ArticlePDF Available

Anoxic-epileptic seizures: Observational study of epileptic seizures induced by syncopes

Authors:
  • Independent Researcher

Abstract and Figures

To describe a large series of children with anoxic-epileptic seizures (AES)--that is, epileptic seizures induced by syncopes. Retrospective case-note review in a tertiary paediatric neurology unit. For all 27 children seen with a definite diagnosis of AES between 1972 and 2002, a review of clinical histories, videotapes, and EEG/ECG studies was undertaken. Main outcome measures were: age of onset, frequency and type of syncopes; age of onset and frequency of AES; type and duration of induced epileptic seizures; effect of treatment of syncopal and epileptic components. Median age of onset of syncopes was 8 months (range 0.2-120), frequency 2 in total to 40/day, median total approximately 200. Syncopes were predominantly reflex asystolic (RAS), prolonged expiratory apnoea (cyanotic breath-holding spells), or of mixed or uncertain origin; there was one each of ear piercing and hair grooming vasovagal syncope and one of compulsive Valsalva. Median age of onset of AES was 17 months (range 7-120), frequency from total 1 to 3/day, median total 3. The epileptic component was almost always bilateral clonic; three had additional epilepsy, one each with complex partial seizures, myoclonic absences, and febrile seizures plus. Median duration of epileptic component was 5 minutes (range 0.5-40, mean 11). Cardiac pacing prevented RAS in two patients: most other anti-syncope therapies were ineffective. Diazepam terminated the epileptic component in 6/8. Valproate or carbamazepine abolished AES in 5/7 without influencing syncope frequency. Although uncommon compared with simple syncopes, syncope triggered epileptic seizures (AES) are an important treatable basis of status epilepticus.
Content may be subject to copyright.
ORIGINAL ARTICLE
Anoxic-epileptic seizures: observational study of
epileptic seizures induced by syncopes
I A Horrocks, A Nechay, J B P Stephenson, S M Zuberi
...............................................................................................................................
See end of article for
authors’ affiliations
.......................
Correspondence to:
Prof. J B P Stephenson,
Fraser of Allander
Neurosciences Unit, Royal
Hospital for Sick Children,
Yorkhill, Glasgow G3 8SJ,
UK; john@jbpstephenson.
com
Accepted 25 August 2005
Published Online First
15 September 2005
.......................
Arch Dis Child 2005;90:1283–1287. doi: 10.1136/adc.2005.075408
Aims: To describe a large series of children with anoxic-epileptic seizures (AES)—that is, epileptic seizures
induced by syncopes.
Methods: Retrospective case-note review in a tertiary paediatric neurology unit. For all 27 children seen
with a definite diagnosis of AES between 1972 and 2002, a review of clinical histories, videotapes, and
EEG/ECG studies was undertaken. Main outcome measures were: age of onset, frequency and type of
syncopes; age of onset and frequency of AES; type and duration of induced epileptic seizures; effect of
treatment of syncopal and epileptic components.
Results: Median age of onset of syncopes was 8 months (range 0.2–120), frequency 2 in total to 40/day,
median total ,200. Syncopes were predominantly reflex asystolic (RAS), prolonged expiratory apnoea
(cyanotic breath-holding spells), or of mixed or uncertain origin; there was one each of ear piercing and
hair grooming vasovagal syncope and one of compulsive Valsalva. Median age of onset of AES was
17 months (range 7–120), frequency from total 1 to 3/day, median total 3. The epileptic component was
almost always bilateral clonic; three had additional epilepsy, one each with complex partial seizures,
myoclonic absences, and febrile seizures plus. Median duration of epileptic component was 5 minutes
(range 0.5–40, mean 11). Cardiac pacing prevented RAS in two patients: most other anti-syncope
therapies were ineffective. Diazepam terminated the epileptic component in 6/8. Valproate or
carbamazepine abolished AES in 5/7 without influencing syncope frequency.
Conclusions: Although uncommon compared with simple syncopes, syncope triggered epileptic seizures
(AES) are an important treatable basis of status epilepticus.
C
onvulsive syncope is common in children, and particu-
larly so in infants and toddlers.
1
In most cases these are
simply non-epileptic anoxic seizures
12
—well known to
all paediatricians—in which abrupt loss of cerebral energy
supply leads to brief extensor stiffening and a few seconds of
irregular spasms or jerks. Less well known is the situation in
which a syncope—that is, an anoxic seizure—provokes a true
epileptic seizure. This combination is called an anoxic-
epileptic seizure (AES) and was first described as such by
one of us in 1983.
3
Since then there have been only 11
patients with AES from outwith our unit described in any
detail.
4–11
The paucity of reports may have given the
impression that AES are rare, and even the existence of
AES has been questioned.
12
We therefore thought it worth-
while to describe the features of a large series of children
with AES seen in a single tertiary paediatric neurosciences
unit.
METHODS
Setting
The Fraser of Allander Neurosciences Unit is a tertiary
referral unit for paediatric neurology which serves the West
of Scotland (population 3 million) but may take referrals
from elsewhere. Since its opening in 1972 a diagnostic index
has been maintained of all patients seen.
