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Complex partial status epilepticus in young children.

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ORIGINAL ARTICLES
Complex Partial Status
Epilepticus in Young Children
M. C. McBride, MD, E. C. Dooling, MD, and E. Y . Oppenheimer, MD
Complex partial status epilepticus (CPSE) has rarely been described in children. We have recently studied four girls,
aged I to 4 years, who presented with CPSE. Their seizures were characterized by decreased level of consciousness,
lack of response to familiar persons, diminished response to pain, staring, slow visual tracking, eye deviation, picking at nearby objects, and lip smacking. Three patients developed focal clonic activity during their seizures and one
progressed to a generalized motor seizure after 4 hours of CPSE. Two patients had ictal electroencephalograms
demonstrating temporooccipital polyspikes and slow waves.
McBride MC, Dooling EC, Oppenheimer EY: Complex partial status epilepticus in young children.
Ann Neurol 9526-530, 1981
Complex partial status epilepticus (CPSE) has been
reported rarely in children [12, 151. The youngest
child described with CPSE has been a 5-year-old in
whom it occurred 1 hour after resection of a
craniopharyngioma [ 121. We have observed CPSE in
four children, aged 1 to 4 years, within a 6-month
period. Because of lack of information about this
type of seizure in children, the details of the episodes
and the 18-month follow-up of these four patients
are described.
Patient I
A 15-month-old girl was the product of an uncomplicated
pregnancy and was delivered by cesarean section because
of arrest of labor and fetal tachycardia. Apgar scores were 7
and 8. She had no postnatal difficulties, her development
was normal, and she had not sustained head trauma.
On the day of admission she was “feverish” and subdued.
While standing in the center of a room, she had begun to
reach out “as if groping for something.” Her legs “shivered” but she did not fall. When held, she had truncal
hypotonia and occasional jerks of her shoulders or arm.
She stared ahead without responding to her family. This
behavior persisted en route to the hospital, where her temperature was found to be 39.5”C. Her eyes remained open
and she watched movements made by her mother, but
did not call or reach out to her. She weakly resisted
venipuncture and lumbar puncture. Thirty minutes later
she developed lip smacking, her left arm twitched, and she
picked at the sheet with her right hand. Her head intermittently turned to the left. She stared ahead or had roving
eye movements or eye deviation to the left. She had irregular extensor posturing of the left arm and leg. After
From the Departments of Pediatrics and Neurology (Pediatric
Neurology), Boston University School of Medicine, Boston City
Hospital, Boston, MA 021 18.
Received July 14, 1980, and in revised form Nov 3. Accepted for
publication Nov 10, 1980.
1%hours in this state, she was given 150 mg of phenytoin
intravenously. Within 20 minutes she became responsive,
clutched her mother, cried, and made appropriate movements. Mildly increased tone, hyperactive reflexes, and a
left extensor plantar response persisted for the next 2
hours.
General physical and neurological examination later that
day as well as cerebrospinal fluid (CSF) examination were
normal. An electroencephalogram (EEG) obtained 36
hours later showed high-amplitude delta activity over the
posterior quadrants, predominantly on the right side. A C T
scan was normal. An EEG obtained 3 months afterward was
normal.
The child was maintained on phenobarbital and remained seizure free for one year, when she developed a
second episode of status epilepticus. This again was associated with fever, started with a dazed expression, became
generalized, continued for 1 hour, and was terminated by
intravenous phenytoin, intramuscular phenobarbital, and
rectal paraldehyde.
At 2% years of age the child continues to take phenytoin
and phenobarbital and has had no further seizures. She
speaks in three-word phrases appropriately but infrequently and has better receptive than expressive language abilities. Her cognitive, motor, and social skills are
age appropriate.
