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Hair-Grooming Syncope Seizures DONALD W. LEWIS and L.

MATTHEW FRANK Pediatrics 1993;91;836

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright 1993 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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(rubeola) vaccine in patients hypersensitive to egg protein.Pediatr. J Despite these findings, some investigators have 1983;102:196-199 endorsed simplified skin test methods, ie, either skin7. Johnstone R,Trotter AC, Georgitis JW, Stone BD, Laszio DJ. Measles, prick test or intradermal testing aloneY Others mumps administration in egg sensitive children: systemic reactions durhave stated that skin testing unnecessary.5123 is The big desensitization. Concurrent Sessions American College of Allergy Immunology, 48th Annual Meeting, 1991 Abstract most recent3 selected 140hildren c with various de8. B, Koren G, Gold R. Administration of measles, grees of egg sensitivity from a clinic population in Lavi 5, Zimmerman mumps, and rubella virus vaccine (live) to egg-allergic children. JAMA. which atopic dermatitis and food hypersensitivity 1990;263-271 were being studied. Of these, 69 had tolerated MMR. 9. Greenberg MA, Birx DL. Safe administration of measles-mumps-rubella vaccine in egg allergic children. Pediatr. I 1988;113:504-506 Only 8 had experienced severe life-threatening reacLW, Shirrell MA, Burks AW. Egg hypersensitivity and tions comparable with those of 4 of our patients, and 10. Beck SA, Williams measles-mumps-rubella vaccine administration. Pediatrics. 1991;88:913urticaria developed in I of these after MMR. The 917 stated confidence limits for 97.5% safety of direct 11. Miller JR. Orgel HA, Meltzer ED. The safety egg containing of vaccines. administration ofMMR must be interpreted in terms I Allergy Clin Immunol. 1983;71 :568-573 12. Kemp A, van Asperen P. Mukhi A. Measles immunization in children of the overall study population and do not apply to with clinical reactions togg protein. e AJDC. 1990;144:33-35 a subgroup with severe life-threatening reactions.



Fasano ity and


MB, Wood RA, ooke C adverse reactions


5K, SB, Sampson HA. Egg hypersensitivto measles, mumps and rubella


Egg allergy is very common during the early years of life. There is a general consensus that MMR vaccine can be given to children with milder forms of egg allergy. Studies have shown IgE antibodies to Syncope Seizures ovalbumin and measbes/MMR vaccine in patients Hair-Grooming who reacted to MMR vaccine.6 IgE-mediated reactions to vaccine even in patients with severe egg senPediatricians are commonly consulted to evaluate sitivity are rare but may, nevertheless, be life-threatchildren following their first, apparently unproening. If such reactions occur in I 100 chances in or yoked, convulsion. Keenly aware of the old adage less there is a high probability of missing them when that all that shakes isnt epilepsy, we are ever the study population is less than 1005,12,13 Therefore, mindful of the common mimickers of epileptic seisuch studies may not have included the rare, but zures that include breath-holding spells, benign panextremely important patient who is exquisitely sen-oxysmal vertigo, hyperventilation syndromes, narcositive to both egg and vaccine. lepsy-cataplexy, night terrors, and syncopal events. Based on our experience and the experience of We present the clinical features 15 of children neothers, we think it is not only prudent to follow the ferred for evaluation of witnessed convulsions at recommendations of the AAP (Red Book) for testing home with similar histories of seizures precipitated and desensitization, but also to consider initial skin- by hair grooming and accompanied by presyncopal prick test and intradermal test with more dilute masymptoms. terial in individuals with particularly severe egg senMETHODS sitivity. Desensitization should be performed by an The records of children referred to our neurology clinics from expert in a setting where resuscitative equipment is 1982 to 1992 for evaluation of seizures in temporal relationship to immediately available. We have not attempted desensitization in patients with a generalized reaction both from the to skin testing. The relative risk of contracting Details regarding rubeola and mumps must be weighed against the acts were mg Physical and risk of desensitization in such patients.








were retrospectively reviewed. Clinical histories, witnessing parent and the patient, were recorded. the position, duration, and type of hair-groomnoted. Characteristics of the seizures were detailed. neurologic examinations were recorded with specific attention to vital signs and postural blood pressure measurements. Complete blood cell counts (to exclude anemia); serum electrolyte levels with glucose, calcium, and magnesium; electrocardiograms; and electroencephalograms were obtained. Clinical follow-up on the patients ranged between 1 and 10 years (median 5 years).