Information retrieval
The diagnostic index was searched for the coded diagnosis of
AES from 1972 until the end of 2002. All case records were
reviewed in detail by the authors. All available home or
hospital video recordings were reviewed as well as EEG and
ECG traces. The coded diagnosis of AES was only accepted if
both the history of syncope and of the triggered epileptic
seizure was convincing to all authors. Box 1 shows the levels
of evidence we used for establishing the epileptic component.
Case histories, EEG/ECG records, and/or video recordings
of 14 of the children were already in the literature,
1 2 11 13–15
and these publications were reviewed.
RESULTS
Patient demographics
A total of 27 children had a convincing history of AES, with
the first patient being recognised in 1978 and the next two in
1982. Of this total of 27, 21 were referred from the West of
Scotland, 1 from Northeast Scotland, 3 from England, 1 from
Wales, and 1 from the USA. All patients were seen at one
time by the corresponding author (JBPS). There were 11 boys
and 16 girls.
Age of onset and frequency of syncopes
The age of onset of syncopes ranged from 5 days
(0.2 months) to 10 years (120 months) with a median of
8 months.
The frequency of syncopes varied from 2 in total to 40/day.
The median was of the order of 200 syncopes, but since 11 of
the children had a history of daily syncopes, the figures for
total number of syncopes were in the main approximations.
Type of syncopes
We grouped the syncopes into five types on the basis of
clinical description, aided when possible by the appearances
of recorded events.
Abbreviations: AES, anoxic-epileptic seizures; PEA, prolonged
expiratory apnoea; RAS, reflex anoxic seizures/reflex asystolic syncope
1283
www.archdischild.com
Reflex anoxic seizures/reflex asystolic syncope
(RAS)
There were 9 children whose syncopes were judged to be of
this nature, with a typical history of short latency onset
syncopes most often precipitated by unexpected hurt.
16
Of
these, 7 had ECG recordings during ocular compression (5
children), on head-up tilt (1 child), or on cardiac monitoring
at home (1 child ). Median cardiac asystole was 28 seconds
(range 17–31).
Prolonged expiratory apnoea/cyanotic breath-
holding spells
In 6 children the clinical description was of ‘‘blue breath-
holding’’
13
or cyanotic breath-holding spells,
17
otherwise
called prolonged expiratory apnoea,
18
with expiratory grunt-
ing apnoea and deep cyanosis in response to frustration.
When syncopes occurred during monitoring (3 children),
prolonged cardiac asystole was not seen.
Infantile syncopes of uncertain or mixed origin
In 9 children either we could not decide which of the above
two mechanisms was operative or else there was evidence of
a mixed mechanism. In the most dramatic example
214
there
was simultaneous prolonged expiratory grunting and pro-
longed asystole (23–24 seconds).
Vasovagal syncope
Two children had more typical vasovagal syncope, 1 while
having her pierced ears cleaned, and 1 (aged 10 years) during
hair grooming.
19
Compulsive Valsalva manoeuvres
One boy
11
with autistic spectrum disorder had very frequent
daily compulsive Valsalva manoeuvres.
Anoxic-epileptic seizures: age of onset and frequency
The median age of onset of AES was 17 months (range 7–
120). The frequency varied from a total of just one to 3/day,
the median being 3 AES in total.
Type of epileptic component of AES
In almost all children the epileptic component could be
described as bilateral clonic. Sometimes there was horizontal
eye deviation or rhythmically interrupted vocalisation.
11
In
one child, what appeared to be absence status followed the
clonic epileptic component. In 3 children there were in
addition unprovoked epileptic seizures, that is epilepsy, the
syncope triggered epileptic seizures being reportedly identical
to the spontaneous episodes. There was one example each
of myoclonic absences, generalised epilepsy with febrile
seizures—clonic epileptic seizures with and without fever—
and temporal lobe complex partial seizures.
Duration of epileptic component of AES
The duration of the syncope triggered epileptic seizures varied
from 28 seconds (Stephenson,
1
case 11.2) to 40 minutes
(mean 11 minutes, median 5 minutes). In about half (12
children) the epileptic component lasted for 10 minutes or
more, and in 4 children for 30 minutes or more. The epileptic
component was witnessed by medical staff in 16 children,
and in these the duration of the epileptic component was
10 minutes or more in 8 and 30 minutes or more in 4.
Treatment of the syncopal component of AES
Most medications, including iron supplements and piracetam
were unhelpful. In one child with RAS, atropine was
associated with improvement, and in another was ineffective.
In the autistic boy with compulsive Valsalva manoeuvres,
naltrexone had at least temporary benefit. Permanent cardiac
pacing
14 15
prevented RAS, and therefore AES in 2 severely
affected children. In one child with very frequent AES from
‘‘breath-holding’’ (prolonged expiratory apnoea), psycholo-
gical methods
9
led to no benefit.
Acute treatment of the epileptic component of AES
Rectal or intravenous diazepam was given by emergency
doctors in 7 children and was effective in 6, albeit one child
required two injections. In the diazepam non-responder,
intravenous phenobarbitone and rectal paraldehyde were
required in the intensive care unit, and this child later had
cardiac pacing.
