Abdominal epilepsy
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Transcript of Abdominal epilepsy
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Best Practice & Research Clinical GastroenterologyVol. 19, No. 2, pp. 263–274, 2005
6
Abdominal epilepsy
Noah T. Zinkin1MD
Mark A. Peppercorn* MD
Professor of Medicine
Beth Israel Deaconess Medical Center, Harvard Medical school, 330 Brookline Ave.,
Rabb 4, Cambridge, MA 02215, USA
Abdominal epilepsy is an uncommon syndrome in which gastrointestinal complaints, mostcommonly abdominal pain, result from seizure activity. It is characterized by (1) otherwiseunexplained, paroxysmal gastrointestinal complaints, (2) symptoms of a central nervous systemdisturbance, (3) an abnormal electroencephalogram with findings specific for a seizure disorder,and (4) improvement with anticonvulsant medication. We review the history of the syndrome andanalyze all 36 cases reported in the English literature from the last 34 years. The most commongastrointestinal symptoms include abdominal pain, nausea and vomiting, while the most commonneurological symptoms include lethargy and confusion. After exclusion of more commonetiologies for the presenting complaints, workup should proceed with an electroencephalogram.Where the diagnosis is seriously considered, neurological consultation should be considered.Treatment typically begins with anticonvulsant medication, and resolution of symptoms withtherapy helps to confirm the diagnosis.
Key words: epilepsy; temporal lobe; nausea; vomiting; abdominal pain; anticonvulsants;electroencephalography.
DEFINITION
Abdominal sensations will commonly herald the onset of a motor seizure. This so calledabdominal aura, which may include sensations such as pain, nausea, gassiness andhunger, has been reported to be the most common aura in epilepsy, especially temporallobe epilepsy.1 On occasion, however, gastrointestinal signs and symptoms may be
doi:10.1016/j.bpg.2004.10.001available online at http://www.sciencedirect.com
1521-6918/$ - see front matter Q 2004 Elsevier Ltd. All rights reserved.
* Corresponding author. Tel.: C1 617 667 8926; Fax: C1 617 667 1071.
E-mail addresses: [email protected] (N.T. Zinkin), [email protected]
(M.A. Peppercorn).1 Tel.: C1 617 667 9355; Fax: C1 617 667 8242.
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264 N. T. Zinkin and M. A. Peppercorn
the primary or only manifestation of seizures. This phenomenon defines abdominalepilepsy.
The main criteria that establish a diagnosis of abdominal epilepsy include (1)paroxysmal gastrointestinal complaints that are unexplained by complete evaluation,including laboratory tests, radiographic imaging, and endoscopy, (2) symptoms of acentral nervous system (CNS) disturbance, (3) an abnormal electroencephalogram(EEG) with findings specific for a seizure disorder, and (4) a sustained abolition ofsymptoms on anticonvulsant medication.2 EEG recordings during events have been onlyrarely reported3,4 and are not necessary for the diagnosis.
HISTORY AND EVOLUTION
Despite its seeming rarity, many cases of abdominal epilepsy have been reported in theliterature, although many of the earlier cases are of questionable validity. Trousseau iscredited with the first description of abdominal epilepsy in 1868. He described a boywith paroxysmal attacks of abdominal pain, vomiting, giddiness, and pallor, who laterdeveloped grand mal seizures.5
In his landmark paper in 19446, Moore reported a 32 year old man with paroxysmsof abdominal pain followed by exhaustion, that had started at age 9 months. He had anextensive workup at multiple hospitals over many years with no etiology found. An EEGwas finally performed which revealed ‘low voltage abnormal waves seen particularly inthe right and left frontal regions.’ His attacks of pain resolved completely on phenytoin,and then recurred when therapy was stopped. Moore then published a second part tothis article in 1945 in which he presented 5 additional patients with paroxysmalabdominal pain.7 Of the total of 6 patients, 5 were treated with anticonvulsants andresponded favorably, although not all of the patients had abnormal EEGs. He alsoproposed a practical set of criteria for diagnosing abdominal epilepsy, similar to thosenoted above.
Moore later defined abdominal epilepsy as ‘a disorder characterized by bouts ofparoxysmal abdominal pain. provoked by abnormal discharges of certain neurones inthe vicinity of biochemically or structurally altered cerebral tissue.’.8 By 1952, he hadreported 28 cases of abdominal epilepsy.9 His reports were somewhat unusual in thatmost cases were in adults.
