Editorials
becoming incontinent of urine, how would we be 2 Farrell SA, Allen VM, Baskett TF. Parturition and urinary incontinence in
primiparas. J Obstet Gynecol 2001:97:350-6.
judged? 3 Chaliha C, Sultan AH, Bland JM, Monga AK, Stanton SL. Anal function:
effect of pregnancy and delivery. Am J Obstet Gynecol 2001;185:427-32.
Linda Brubaker professor and fellowship director 4 Donnelly V, Fynes M, Campbell D, Johnson H, O’Connell PR, O’Herlihy
C. Obstetric events leading to anal sphincter damage. Obstet Gynecol
([email protected])
1998;92:955-61.
Department of Obstetrics and Gynecology and Urology, Female Pelvic 5 MacLennan AH, Taylor AW, Wilson DH and Wilson D. The prevalence of
Medicine and Reconstructive Surgery, 2160 S, First Avenue, Maywood, pelvic floor disorders and their relationship to gender, age, parity and
Illinois 60153, USA mode of delivery. Br J Obstet Gynecol 2000;107:1460-70.
6 Al-Mufti R, McCatrhy A, Fist NM. Survey of obstetrician’s personal pref-
erence and discretionary practice. Eur J Obstet Gynecol Reprod Biol
1997;73:1-4.
1 Chiarelli P, Cockburn J. Promoting urinary continence in women after 7 Viktrup L. The risk of lower urinary tract symptoms five years after the
delivery: randomised controlled trial BMJ 2002;324:1241-4. first delivery. Neurourol Urodyn 2002;21:2-29.
Panayiotopoulos syndrome
A common benign but underdiagnosed and unexplored early childhood seizure
syndrome
E
pilepsy affects 1% of the general population and visual seizures, and has less predictable outcome. Elec-
4% of children, encompassing heterogeneous troencephalography shows occipital spikes.2
seizure syndromes.1 These are defined by Autonomic seizures are the hallmark of the Panayi-
distinct aetiology, age at onset, seizure type, and otopoulos syndrome.4–9 Autonomic symptoms and
electroencephalographic features, which taken signs (mainly vomiting) occur from the onset in 80% of
together provide the key to diagnosis, prognosis, and seizures, with half of them lasting for more than 30
optimal management. Over the past two decades vari- minutes to hours, thus amounting to autonomic status
ous distinct paediatric epilepsy syndromes, such as epilepticus. Two thirds of the seizures occur during
rolandic epilepsy, have been formally recognised.2 nocturnal sleep or brief daytime naps. In a typical day-
Panayiotopoulos syndrome is a new idiopathic time seizure the child looks pale, complains, “I want to
childhood epilepsy, recently recognised by the be sick,” and vomits. If in sleep, the child wakes up with
International League Against Epilepsy.2 3 It is common, similar complaints or is found vomiting, confused, or
benign, and may mimic other common illnesses. unresponsive. Vomiting occurs in three quarters of sei-
Awareness of this syndrome is important for all zures. Other autonomic manifestations may occur
professionals who care for children with epileptic either concurrently with vomiting or later in the course
seizures, including general practitioners and commu- of the seizure, and include pallor, mydriasis, cardiores-
nity nurses, paediatricians and paediatric neurologists piratory, gastrointestinal and thermoregulatory altera-
and clinical neurophysiologists, for the following tions, incontinence, and hypersalivation. In at least a
reasons. Firstly, it is common. It probably affects about fifth of the seizures the child becomes unresponsive,
13% of children of 3-6 years old with one or more non- pale, and flaccid (ictal syncope)4 either before convuls-
febrile seizures (peak age 4-5 years), and 6% of the age ing or in isolation.