15
In addition, the child with compulsive
Valsalvas was given diazepam at home without evident
benefit.
Prophylactic treatment of the epileptic component
of AES
Sodium valproate or carbamazepine was effective in 5 of 7
children to whom it was given. By effective we mean that the
epileptic component of the AES was abolished, but the
syncopes continued unchanged.
Frequency of AES compared with frequency of
syncope
Except in a few instances, there were in any child vastly more
syncopes, that is pure anoxic seizures, than there were AES.
In 3 children in whom a more precise ratio could be
calculated, about 7–8% of syncopes developed into AES.
These results are summarised in table 1.
DISCUSSION
This is the first large published series of definite epileptic
seizures induced by syncopes. It establishes that such AES are
not rare and should be considered when prolonged rhythmic
jerking follows a noxious stimulus. While any type of
neurally mediated syncope from impaired cerebral perfusion
or hypoxia may be the stimulus for the epileptic component
of AES, the epileptic component is more stereotyped and less
diverse. In the vast majority of AES the epileptic component
could best be described as clonic, with no examples known of
a tonic-clonic epileptic seizure being induced. Some authors
5
have stated that tonic-clonic epileptic seizures may be
induced by syncopes, but in our opinion the tonic phase
was the tonic component of the syncope or anoxic seizure
Box 1: Levels of evidence for epileptic
component in AES
(1) Syncope induced epileptic seizure recorded on EEG
(2) Epileptic component of AES video recorded
(a) video included transition from syncope to epileptic
seizure
(b) video was of epileptic seizure immediately after
transition from provoking syncope
(c) video was of prolonged epileptic jerking which,
although not on that occasion syncope induced,
had also by history been so induced
(3) Prolonged post-syncopal jerking or other epileptic
activity witnessed by attending medical staff
(4) Clinical history of post-syncopal rhythmic jerking or other
convincing epileptic activity
(a) duration 10 minutes or more
(b) duration less than 10 minutes
1284 Horrocks, Nechay, Stephenson, et al
www.archdischild.com
Table 1 Clinical data on 27 children with a history of AES
Case no./
sex/from Ref./case
Syncope
onset (mth)
Syncope
type/
frequency
AES
onset
(mth)
Epileptic
component/
duration (min)
Evidence of
epileptic
component AES total
Therapy (bold
type = effective)
Other
epileptic
seizures Other details/FH
1M
1
6–7 RAS 15 Clonic 1 3 OC 22s
11.1 3/day 10
2M
1
9 RAS 11 Clonic 1 2 OC 30 s
11.2 Several K
(28s.)
3 F 15 RAS 48 Focal 1 1+ Carbamazepine Same OC 17 s
1/month 3
4M
11
0.5 BHS 7 Myoclonic absence 2c Many Clonazepam Same
5 3/week 1
5M
U
11
3
30 CVM
Daily
30+ Clonic (vibratory)
L
2a Many Wrestler’s belt
Naltrexone
Diazepam
6M
11
4
5 PEA
Daily
10 Clonic
1
2b Many Iron
Piracetam
Psychology
Valproate
Brother PEA & FS
up to 20 m
7 F 30 VVS 84 Clonic 4b 1 Ear piercing
Total 2 5
8 F 6 RAS 18 Clonic 4a 10 CM 31 s
3/day 25
9F
1
4 PEA 14 Clonic 3 20 OC 5 s
8.1 3/day 5 Seen in ward
10 F
1
6 BHS 11 Clonic 4a .3– OC12s
15.45 2/week 20 One AES febrile
11 F
2
8 PEA 15 Clonic 4b 4 OC 7 s
2 Daily 3 PEA 8 s
12 F 42 RAS 108 Clonic 4a 1 Pacing Tilt 30 s
3/month 15
13 M
W
12 BHS
1/month
17 Clonic
30
33Diazepam
Valproate
OC 1.5 s
Sister FS with status
14 F
1
12 RAS ,20 Clonic 3 1 Diazepam –OC28s
E 4.8 Total 12 15
15 F 8 BHS 13 Clonic 4b 3 OC 2 s
6/year 1 ‘‘That’s it!’’
16 F
S
15
7
0.2 RAS
4/day
19 Clonic
40
3 1 Diazepam
Paraldehyde
Valproate
–CM20s
Pacing
17 M
1
9 PEA 18 Clonic 4b 3 OC 0 s
11.3 Daily 2
18 F
1
,16 BHS 16 Clonic 3 2 Diazepam –OC15s
11.4 Total 3 20
19 M 9 PEA 17 Clonic 4b 4
Daily 2 ‘‘1/s. jerks’’
20 F 120 VVS 120 Clonic 3 3 Diazepam Hair groom
Total 3 10 M: epilepsy
21 F
1
12 RAS 18 Clonic 3 1 Diazepam
11.5 5/year 30 Repeat needed
22 F 4 BHS ,30 Clonic 2c 6 Valproate Same,
also FS
=FS+
M’s sister FS
Total 13 1
23 M 8 RAS 15 Clonic 4b 6 Carbamazepine
E Up to 5/day 5
24 F 8 BHS
3/week
11 Clonic + absence
status
31Diazepam
30
25 M 14 BHS 20 Clonic 4b 1
E 3/week 5
26 M 0.6 PEA 12 Clonic 4a 10 Phenobarb. OC 0 s
Uncertain 15
27 F
14
8 BHS
Up to 40/day
12 Clonic
2
1Upto
3/day
Valproate
Carbamazepine
Atropine,
Pacing
–OC24s
E PEA 23 s
Twin RAS
All cases were referred from the west of Scotland, except those initialled in the first column as: S, from elsewhere in Scotland; E, from England; W, fro m Wales, U,
from USA. In the second column Ref./case gives the reference and the published case number (if any).