Dept of Allergy The Long Island Brooklyn, NY REFERENCES

1. American

and Immunology College Hospital Fifteen ages of temporally consisted braiding

RESULTS children (14 girls and I boy) between the 5 and 13 years (mean 11 years) had seizures related to hair grooming. Hair grooming of combing in 4 (26%), brushing in 6(40%), in 2 (13%), and blow-drying in 3 (20%). The

(Red Insert:

Book). Elk

2. Package

Publications; West ER.

of Pediatrics. Report Grove Village, 1991:22-30

II (Measles,






IL: American
mumps, and

rubella virus

of Pedi-


vaccine, Received Presented

live). 3. Stiehm

Point, PA: Merck Skin testing prior


Sharp and to measles

Dohme; 1991 vaccination






US Dept of Health and Human Services. easles, M Mumps, and Rubella: What You Need to Know. Atlanta, GA: Public Health Service, Centers for Disease Control; 1992:6 5. Kamin PB, Fein BT, Britton HA. Usef live, attenuated o measles virus vaccine in children allergic to egg protein. JAMA. 1965;193:143-144 6. Herman JJ, Radin R, Schneiderman R. Allergic reactions to measles

for publication Nov 30, 1992; accepted Jan 12, 1993. at the 21st Annual Meeting of the Child Neurology Society, October 1992. The opinions or assertions contained herein are those of the authors are and not to be construed as official or reflecting the views of the Department of the Navy or Department of Defense. Reprint requests to (D.W.L.) Dept of Pediatrics, US Naval Hospital, Portsmouth, VA 23708-5100. PEDIATRICS (ISSN 0031 4005). Copyright 1993 the by American Academy of Pediatrics.