In the 25 years after Moore’s initial formal description of the entity, many moreauthors reported case series of patients, mostly children, with suspected abdominalepilepsy.5,10–17 However, while many of these were no doubt genuine cases ofabdominal epilepsy, the standards by which cases were diagnosed and reported werepoor, with many patients having atypical presentations, normal or non-specific EEGs,and no improvement with anticonvulsants. For example, several authors reported onpatients with recurrent abdominal pain and the so called ‘14 and 6 positive spikes’pattern on EEG.18 It was postulated that this pattern was actually indicative ofabdominal epilepsy.19 However, this pattern was later identified in over 50% of normalchildhood and adolescent EEGs, with no association with recurrent abdominal pain.20,21
It is now considered a normal variant.22
Then, in 1971, Douglas and White23 published another landmark article in whichthey reviewed the literature on abdominal epilepsy and noted that previous authors hadtoo often based the diagnosis on insufficient evidence. They investigated 28 patientsreferred to a neurology clinic for recurrent abdominal pain and found that only 5 had
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Abdominal epilepsy 265
clinical and EEG findings strongly suggestive of epilepsy. They stressed the importanceof both disturbances of consciousness and specific EEG abnormalities as the types ofevidence that could truly separate abdominal epilepsy from other causes of recurrentabdominal pain. They also pointed out that, although helpful, response to antic-onvulsants was non-specific since their sedating properties alone could improve non-epileptic symptoms.
CLINICAL FEATURES
The practicing physician familiar with abdominal epilepsy is aware that a true case isquite uncommon. Most reported cases are in children and adolescents. In onestudy of 858 patients with epilepsy, only 3 had abdominal pain as a prominentfeature of their seizures24, while another found no cases of abdominal epilepsyamong 127 patients with somatosensory seizures.25 However, its true prevalence isunknown.
Since Douglas and White laid the groundwork for more rigorous standards inthe reporting of cases of abdominal epilepsy in 1971, we found only 36 reportedcases in the English literature. We reviewed all such cases and present thefeatures of each with regard to age at presentation, sex, gastrointestinal symptoms,CNS and other non-gastrointestinal features, EEG, treatment, and outcome(Table 1).
Patients ranged from 1 to 66 years old at presentation and 19/36 (53%) werefemale. Gastrointestinal manifestations of seizures included abdominal pain, mostcommonly sharp or colicky, in 31 (86%), nausea and/or vomiting in 10 (28%),diarrhea in 2 (5%), and bloating in 1 (3%). Where reported, the abdominal pain wasmost commonly periumbilical or upper abdominal, including epigastric and rightupper quadrant, but left lower quadrant, lower abdominal, and migratory pains werereported in one patient, each. Neurological and other non-gastrointestinalmanifestations that were reported in more than one patient included lethargy,fatigue and/or post-ictal sleep in 13 (36%), some level of disturbance inconsciousness in 23 (64%), including loss of consciousness or generalized tonic–clonic seizures in 13 (36%), dizziness in 3 (8%), headaches in 5 (14%), pallor/sweatsin 4 (11%), fever in 2 (6%), extremity pain, numbness and/or paresthesias in 2 (6%),and blindness in 2 (6%).
However, in any individual case, each episode was not necessarily the same. Forexample, many of the patients with recurrent abdominal pain had associateddisturbances of consciousness, but not necessarily with every episode. Some patientshad typical generalized tonic–clonic seizures at times, but then purely sensoryseizures, characterized only by abdominal pain, at other times.
The duration of episodes, where reported, was typically no more than a fewminutes, but 2 had symptoms lasting for an hour or more. Most patients did haveabnormalities on EEG characteristic of epilepsy but one patient actually had normalEEGs, even during events.
Where reported, all patients were treated with anticonvulsants, most commonlyphenytoin, phenobarbital, or carbamazepine. Four patients received anticonvulsantsand surgery, one of whom also underwent radiation therapy. The outcome for allpatients was favorable in that all had at least some improvement with therapy, mostwith complete or near complete resolution of symptoms.
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Table 1. Clinical characteristics of all reported cases of abdominal epilepsy since 1971.
