuromuscular junction.
Antibodies to human muscle acetylcholine are detected in the
serum.
Therapeutic Management
Administration of cholinesterase inhibiting drugs (neostigmine –
Prostigmin) IM or as oral neostigmine bromide.
Pyridostigmine (Mestinon) – less toxic.
Starting dose of neostigmine is usually 0.04mg/kg, administered
IM every 4 to 6 hours.
Dosage is gradually increased until a satisfactory result is
obtained.
The child must be observe for sins of parasympathetic
stimulation from overmedication. (lacrimation, salivation,
abdominal cramps, sweating, diarrhea, vomiting, bradycardia,
and weakness of respiratory muscles).
Thymectomy (removal of the thymus) - not effective for familial and
congenital forms of MG.
Plasmapheresis
Long term corticosteroid treatment
IV immunoglobulin.