group 1-15.4 5 Secondly, seizures can be prolonged, may Behavioural disturbances, headache, or various
mimic non-epileptic disorders, and may vary in severity non-painful cephalic sensations are common particu-
from trivial to apparently life threatening—implying larly at onset. More conventional manifestations of sei-
that the diagnosis may need to be considered in a vari- zures often ensue: the child becomes confused or
ety of clinical settings and by medical professionals of unresponsive, eyes may deviate to one side (in 60%) or
different specialties. Thirdly, it is benign—its recognition the patient may stare. Half of the seizures end with
therefore can provide firm reassurance to families in hemi or generalised convulsions. Other, less frequent
situations that can be very alarming. Finally, clinical ictal features include speech arrest, hemifacial spasms,
research, necessary in any new syndrome, would visual hallucinations and oropharyngolaryngeal move-
require a multidisciplinary approach. ments, suggesting a maturation related continuum
Panayiotopoulos syndrome can be best defined as with Rolandic epilepsy.10
idiopathic susceptibility to early onset benign childhood Diagnosis of Panayiotopoulos syndrome may be
seizures with electroencephalograhic occipital or extra easily missed—mild and brief ictal autonomic symp-
occipital spikes, and manifests mainly with autonomic toms in the presence of clear consciousness would sug-
seizures.4 Large independent studies have accumulated gest trivial non-epileptic conditions such as atypical
impressively concordant information on the clinical and migraine, gastroenteritis or syncope, while prolonged
electroencephalographic features of this syndrome.4–9 and severe attacks may simulate life threatening insults
Rolandic epilepsy, another common syndrome, such as encephalitis, for which many of these children
affects 15% of children with seizures and has as good a are treated.3 4 9
prognosis as febrile convulsions. Seizures usually start Characteristically, even after the most severe
between 7-9 years of age, occur mainly during sleep seizures and status, the child is normal after a few
and consist of hemifacial convulsions, speech arrest, hours of sleep—this is both reassuring and diagnostic.
oropharyngolaryngeal movements, and hypersaliva- Electroencephalography, which should be done after a
tion. EEG shows centrogyral spikes. Gastaut’s occipital first non-febrile seizure, is confirmatory. This usually
epilepsy is much less frequent, manifests with mainly shows multifocal spikes at various locations.3–5 7 11 BMJ 2002;324:1228–9
1228 BMJ VOLUME 324 25 MAY 2002 bmj.com
Editorials
Though occipital spikes predominate they are neither again. It is currently hypothesised that in Panayiotopou-
a prerequisite nor specific.12 Normal recordings may los syndrome an inherent autonomic instability
occur in 25% of children11 and should prompt an EEG responds by generating autonomic seizures and status
during sleep to activate the spikes; on strong clinical when cortical hyperexcitability triggers susceptible brain
suspicion a sleep EEG should be done. A useful rule of circuits.4 As the current epidemiological data seem to
thumb is that Panayiotopoulos syndrome should be indicate, this hyperexcitable loop is mainly related to the
considered if a normal child with a single or a few sei- early childhood and is short lived. Careful prospective
zures has an EEG with multifocal spikes.3 4 10 11 and controlled studies of autonomic function will clarify
Panayiotopoulos syndrome is remarkably benign. the roles of the cortex and the brainstem in the genera-
Remission usually occurs within two years from tion and expression of seizures.
onset.4 6–9 A third of these children have a single
Michael Koutroumanidis senior lecturer in neurology
seizure, and only 5-10% have more than 10 seizures
Guy’s, King’s and St Thomas’s School of Medicine, Denmark Hill
that may be very frequent sometimes but the outcome
Campus, London SE5 9RS
is still favourable.4 Lengthy seizures do not appear to ([email protected])
result in residual deficits or have adverse prognostic
significance. One fifth of children with this syndrome
may develop other types of infrequent, usually rolandic 1 Berg AT, Panayiotopoulos CP. Diversity in epilepsy and a newly
seizures, but these also remit before the age of 16 years. recognized benign childhood syndrome. Neurology 2000;55:1073-4.
2 Engel J Jr. A proposed diagnostic scheme for people with epileptic
It follows that treatment with antiepileptic drugs seizures and with epilepsy: report of the ILAE task force on classification
(mainly carbamazepine) is usually unnecessary but may and terminology. Epilepsia 2001;42:796-803.