Patient
number Age Sex
Gastrointestinal
symptoms
CNS disturbance
and other non-gas-
trointestinal
symptoms
Episode
duration EEG Treatment Outcome
123 11 F Paroxysmal, peri-
umbilical abdominal
pain
Lassitude, post-ictal
sleep, fever, head-
ache, confusion
‘Brief’ Irregular 3 Hz spike-wave activity Phenobarbital Symptom free
223 5 F Crampy, paroxys-
mal abdominal pain
Lethargy, post-ictal
sleep
Few minutes Episodic 6–7 Hz activity in L temporal
area, bursts of generalized irregularly
intermixed spikes and slow waves
Phenobarbital Lost to
follow-up
323 18 F Colicky pain Post-ictal sleep 10 minutes Bitemporal spiking Phenobarbital Symptom free
423 6 M Paroxysmal pain Lethargy, confusion,
fever
Minutes Paroxysmal spike-wave activity, frontal
or generalized
Anticonvul-
sants
Seizure free
523 26 M Colicky abdominal
pain
Lethargy, confusion,
post-ictal sleep
Few minutes ‘Compatible with temporal lobe epi-
lepsy’
Phenytoin Seizure free
626 39 M Abdominal pain
(variable location)
Fatigue, headache,
sweating, numb-
ness, paresthesias
NR ‘Abnormal electrical activity suspicious
for epilepsy’
Phenytoin,
phenyl-ethyl
barbiturate,
carbamaze-
pine
Complete res-
olution
72,27 47 F Periumbilical pain Loss of awareness,
confusion
NR Irregular spikes bilaterally over tem-
poral areas
Phenytoin Complete res-
olution
82,27 44 F Periumbilical pain Headaches NR Low voltage spikes in left anterior
temporal region, sharp waves on right
during light sleep
Phenytoin and
phenobarbital
Near complete
resolution
92,27 30 F Periumbilical pain Syncope NR Intermittent sharp wave complexes in
right and left anterior temporal regions
Phenobarbital Complete res-
olution
102,27 17 F Periumbilical pain Blindness NR Diffuse bursts of theta activity over left
post. temporal area, and left post.
quadrant reversals of slow theta waves
and sharp waves
Phenytoin Complete res-
olution
266
N.T
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inkin
and
M.A
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1128 3.5 M Abdominal pain,
vomiting
Confusion, cyano-
sis, urinary inconti-
nence, blindness
Minutes Scattered high voltage slow activity and
high voltage sharp waves
Phenytoin,
phenobarbital
Symptom free
1228 38 M Periumbilical pain Confusion, per-
spiration, pallor
NR Diffuse high voltage sharp waves with
hyperventilation
Phenobarbital Symptom free
1328 16 M Upper abdominal
pain, nausea
Disturbance of
consciousness,
convulsions
3–5 minutes High voltage slow waves; high voltage
sharp waves with hyperventilation
Phenobarbital Symptom free
1428 35 M Lower abdominal
pain, nausea,
vomiting
Confusion, loss of
consciousness
Half an hour Paroxysmal high voltage single spike-
wave complexes with secobarbital acti-
vation
Phenytoin,
primidone,
phenobarbital
Symptom free
1528 11 F Periumbilical
abdominal pain
Disturbance/loss of
consciousness
Minutes to
an hour
Bilateral high voltage spikes, complexed
slow waves
Phenytoin and
phenobarbital
Symptom free
1629 31 M Projectile vomiting
without nausea
Unresponsiveness,
(patient also had
separate atonic,
tonic–clonic,
absence and focal
motor seizures at
other times)
NR Bilateral central 16 Hz paroxysmal
sharp waves preceding each episode
Phenytoin,
phenobarbital,
clonazepam
Seizure free
1730 6 M Vomiting Bad smell, fatigue 20–
40 seconds
Ictal and interictal high voltage arrhyth-
mic delta waves, sometimes sharply
contoured
Multiple anti-
seizure medi-
cations, then
surgery and
radiation (for
astrocytoma)
Decreased fre-
quency of epi-
sodes
1824 18 M Epigastric pain Loss of awareness,
staring, elevation of
right arm
NR Left midtemporal spikes NR NR
1924 21 M Sharp abdominal
pain
Loss of conscious-
ness, automatisms
NR Multiple independent spikes NR NR
(continued on next page)
Abdom
inal
epilep
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Table 1 (continued)
