Colin ferrie and richard grunewald, in their march 17 commentary,1 highlight a common and benign childhood epileptic syndrome and emphasise the predominant autonomic manifestations, which have immense clinical implications. A clinical guide to epileptic syndromes and their treatment. Dr panayiotopoulos has written this monograph cum swan song about the syndrome that he has put on the diagnostic map and to which his name has been attached. Panayiotopoulos makes this a reference book for specialists. A 4yearold girl with panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. Thirteen ps patients with mean age 5 3 to 14 years were studied. Panayiotopoulos syndrome american academy of pediatrics. To assess the contribution of the eeg technologists in the diagno sis of children.
Misdiagnosis and pitfalls in panayiotopoulos syndrome. Here are links to possibly useful sources of information about panayiotopoulos syndrome. Panayiotopoulos syndrome with convulsive status epilepticus. Seizures are infrequent in most patients, with 25% having a single seizure which may be autonomic status epilepticus and 50% having six seizures or less. Although ps is considered to be a benign childhood epilepsy syndrome usually not requiring treatment with antiepileptic drugs, our case and the small number of. Panayiotopoulos syndrome list of high impact articles. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. While there are several case reports and case series describing the mri findings in patients with panayiotopoulos syndrome, there is no literature on the diffusion tensor imaging dti in these patients. This book aims to describe the clinical and eeg manifestations of benign childhood partial seizures with particular emphasis on the occipital seizures which are often more characteristic. During a seizure, children with panayiotopoulos syndrome often turn pale, complain of feeling sick and vomit.
Panayiotopoulos syndrome, a common and benign childhood. Panayiotopoulos syndrome an overview sciencedirect topics. For more information about panayiotopoulos syndrome visit the epilepsy foundation usa. Panayiotopoulos syndrome is a form of idiopathic benign childhood focal epilepsy in which the seizures are associated with signs of increased autonomic activity. This is a video of what our sons seizures look like. The important advance in epileptology is the recognition of epileptic syndromes and diseases, most of which are well defined and easy to diagnose. Further, in agreement with ferrie and grunewald, children with panayiotopoulos syndrome had an excellent prognosis although some of them later developed rolandic seizures. Jul 22, 2010 a novel inherited mutation in the voltage sensor region of scn1a is associated with panayiotopoulos syndrome in siblings and generalized epilepsy with febrile seizures plus. Panayiotopoulos syndrome is a common idiopathic childhoodspecific seizure disorder formally recognized by the international league against epilepsy. Panayiotopoulos syndrome and diffuse paroxysms as the first.
Initially described in 1989, this syndrome affects otherwise healthy children between the ages of 1 and 14 years with 76% of cases starting at 36 years of age. Panayiotopoulos syndrome with convulsive status epilepticus at the. This childhood syndrome certainly breaks many epilepsy rules. Scribd is the worlds largest social reading and publishing site. Physicians should be aware of the different ways in which this syndrome can present to ensure its early diagnosis and treatment. An expert consensus has defined panayiotopoulos syndrome as a benign agerelated focal seizure disorder occurring in early and midchildhood. Autonomic seizures in panayiotopoulos syndrome consist of. Recent reports document hypertension, tachycardia, and release of vasopressin during the seizures, suggestive of activation of the central autonomic network.
My son is now 7 years old and has not had an episode in over a year. Panayiotopoulos is a common idiopathic childhoodrelated seizure disorder that occurs exclusively in otherwise normal children idiopathic epilepsy and manifests mainly with autonomic epileptic seizures and autonomic status epilepticus. May 25, 2002 over the past two decades various distinct paediatric epilepsy syndromes, such as rolandic epilepsy, have been formally recognised. Panayiotopoulos syndrome ps is a frequent and benign epileptic syndrome. Panayiotopoulos syndrome or benign epilepsy with occipital paroxysms is an uncommon benign epilepsy syndrome described in children. Panayiotopoulos syndrome presenting with respiratory arrest. The clinical features of her seizures included, in order of occurrence, blank staring. Prophylactic treatment with antiepileptic medication may not be needed for most patients.