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hyperventilation, orthostasis, and Vabsabva majority of the patients (12) were standing, 2 werebowing had seizures. Using video analysis, the seated, and 1 was kneeling. Mothers were usually maneuver seizures were characterized as arrhythmic, asynchnostanding behind the patients (13), with the exception myocbonic convulsions and up to two thirds of two cases in which the mothers were in front of nous, a had complex automatisms. The investigators proseated on kneeling child. The childs head was in the posed that transient global hypoxia leads to cortical neutral position in 12 (80%), flexed in I (7%), and suppression with disinhibition of bimbic and subcorextended in 2 (13%). Each parent reported a witnessed event during ticab structures.7 In this series, 15 children, predominately girls, which the child collapsed and had a seizure. Unless were found to have hair-grooming syncope seizures. specifically asked, none of the mothers initially reThe events were strikingly similar. As the childs hair ported the preceding activity of hair grooming. Hisbeing dressed by the mother, the child expenitory from the patient included pnodnomal nausea (3),was presyncopab symptoms followed by loss of light-headedness (12), diaphonesis (8), dimmed vi- enced consciousness. The startled parents later reported sion (15), and boss of consciousness (15). None of the only that their children collapsed into a convulsion; children reported pain before losing consciousness. the parents consistently failed to report the preceding Witnessed convulsions were described as tonic (8), grooming unless specifically asked. clonic (2), tonic-clonic (2), and atonic (3). Reported hair presumed primary mechanism is vasovagab duration of the convulsions ranged between 5 sec- The syncope followed by a hypoxic-ischemic convulsion. onds and 3 minutes (average, 20 seconds). Longer Why hair grooming triggers this phenomenon is seizures occurred in patients who fell into the panopen to speculation. This may be a variation of paents arms and were supported upright rather than in nade ground syncope. Pain from hair pulling on a supine position. scalp stimulation of the tnigeminab nerve on cervical Within I week of the event, all children had normal roots may precipitate the syncopal events. Other exphysical and neunobogic examinations including vital planations include neck extension on flexion causing signs with specific attention toward onthostatic compression of carotid banoneceptons or causing imchanges in blood pressure and heart rate. Each had a pedance of ventebnobasilan blood flow; however, the normal electrocardiogram without evidence of premajority of our patients held their head in the neutral excitation or prolonged QTc intervals and a normal position. electroencephalogram, which included hyperventilaHair-grooming syncope seizures can be easily contion and photic stimulation. fused with epilepsy. Specific inquiry both into the Two patients had subsequent single events of activity preceding a seizure and into the childs subonthostatic syncope, unassociated with hair groomjective experience during the event are important in ing, but were otherwise indistinguishable from the reaching a correct diagnosis. When the characteristic remaining 13. history is elicited, evaluation may be limited to history, physical examination, hematocnit, and an ebectnocandiogram. Since the events are syncopab in naSyncope may be defined as boss of consciousness tune, further diagnostic evaluation with cranial due to transient, reversible impairment of cerebral imaging and electroencephalography is not warrantl Generally classified into vasodepnessor/ ed. vasovagal and candiogenic categories, the ultimate ACKNOWLEDGMENT basis of syncope is cerebral hypoxia-ischemia.2 AlWe thank Sara Crispen for help with preparation of this manuthough thought to occur uncommonly, convulsions and other seizure-bike activities may follow primary script. syncopal events. Stephenson3 refers to these as anDONALD W. LEWIS, MD oxic seizures, although alternate terms in the litenaL. MATTHEW FRANK, MD tune include convulsive syncope, ictal syncope, and Depts of Pediatrics and Neurology syncope-seizunes. Naval Hospital Portsmouth, VA Several series have reported an association between syncope and seizures. Gastaut and Gastaut4 Childrens Hospital of the Kings Daughters produced vagalby mediated bradycandia with synEastern Virginia Medical School cope by using ocular compression in 218 patients and Norfolk, VA observed convulsions in 46 (21%). Duvoisen5 proREFERENCES duced syncope in 61 of 200 healthy men by using the Weben Vabsabva maneuver and reported tonic sei- 1 Fitzpatrick A. How and when to investigate syncope. I Hosp Br Med. zures in 40 (65%). Gnubb et al,6 using head-up tilt 1991;46:386-390 and Epilepsy. Philadelphia, PA: FA Davis Company; table testing with and without isopnoterenol infu- 2. Engel J Jr.Seizures 1989:340-379 sion, produced syncope with seizure in 10 (67%) of 15 Stephenson 3. JBP. Fits and Faints. New York, NY: Cambridge University selected patients. The seizures were rigid flexion fob- Press; 1990 H, Gastaut Y. EEC and clinical study of anoxic convulsion in bowed by extensor spasms. Four patients had vocal- 4. Gastaut their location within the group of infantile convulsions and izations, 4 had urinary incontinence, and 5 had children: differentiation from epilepsy. Electroencephagr Clin Neurophysiol. 1958; rhythmic myocbonic jerking.6 10:607-620 Recently, Lempent et al7 reported that 42 (71 %) of5. Duvoisen RC. Convulsive syncope induced by Weber maneuver. Arch 59 healthy volunteers who were induced to faint fob- Neurol. 1962;7:219-226 DISCUSSION

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Grubb syncope

BP, and

Geraed epilepsy


Rousch with head-up



al. tilt

Differentiation of testing. Ann Intern

convulsive Med. 1991;

7. Lempert of induced

the anal sphincter.4 did not disappear identified as deep

skin.4 Wedge-shaped

Skin tags were defined with stretching of nearby linear clefts, and ulceration
smooth areas were

as excess skin tissues.4 Fissures as superficial


which were loss of

to the

R, Bauer

M, Schmidt

D, Berlin

C. The




perianal skin folds. Location with the infant in the supine located superiorly near the position, inferiorly.