Patient
number Age Sex
Gastrointestinal
symptoms
CNS disturbance
and other non-gas-
trointestinal
symptoms
Episode
duration EEG Treatment Outcome
2024 15 F Abdominal pain Vague ‘head sen-
sation,’ generalized
tonic seizures
NR Multiple independent spikes NR NR
2131 24 F Crampy periumbili-
cal pain, nausea,
diarrhea
Weakness, lethargy,
syncope
10 minutes
to 4 hours
Rare bursts of 14 and 6/s positive spikes
on posterior temporal areas
Phenobarbital Complete res-
olution
2232 10 M Periumbilical pain Pallor, sweats,
lethargy, post-ictal
sleep
Minutes Sharp spikes, and spike and wave activity
arising over the central region and
becoming generalized
Phenytoin Complete res-
olution
232 29 F Diarrhea Confusion, mem-
ory loss, difficulty
concentrating
NR Bursts of spike activity over right
temporal region
Valproic acid Symptoms well
controlled
242 34 F Nausea, bloating Dizziness Several
hours
Bitemporal sharp waves and spikes Carbamaze-
pine
Complete res-
olution
252 38 F RUQ pain, nausea Fatigue NR Bursts of sharp theta slowing over left
temporal area
Phenytoin Complete res-
olution
262 48 F RUQ pain Headaches and diz-
ziness
NR NR Phenytoin Complete/near
complete resol-
ution
272 18 F RUQ pain Confusion NR NR Phenobarbital Complete/near
complete resol-
ution
282 35 F Periumbilical pain Fatigue NR NR Carbamaze-
pine
Complete/near
complete resol-
ution
2933 6 M Colicky periumbili-
cal pain
Lassitude, post-ictal
sleep
Half an hour Generalized slowing, right posterior
spikes
Carbamaze-
pine
Symptom free
268
N.T
.Z
inkin
and
M.A
.Pep
perco
rn
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303 14 F Colicky periumbili-
cal pain
Headache, pallor,
dizziness, multico-
lored photopsia
Seconds to
minutes
Interictal-bursts of sharp and slow waves Valproic acid Near complete
resoluton
Ictal-repetitive spikes, sharp waves,
high-voltage slow waves
3134 26 M Sharp RLQ pain Occasional general-
ized seizures
1–2 minutes Interictal and ictal EEGs normal NR NR
3234 30 M Nausea, crampy
LLQ pain
Pain and numbness
in left arm, alien
hand, occasional
generalized sei-
zures
!30 min-
utes
Right parietal lobe focus Resection of
gliotic scar
from prior
tumor resec-
tion
Rare seizures
3334 1 F Crampy periumbili-
cal pain
Occasional general-
ized seizures
Few seconds Right parietal focus NR NR
3435 66 M Nausea without
vomiting
Disorientation and
confusion, fatigue
1–2 minutes Right anterior temporal sharp waves Carbamaze-
pine
Nausea free
354 37 M Epigastric pain Disturbance of
consciousness,
occasional general-
ized tonic–clonic
seizures
Seconds to
hours
Interictal-epileptogenic focus in left
frontotemporal region
Carbamaze-
pine, then left
amygdalo-
hippo-cam-
pectomy for
mesial tem-
poral sclerosis
Complete res-
olution
Ictal-flattening in same region before
seizure onset
3636 6 F Abdominal pain Disturbed aware-
ness, occasional
generalized tonic–
clonic seizure
Seconds to
minutes
Spike and slow waves over left temporal
area
Anticonvul-
sants, surgical
resection of
oligoastrocy-
toma
Seizure free
NR, not reported.
Abdom
inal
epilep
sy269
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270 N. T. Zinkin and M. A. Peppercorn
DIAGNOSIS
The diagnosis of abdominal epilepsy begins with eliciting a history typical for the syndrome,namely paroxysmal and brief episodes of abdominal pain or other gastrointestinalsymptoms. Chronic symptoms are not consistent with the diagnosis, but even episodes ofsymptoms that last more than a few hours are unlikely to be from abdominal epilepsy. Thepresence of associated neurological symptoms, including convulsions, impaired con-sciousness, and other sensory phenomenon, is an important clue and should be elicitedwhen considering the diagnosis. However, neurological symptoms may not be present withevery episode and, in any event, may not be recognized by the patient.
