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BRIEF COMMUNICATION
Benign focal epilepsy of childhood and
gastroesophageal reflux
ANNE G SHEEHAN MB MRCPI, SHERRY PELENSKY RN, COLIN VAN ORMAN MD FRCPC, STEVEN R MARTIN MD FRCPC
AG SHEEHAN, S PELENSKY, C V<A,N ORMAN, SR MARTIN. Benign focal epilepsy of childhood and ~ux. Can J Gastroenterol 1994; 8( l ):45-48. Gastrnesophageal te6m( has~ iwociated with, and implicated in, a number of conditions, inc~teSl)~ disease (recurrent pneumonia, chronic cough, asthma), sudden infiot dead>. syndrome, dysphagia and central nervous disorders. An eight-year-old girl p:.ented with an acute history that suggested gastroesophageal reflux. AD~~l motility study was abnormal and 24 h pH study demonstrated~ reflux. Before the manometric study, a seizure was ob~erved and ~l\eU(Ological evaluation confirmed the diagnosis of benign focal epilepsy of dilldhood, which was treated with carbamazepine. The symptoms resolved after eight weeks and the repeat reflux investigations were essentially normaL Ompbuyngeal symptoms are common in benign focal epilepsy of childhood, a condition which is very responsive to therapy. Symptoms suggestive of this dlagnostS - acute onset, with unusual oropha1y ngeal sensations, or seizures-occUl;ring mainly at night may initially be confused with gastroesophageal reflux. Benign focal epilepsy of childhood should he considered in reflux presenting outside infancy.
Key Words: Children, Epilepsy, GastroesophageQlreflux, Manomecry
Epilepsie partielle benigne infantile et reflux gastro,oesophagien
RESUME : Le reflux gastro-oesophagien a &l associe a divers etats morbides, notamment a ccrtaines affections respiratoires (pneumonie recidivante, toux chroniquc, asthme), syndrome de mort subite du nourrisson, dysphagie e t affections tlu systeme nerveux central. Une fillette de huit ans a presente un tableau a igu indicatcur de reflux gastro-oesophagien. l'epreuve de motilite oesophagienne s'est revelec anormale et la mesure du pH sur 24 heures a confirme le reflux gascro-oesophagien. Avant !es epreuves de manometrie, une convulsion a etc observee et l'evaluatinn neurologique subsequente a confirme un diagnostic d'epilcpsie partic lle benigne infantile qui a ete traitee avec de la carbamazepine. Les symptomes sont rcmres clans l'ordre apres huit semaines et a la reprise, les cpreuvcs sur le reflux sc sonc revelee~ essentiellement nonnalcs. Les symptomes
Department of Pediatrics, Alberta Children's Hospital; and University of Calgary, Calgary, Alberta
Corres/JOndence: Dr SR Martin, Gastroenterowgie/Nutrwon, I lopital Ste-Justine, 3175 Core Ste Catherine, Montreal, Quebec l-l3T 1C5. Telephone (514) 345-4626, Fax (514) 345-4999
Received for publication October 15, 1992. Accepted]uly 15, 1993
CAN J GASTROENTEROL VOL 8 No 1 JANUARY/FEBRUARY 1994
BENIGN FOCAL EPILEPSY OF CHILD
hood (BFEC) is defined by five main characteristics. It is age-related, beginning between the ages of two and 13 years, with a spontaneous remission during adolescence ( 1). It occurs in otherwise normal children without neurological or intellectual deficit; in the majority of cases the seizures are partial, with motor signs frequently associated with somatosensory symptoms, and are sleep-related in 75% of patients. The incerictal electroencephalogram displays a spike focus locate<l in the centrotemporal (rolandic) area with normal background activity.
A patient is presented with BFEC, whose clinical history and marked oropharyngeal symptoms initially suggested gastroesophageal reflux which was confirmed in a 24 h pH study. C linical symptoms and documented reflux resolved with seizure therapy. We suggest that gastroesophageal reflux may occur in BFEC and may account for the marked oropharyngeal symptoms described in this condition.
