S Staring spells in children: Descriptive features

distinguishing epileptic and nonepileptic events

Felix Rosenow, MD, Elaine Wyllie, MD, Prakash Kotagal, MD, Ed Mascha, MS,
Barbara R. Wolgamuth, REEGT, and Hajo Hamer, MD

In these cases definitive diagnosis may

Objective: To identify questions sensitive and specific for staring spells of sometimes require video EEG of an actual
epileptic (absence seizures [AS]) or nonepileptic etiology to increase the yield event, and approximately 10% of admis-
of history taking. sions to pediatric epilepsy monitoring
units consist of children with staring spells
Study design: A questionnaire was completed by parents of 40 children who
that are eventually shown to be nonepilep-
presented with staring spells. Results from 17 children with AS and 23 with
tic.10,11 However, video EEG is an expen-
nonepileptic staring (NES) were compared.
sive resource, available only to a minority
Results: Features with moderate sensitivity (43% to 56%) but high specificity of children with staring spells, and results
(87% to 88%) for NES included preserved responsiveness to touch, lack of in- may not be definitive if a clinical event
terruption of playing, and initial identification by a teacher or health profession- does not occur during the recording peri-
al. These features were more frequent in NES than in AS (P = .013, .016, .030). od.17,18 The goal of our study was to offer
Body rocking occurred only in NES, but sensitivity was low (13%). Features a means to increase the diagnostic certain-
with high specificity (91% to 100%) for AS included limb twitches, upward eye ty of the parental interview by identifying
movements, and urinary incontinence; but sensitivities were low (13% to 35%). questions specific and sensitive for
nonepileptic and epileptic staring spells.
Conclusion: In children with normal interictal electroencephalography find- Because the most common diagnostic
ings and without neurologic disease, staring spells are most likely nonepileptic problem involves inattention versus AS,
when parents report preserved responsiveness to touch, body rocking, or initial we focused on this distinction in our study.
identification by a teacher or health professional without limb twitches, upward
eye movements, interruption of play, or urinary incontinence. In these cases a AS Absence seizures, epileptic staring spells
EEG Electroencephalography
diagnosis of NES may be confidently applied, with confirmation based on long- NES Nonepileptic staring spells
term follow-up. (J Pediatr 1998;133:660-3)
PATIENTS AND
METHODS
Staring spells are a frequent presenting cephalography.1,2 Generalized 3 Hz
symptom in childhood and may be spike-wave complexes strongly support We compiled a questionnaire with 25
epileptic (absence or complex partial a diagnosis of absence seizures,1,2,4,14 questions based on observations from the
seizures)1-10 or nonepileptic (inattention whereas focal sharp waves, especially in literature* and our personal clinical expe-
or daydreaming).10-13 Diagnosis is usu- the temporal lobe, suggest complex par- rience. Questions explored features such
ally based on parental description of the tial seizures.5,6 Additional diagnostic as arrest of activity, unresponsiveness, eye
events and results of routine electroen- clues are often present in children with blinking, upward eye rolling, myoclonic
complex partial seizures, such as abnor- twitches, body stiffening, dropping of the
mal neuroimaging,5-9 focal findings on head or jaw, complex movements or au-
From the Department of Neurology, The Cleveland Clinic
Foundation, Cleveland, Ohio. Drs Rosenow and Hamer are neurologic examination, or the presence tomatism, and body rocking. Questions
now at the Neurologische Universitätsklinik, Marburg, of other seizure types in addition to the also addressed age of onset, duration, and
Germany. staring spells. Therefore the most fre- frequency of the staring spells; presence or
Submitted for publication Feb 20, 1998; revision re- quent diagnostic dilemma in children absence of learning difficulties; the person
ceived June 30, 1998; accepted Sept 4, 1998.
with normal interictal EEG findings is responsible for the initial identification of
Reprint requests: Elaine Wyllie, MD, Department
of Neurology, S51, The Cleveland Clinic Founda- AS versus nonepileptic inattention. the spells; and the emotional response of
tion, 9500 Euclid Ave, Cleveland, OH 44195. Diagnosis may be challenging when the parental witness during a spell.
Copyright © 1998 by Mosby, Inc. staring spells are the only symptom and re-
0022-3476/98/$5.00 + 0 9/21/94315 sults of the routine EEG are normal.14-16 *References 1-4, 14, 16, and 18-21.

