Transcript Approach to Floppy Infant

Dr. AMINA H. Harharah
Non covulsive satus epilepticus
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Status Epilepticuc
Status epilepticus (SE) is a major clinical
problem,
frequently
occurring
in
childhood, with a high potential for
morbidity. The condition is subdivided
into convulsive and
nonconvulsive
forms (CSE and NCSE, respectively), and
the seizures can be generalized or partial.
.
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Non convulsive status epilepticus
is difficult to diagnose in children
as it can present with altered
mental status and change in
behaviour. It is important to rule
out other serious differential
diagnoses.
It may be one of the most
frequently missed diagnoses in
patients with altered neurologic
function..
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Historical
Background
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One of the first detailed accounts of
NCSE or ‘automatisme ambulatoire’,was
by Charcot in 1888, who described a
patient who got into trouble with the
law because he had boarded a train
without a valid ticket.
Lennox in 1949 described a boy
aged 11 years who had occasional days
of being confused, when he was able to
eat but not to converse. periods of what
he then termed ‘petit mal’ status
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NCSE represents a prolonged state
of seizure(s) (status epilepticus—
SE) without marked motor
manifestations. NCSE typically
encompasses an ictal impairment
of cognition, subtle facial or limb
twitches, eyes-open mutism, head
or eye deviation, automatisms, and
behavioral change.
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NCSE
has
traditionally
been
considered to occur less frequently and
to be less harmful than CSE. Increasing
evidence,
however,
indicates
that
NCSE is under diagnosed, particularly
in children, and might not be as benign
as was previously thought.
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NCSE has been defined as a cognitive
or behavioral change that lasts for at
least 30 minutes, with evidence of
seizures on electroencephalogram
(EEG). Some authors believe that a
more-precise definition of NCSE
should include "unequivocal
electrographic seizure activity,
rhythmic electrographic discharges
with clinical seizures, and clinical or
electrographic response to treatment".
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The recognition of a significant
cognitive or behavioral change is
often challenging, if not impossible,
in children, particularly in:
- Very young
- Children with pre-existing
Developmental delay
- Those with intractable recurrent
seizures treated with multiple
medications—all categories that
carry a relatively high risk for
NCSE.
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The following criteria have been
suggested for the diagnosis of NCSE
in children : "clear and persistent
clinical change in behavior (which
includes changes in cognition, memory,
arousal or motor behavior), confirmed
by comparisons with previous
functioning observations and/or by
neuropsychological examination, in the
presence of continuous paroxysmal
electrographic activity, and in the
absence of clonic, tonic or tonic–clonic
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seizures”
Classification
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Classification based on the age at
which NCSE occurs has recently been
suggested
1. NCSE occurring in the neonatal
and infantile epilepsy syndromes
1a. West syndrome
1b. Ohtahara syndrome
1c. Severe myoclonic
encephalopathy of infancy (SMEI;
Dravet syndrome)
1d. NCSE in other forms of
neonatal or infantile epilepsy
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2. NCSE occurring only in childhood
2a. NCSE in Early-onset benign childhood
occipital epilepsy (Panayiotopoulos
syndrome)
2b. NCSE in other forms of childhood
epileptic encephalopathies, syndromes
and etiologies, e.g., Ring chromosome X
and other karyotype abnormalities,
Angelman , Rett syndrome, myoclonicastatic epilepsy, other childhood
myoclonic encephalopathies.
2c. Electrical status epilepticus in slow
wave sleep (ESES)
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2d. Landau-Kleffner syndrome
3. NCSE occurring in both childhood
and adult life
With epileptic encephalopathy
3a. NCSE in the Lennox-Gastautsynd
i. Atypical absence status epilepticus
ii. Tonic–status epilepticus
3b. Other forms of NCSE in patients
with learning disability or disturbed
cerebral development (cryptogenic
or symptomatic)
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Without epileptic encephalopathy
3c. Typical absence status epilepticus
in idiopathic generalized epilepsy
3d. Complex partial status epilepticus:
3e. NCSE in the postictal phase of
tonic–clonic seizures
3f Subtle Status epilepticus (myoclonic
SE occurring in the late stage of
convulsive SE)
3g. Aura continua
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4. NCSE occurring in late adult life
4a. De novo absence status epilepticus
of late onset
5. Boundary syndromes
5a. Some cases of epileptic
encephalopathy.
5b. Some cases of coma due to acute
brain injury with epileptiform EEG
changes.
5c. Some cases of epileptic behavioral
disturbance or psychosis.
5d. Some cases of drug induced or
metabolic confusional
state with
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epileptiform EEG changes.