CASE PRESENTATION An eight-year-old Caucasian girl
presented to the gastrocnterology clinic of Alberta Children's Hospital with a seven-week history of 'retching episodes', abdominal pain and choking spells. The patient described episodes beginning with an unusual sensation arising in her stomach, which would
45
SHEEI JAN er al
oropharynges sont frequents clans l'cpilcpsie partielle bcnigne infantile, une maladie qui repond tres bien au traitement. Les symptomes qui concordenr avec ce diagnostic sont le declenchement rapide, les sensations oropharyngees inhabituelles ou !es convulsions survemmt surtout la nuit et la possibilite d'une confusion initiate accompagnant le reflux gastro-oesophagien. L'epilepsie partielle benigne infantile doit etre envisagce lorsqu'il y a reflux, si l'enfant n'est plus un nourrisson.
TABLE 1 Esophageal manometry and 24 h pH study results before and following eight weeks of therapy with carbamazepine
At presentation After therapy Normal values
Esophageal manometry LESP (mmHg) 5 4 30.6±9.2 (reference 2)
UESP (mmHg) 120 30 21.3±7 .4 (reference 3)
24 h pH study Mean ± so• Mean ± 2S0
%time pH <4 31 3 2.3±1.9 6.1
Number of refluxes 164 50 11.1±6.1 23.3
Longest reflux 124 4 12.6±9.4 31.4 (mins)
Refluxes >5 mins 13 0 2. 1±1.8 5.7
LESP Lower esophageal sphincter pressure; UESP Upper esophageal sphincter pressure. 'Reference 17
migrate to the back of her throat. She would often notice sour tasting fluid in her mouth and begin to retch and cough. These episodes lasted 10 to 15 mins and occurred at any hour, but mostly at night, often waking her from sleep. They gradually increased in frequency to JO to 20 times daily.
The patient's physical examination was essentially unremarkable. Her weight was 30 kg (above the 50th percentile), her height was 134.5 cm (on the 90th percentile) and she had normal vital signs.
Investigations revealed a normal complete blood count, electrolytes, glucose, blood urea nitrogen, creatinine, total protein, albumin, liver function tests, amylase and lipase. Urinalysis was unremarkable. A barium swallow demonstrated no evidence of anatomical obstruction, showing only mild gastroesophageal reflux.
The patient's initial symptoms, which preceded the retching and coughing, suggested that gastroesophageal reflux was precipitating these episodes, so an esophageal motility study, 24 h pH study (Table 1) and esophageal biopsy were carried out without sedation. On insertion of the motility catheter, however, the patient
developed central cyanosis with twitching movements of her eyelids lasting 2 mins, during which she was unresponsive to voice. The catheter was withdrawn and after she had recovered from the episode the motility study was carried out without problem. T he lower esophageal sphincter pressure was extremely low. Normal peristaltic swallow waves were demonstrated in the esophageal body but the upper esophageal sphincter showed extremely high pressures (Table 1). The 24-hour pH study proved co be grossly abnormal (Table 1). Esophageal biopsy, from 3 cm above the lower esophageal sphincter, was normal.
In view of the seizure activity that had occurred during the study, a neurological opinion was sough t, and an electroencephalogram and computerized tomographic (CT) scan of the brain were performed. The electroencephalogram was abnormal with a sharp spike focus in the right central region, the field of distribution extending into the right midtemporal and midfrontal areas; CT scan was normal. The findings were considered to be diagnostic of BFEC and the patient was started on carbamazepine. Because of the abnormal pH study and the concern that
acidification of the esophagus might be stimulating seizures, she was also started on ranitidine. In the following weeks, the patient's seizure episodes gradually reduced in frequency and severity, and eight weeks later had stopped completely.
Two weeks after disconttnuing rarn tidine, the motility study and 24 h pH study were repeated. The lower esophageal sphincter pressure was again low but the upper esophageal sphincter pressure was normal. Repeat 24 h pH study on this occasion was entirely different from the previous one, the only abnormality being nn increase in mnnber of reflux episodes. An esophageal biopsy was not repeated because it was previously normal. The patient remains asymptomatic on carbamazepine.
DISCUSSION Primary gastrocsophageal reflux is
commonly seen in infancy with an estimated incidence of l :500 ( 4 ). The majority of these patients have functional reflux which may be perceived as a developmental d isorder which resolves in most patients by age four years ( 5). A small percentage of these patients may develop pathological gastroesophageal reflux associated with dysphagia, esophagitis, strictures, poor growth, respiratory problems or Sandifer's syndrome, and these patients usually require chronic medical therapy or surgical intervention (5,6).