660
THE JOURNAL OF PEDIATRICS ROSENOW ET AL
VOLUME 133, NUMBER 5

The questionnaire was presented by Table I. Age at evaluation, duration since onset of spells and mean and maximum dura-
one investigator (F.R.) to parents or tion and frequency of spells in patients with AS (n = 17) and NES (n = 23)
guardians of 40 children who presented
AS NES
with a chief complaint of staring spells.
(median (mean P value
Two patient groups were compared: 17
Parameter [25%-75%]*) [25%-75%]*) (MWU test)
patients had AS with generalized seizure
patterns recorded during routine EEG Age (mo) 103.4 ± 52.3, 90† 80.8 ± 52.2, 72† 0.19
(10 patients) or prolonged video EEG (7 Duration since onset (mo) 16 (8-60) 24 (4-43) 0.58
patients), and 23 patients had NES, di- Average frequency/month 150 (16-300) 30 ( 16-150) 0.28
agnosed after a full clinical evaluation by Average duration (sec) 10 (5-15) 15 (10-30) 0.19
a pediatric epileptologist (E.W. or P.K.) Maximal duration (sec) 25 (10-60) 30 (20-60) 0.39
who had no knowledge of the question- MWU test, Mann-Whitney U test.
naire results. Children with NES all had *Interquartile range.
†For age only, results are given as mean ± SD, median.
normal interictal EEG findings during
hyperventilation, photic stimulation, and
usually with sleep. No types of events or Table II. Nonepileptic staring spells (inattention): Specificity and sensitivity of reported
seizures other than staring spells and no clinical features
neurologic disease or findings other than
Clinical features n Specificity (95% CI) n Sensitivity (95% CI)
mild delay of cognitive development (5
of 23, 22%) were seen in children with 1. No interruption of playing 17 0.88 (0.63-0.99) 23 0.48 (0.27-0.69)
NES in this series. In 10 children with 2. First seen by professional 17 0.88 (0.64-0.99) 23 0.43 (0.23-0.66)
NES, ictal EEG was obtained during a 3. Responsive to touch 15 0.87 (0.60-0.98) 16 0.56 (0.30-0.80)
staring spell on prolonged video EEG, 4. Body rocking 17 1.0 (0.80-1.00) 23 0.13 (0.03-0.34)
and in each case the recording was nor- 1 or 2 17 0.82 (0.57-0.96) 23 0.70 (0.47-0.87)
mal. None of the children diagnosed with 1 and 2 17 0.94 (0.71-1.00) 23 0.22 (0.07-0.44)
NES presented during the follow-up pe- 1 and 3 15 1.0 (0.78-1.00) 16 0.31 (0.11-0.59)
riod (15.7 ± 5.5 months) with new fea- 2 and 3 15 1.0 (0.78-1.00) 16 0.31 (0.11-0.59)
tures suggesting epilepsy, and no new
concerns were described by any of the
parents of 16 children with NES who differences in age at evaluation, duration playing, the sensitivity for NES increased
were reached for telephone contact. from onset to evaluation, the average fre- but the specificity remained high (Table
The 2 patient groups were compared quency of staring spells, or the average II), and this “either/or” combination was
by using univariate descriptive statistics and maximum duration of spells (Table I). significantly more frequent in NES than
(Mann-Whitney U test and χ2 test in- Problems with learning or attention were AS (P = .001). Body rocking occurred ex-
cluding Fisher’s exact test). No adjust- reported in 40% of children in both clusively during NES but was reported
ment for multiple testing was performed groups. A family history of staring spells for only 13% of patients in this group. Sig-
because this was an exploratory study. or epileptic seizures tended to be more fre- nificantly more parental witnesses report-
The results will be used to foster further quent in the AS group (35% vs 17%), but ed feeling worried during NES than dur-
studies testing specific hypotheses. the difference was not significant (P = .2). ing AS (P = .03), but this feature had low
Of the 25 items on the questionnaire, specificity (35%). The proportion of
only a few showed significant differences parental witnesses answering “yes” to
RESULTS between patients with AS and those with each of the related questions is shown in
NES. Features with high specificity (87% the Figure.
The staring spells of the patients in to 88%) for NES included preserved re- Features with high specificity for AS
both groups were characterized by a sponsiveness to touch, lack of interrup- (91% to 100%) included limb twitches, up-
dazed or vacant facial expression with tion of playing, and initial identification ward eye movements, and urinary inconti-
sudden onset and abrupt cessation. The by a teacher or health professional rather nence during stares; but each of these fea-
stares lasted for several seconds, were as- than by a parent (Table II). Each of these tures had low sensitivity (13% to 35%)
sociated with reduced interaction and re- features had moderate sensitivity (43% to (Table III). Typical occurrence when tired
sponsiveness, and were not preceded by 56%) for NES, and each was significantly had moderate sensitivity and specificity for
a warning or aura. No differences be- more frequent in NES than in AS (Fig- AS. Limb twitches and typical occurrence
tween children with AS and those with ure). When 2 or more of these features when tired were significantly more fre-
NES were detected by questions explor- were present, the specificity for NES fur- quent in AS than NES, whereas upward
ing these features. ther increased above 94%. When parents eye movements tended to be more frequent
For children with NES compared with reported initial identification by a teacher in AS than in NES (Figure). Sensitivity for
those with AS, there were no significant or health professional or interruption of AS increased slightly for the “either/or”