Syncopes are abbreviated: RAS, reflex anoxic seizures/reflex asystolic syncope; PEA, prolonged expiratory apnoea (cyanotic breath-holding); BHS, breath-
holding spells of uncertain mechanism or combined asystole and expiratory grunting; VVS, vasovagal syncope of adult type; CVM, compulsive Valsalva
manoeuvre.
Under Evidence of epileptic component, the scoring of level of evidence is as in box 1, with ‘‘seen in ward’’, observed during ward-round; ‘‘that’s it!’’, recognition
of event by parents from video demonstrations;
20
and ‘‘1/s jerks’’, parents described and mimed 1/second jerking.
In Other epileptic seizures: FS, febrile seizures; FS+, febrile seizures plus.
In the last column: FH, family history; M, mother; m, month; the figures before s., seconds refer to duration of asystole; OC, ocular compression; CM, cardiac
monitoring; tilt, head-up tilt testing.
Other abbreviations are as previously, except that when PEA is followed by a number of seconds, that is the duration of asystole accompanying the PEA event.
Anoxic-epileptic seizures 1285
www.archdischild.com
and what followed was simply a clonic epileptic seizure.
1
In
support of this assertion is the EEG/ECG appearance of
published examples of AES, in particular that of our case 2, as
shown in fig 1.
Why some patients have epileptic seizures after syncopes
while most do not is a most interesting question. It has been
known for over 40 years that hypoxia, experimentally
induced by nitrogen inhalation, could provoke generalised
21
or focal
22
EEG discharges in adults with respectively general-
ised or focal epilepsy. Most of the children in our series did
not have additional epilepsy—in the sense of recurrent
‘‘spontaneous’’ epileptic seizures—but four of them (cases
6, 13, 20, and 22) had a family history of probable epileptic
seizures, including prolonged epileptic febrile seizures in two
cases. In one large family (case 6) there was a history of both
infantile epileptic convulsions and PEA, suggesting that an
epileptic seizure gene and a neurally mediated syncope gene
might have come together to give a propensity to AES.
It may also be asked whether the syncopes in our cases
differed in any identifiable way from the background
population of children with neurally mediated syncopes.
There is little good information on the relative frequency of
the different types of infantile syncope, but in a prospective
study Lombroso and Lerman
23
found that of their total of 140,
62% had cyanotic breath-holding or what we would now call
prolonged expiratory apnoea, 19% had pallid spells (reflex
anoxic seizures or reflex asystolic syncope), and 19% were
indeterminate. Our series was different, in that asystolic
events were more frequent, but not sufficiently so to provide
an explanation. We suggest that future combined clinical and
genetic studies should address origins of both the syncopal
and epileptic components of AES.
In the literature and in our cases, status epilepticus—
whether defined as an epileptic seizure lasting 30 minutes or
10 minutes
24
—is a frequent complication of AES. Whether
this is because AES with only a brief epileptic component are
difficult to recognise is not clear.
We think that AES are important for paediatricians,
paediatric cardiologists, and paediatric neurologists to recog-
nise because the common tendency to progress to status
epilepticus may be prevented, either by acute administration
of a benzodiazepine (in our series rectal or intravenous
diazepam) or by daily prophylaxis with standard antiepileptic
medication (in our series valproate or carbamazepine). The
uncomplicated syncopes (anoxic seizures) which are usually
much more frequent than AES in any given patient can
seldom be prevented, with one important exception: if the
syncopal component is RAS, with documented prolonged
reflex asystole, specific cardiac therapy (even pacing
15
) may
prevent the need for antiepileptic medication.
In conclusion, syncopes may trigger true epileptic seizures,
and children’s doctors should be aware of this. The paucity of
published reports suggests that most AES are either
unrecognised or misdiagnosed.
T3–O1
FP1–T3
T4–O2
FP2–T4
P3–T5
PZ–P3
P4–PZ
T6–P4
F3–F7
FZ–F3
F4–FZ
F8–F4
CZ–PZ
O2–O1
ECG
ECG
T3–O1
FP1–T3
T4–O2
FP2–T4
P3–T5
PZ–P3
P4–PZ
T6–P4
F3–F7
FZ–F3
F4–FZ
F8–F4
CZ–PZ
O2–O1
ECG
ECG
T3–O1
FP1–T3
T4–O2
FP2–T4
P3–T5
PZ–P3
P4–PZ
T6–P4
F3–F7
FZ–F3
F4–FZ
F8–F4
CZ–PZ
O2–O1
ECG
ECG
Figure 1 EEG/ECG recording showing AES induced by ocular
compression (OC). Each panel lasts about 20 s. In the upper panel OC
induces instant asystole and the EEG becomes isoelectric within 12 s (the
fast deflections in the 9th and 11th channels correspond to down-beat
nystagmus). In the centre panel the tonic phase of the anoxic seizure is
shown on the ECG line as black ‘‘fuzz’’ due to tonic EMG activity.