In addition, in infancy- but more so in childhood - gastroesophageal reflux may occur as a secondary phenomenon in other <lisease states, cg, cystic fibrosis (7) and motility disorders of the esophagus (8), but most commonly in association with central nervous system disorders (9). A recent report described the association of gastroesophageal reflux with brain-stem glioma in three infants, the suggested mechanism being that the tumour infiltration of the brain-stem impaired neurogenical contro l of esophageal motility, resulting in gastroesophageal reflux (10). This report <lcscri bes gastroesophageal reflux in association with untreated BFEC, further supporting the central influence on esophageal function.
RFEC' is the most common type of
46 CAN J GASTROENTEROI VOL 8 N O l jANUARY/FEBRl.,ARY 1994
partial minor epilepsy in childhood, accounting for 11 .5 to 25% of the epilepsies of ~chool-age children (11 ). Oropharyngeal symptoms arc reporred by more than half rhe patients and include hypcrsalivation with inability to swallow, 'sounds from the throat', gurgling noise as if the child were about to
vom1r, moven1l'nts nf the mouth and speech arrest Jue to tonic or clonic phenomena mvolving the mouth (anJ probably the larynx) (1,11 ). In the case descnhed here, we documented severe gastroesophageal reflux over 24 h before commencing therapy for seizures due to Bf-TC. A repeat stuJy after eight weeb of anricon vu lsant therapy demonstrated a dramatic reduction in number and duration of reflux episodes (Table I). Jc was fortuitous that during insertion of the motility catheter, the patient developed an episode of eyelid twi tching and cyanosis which her mother had not previously described (possibly since most episodes occurred in sleep). The very high upper esophageal sphincter pressure demonstrated afterwards was no longer evident on repeat study e ight weeks after carbamazepine therapy. Lability of upper esophageal sphincter pressure has been described previously ( 12- 14 ); reductiorn, in pressure occur wi th sedation and increases occur with esophageal acidification, arousal, emotional st ress and 111creased abdom111al and tho racic. pressure Jue to stra ining. We cannot discount the possible effects of a seizure within the previous 10 or 15 mins, al-
ACKNOWLEDGEMENTS: The authors thank Chem Br(x1ks an<l Bev Harley for their assistance m preparing the manuscript.
REFERENCES l. Lrnseau P, Duche B. BeniJ,?"n <.:h1l<lhnod
epi lepsy with centrotemporal spike~. CleveClm J Med 1989;56:17-21.
2. Moroz SP, Espinoza J, CumminJ,?' WA, Diamant NE Lower esophageal sphincter function in children with an<l wichout ga.,troesophageal reflux. Gastroenterology l 97 6; 71 :236-41.
}. Sondheimer JM. Upper esophageal sphincter an<l phar,ngoesophagcal motor function in infants with an<l without gastrnesophageal reflux. Gastroenterology 1983;85:301-5.
4. Boyle J Gastroesophageal reflux m the
though the patient was not drowsy and was not experiencing any symptoms du ring the study. The measured upper sphincter pressure was well above that previously dcscriheJ in studies of factors increasing upper esophageal sphincter pressure, and the tnplc lumen, continuously perfused catheter system used here ha::- hcen thought to
underestimate the true sphincter pressure compared with sleeve catheters ( l 2-14). Perhaps high upper esophageal sphincter pressure explaim the 'lump in rhe throat' feeling and the hypersal1vatilln that is descnbed 111 this form of partial epilepsy. Possibly it is a protective mechamsm to prevent aspiration from gastroesophageal reflux during these episodes.
The pathophysiological mechanisms of gastroesophageal reflux are multifactorial. Enological mechanisms include a low basal sphincter pressure, inappropriate sphincter rclaxanon which is enher synchronous or asynchronous with swallowing, and transient increases in intra-abdominal or gastric pressure alone or in comh111ation with the above mechanisms (15,16). Resting tone in the lower esophageal sphinc ter 1s influenced hy neural factors. The typical electroencephalogram seen in BFEC is that of centrotemporal spikes on normal ha<.kground activ ity. Perhaps discharges from this area md1recrly lead to mappropriatc relaxation of the lower esophageal sphmcter, resulting m reflux, and an associated e levation in up-
pediatric patient. Gastmenternl Clin Nllrth Am 1989;18:315-37.
5. Came IJ. A H1s1nncal Review of thl· Clm1cal Cnnscquen<.:l'\ of H1aral I lcrma (p.irt ,al thorauc st111rn1d1) ,md Gasrrnes11phageal Reflux. In, Gellin SS, ed. Gastroesophageal Reflux. Rep,m of the Ros, Confl'rence on Ped1atrn: Research. C11lumhu,. Ross, 1979· 1-l 2.