661
 ROSENOW ET AL
Figure. Clinical features more commonly reported in NES (first 4 features on left) or AS (last 4 features
on right) and relative frequency of reported features (ie, sensitivity of questions) with CIs.
Table III. Epileptic staring spells (AS): Specificity and sensitivity of reported clinical
features
Clinical features n Specificity (95% CI)
1. Twitching of arms or legs 23
2. Urine loss 22
3 Upward eye movements 23
4. Occurrence when tired 23
1 or 2 22
3 and 4 23
combination of limb twitching or urinary
incontinence, whereas specificity was mod-
erate (Table III), and this was significantly
more frequent during AS than NES (P =
.003). Compared with this “either/or” com-
bination, upward eye movements had simi-
lar specificity and sensitivity for AS, but
frequency of occurrence was low (Figure)
and not significantly different between pa-
tient groups. Also infrequently reported in
both groups were eye blinking (3 of 17 pa-
tients with AS and 1 of 23 patients with
NES) and “complex movements such as
finger rubbing” (automatism) (2 of 17 chil-
dren with AS and 3 of 23 children with
NES). Typical occurrence when tired had
moderate specificity and sensitivity for AS
but was not significantly more frequent in
AS versus NES (Table III, Figure).
DISCUSSION
We found that in children with normal
interictal EEG findings and without
662
n Sensitivity (95% CI)
1.0 (0.85-1.00) 17 0.23 (0.07-0.50)
1.0 (0.85-1.00) 16 0.13 (0.02-0.38)
0.91(0.72-0.99) 17 0.35 (0.14-0.62)
0.74 (0.52-0.90) 17 0.58 (0.33-0.82)
1.0 (0.85-1.00) 16 0.35 (0.15-0.65)
0.96 (0.78-1.00) 17 0.29 (0.07-0.50)
other seizure types or neurologic abnor-
malities (except mild cognitive delay or
learning problems), staring spells were
highly likely to be nonepileptic when par-
ents reported preserved responsiveness
to touch, no interruption of playing by
the stares, or initial identification of the
stares by a teacher or health professional
rather than by a relative. Body rocking
occurred only in NES, but sensitivity was
low. On the other hand, features strongly
suggesting AS included limb twitches,
upward eye movements, and urinary in-
continence during stares; but each of
these features had low sensitivity.
Our results suggest that in otherwise
uncomplicated cases positive for features
suggesting NES and negative for fea-
tures suggesting AS, a diagnosis of
nonepileptic staring or inattention may
be confidently applied without ictal
video EEG recording. Diagnostic confir-
mation over time is based on long-term
follow-up. Results gained from parental
interview may be especially helpful in
THE JOURNAL OF PEDIATRICS
NOVEMBER 1998
avoiding the need for video EEG in the
high number of children with attention
deficit disorder (3% to 5% of school-aged
population3) who “tune out” during
classroom instruction.
Compared with nonepileptic staring,
AS are less frequent, with an estimated
incidence of 9.6/100,000 children be-
tween 0 and 15 years of age.21 In most
cases the diagnosis is clarified by the
presence of generalized spike-wave dis-
charges and AS during routine EEG.
However, Wirrel et al15 were unable to
reliably provoke AS by hyperventilation-
induced hypocapnia during EEG record-
ing for 33% of patients. More prolonged
video or ambulatory EEG may be appro-
priate for children with suspicious clini-
cal features and normal routine EEG
findings. Symptoms identified during
video EEG analyses of AS have included
increased frequency during tiredness,
abrupt onset and cessation of the staring,
arrest of activity, reduced responsive-
ness, upward eye rolling, eyelid twitch-
ing, changes in tone or posture, oral or
gestural automatism, and urinary incon-
tinence.4,16,20 We found that with regard
to these features, only parental report of
limb twitches, urinary incontinence, and
upward eye movements had high speci-
ficity for AS versus NES. In a video
EEG study comparing smaller numbers
of children with epileptic and nonepilep-
tic staring spells, Nagarajan and Bye10
found no significant differences between
groups in the frequency of accompanying
motor features.10
In our series several features were re-
ported for the majority of children in both
groups. These included a dazed or vacant
facial expression, arrest of ongoing activity,