Patient 2
A 21/2-year-old girl was the product of an uncomplicated
full-term pregnancy, labor, and delivery. Her development
was normal and she had not sustained head trauma. One
hour before admission she developed a dazed look and
flexed posture of the right hip and knee. She sat unrespon-
Address reprint requests to Dr McBride, Division of Pediatric
Neurology, Strong Memorial Hospital, 60 1 Elrnwood Ave, Box
631, Rochester, NY 14642.
526 0364-5 134/81/060526-05$01.25 @ 1980 by the American Neurological Association
sively on the floor, motionless and staring ahead. In the
emergency room she followed simple commands but did
not speak or interact with her mother. While lying supine
on the litter, she postured her extremities bilaterally in
awkward positions or moved them slowly and deliberately.
She only weakly resisted painful procedures. Intermittently she picked at her clothing or at nearby objects. Her
eyes remained open and either followed the movement of
people in the room or stared ahead.
Because of the dystonic posturing, she was given
diphenhydramine intravenously. After showing a transient
increase in reactivity to her environment, she became less
responsive, resumed staring, and stopped moving except
for occasional picking. An EEG showed continuous right
temporal and occipital high-voltage spike, polyspike, and
slow wave discharges (Fig 1). The voltage of this activity
waxed and waned, and intermittently slow waves appeared
synchronously in the right temporal area and over the left
hemisphere. While phenytoin was being administered
intravenously, she began to have left arm and leg twitching
followed by generalized tonic-clonic seizure activity, and
the focal spike activity became generalized. Both clinical
and electrical seizure activity stopped within 3 minutes,
terminating the episode, which had lasted 4 hours. Postictally, the child remained drowsy for 30 minutes and had
left-sided hypotonia and hyperreflexia with neglect of the
left side for 2 hours.
Subsequent physical and neurological examinations were
unremarkable. She was playful, verbal, and develFig 1 . Ictal EEG in Patient 2, showing right temporooccipital
discharges recorded during quiet staring. (Calibration: 1 sec,
300 pu.)
T3 -T5
T5
-ol\\
opmentally advanced for her age. The CSF contained 36
noncrenated red blood cells per cubic millimeter and 86
mgldl of protein. A CT scan was normal. She was discharged on Phenobarbital therapy.
Three months after this episode, her mother discontinued the phenobarbital and the child developed a left
focal tonic-clonic seizure that continued for 30 minutes
until she was given intravenous phenobarbital. The CSF at
that time contained 28 mg/dl protein. Two months later an
EEG showed frequent spikes and spike and wave discharges independently in the left temporooccipital and
right posterior temporal regions. Five months later an EEG
showed frequent runs of spikes and slow waves arising
from the occipital regions bilaterally. She has had two more
seizures while on anticonvulsant therapy, each lasting 10 to
15 minutes and including prominent motor components.
She is currently taking mephobarbital. Her development
remains above normal for her age.
Patient 3
This girl was the product of an uncomplicated full-term
pregnancy and had no perinatal difficulties. Her development was normal and she had not sustained head trauma.
An older half-sibling had seizures. At 3% years of age the
patient experienced a seizure characterized by "eyes rolled
back into her head," rigid flexion of the arms, and extension of the legs. An EEG two days later showed intermittent high-voltage sharp waves in the right occipital area and
one burst of generalized high-voltage spike and slow wave
activity. Phenobarbital made her overactive, and sodium
valproate was substituted.
Two weeks later, two additional seizures occurred and
she was hospitalized. During the first episode, which lasted
FP2-F8
T3 -T5
#
McBride et al: Complex Partial Status Epilepticus 527
5 minutes, she stared ahead and her eyebrows twitched
while both arms and legs remained flaccid. One and a half
hours later, when playing normally, she developed staring
and eyebrow twitching with her legs alternately extended
or limp. This persisted for 35 minutes. O n arrival at the
hospital her general physical and neurological examinations
were normal. A serum valproate level was subtherapeutic.