6. Carrn-Sm1th AC, Mad1id.1 11, Bui:nl'r JD, Gall DG, Swtt RB. The role of gastmc,ophageal reflux m pe<lmtnc <lysphag1a. J Pe<liatr Gastrocnteml Nutr l991;12:l59-65.
7. Scott RB, O'LiuJ.!hlm EV. Gall DG. Oastrocsophagl'al reflux in pauents with cystt( fihrosis. J Ped1.1rr 1985;106:22.3-7.
8. Mahony MJ, Migltavan:a M, '>ptt: L,
CAN J GASTROENTEROI. VOL 8 NO I JANUARY/FFRRUARY 1994
Gastroesophageal reflux and epilepsy
per esophageal sphincter pressure. lt is possible that centrally ml.!diated me(han1sms play a higger role 111 the pachophys1ology of gastrocsophageal reflux rhan is realized; iJcally, m dus case, chi~ might have bl.!en examined wtth simultaneous electrocnci.!phalogram and esophageal pl I mon1tonng. Due to the frequency llf sei:urcs, we were unable ro ohtam pt:nmsston to perform these further studies, but they should be considered 1f the opportunity arise:,, 111 future <.<1scs.
CONCLUSIONS C,astroesophageal reflux, whteh 1s
common in infancy, b,s commonly presents in childhood. In the appropriate settmg, one should consider the possibility llf an associated under lying disease state. HFE< 1s the most common form of p,irtial t:pilepsy in childhood and, 111 the absence of observed seizures, the clinical history of ornpharynge,11 symptoms may suggest gastroe,ophagcal reflux. In the presented GN:, reflux was do<.umenced 111 associa tion with RFEC which rcsolve<l with trl'Htmcnt of the epilepsy. G iven the like lihood of central effects on esophageal functmn , further studies arc required of oth l.!r ch ildren with BFEC or cnmplcx partial ~e1zurcs with oropharyngeal phenomena to evaluate the prc~cncc of gastroesophageal reflux anJ to ohra111 more precise in
formation on the effoct of seizure activity on upper esophagl.'al sph111ctcr pressure.
Milla PJ. M11tor disorder, of the oe,ophagll', m gastr11esophageal reflux. Arch Dis Ch ild 1988;6 3: I H3-8.
9. Jolley SG, I krhst JJ, John,on DG, M;itl,1k ME, Boni LS. Surgery 111
childrL·n with gast rocsophageal reflux an<l re,p1ralnr} symptnm,. J Pc,li.itr 1980;96; 194-8.
10. Mahony MJ, Kennedy JD, Leaf H, Matthew DJ, Milla PJ. Rram stem gl1oma pre,entmg as J.?'ilstroesnphagcal rdlux. Arch Dis Child 1987;62:731-7.
11. A1cardi J. Simple partial seizures. In: Epilepsy m Ch1l<lren {lnternauonal Review of Chil<l NeurokiJ,?'y Scnes). New Y,irk: Raven Press, l986:l l9-128.
12. Davidson O P, Dent J, WillmgJ. M1,111wrmg of upper esophageal ,phmcrer pressure m children. Gut 1991;32:607 11.
47
SHEEHAN ec al
13. Cook lJ, Dent J, Shannon S, Collins SM. Measurement of upper esophageal sphincter pressure: Effect of acute emotional stress. Gastroenterology 1987:93:526-32.
14. Kahrilas PJ, Dodds WJ, Dent), Haeberle B, Hogan WJ, Arndorfer RC.
48
Effect of sleep, spontaneous gasrroesophageal reflux and a meal on upper esophageal sphincter pressure in normal human volunteers. Gastrocnterology 1987;92:466-71.
15. Milla PJ. Reflux vomiting. Arch Dis Child. 1990;65:996-9.
16. Werlin SL, Dodds WS, Hogan WJ, er al. Mechanics of gastrocsophageal reflux in children. J Pe<liatr I 980;97:244-9.
17. Euler AR, Byrne WJ. Twency-four hour esophageal intraluminal pH probe testing: A comparative analysis. Gastroenterology 1981;80:957-61.
CAN J GASTROENTEROL VOL 8 Nol JANUARY/FEBRUARY 1994
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