Classification
The term NCSE encompasses several
conditions that differ widely in their
etiology, prognosis and management:
Absence SE
Myoclonic SE
Complex partial SE
Simple partial SE
SE in coma
SE in patients with learning difficulties.
Atypical absence SE
Autonomic SE.Non covulsive satus epilepticus
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Absence status epilepticus
Absence SE is characterized by a
prolonged confusion state, but
agitation, violent behavior and
hallucinations can occasionally occur.
Associated automatisms, blinking, and
jerks of the face and limbs might also
be observed. Absence SE has a strong
tendency to recur.
In childhood, absence SE is almost
exclusively noted in patients with
known idiopathic generalized
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epilepsies.
Absencestatus epilepticus in a 6-year-old child who presented with unresponsiveness
with subtle twitching of the corner of the mouth. The electroencephalogram m
shows continuous rhythmic generalized spike-and-wave discharges with frontal predominance.
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FIG. 1. Rapid generalized epileptiform spikes and slow waves
following a few seconds of a normal EEG.
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Myoclonic status epilepticus
It is characterized by continuous,
usually generalized, myoclonias of
cortical origin. The inclusion of
myoclonic SE in the category of
nonconvulsive SE is controversial,
because myoclonias might be
considered as convulsions. Myoclonic
manifestations might be subtle,
however, and they frequently go
unnoticed in patients with chronically
disturbed cognitive
functioning
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Complex partial status epilepticus
Differentiation of complex partial SE from
absence SE is difficult to achieve on clinical
grounds alone. patients in complex partial SE
are confused, and clear lateralizing signs
might be missing. Criteria for the clinical
diagnosis of complex partial SE have been
summarized as follows: "recurrent complex
partial seizures without full recovery of
consciousness between seizures or
continuous 'epileptic twilight state' with
cycling between unresponsive and partially
responsive phases“..
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Symptoms of complex partial SE can
include amnesia, aphasia, bizarre
behavior and hemiparesis.
EEG is essential to make a correct
diagnosis: the ictal abnormalities are
focal and include spike-and-slow
waves, polyspikes and rhythmical
slowing. A secondary generalization
might occur, potentially leading to
diagnostic confusion.
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Periodic discharges of nonconvulsive status epilepticus following convulsive status
epilepticus
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Periodic discharges of nonconvulsive status epilepticus following
convulsive status epilepticus. A 10-year-old girl presented in convulsive status
epilepticus, and this electroencephalogram was obtained after convulsions
resolved but while she was still unresponsive. There are right hemisphere
periodic sharp waves with a slow repetition rate
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Periodic lateralized epileptiform discharges in nonconvulsive status epilepticus
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Bilateral PLEDs (rhythmic 1 Hz spikeand-wave discharges) in a 12-year-old girl.
3
Fp1–F3
F3–C3
C3–P3
P3–O1
Fp2–F4
F4–C4
C4–P4
P4–O2
Fp1–F7
F7–T3
T3–T5
T5–O1
Fp2–F8
F8–T4
T4–T6
T6–O2
Fz–Cz
Cz–Pz
EKG–Ref
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NCSE with learning difficulties
It is diagnosed mainly in children. It
encompasses several pediatric
conditions that share variable degrees
of developmental delay and continuous
or near-continuous electrographic
epileptic activity.
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It is commonly divided into electrical
SE during sleep (ESES), atypical
absence SE, and tonic SE.
ESES is characterized by diffuse 1.5–
3.5 Hz spike-and-wave discharges
occurring in 85–100% of non-rapid eye
movement sleep, usually associated
with various pediatric epilepsy
syndromes, such as typical or atypical
benign epilepsy with centrotemporal
spikes, Landau–Kleffner syndrome, and
epileptic encephalopathy with
continuous spike-and-wave
discharges
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during sleep.
Autonomic status epilepticus
Most cases of autonomic SE have been
observed in Panayiotopoulos syndrome,
the most important clinical
manifestations of which include
nausea, vomiting, pupillary
abnormalities, and cardiorespiratory
and thermal alterations. Despite the
fact that some of these episodes end in
convulsions, they are considered to be
autonomic NCSE because of the large
predominance Non
ofcovulsive
vegetative
features.31
satus epilepticus
Electrographic
criteria for
nonconvulsive
status epilepticus.