One day after admission, she began staring into space
and smacking her lips. She was able to walk to her bed but
continued staring and began grasping and picking at her
clothing and nearby objects. Her eyes intermittently deviated to the right. Lip smacking alternated with other
mouthing movements. She was unresponsive to voice and
showed decreased responsiveness to venipuncture. This
behavior continued for 45 minutes. After receiving 3 mg of
diazepam intravenously, she fell asleep. Upon awakening 1
hour later, she appeared normal and answered questions
appropriately. An EEG obtained 48 hours after this
episode showed slowing, most prominently in the right
posterior quadrant. A CT scan with contrast was normal.
CSF protein was 12 mg/dl.
She has been maintained at therapeutic levels of sodium
valproate and has had no further seizures in the ensuing 16
months. Her development remains normal. An EEG 16
months after the episode of status epilepticus showed
slowing and paroxysmal high-voltage sharp activity in the
right posterior area.
Patient
times, speech development was slow. At age 3 years her
receptive and expressive language was at the 12- to 15month level. She had frequent upper respiratory tract and
ear infections. She had not sustained head trauma. A 14year-old sibling had had febrile convulsions as a toddler and
a cousin had Down's syndrome.
At age 4b$ years the child was hospitalized for assessment of developmental delay. Physical findings included
height at the twenty-fifth percentile, weight below the third
percentile, and head circumference of 47.5 cm, below the
third percentile. The tendon reflexes were symmetrically
hyperactive. She had a ten-word vocabulary and rarely
joined two words together. Her motor and cognitive skills
were two years delayed. She had a right hand preference.
An EEG showed background slowing, especially in the
right posterior quadrant.
Two days after admission she developed eye deviation to
the right and began smacking her lips. She remained seated
but did not respond to her name. A few minutes later her
eyes deviated to the left and she began picking at her
clothing with her left hand. Her eyes deviated to the right
again and the right side of her mouth twitched. An EEG
showed spikes, polyspikes, and slow waves in the left posterior temporal and occipital areas (Fig 2). The focal discharges persisted while her eyes deviated to the left with
left hand picking, followed by eyes in midposition with left
hand groping, and then eye deviation to the right with per-
4
This girl was the product of a full-term, uncomplicated
pregnancy. At delivery the umbilical cord was wrapped
around her neck twice, but her Apgar scores were 9 and 10
and she had no subsequent perinatal difficulties. Although
her gross motor milestones were achieved at the normal
Fig 2. lctal EEG in Patient 4, showing left posterior temporal
discharges recording during (A) left eye deviation with left
handpicking, (B) eyes in midposition with ldt band groping,
and (C) right eye deviation with lefi band groping. (Calibration: 1 sec, 300 pv.)
FP2-F8
F8 -T4
,
T4 -T6--F
T6 -02
1
B
A
528
Annals of Neurology
Vol 9
N o 6 June 1981
L
sistent left hand groping. Occasionally small-amplitude discharges were also seen in the right posterior temporal area.
One hour after the onset of her seizure, intravenous
diazepam abolished the spike activity and she fell asleep.
Postictally she was found to have a temperature of 39.5”C
associated with an upper respiratory tract infection. A CT
scan showed mild ventricular enlargement, CSF protein
was 10 mg/dl, and karyotype was normal. She was discharged on phenytoin therapy. A follow-up EEG showed
diffuse background slowing.
Eight months later, with fever and a respiratory infection, she had two seizures characterized by deviation of the
eyes to the right, extension of the arms above the head, and
tonic-clonic movements of the legs lasting 3 to 5 minutes.
Phenytoin was at therapeutic level, and phenobarbital was
added. One further seizure has occurred. Her EEG continues to show background slowing, predominantly in the
right posterior quadrant.