Midway across the centre panel while the EEG is still isoelectric, rhythmic
2–3/s jerk artefacts are seen on EEG and ECG channels. By the time of
the lower panel, rhythmic EEG spike wave is visible, slowing towards the
conclusion. Jerking lasted 28 s but the child was unresponsive for a
further 8 minutes (reproduced with permission from Stephenson,
1
Mac Keith Press, fig. 11.4).
What is already known on this topic
N
Convulsive syncope usually means non-epileptic anoxic
seizures
N
Published examples of convulsive syncope in which true
epileptic seizures were induced by syncopes have been
few in number, giving the impression that anoxic-
epileptic seizures (AES) are rare
What this study adds
N
In this, the first large systematic study of AES from a
single referral centre, the numbers seen suggest that
AES—triggered by a variety of syncopes—are not rare
N
Clonic status epilepticus—however defined—was
common in AES, but was treatable or preventable,
whereas the syncopal component could only occasion-
ally be treated
1286 Horrocks, Nechay, Stephenson, et al
www.archdischild.com
ACKNOWLEDGEMENTS
Thanks are extended to Dr William Whitehouse for referring one of
the children and for sharing additional information.
Authors’ affiliations
.....................
I A Horrocks, J B P Stephenson, S M Zuberi, Fraser of Allander
Neurosciences Unit, Royal Hospital for Sick Children, Glasgow, UK
A Nechay, Fraser of Allander Neurosciences Unit, Royal Hospital for
Sick Children, Glasgow, UK, and Neurology Department, Paediatric
Hospital N
˚
1, Kiev, Ukraine
Competing interests: none
REFERENCES
1 Stephenson JBP. Fits and faints. London: MacKeith Press, 1990: 19–23,112–
24.
2 Stephenson JBP. Anoxic seizures: self-terminating syncopes. Epileptic Disord
2001;3:3–6.
3 Stephenson JBP. Febrile convulsions and reflex anoxic seizures. In: Rose FC,
ed. Research progress in epilepsy. London: Pitman, 1983:244–52.
4 Aicardi J, Gastaut H, Mise`s J. Syncopal attacks compulsively self-induced by
Valsalva’s maneuver associated with typical absence seizures. A case report.
Arch Neurol 1988;45:923–5.
5 Battaglia A, Guerrini R, Gastaut H. Epileptic seizures induced by syncopal
attacks. J Epilepsy 1989;2:137–45.
6 Emery ES. Status epilepticus secondary to breath-holding and pallid syncopal
spells. Neurology 1990;40:859.
7 Nirale S, Bharucha NE. Breath-holding and status epilepticus. Neurology
1991;41:159.
8 Bergey GK, Krumholz A, Fleming CP. Complex partial seizure provocation by
vasovagal syncope: video-EEG and intracranial electrode documentation.
Epilepsia 1997;38:118–21.
9 Kuhle S, Tiefenthaler M, Seidl R, et al. Prolonged generalized epileptic
seizures triggered by breath-holding spells. Pediatr Neurol 2000;23:271–3.
10 Kelly AM, Porter CJ, McGoon MD, et al. Breath-holding spells associated with
significant bradycardia: successful treatment with permanent pacemak er
implantation. Pediatrics 2001;108:698–702.
11 Stephenson J, Breningstall G, Steer C, et al. Anoxic-epileptic seizures: home
video recordings of epileptic seizures induced by syncopes. Epileptic Disord
2004;6:15–19.
12 Appleton R. Reflex anoxic seizures. BMJ 1993; 24:214–15.
13 Stephenson JBP. Blue breath holding is benign. Arch Dis Child
1991;66:255–7.
14 Sreeram N, Whitehouse W. Permanent cardiac pacing for reflex anoxic
seizure. Arch Dis Child 1996;75:462.
15 Stephenson JBP, McLeod KA. Reflex anoxic seizures. In: David TJ, ed.
Recent advances in paediatrics 18. Edinburgh: Churchill Livingstone,
2000:12.
16 Stephenson JBP. Reflex anoxic seizures (white breath-holding) nonepileptic
vagal attacks. Arch Dis Child 1978;53:193–200.
17 Breningstall GN. Breath-holding spells. Pediatr Neurol 1996;14:91–7.
18 Southall DP, Talbert DG, Johnson P, et al. Prolonged expiratory apnoea: a
disorder resulting in episodes of severe arterial hypoxaemia in infants and
young children. Lancet 1985;2:571–7.
19 Igarashi M, Boehm RM Jr, May WN, et al. Syncope associated with hair-
grooming. Brain Dev 1988;10:249–51.
20 Stephenson JBP. Nonepileptic seizures, anoxic-epileptic seizures, and
epileptic-anoxic seizures. In: Wallace SJ, ed. Epilepsy in children. London:
Chapman & Hall, 1996:23.