reduced responsiveness, and abrupt onset
and cessation of the episodes. The high fre-
quency of these features in these children
with NES may reflect referral bias, with
these features raising concerns on the part
of the parents or pediatrician so that neu-
rologic consultation was required. Howev-
er, 2 specific questions were helpful for ex-
ploring the degree of unresponsiveness.
Interruption of playing by a spell and un-
responsiveness to touch during a spell are
features that may suggest the possibility of
unconsciousness rather than inattention.
Consistent with results from the study by
THE JOURNAL OF PEDIATRICS
VOLUME 133, NUMBER 5
Nagarajan and Bye,10 we found no signifi-
cant differences in duration and frequency
of AS versus NES. In contrast, Carmant et
al22 reported no differences in the length of
the events but a higher frequency of AS as
compared with behavioral NES in a video
EEG study.
At first glance it seems counterintuitive
that initial identification of the spells by a
teacher or health professional was an in-
dicator of NES rather than AS. One
might expect that a teacher, speech thera-
pist, or nurse would be more accurate
than a lay parent in the identification of
epileptic seizures. However, this did not
appear to be the case. In our series initial
recognition by a teacher or health profes-
sional appeared to be part of a scenario
that was typical for children with NES.
The professional noted the staring, was
aware that AS could be a cause of poor
school performance, and notified the par-
ents. The parents then began to watch the
child more closely and started noticing
periods when the child seemed inatten-
tive or did not respond when called (eg,
while watching television or sitting at the
dinner table). This led to consultation
with a pediatrician and then referral to a
child neurologist. When something about
the staring worried the parents indepen-
dently, the events were more likely to be
epileptic than when parents first became
concerned at the advice of a professional.
Compared with parents of children with
AS, the parents of children with NES
more frequently reported feeling worried
during the stares, although this feature had
low specificity and sensitivity for NES.
This result also seems counterintuitive, be-
cause one might expect epileptic seizures
to appear more worrisome than nonepilep-
tic events. A possible explanation is again
referral bias, with greater parental concern
possibly playing a larger role in the deci-
sion for neurologic consultation among pa-
tients with NES versus AS.
Some of our patients with NES did not
have EEG recording of a staring spell. AS
usually occurred during routine EEG,
whereas NES rarely did. For financial rea-
sons it was not possible to perform pro-
longed video EEG on every child with
NES, and this was reserved for cases with
diagnostic uncertainty. Therefore the pa-
tients with NES with recorded events
were an especially worrisome subgroup
compared with the children with NES di-
agnosed on the basis of clinical features
and normal interictal EEG findings. By in-
cluding a sizeable proportion of children
with NES confirmed on video EEG, we
may have biased our NES population to-
ward those with clinical features more
similar to those reported in AS. On the
other hand, by including children without
ictal EEG confirmation of NES, we may
have inadvertently diagnosed some pa-
tients with NES who actually had epilep-
tic seizures. Either way, these potential er-
rors would be expected to reduce the
differences between our groups rather
than enhance them. Overcoming these
limitations would require an extremely ex-
pensive study with prolonged video EEG
in every case.
In conclusion, our results suggest that
some parental observations may increase
the diagnostic certainty for NES among
children without other seizure types or
neurologic abnormalities who have nor-
mal interictal EEG findings on record-
ings obtained during hyperventilation,
photic stimulation, and sleep. The pres-
ence of reported features suggesting
NES in combination with the absence of
features that suggest AS may allow chil-
dren’s physicians to more confidently
defer an expensive prolonged video EEG
evaluation in selected cases.
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