Next, an appropriate evaluation, including physical and neurological examination,laboratory studies, endoscopy, and abdominal imaging, usually with computed tomographyand ultrasound, should be performed to exclude other, more common etiologies. Wherenone is found, or the history is still suggestive, EEG should be performed to look forabnormalities specific for epilepsy. These so called interictal epileptiform discharges (IEDs)consist of paroxysmal spikes and/or sharp waves. Other abnormalities are either less wellor not at all correlated with epilepsy.37 If clinical and EEG findings are consistent withabdominal epilepsy, imaging of the brain, preferably with MRI, should be performed, sinceseveral cases have resulted from structural lesions of the brain.
It should be noted, though, that while the interictal EEG is usually abnormal, the changescan be non-specific.38 No studies on the sensitivity of EEG for abdominal epilepsy have beenperformed. However, in epilepsy as awhole, the initial EEG will show IEDs in only 29–55% ofcases. The yield can be substantially increased, however, by activation methods such as sleepdeprivation, by obtaining sleep recordings, and by performing repeated EEGs at differenttimes.37 Non-specific abnormalities may warrant further investigation, but they are notsufficient alone to confirm the diagnosis of abdominal epilepsy. Even an EEG recorded duringaneventcanbenormal if there isnoassociatedconvulsionor impairmentofconsciousness.39
In addition, no particular EEG findings will distinguish abdominal epilepsy from other formsof epilepsy. Where doubt remains, consultation with a neurologist should be considered.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis will typically include other elusive causes of paroxysmalgastrointestinal symptoms, such as porphyria, familial Mediterranean fever, cyclicalvomiting, and abdominal migraine. The latter deserves special notice because it hasoften been confused in the literature with abdominal epilepsy. Abdominal migraine ischaracterized by recurrent attacks of periumbilical or poorly localized abdominal pain,commonly associated with anorexia, nausea, vomiting, and pallor.40 Headache is not anecessary feature. Although more common in children, it is seen in adults, but itsprevalence in adults is unknown.41 A family history of migraine is common.42
In the years after Moore first fully characterized abdominal epilepsy, some postulatedthat previous cases of abdominal migraine were actually cases of abdominal epilepsy.Moore doubted the very existence of abdominal migraine43, while Prichard suggestedthat abdominal epilepsy could be distinguished from abdominal migraine by the pattern ofpain and EEG findings.14 In practice, they can be difficult to differentiate, but there aresome helpful distinguishing features. In abdominal migraine, the pain is characteristicallymore dull and of longer duration, with episodes usually lasting between 1 hour and 1–2
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Abdominal epilepsy 271
days.41,42 In addition, EEG abnormalities characteristic of epilepsy and a response toanticonvulsant therapy favor a diagnosis of abdominal epilepsy.
PATHOPHYSIOLOGY
Seizures are commonly categorized as either simple partial seizures, where consciousness ispreserved, complex partial seizures, where consciousness is impaired, and generalizedseizures, where consciousness is lost. Seizures can manifest with sensory symptoms, such asstrange odors or abdominal pain, motor symptoms (convulsions), or both. Where sensorysymptomsareassociatedwithan impairmentor lossofconsciousness, thesensorysymptomsare referred to as an aura. Multiple types of seizures may be present in the same person.35
How seizures can induce abdominal sensations has been studied but is still notprecisely known. Stimulation of temporal lobe structures, including the amygdala,hippocampus, and insular cortex, has been shown to induce abdominal sensations,including nausea, hunger, and ‘funny feelings’, in humans.1,44,45 Others have producedchanges in gastrointestinal secretion and motility in animals by stimulation of the limbicsystem.46 Thus, one might postulate that the seizures of abdominal epilepsy areactivating those neurons that ordinarily are responsible for abdominal sensations.