21 Gastaut H, Bostem F, Fernandez-Guardiola A, et al. Hypoxic
activation of the EEG by nitrogen inhalation. 1. Preliminary
observations in generalised epilepsy. In: Gastaut H, Meyer JS, eds.
Cerebral anoxia and the electroencephalogram. Springfield, IL: Charles C
Thomas, 1961:343–54.
22 Gastaut H, Bostem F, Fernandez-Guardiola A, et al. Hypoxic activation of the
EEG by nitrogen inhalation. 2. Preliminary results in partial epilepsy. In:
Gastaut H, Meyer JS, eds. Cerebral anoxia and the electroencephalogram.
Springfield, IL: Charles C Thomas, 1961:355–64.
23 Lombroso CT, Lerman P. Breathholding spells (cyanotic and pallid infantile
syncope). Pediatrics 1967;39:563–81.
24 Livingston JH. Status epilepticus. In: Wallace SJ, Farrell K, eds. Epilepsy in
children, 2nd edn. London: Arnold, 2004:290–303.
IMAGES IN PAEDIATRICS ..............................................................................
doi: 10.1136/adc.2005.084160
An unwell child with a florid rash
A
3 month old girl presented with a
striking erythematous rash on her
lower limbs. She had become increas-
ingly oedematous and had started to vomit
frequently. Despite being breast fed, her
stools were frequent, offensive, and pale.
Investigations revealed anaemia, hypoprotei-
naemia, and deranged liver function. Serum
zinc levels were normal.
This is necrolytic migratory erythema. In
children, it may occur in association with
liver disease, inflammatory bowel disease,
pancreatitis, and malabsorption syndromes.
A deficiency of amino acids, essential fatty
acids, and/or zinc is thought to be the cause.
Our child was subsequently found to have
cystic fibrosis. Creon (pancreatic enzymes)
and Ketovite (multivitamins and folic acid)
supplementation resulted in a dramatic
resolution of the rash and a sustained
improvement in the child’s condition.
W Hussain, N Craven
Department of Dermatology, Burnley General
Hospital, Burnley, UK
I Swann
Department of Paediatrics, Burnley General
Hospital, Burnley, UK
Correspondence to: Dr W Hussain,
Department of Dermatology, Burnley General
Hospital, Casterton Avenue, Burnley BB10
2PQ, UK; dr_w_hussain@hotmail.com
Competing interests: none
Parental consent was obtained for publication of these
figures
Anoxic-epileptic seizures 1287
www.archdischild.com
... In some cases, the syncopal episode can induce a true epileptic seizure, referred to as an anoxic-epileptic seizure. These episodes are rare and less commonly reported, and may develop into status epilepticus [3]. Anoxic-epileptic seizures have also been reported in adults [4]. ...
... Anti-epileptics should not be used in RAS, although their use in anoxic-epileptic seizures requires further research. In an observational study, anti-epileptic medications prevented the epileptic component, but not the syncopal component, in five out of seven cases [3]. Anti-epileptics were not recommended for our patient given her infrequent episodes which were only induced by pain. ...
Article
Full-text available
Reflex anoxic seizures are a type of convulsive syncope seen more commonly in children. In rare cases, they may develop into true epileptic seizures. There is no current consensus on treatment. In this case report, we present an adult patient with reflex anoxic seizures, along with EEG monitoring from an event. Our patient had a successful trial with intranasal midazolam preventing the episode, suggesting that anxiety played a significant role in her case. Reflex anoxic seizures in adults are rare and need to be properly identified, as treatment can lead to significant improvements in patient quality of life.
... Most cases concern children who have had generalized epileptic seizures associated with their syncopal events. Some patients had absence seizures [20] or generalized convulsive seizures, either clonic or tonic-clonic [21]. These seizures, also known as anoxic epileptic seizures, were recorded during a variety of syncopes, ranging from reflex asystolic, prolonged expiratory apnoea (cyanotic breath-holding spells), compulsive Valsalva maneuvers, or of mixed or uncertain origin. ...
Article
Full-text available
The heart and seizures are closely linked by an indissoluble relationship that finds its basis in the cerebral limbic circuit whose mechanisms remain largely obscure. The differential diagnosis between seizures and syncopes has always been a cornerstone of the collaboration between cardiologists and neurologists and is renewed as a field of great interest for multidisciplinary collaboration in the era of the diffusion of prolonged telemonitoring units. The occurrence of ictal or post-ictal arrhythmias is currently a cause of great scientific debate with respect to the role and risks that these complications can generate (including sudden unexpected death in epilepsy). Furthermore, the study of epileptic seizures and the arrhythmological complications they cause (during and after seizures) also allows us to unravel the mechanisms that link them. Finally, intercritical arrhythmias may represent great potential in terms of the prevention of cardiological risk in epileptic patients as well as in the possible prediction of the seizures themselves. In this paper, we review the pertaining literature on this subject and propose a scheme of classification of the cases of arrhythmia temporally connected to seizures.
... Syncope in infants can rarely trigger a tonic-clonic seizure; these events have been termed 'anoxic epileptic seizures' [31]. ...