was noted using position, with the posterior fourchette

the face of a clock 12 oclock position and the 6 oclock

Perianal Months

female infants ranging in age from 1 to were photographed. The mean age of was 10.7 months. The racial distribution population was 44% (39/89) black, 29% Due to the recent increase in the number of cases white, non-Hispanic, 26% (23/89) Hispanic, suspected sexual assault, a need has arisen for better (26/89) knowledge and understanding of the genital anatand 1% (1/89) Asian. or decrease of the skins pigmentation omy in the prepubertal girl. In spite of the fact that An increase near the anus was noted in nine infants (Table). Six abuse involving the rectum has been reported in up and one white subject had increased pigmento 40% of young girls assaulted, little normative black A decrease in pigmentation was observed in data exists on the appearance of the anonectal region. tation. one black infant, and a second black infant had both In the single study of penianal findings in nonabused of increased and decreased pigmentation. Five girls, McCann et a!2 documented that findings pre-areas had a rash. A confluent area of redness was viously reported to be suggestive of sexual abuse, infants observed near the anus in an additional six subjects. including erythema, smooth areas, skin tags, and areas adjacent to the perianal folds were anal dilation, commonly occurred in 161 female sub- Smooth in 26% (23/89) of subjects ranging in age from jects up to 11 years old. However, this study lacked found old (Fig 1). This characteristic, which adequate numbers of infants, with only 12 subjects 3 to 15 months was observed in the midline only, occurred at the 6 younger than 2 years of age. Comparison data on in 83% (19/23) of the infants and at anorectal anatomy of very young patients are espe- oclock position 12 oclock position in 26% (6/23) of those who cially critical, as anal abuse is more common thanthe had this finding. Two of the 23 girls had a smooth vaginal penetration in girls aged 0 through5#{149}3 both inferionly and superiorly. A smooth area The purpose of this study was to document in area a was observed more often in white non-Hispanic intriethnic population the anatomy of the perianal refants (48%) than in blacks (30%) on Hispanics (22%). gion in infants 18 months of age without a history of infants were observed to have an anal skin sexual assault. This information should help clini- Three A 15-month-old white and a 1-month-old Hiscians who evaluate infants for possible sexual assault tag. child had this finding superiorly at the 12 differentiate normal anatomical findings from post- panic traumatic changes. oclock position, (Fig 2). Photographs of a 13-monthold white child, who had been in the supine position MATERIALS AND METHODS for approximately 2 minutes, showed both a skin tag Infants were recruited for this study at a single center between at the 6 oclock position and venous pooling inferiJanuary 7, 1990, and January 31, 1991. Recruitment of infants 18 only (Fig 3). A fissure was observed at the 12 oclock months of age took place at the time of a health supervision visit position in one 14-month-old girl. Warts, areas of (n = 29) or through a letter sent to children born at this institution or anal gaping were not observed in a (n 60) between August 1989 and February 1990. The parents of ulceration, infant. all girls were asked whether they suspected that their daughter single

Findings of Age

in Infants or Younger

Eighty-nine 17.6 months the subjects of of the study



have been of possible

sexually sexual

abused assault

in were

the past. excluded

Children from

with participation.

a his-


With Institutional ten parental consent,

by one of two

Perianal findings reported to be associated with abuse include anal gaping,7 rectal fissures,57 tion table in the supine position with legs extended. Gentle lateral wedge-shaped smooth or thickened areas,6 penianal traction was applied to the buttocks to expose the anus. A handerythema,57 penianal swelling,5 venous engorgeheld Nikon F3 camera was used to take approximately two phoment,57 skin tags,47 perineal scarring,47 rectal and tographs of the perianal area of each infant. A 105-mm microsphincter tears, and human papilloma virus lesions.4 Nikkor lens with attached extension ring and ring flash provided 1:1.2 magnification. Limited information, however, is known on the freexaminers. Infants were positioned on an examina-

Review Board approval each child was examined

and informed, writand photographed

Findings reported corded characteristics


reflect those observed included the number

photographically. and location skin of

ReFindings in the Sample


fissures, smooth or ulcerated areas, and human papifioma TABLE. Perianal lesions (warts) and the presence of anal gaping. Variations in pigmentation, classified as increased or decreased, were noted as were rashes or areas of redness. A rash was defined as discrete Smooth area maculopapular lesions, and redness as a confluent area of redness Ti.1 pigmentation near the anal orifice. Anal gaping was defined as 1 cm dilation of Redness Skintag Received Reprint
atrics, PEDIATRICS for University publication of Texas 4005). Jul 13, 1992; of Medical accepted Obstetrics Branch, Oct and Galveston, 13,1992. Gynecology TX and 77555-0587. Pedi-

(n 89)

n 23

% 26





1 0

I 0


to (A.B.B.) Depts
(ISSN 0031








of Pediatrics.

Fissure Ulceration Anal gaping

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Hair-Grooming Syncope Seizures DONALD W. LEWIS and L. MATTHEW FRANK Pediatrics 1993;91;836
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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright 1993 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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