3 Ferrie CD, Grunewald RA. Panayiotopoulos syndrome: a common and
be considered in children with multiple seizures. The benign childhood epilepsy. Lancet 2001;357:821-3.
decision should also take into account a likely traumatis- 4 Panayiotopoulos CP. Panayiotopoulos syndrome: a common and benign child-
hood epileptic syndrome. London: John Libbey, 2002.
ing parental experience as in febrile convulsions.3 4 8 5 Panayiotopoulos CP. Vomiting as an ictal manifestation of epileptic
Appropriate advice by the family doctor is expected to seizures and syndromes. J Neurol Neurosurg Psychiat 1988;51:1448-51.
shape parental attitude and prevent chronic anxiety. 6 Ferrie CD, Beaumanoir A, Guerrini R, Kivity S, Vigevano F, Takaishi, et al.
Early-onset benign occipital seizure susceptibility syndrome. Epilepsia
There is a lot more to learn about this syndrome. 1997;38:285-93.
Prospective epidemiological and clinical studies 7 Oguni H, Hayashi K, Imai K, Hirano Y, Mutoh A, Osawa M. Study on the
early-onset variant of benign childhood epilepsy with occipital
should assess the actual prevalence, delineate the clini- paroxysms otherwise described as early-onset benign occipital seizure
cal spectrum, and define possible clinical and susceptibility syndrome. Epilepsia 1999;40:1020-30.
8 Caraballo R, Cersosimo R, Medina C, Fejerman N. Panayiotopoulos-type
electroencephalography markers of atypical clinical benign childhood occipital epilepsy: a prospective study. Neurology
presentations and complicated evolution that is 2000;55:1096-100.
9 Kivity S, Ephraim T, Weitz R, Tamir A. Childhood epilepsy with occipital
occasionally seen.4 Possible genetic links with the paroxysms: Clinical variants in 134 patients. Epilepsia 2000;41:1522-3.
rolandic phenotype may provide further information 10 Panayiotopoulos CP. Benign childhood partial epilepsies: benign
childhood seizure susceptibility syndromes. J Neurol Neurosurg Psychiat
about the age related continuum of “benign childhood 1993;56:2-5.
seizure susceptibility syndrome.”10 11 Koutroumanidis M, Ferrie CD, Sanders S, Rowlinson S. Panayiotopoulos
syndrome of early onset benign childhood seizures: EEG variability and
Finally, in the light of this syndrome, the concept of management issues. Clin Neurophysiol 2001;112:1269.
ictal syncope in childhood clearly needs to be evaluated 12 Martinovic Z. Panayiotopoulos syndrome. Lancet 2001;358:69.
Regulating cosmetic surgery
Members of the public would be better protected if they consulted their general
practitioners first
C
osmetic surgery has become a growth industry fundamentally medical. Self referral to a clinic is an
and a public obsession. The demand for the top easier option.
three procedures in the United States grew by Standards in cosmetic clinics vary, but the clinics
26% between 1999 and 2000, and this growth is often send a representative to the home of the patient
mirrored in the United Kingdom.1 The public percep- in response to a reply to an advertisement. These rep-
tion of cosmetic surgery is that it is quick and easy. In fact resentatives are not medically qualified but recom-
most cosmetic surgery operations are extremely mend operations and book dates for surgery, often
complex and require a high degree of anatomical offering discounts if the patient signs immediately.
knowledge and surgical skill as well as aesthetic Starved of food before having general anaesthesia and
appreciation. having paid the fee, the patient briefly meets the
The public’s increasing interest is accompanied by surgeon before the operation.
a reduction in the provision of cosmetic surgery in the New government regulations insist on preoperative
NHS, so that patients look to the private sector, financ- consultations by the surgeon and ban surgery within
ing their treatment through bank loans and finance two weeks of consultation.4 The regulations also insist
agreements.2 3 These patients have been prey to that clinics are inspected regularly and that written
organisations that offer discounts, privacy, and no wait- information is realistic.
ing time but are not staffed by accredited surgeons. The training of junior surgeons in cosmetic surgery
Many patients do not seek a referral from their is proving an extremely contentious issue since less
general practitioner because they fear an unsympa- training is now provided in the NHS than ever before
BMJ 2002;324:1229–30 thetic response or they feel that cosmetic surgery is not and in any case many cosmetic procedures were never
BMJ VOLUME 324 25 MAY 2002 bmj.com 1229