Article
Full-text available
This paper aims to improve the diagnosis of syncope and transient loss of consciousness (TLOC) in children. Diagnostic problems stem, first, from some causes spanning various disciplines, e.g. cardiology, neurology and psychiatry, while the most common cause, vasovagal syncope, is not embraced by any specialty. Second, clinical variability is huge with overlapping signs and symptoms. Third, the approach to TLOC/syncope of the European Society of Cardiology (ESC) is underused in childcare. We explain the ESC guidelines using an additional paediatric literature review. Classification of TLOC and syncope is hierarchic and based on history taking. Loss of consciousness (LOC) is defined using three features: abnormal motor control including falling, reduced responsiveness and amnesia. Adding a < 5 min duration and spontaneous recovery defines TLOC. TLOC simplifies diagnosis by excluding long LOC (e.g. some trauma, intoxications and hypoglycaemia) and focussing on syncope, tonic–clonic seizures and functional TLOC. Syncope, i.e. TLOC due to cerebral hypoperfusion, is divided into reflex syncope (mostly vasovagal), orthostatic hypotension (mostly initial orthostatic hypotension in adolescents) and cardiac syncope (arrhythmias and structural cardiac disorders). The initial investigation comprises history taking, physical examination and ECG; the value of orthostatic blood pressure measurement is unproven in children but probably low. When this fails to yield a diagnosis, cardiac risk factors are assessed; important clues are supine syncope, syncope during exercise, early death in relatives and ECG abnormalities. Conclusions: In adults, the application of the ESC guidelines reduced the number of absent diagnoses and costs; we hope this also holds for children.What is Known: • Syncope and its mimics are very common in childhood, as they are at other ages. • Syncope and its mimics provide considerable diagnostic challenges. What is New: • Application of the hierarchic framework of transient loss of consciousness (TLOC) simplifies diagnosis. • The framework stresses history-taking to diagnose common conditions while keeping an eye on cardiac danger signs.
... Согласно J. Stephenson et al. [7] домашние видеозаписи эпилептических приступов, вызванных обмороком, зарегистрированы у трех пациентов с «аноксическими эпилептическими приступами». Эти авторы также сообщили о 9 детях с обмороком, у которых первоначальный аноксический приступ спровоцировал настоящий эпилептический приступ [8]. В противоположность этим сообщениям детский невролог R. Appleton утверждает, что за 22 года наблюдений он никогда не видел ребенка с эпилептическим приступом, развившимся как осложнение РАС [9]. ...
Article
Full-text available
Reflex asystolic syncope — episodes of loss of consciousness and non-epileptic postural tone due to short-term reflex cardiac asystole caused by vagus nerve disorder and leading to subsequent transient cerebral hypoperfusion. Reflex asystolic syncope is characterized by rapid development, short duration and spontaneous recovery. Most commonly found in infants (first and second year of life). The condition is differentiated with epilepsy, syncope of another etiology, affective-respiratory attacks of the blue type, somatic diseases. During physical examination, special attention should be paid to the state of the cardiovascular system to suggest organic heart disease. History and physical examination data commonly reveal a possible cause of syncope. A small number of children need pharmacological therapy either with very frequent episodes in response to relatively minor triggers, or in cases of significant parental anxiety. Keywords: epilepsy, seizures, asystole, syncope, fainting, apnea, breathing.
... In many studies, piracetam was reported to make partial or complete remission and to have a significant difference with a placebo (7,12,28). prophylactically and in 5 of them seizures stopped (33). And a case of a girl having generalized seizures due to BHS was cured by psychotherapy administered to the mother and child (10 first-month and third-month values in terms of a decrease in seizure numbers as well as no seizure rates may be due to peak plasma concentration of levetiracetam attaining maximum value within one hour and attaining constant plasma concentration within two days when taken two doses a day (31). ...
Article
Full-text available
Introduction: In this study, the patients diagnosed with breath holding spell (BHS) or anoxic epileptic seizures and initiated at least one out of iron or piracetam or levetiracetam therapies were evaluated. Material and Methods: We retrospectively evaluated 194 BHS patients. Iron therapy was initiated in case of iron deficiency anemia or case of ferritin values under 12 ng/dl even if there was no anemia. The patients having no iron deficiency anemia, low ferritin and anoxic epileptic seizures were administered piracetam and the patients diagnosed with anoxic epileptic seizures were administered levetiracetam. Results: One hundred and eight patients (55.7%) were male. The mean age was 21.39±12.78 months. Iron therapy was initiated in 87 patients, piracetam to 96, and levetiracetam in 11 patients. Seizure numbers were manifestly decreased in all groups by the end of the first month after treatment concerning pretreatment levels (p<0.05). Conclusions: We determined that the spells were reduced or completely stopped in all groups. Levetiracetam seems to be considerably effective in patients developing anoxic epileptic seizures after BHS.
... (2004), домашние видеозаписи эпилептических припадков, вызванных обмороком, зарегистрированы у 3 пациентов с «аноксическими эпилептическими приступами» [118]. Эти авторы также сообщили о когорте из 9 детей с обмороком, у которых первоначальный аноксический приступ спровоцировал настоящий эпилептический приступ [68]. ...