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•Frequent or continuous focal electrographic
seizures with ictal patterns that change in
amplitude frequency or localization
•Frequent or continuous generalized spikeand-waves in patients without a prior history
of epilepsy
•Frequent or continuous generalized spikeand-waves, significantly different in
amplitude or frequency as compared to
previous findings, in patients with a history of
epileptic encephalopathy
Periodic lateralized epileptiform discharges
(PLEDs) in comatose patients after convulsive
status epilepticus
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Conditions
associated with
NCSE in children
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Although rare, NCSE is found in a wide
variety of situations in childhood these
conditions can be grouped in three
separate categories:
1- Acute neurological injuries
2- Specific childhood epilepsy
syndromes with prominent tendency
for NCSE
3- Nonprogressive encephalopathies
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Acute neurological injuries:
NCSE in children often occurs as a
consequence of one of a variety of
Acute or subacute neurological
injuries, such as stroke, encephalitis.
Exacerbation of a chronic metabolic
encephalopathy.
Trauma
Intoxication.
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Specific epilepsy syndromes
Children with epilepsy are at risk of
presenting with NCSE for two main
reasons.
1- Certain antiepileptic drugs, such as
levetiracetam, lamotrigine, valproate
and tiagabine, have been associated
with NCSE. Tonic SE induced by
benzodiazepines, sometimes subtle
enough to be considered as a
nonconvulsive.
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2- Several pediatric epilepsy
syndromes show a prominent
tendency for NCSE. These include
Dravet syndrome, myoclonic–
astatic epilepsy, malignant
migrating partial seizures in
infancy, and LGS.
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Dravet syndrome (severe myoclonic
epilepsy in infancy)
Is a severe form of epilepsy that begins in
infancy. It is associated with mutations in the
gene that encodes the sodium channel.
Infants with Dravet syndrome often have
prolonged febrile convulsions at disease
onset, but afebrile focal seizures, atypical
absence seizures and myoclonic seizures
might follow at a later stage. NCSE occurs in
at least 40% of cases usually after the age of
2 years.
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Myoclonic–astatic epilepsy
Is characterized by isolated
myoclonias and epileptic falls or head
drops, often preceded by subtle
myoclonias of the limbs, that begin in
infancy. NCSE frequently occurs in this
condition, and takes the form of stupor
associated with perioral or distal
muscle twitching and repeated head
nods, all lasting for several hours.
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Malignant migrating partial seizures in
infancy
Infants with this condition develop
normally before the onset of seizures,
which usually begin during the first 6
months of life. The seizures are focal
and nearly continuous, and they shift
from one area of the brain to the next,
giving the appearance of migration.
Clinical manifestations can be very
subtle, with behavioral arrest.
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Although not often formally
described as NCSE, patients with
this syndrome have prolonged and
nearly continuous electroclinical
seizures with subtle manifestations
and altered responsiveness.
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Lennox–Gastaut syndrome
Is characterized by multiple seizure
types, almost invariably including
nocturnal tonic seizures and atypical
absences, in patients with severe
developmental delay and interictal
diffuse slow spike-and-wave discharges
on EEG.
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NCSE occurs in 50–75% Subtle
tonic SE with progressive
attenuation of motor manifestations
and prolonged obtundation with
myoclonias are two forms of NCSE
also observed in this syndrome.
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Nonprogressive encephalopathies
Myoclonic SE can occur in various
nonprogressive encephalopathies
one of the best-recognized being
Angelman syndrome.
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Angelman syndrome
is a genetically determined disorder,
Children with this condition have:
Developmental delay
Mild facial dysmorphism
Ataxia
Hypotonia
Cheerful disposition
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The interictal EEG often shows a
characteristic pattern that is
sometimes referred to as 'notched'
delta. The pattern can occur without
any clear clinical manifestations, but
affected children might have
prolonged periods of altered
responsiveness with admixed
myoclonias and eye blinking timelocked with the rhythmic EEG activity.
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Ring chromosome 20 epilepsy
syndrome
Is a rare entity, the most important
clinical characteristics of which include
repeated episodes of NCSE. onset of
seizures ranged from 1 day to 14 years.
Most of the patients had convulsions
that were very difficult to treat in
addition to prolonged confusional
states.
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EEG show asymmetric high-voltage
slow waves with occasional spikes,
predominantly in frontal regions
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Conclusion
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Cognitive or behavioral change for at least
30 minutes, with evidence of seizures on
electroencephalogram
•The diagnosis is difficult and requires a
high index of suspicion
• Lack of agreement on diagnostic criteria
•Observed in various childhood epilepsy
syndromes, encephalopathies and acute
conditions
• Data scarce regarding diagnosis, treatment
and outcome .
Further studies analyzing the EEG features
and clinical presentation, especially the very
young, are needed
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Criteria for Diagnosis of NCSE
1.Diminished level of consciousness
or other neurologic deficit
2.EEG: typical discrete seizures or
continuous discharges
3.Response to anticonvulsants:
clinical and EEG (controversial,
often after long delay)
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