Fandi

405110174

LO 1 SEIZURE

Definition
instantaneous loss of consciousness, alteration of
perception or impairment of psychic function, convulsive
movements, disturbance of sensation, or some combination
thereof
Convulsion
an intense paroxysm of involuntary repetitive muscular
contractions, is inappropriate for a disorder that may
consist only of an alteration of sensation or consciousness
Seizure
preferable as a generic term, since it embraces a diversity
of paroxysmal events and also because it lends itself to
qualification

Etiology
Primary neurologic
disorders

Systemic disorders

Benign febrile convulsions of

childhood
Idiopathic/cryptogenic seizures
Cerebral dysgenesis
Symptomatic epilepsy
Head trauma
Stroke or vascular malformations
Mass lesions
CNS infections
Encephalitis
Meningitis
Cysticercosis
HIV encephalopathy

Hypoglycemia
Hyponatremia
Hyperosmolar states
Hypocalcemia
Uremia
Hepatic encephalopathy
Porphyria
Drug toxicity
Drug withdrawal
Global cerebral ischemia
Hypertensive encephalopathy
Eclampsia
Hyperthermia

Pathophysiology

Mechanisms of Seizure Initiation and Propagation

Initiation phase
high-frequency bursts of action potentials
relatively long-lasting depolarization of the neuronal
membrane due to influx of extracellular calcium
opening of voltage-dependent sodium (Na +) channels
influx of Na+ repetitive action potentials
Hypersynchronization
Followed by hyperpolarizing afterpotential mediated
by G-aminobutyric acid (GABA) receptors or
potassium (K+) channels, but it is not functioning
well synchronized bursts from a sufficient number
of neurons spike discharge on the EEG

Classification
Partial / focal
seizures
Simple
consciousness is
undisturbed

Complex
consciousness is
altered

Generalized seizures
Convulsive seizures
Tonic-clonic (grand mal)
seizures
Less common
Purely tonic, purely
clonic, generalized
clonic-tonic-clonic

Non-convulsive seizures
brief lapse of
consciousness or absence
(petit mal)
minor motor phenomena
(brief myoclonic, atonic,
or tonic seizures)

Partial Seizure

SIMPLE PARTIAL SEIZURE

 Simple partial seizures begin with motor, sensory, or

autonomic phenomena, depending on the cortical region
affected.
For example, clonic movements of a single muscle group
in the face, a limb, or the pharynx may occur and may be
self-limited; they may be recurrent or continuous or may
spread to involve contiguous regions of the motor cortex
(jacksonian march).
Autonomic symptoms may consist of pallor, flushing,
sweating, piloerection, pupillary dilatation, vomiting,
borborygmi, and hypersalivation.
Psychic symptoms include distortions of memory (e.g.,
d j vu, the sensation that a new experience is being
repeated), forced thinking or labored thought processes,
cognitive deficits, affective disturbances (e.g., fear,
depression, an inappropriate sense of pleasure),
hallucinations, or illusions

 During simple partial seizures, consciousness is

preserved unless the seizure discharge spreads
to other areas of the brain, producing tonicclonic seizures (secondary generalization).
The aura is the portion of the seizure that
precedes loss of consciousness and of which the
patient retains some memory.
The aura is sometimes the sole manifestation of
the epileptic discharge.
In the postictal state, a focal neurologic deficit
such as hemiparesis (Todd paralysis) that
resolves over a period of 30 minutes to 36 hours
is a manifestation of an underlying focal brain
lesion.

COMPLEX PARTIAL
SEIZURE

 Complex partial seizures, formerly called psychomotor

seizures, are partial seizures in which consciousness,
responsiveness, or memory is impaired.
The seizure discharge usually arises from the temporal
lobe or medial frontal lobe but can originate
elsewhere.
The symptoms take many forms but are usually
stereotyped for the individual patient.
Episodes may begin with an aura. Epigastric
sensations are most common, but affective (fear),
psychic (d j vu), and sensory (olfactory
hallucinations) symptoms also occur.
Consciousness is then impaired. Seizures generally
persist for less than 30 minutes (on an average, 1-3
minutes).

 The motor manifestations of complex

partial seizures are characterized by
coordinated involuntary motor activity,
termed automatism, which takes the
form of orobuccolingual movements in
approximately 75% of patients and
other facial or neck or hand movements
in approximately 50%. Sitting up or
standing, fumbling with objects, and
bilateral limb movements are less
common.
Secondary generalization may occur

Generalized Seizure

GRAND MAL SEIZURE

Grand Mal Seizure

Generalized tonic-clonic seizures are
attacks in which consciousness is
lost, usually without aura or other
warning. When a warning does occur,
it usually consists of nonspecific
symptoms.