Book
Full-text available
In the textbook, from modern positions, the issues of sy etiology, pathogenesis, clinical polymorphism, diagnostic ki, treatment, prevention of non-epileptic paroxysmal races structures in children. The manual is intended for neurologists, psychiatrists, clinical psychologists, as well as doctors of other specialties.
Article
Reflex epilepsy is a relatively rare form of epilepsy, occurring only in five percent of all cases of this disease. The genetic factors of reflex epilepsy are diverse and, in general, poorly studied. This review examines the main results obtained in recent years in the study of molecular genetic factors of reflex epilepsy, including new data on the mechanisms of genetic regulation in reflex epilepsy caused by triggers such as audio and video stimulation, food consumption, reading, contact with water and hypoxia. The results obtained in studies on animal models and patients using next-generation sequencing technology are presented.
Article
Many factors acting during the neonatal period can affect neurological development of the infant. Neonatal seizures (NS) that frequently occur in the immature brain may influence autonomic maturation and lead to detectable cardiovascular signs. These autonomic manifestations can also have significant diagnostic and prognostic value. The analysis of Heart Rate Variability (HRV) represents the most used and feasible method to evaluate cardiac autonomic regulation. This narrative review summarizes studies investigating HRV dynamics in newborns with seizures, with the aim of highlighting the potential utility of HRV measures for seizure detection and management. While HRV analysis in critically ill newborns is influenced by many potential confounders, we suggest that it can enhance the ability to better diagnose seizures in the clinical setting. We present potential applications of the analysis of HRV, which could have a useful future role, beyond the research setting.
Chapter
Status epilepticus (SE) is common in pediatric practice, particularly in infants: 40 per cent of all SE occurs in children under 2 years.1-4 In a prospective study of patients with newly diagnosed epilepsy, 9 per cent of 613 children had had one or more episodes of SE by the time of diagnosis.5 In a long-term follow-up study of a populationbased cohort of 150 children with epilepsy, 27 per cent had at least one episode of SE, usually within 2 years of onset.6 Gastaut7 defined SE as
Article
Two particularly common, and frequently frightening, forms of syncope and anoxic seizure in early childhood are pallid and cyanotic breath-holding spells, Pallid breath-holding spells result from exuberant vagally-mediated cardiac inhibition, Cyanotic breath-holding spells are of more complex pathogenesis, involving an interplay among hyperventilation, Valsalva maneuver, expiratory apnea, and intrinsic pulmonary mechanisms, The history is the mainstay of diagnosis; videotape documentation may be possible, Performance of an electrocardiogram to evaluate for prolonged QT syndrome should be strongly considered, in patients with pallid breath-holding spells, a characteristic sequence of changes may be documented on an electroencephalogram with ocular compression, if this study is performed, Spontaneous resolution of breath-holding spells, without sequelae, is anticipated, Reassurance is the mainstay of therapy, Occasionally, pharmacologic intervention may be of benefit.
Article
Three white female children (age range: 2 years, 7 months, to 11 years), who presented with syncopal attacks due to asphyxic (Case 1), ischemic (Case 2), and ischemic-asphyxic (Case 3) cerebral anoxia, are reported. All of them showed epileptic seizures, in the form of grand mal type (Case 1), “absence status” (Case 2), and absences (Case 3), induced by their syncopes. Case 1 showed, in addition, some massive myoclonic jerks facilitated by intermittent photic stimulation, whereas seizures in Case 3 also occurred spontaneously. The authors suggest that all patients had a constitutional (genetic) predisposition to epilepsy, and that under anoxic conditions overt epileptic phenomena were triggered.
Article
From clinical history 58 children were diagnosed as having reflex anoxic seizures secondary to provoked cardioinhibition (also known as white breath-holding attacks). Before referral, these seizures were commonly misdiagnosed as epileptic either because the provocation was ignored, not recognised, or was a febrile illness, or because there was no crying, no obvious breath-holding, little cyanosis, and often no pallor to suggest syncope and cerebral ischaemia. The duration of cardiac asystole after ocular compression was measured in these children and in 60 additional children with other paroxysmal disorders. In 45 (78%) of the 58 with reflex anoxic seizures asystole was 2 seconds or over, and in 32 (55%) it was 4 seconds or greater, an abnormal response. Review of the literature supports the concept that these seizures result from vagal-mediated reflex cardiac arrest which can if necessary be prevented by atropine. The simple name 'vagal attack' is proposed. Ocular compression under EEG and ECG control supports the clinical diagnosis if asystole and/or an anoxic seizure is induced; the procedure described is safe and should be routine in seizure or syncope evaluation, when a meticulous history still leaves room for doubt.
Article
In their recent publication in this journal, Southall et al described typical cyanotic breath holding spells, both in otherwise healthy children and in those with brainstem lesions and other malformations. Their suggestions regarding possible autonomic disturbances may require further study, but they have adduced no scientific evidence to contradict the accepted view that in the intact child blue breath holding spells are benign. Those families in which an infant suffers an 'apparently life threatening event' deserve immense understanding and help, and it behoves investigators to exercise extreme care and self criticism in the presentation of new knowledge which may bear upon their management and their morale.