However, some have postulated that the etiology of the abdominal symptoms couldresult from true visceral stimuli which, through connections to the brain, could therebyinduce seizures.2 For example, Gastaut and Poirier reported a case of a 9-year-old boywith seizures, always heralded by attacks of abdominal pain.47 They then experimentallyreproduced these seizures with cold water barium enema, postulating that some casesof abdominal pain associated with seizures could be the results of noxious intestinalstimuli lowering the seizure threshold. However, cases of abdominal epilepsy have beentraced to head injury7, brain tumors30, mesial temporal sclerosis4, developmental braindisorders3, and glial scar34, suggesting that the primary disturbance in at least somecases lies within the brain. While most cases of abdominal epilepsy are of temporal lobeorigin2, several cases have also been traced to parietal lobe lesions.34
Of note, the distinction between ‘abdominal epilepsy’ and other forms of epilepsy maybe overestimated. Painful abdominal auras which then herald generalized tonic–clonicseizures are a well described phenomenon and, like the seizures of abdominal epilepsy,most commonly localize to the temporal lobe.48 Furthermore, many patients reported tohave abdominal epilepsy have associated generalized tonic–clonic seizures, at least at times.Thus, rather than considering abdominal epilepsy to be a separate entity, it may be moreappropriate to simply recognize that some patients will have significant gastrointestinalsymptoms as a manifestation of their seizures. In some, these symptoms may herald theonset of a generalized seizure, where they might be referred to as an ‘abdominal aura.’ Inothers, they might stand alone, or only be associated with some more subtle neurologicalsymptoms, such as confusion or lethargy. In these cases, the etiology of the symptomswould be more difficult to recognize, but the astute physician might still recognize thesyndrome and subsequently make a diagnosis of ‘abdominal epilepsy.’
TREATMENT
Once the diagnosis of abdominal epilepsy is confirmed, treatment can commence aswith other forms of epilepsy. Treatment usually starts with anti-seizure medication,
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272 N. T. Zinkin and M. A. Peppercorn
typically phenytoin. Additional medication can be started in addition to, or in place of,the initial choice of therapy, based on the clinical response. Of note, no controlled trialshave been published regarding treatment of abdominal epilepsy.
It should be noted again that, while helpful for confirming a diagnosis of abdominalepilepsy, a response to therapy alone is not diagnostic. Anti-seizure therapy mayimprove many non-epileptic causes of abdominal pain, either by its sedating effect or asa placebo. Furthermore, some cases of epilepsy, including abdominal epilepsy, can berefractory to medication.4,30 Fortunately, the prognosis is generally good, with mostpatients having dramatic improvement in symptoms with antiseizure medication alone.
SUMMARY
Abdominal epilepsy is an uncommon disorder, more often seen in children than adults,in which gastrointestinal symptoms result from seizure activity. Diagnostic criteriainclude (1) otherwise unexplained, paroxysmal gastrointestinal complaints, (2)symptoms of a CNS disturbance, (3) an abnormal EEG, with findings specific for aseizure disorder, and (4) improvement with anticonvulsant medication. After thesyndrome was initially defined by Moore in 1944, many more cases were reported.However, as authors have more recently adhered to more rigid diagnostic criteria,fewer cases have been described, with only 36 cases reported in the English literature inthe last 34 years. Among these cases, the most common gastrointestinal complaintsincluded abdominal pain, which is typically severe and colicky or sharp, and nauseaand/or vomiting. The most common neurological signs and symptoms includedfatigue and disturbance or loss of consciousness. Evaluation should include a carefulhistory and physical, with particular attention to neurological signs and symptoms, andan appropriate evaluation for more common causes of the symptoms. When otherpathology has been adequately excluded, an EEG should be obtained. The EEG is usuallyabnormal, but the findings may be non-specific. Obtaining sleep, sleep deprived, orserial EEGs may improve the yield, and neurological consultation should be considered.The prognosis is generally favorable, with most patients having complete or nearcomplete resolution of symptoms with anticonvulsant therapy.
Practice points
† abdominal epilepsy is defined by the occurrence of gastrointestinal symptomsas the primary or only manifestation of seizures
† abdominal epilepsy causes paroxysmal, but not chronic, gastrointestinalsymptoms
† common gastrointestinal symptoms include abdominal pain, nausea, andvomiting
† associated CNS disturbances are helpful in distinguishing abdominal epilepsyfrom other causes of abdominal pain
† EEG is crucial in confirming the diagnosis, and consultation with a neurologistshould be considered
† treatment with anticonvulsants is generally effective at relieving bothneurological and gastrointestinal symptoms
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*
*
Abdominal epilepsy 273
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