Discussion
Prolonged epileptic stupor and confusional states
have been well described. Usually these are episodes
of absence status [ l , 2 , 4 , 7, 11, 131, characterized by
evenly sustained impairment of consciousness or intellectual dysfunction, facial twitching or eye blinking, staring, and occasional motor automatisms. Less
frequently they are episodes of CPSE [2, 3 , 5, 6, 9,
11, 12, 14, 151, characterized by fluctuating impairment of consciousness, staring, paucity of speech,
and automatisms. Because the clinical features of
these two types of prolonged epileptic stupor overlap, ictal EEGs are necessary to establish an accurate
diagnosis. In absence status the ictal EEG shows
generalized spike and wave or polyspike discharges,
whereas in CPSE it shows focal discharges, usually in
the temporal or temporooccipital region.
The proposed criteria for diagnosis of CPSE thus
incorporate the ictal EEG findings. Belafsky et al [2]
defined CPSE, or psychomotor status, as recurrent
phases of: (1) a continuous twilight state with partial
and amnesic responsiveness and quasipurposeful
complex automatisms associated with bilateral or
unilateral slow waves on the EEG; and (2) staring,
total unresponsiveness, and stereotyped automatisms
associated with temporal lobe discharges on the EEG.
Mayeux and Lueders [12] described CPSE as an
“electroclinical syndrome of prolonged or repetitive
complex partial seizures (with continuous interictal
confusion) accompanied by electroencephalographic
seizure patterns, which are either focal (usually temporal iobe) or secondarily generalized from a focal
pacemaker.
The ictal recordings in our Patients 2 and 4 showed
discharges arising in the temporooccipital regions,
confirming the diagnosis of CPSE. Although Patients
1 and 3 did not have ictal EEGs, the focal clonic ac”
tivity and eye deviation during their episodes of
status epilepticus indicated a focal discharge. Moreover, EEGs obtained within 48 hours postictally in
these patients showed focal slowing in one posterior quadrant. Such focal slowing suggests localization of seizure activity during the preceding episode
of status epilepticus [ 101.
The features of CPSE in our four patients were
consistent. Each child had impaired consciousness
characterized by absence of vocalization, lack of interaction with familiar people, decreased reaction to
pain, intermittent staring alternating with wandering
eye movements or eye deviation, and intermittent
picking at clothing or nearby objects. Three of the
four children had lip smacking. Focal clonic motor
components occurred in three of the children, and
generalization to a full tonic-clonic seizure was seen
in one case. In each child the episode of CPSE was
terminated after 45 minutes to 4 hours by intravenous anticonvulsant medication (phenytoin in two
and diazepam in two). The abruptness of onset, abnormal ictal EEGs, response to anticonvulsants, and
normal laboratory values excluded basilar migraine,
encephalitis, metabolic imbalance, or emotional
reactions.
Nothing in the antecedent histories or neurological examinations of these four children explained
why they developed an uncommon type of prolonged
seizure. One child had mild perinatal difficulties,
another had a family history of seizures, and a third
was developmentally delayed, but these risk factors
do not distinguish them from children who develop
other types of seizures. None of the patients had a
history of head trauma or evidence for a focal structural lesion. High fever in two children may have
contributed to the onset of the seizure disorder, as it
has in other children with epilepsy. The variability of
the interictal EEG findings in these four patients is
similar to that described in a large series of children
with complex partial seizures of usual duration [8]. In
the course of 18 months of follow-up, three of the
four patients have had additional seizures despite
therapeutic serum levels of at least one anticonvulsant.
Because we have seen four cases in a 6-month
period, we suggest that CPSE occurs in young children more frequently than the paucity of reports in
the literature would indicate. Perhaps associated
motor components divert the attention of the
emergency room clinician and the complex symptomatology of such episodes is not recognized or recorded.
~~
~
Presented in part at the 8th Annual Meeting of the Child Neurology Society, Hanover, N H , Sept 13-15, 1979.
McBride et al: Complex Partial Status Epilepticus 529
We are grateful to Dr G. Erba for reviewing the EEGs, to Dr N.
Paul Rosman for reviewing the manuscript, to C. McNamara for
technical assistance, and to E. Berkman, B. Brockl, and D. Pel1 for
aid in preparing the manuscript.
7.
8.
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