Tonic Phase
The initial manifestations are unconsciousness
and tonic contractions of limb muscles for 1030 seconds, producing first flexion and then
extension particularly of the back and neck
Tonic contraction of the muscles of respiration
may produce an expiration-induced
vocalization (cry or moan) and cyanosis, and
contraction of masticatory muscles may cause
tongue trauma.
The patient falls to the ground and may be
injured.

Clonic Phase
The tonic phase is followed by a clonic (alternating
muscle contraction and relaxation) phase of
symmetric limb jerking that persists for an
additional 30-60 seconds, or longer.
Ventilatory efforts return immediately after
cessation of the tonic phase, and cyanosis clears.
The mouth may froth with saliva.
With time, the jerking becomes less frequent, until
finally all movements cease and the muscles are
flaccid.
Sphincteric relaxation or detrusor muscle
contraction may produce urinary incontinence.

Recovery
As the patient regains consciousness, there is postictal
confusion and often headache.
Full orientation commonly takes 10-30 minutes, or
even longer in patients with status epilepticus (see
below) or preexisting structural or metabolic brain
disorders.
Physical examination during the postictal state is
usually otherwise normal in idiopathic epilepsy or
seizures of metabolic origin, except that plantar
responses may be transiently extensor (Babinski sign).
The pupils always react to light, even when the patient
is unconscious.

Status Epilepticus
Status epilepticus is defined arbitrarily as
seizures that continue for more than 30
minutes without ceasing spontaneously, or
which recur so frequently that full
consciousness is not restored between
successive episodes.
Status epilepticus is a medical emergency
because it can lead to permanent brain
damage, from hyperpyrexia, circulatory
collapse, or excitotoxic neuronal damage, if
untreated.

PETIT MAL SEIZURE

 Absence (petit mal) seizures are genetically

transmitted seizures that always begin in
childhood and rarely persist into adolescence.
The spells are characterized by brief loss of
consciousness (for 5-10 seconds) without loss of
postural tone.
Subtle motor manifestations, such as eye
blinking or a slight head turning, are common.
Automatisms are uncommon.
Full orientation immediately follows cessation of
the seizure.

 There may be as many as several hundred spells daily,

leading to impaired school performance and social
interactions, so that children may be mistakenly thought to
be mentally retarded before the diagnosis of petit mal
epilepsy is made.
The spells are characteristically inducible by hyperventilation.
The electroencephalogram (EEG) shows a characteristic 3/s
spike-and-wave pattern during the seizures.
In most patients with normal intelligence and normal
background activity on EEG, absence spells occur only during
childhood; in other cases, however, the attacks continue into
adult life, either alone or in association with other types of
seizures.

OTHER TYPE OF
GENERALIZED SEIZURE

Tonic Seizure
Tonic seizures are characterized by
continuing muscle contraction that can
lead to fixation of the limbs and axial
musculature in flexion or extension
and are a cause of drop attacks; the
accompanying arrest of ventilatory
movements leads to cyanosis.
Consciousness is lost, and there is no
clonic phase to these seizures

Clonic Seizures
Clonic seizures are characterized by
repetitive clonic jerking accompanied
by loss of consciousness. There is no
initial tonic component.

Myoclonic Seizures
Myoclonic seizures are characterized by sudden, brief,
shocklike contractions that may be localized to a few
muscles or one or more extremities or that may have
a more generalized distribution.
Juvenile Myoclonic Epilepsy (JME) is the most common,
with onset usually in adolescence.
There is a family history of seizures in one-third.
Myoclonic seizures may be idiopathic or associated
with a variety of rare hereditary neurodegenerative
disorders, including Unverricht-Lundborg disease,
Lafora body disease, neuronal ceroid lipofuscinosis
(late infantile, juvenile, and adult forms), sialidosis,
and mitochondrial encephalomyopathy (myoclonus
epilepsy with ragged red fibers on skeletal muscle
biopsy).

Atonic Seizures
Atonic seizures result from loss of
postural tone, sometimes following a
myoclonic jerk, leading to a fall or
drop attack. They are most common
in developmental disorders such as
the Lennox-Gastaut syndrome.

EPILEPSY

Epilepsy
Definition
Condition of reccurent unprovoked seizures
an intermittent derangement of the nervous system due
to an excessive and disorderly discharge of cerebral
nervous tissue on muscles (Hughlings Jackson, 1870)
Sudden alteration of central nervous system (CNS)
function resulting from a paroxysmal high-frequency or
synchronous low-frequency, highvoltage electrical
discharge
discharge arises from an assemblage of excitable neurons in
any part of the cerebral cortex / secondarily involved
subcortical structures as well
there need not be a visible lesion

 Etiology
Role of hereditary

 Clinical approach
Is it indeed a seizure?
Hows the clinical & EEG pattern & other characteristics?
Whats the underlying cause?
In the diagnosis of epilepsy history is the key
The examination in children & infant greater value
Finding of dysmorphic & cutaneus abnormalities highly
characteristic cerebral disease that give rise to epilepsy

Some lab studies (CBC, blood chemistries, liver & thyroid

function tests, EEG, imaging studies of brain: MRI, CT for
emergency & very young children)

KEJANG DEMAM

Definition & classification

occur between the age of 6 and 60 mo
temperature of 38C or higher
not the result of central nervous system infection or any metabolic
imbalance
occur in the absence of a history of prior afebrile seizures
Simple febrile seizure
primary generalized; tonic-clonic
attack associated with fever
lasting for a maximum of 15 min
not recurrent within a 24-hour period

Complex febrile seizure

Focal; more prolonged (>15 min)
recurs within 24 hr

Febrile status epilepticus febrile seizure lasting > 30 minutes

Epidemiology
2-5% of neurologically healthy infants and
children experience at least 1, usually
simple febrile seizure
Complex febrile seizure mortality (>2x)
recurrent simple febrile seizures do not
damage the brain
Reccurency
30% of those experiencing a first episode
50% after 2 or more episodes
50% of infants <1 yr old at febrile seizure onset

 Risk factor for recurrency

Genetic factors
Predicted polygenic factors
Identified single genes FEB 1, 2, 3, 4, 5, 6, and 7 genes
on chromosomes 8q13-q21, 19p13.3, 2q24, 5q14-q15,
6q22-24, 18p11.2, and 21q22
Function of FEB 2 a sodium channel gene (SCN1A)

GEFS+ (generalized epilepsy with febrile seizures plus)

autosomal dominant syndrome with a highly variable phenotype
Onset is usually in early childhood and remission is usually in
mid-childhood
Multiple febrile seizures and several types of afebrile
generalized seizures (generalized tonic-clonic, absence,
myoclonic, atonic, or myoclonic astatic seizures)

 Dravet syndrome
Etiology
new mutation (2q24-31 and encodes for SCN1A); inherited in an
autosomal dominant manner

most severe of the phenotypic spectrum of febrile seizures

plus
onset is in the 1st yr of life
febrile and afebrile unilateral clonic seizures recurring every
1 or 2 mo
Induced by fever more prolonged, more frequent, come in
clusters
2nd year of life myoclonus, atypical absences, and partial
seizures occur frequently and developmental delay usually
follows

Work up & evaluation

Febrile seizures often
occur in the context of
otitis media, roseola and
human herpesvirus 6
(HHV6) infection,
shigella, or similar
infections

 Lumbar puncture
children <12 mo of age after their first febrile seizure;
to rule out meningitis
children >18 mo of age, a lumbar puncture is
indicated in the presence of clinical signs and
symptoms of meningitis

EEG
delayed until or repeated after >2 wk have passed
(because if < 2 wk nonspecific slowing)
performed for at least 30 min in wakefulness and in
sleep according to international guidelines

Blood studies
Neuroimaging
CT or MRI is not recommended in evaluating the child
after a first simple febrile seizure

Treatment
antiepileptic therapy, continuous or intermittent, is not
recommended for children with one or more simple febrile seizure
If the seizure lasts for >5 min acute treatment with diazepam,
lorazepam, or midazolam
recurrence of febrile seizure lasting >5 min rectal diazepam
Febrile status epilepticus Intravenous benzodiazepines,
phenobarbital, phenytoin, or valproate
< risk of febrile seizure
intermittent oral diazepam can be given during febrile illnesses (0.33
mg/kg every 8 hr during fever)
Intermittent oral nitrazepam, clobazam, and clonazepam (0.1 mg/kg/day)
intermittent diazepam prophylaxis (0.5 mg/kg administered as a rectal
suppository every 8 hr)
Phenobarbital (4-5 mg/kg/day in 1 or 2 divided doses)
Valproate (20-30 mg/kg/day in 2 or 3 divided doses)

 Antipyretics < discomfort; not <

risk for febrile seizure
Chronic antiepileptic therapy may be
considered for children with a high
risk for later epilepsy
Iron deficiency has been shown to be
associated with an increased risk of
febrile seizures

Reference
Adams & Victors principle of
neurology; 8th & 9th edition
Harrisons principle of internal
medicine; 17th edition
Nelsons pediatric; 19th edition
Clinnical Neurology 7th edition