Panayiotopoulos syndrome ps is described as a benign autonomic epilepsy that affects up to % of children aged 36 years 6% aged 115 years with a history of nonfebrile seizures. The seizures usually start with autonomic symptoms nausea, retching, or vomiting and evolve to. A frequent seizureelectroencephalogram eeg pattern resembles that of benign rolandic epilepsy. Among 150 patients who met the electroclinical criteria of panayiotopoulos syndrome, we identified nine children who presented with the typical clinical manifestations but who, on eeg, only had diffuse paroxysms at onset that continued along the course of the syndrome. Panayiotopoulos syndrome ps is a relatively frequent and benign epileptic syndrome seen in children in the age group of 36 years and is characterised by predominantly autonomic symptoms andor. Understanding panayiotopoulos syndrome 4 about the authors colin ferrie colin ferrie is a consultant paediatric neurologist in leeds. Benign childhood partial seizures and related epileptic. Panayiotopoulos syndrome current problems in epilepsy. Dec 01, 2006 panayiotopoulos syndrome ps is described as a benign autonomic epilepsy that affects up to % of children aged 36 years 6% aged 115 years with a history of nonfebrile seizures. Panayiotopoulos syndrome is characterized by onset of seizures between 1 and 14 years of age majority between 3 and 6 years. Panayiotopoulos syndrome, generalized clonic seizures. Building on the sellout success of the first edition this thorough revision reflects the lat. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care.
A study of 43 patients with panayiotopoulos syndrome, a common and benign childhood seizure susceptibility christina lada, konstantinos skiadas, virginia theodorou, nomiki. Panayiotopoulos syndrome is a relatively frequent and benign autonomic childhood epileptic disorder with significant clinical, pathophysiological and management implications. Panayiotopoulos syndrome in a child masquerading as septic shock. Parent of 2 yr old with panayiotopoulos syndrome epilepsy. He details the clinical and eeg manifestations, prevalence, pathophysiology and management of panayiotopoulos syndrome as it emerged from his longterm prospective studies of the author and worldwide documentation through independent studies. Panayiotopoulos syndrome ps is an early onset benign. Panayiotopoulos syndrome ps is a relatively common earlyonset benign childhood epilepsy, with an estimated prevalence of around % of all epilepsies among 36 yearsold children, with one or more nonfebrileseizures, and 6% in the age group between 1 and 15 years.
Epilepsy is reported to occur in 1020% of individuals with fragile. Panayiotopoulos syndrome ps is an earlyonset benign. An expert consensus statement concluded that such treatment was probably best reserved for children whose seizures were unusually frequent, distressing. He said the seizures were not frontal focused but occipital focused, instead. Case report a case of panayiotopoulos syndrome showing an atypical course makiko saitoha, masaya kubotab, ikumi kimurac, masashi mizuguchia, takashi igarashia adepartment of pediatrics, faculty of medicine, university of tokyo, hongo 731, bunkyoku. Of 1,794 children aged between 1 and 14 years referred for the first afebrile focal seizure, between january 1992 and december 2004, 93 5. The contribution of the eeg technologists in the diagnosis of.
Pronunciation of panayiotopoulos syndrome with 1 audio pronunciation, 1 meaning, 8 translations and more for panayiotopoulos syndrome. We report the results of a magnetoencephalographic study of patients with ps. Find treatment information at the epilepsy foundation online. The premier text in the field with over one thousand images, the atlass highly illustrative approach. To explore the clinical, electroencephalography eeg, neuropsychological features, and prognosis of panayiotopoulos syndrome ps. It affects otherwise normal children with the onset at 36. Abstract the sequential changes in localization of eeg foci with age, and the relation between the clinical manifestations and eeg pattern in panayiotopoulos syndrome ps were analyzed in a study of 76 children 37 boys and 39 girls followed for2 years at tokyo womans medical university, japan. In becop the children are usually older, between 6 and years when the seizures start. At our appointment, the neuro did a fairly comprehensive history of our son and us. In general, overall duration of aeds treatment should last 23 years 3, 35. Atlas of epilepsies is a landmark, allencompassing, illustrated reference work and handson guide to the diagnosis, management and treatment of epilepsy in all its forms and across all age groups. Panayiotopoulos syndrome is a common syndrome affecting about % of children in the 3 to 6year age group who present with one or more afebrile seizures.
A case of panayiotopoulos syndrome showing an atypical course. This point signifies the links of rolandic epilepsy and panayiotopoulos syndrome as a benign childhood seizure susceptibility syndrome. Pdf panayiotopoulos syndrome susceptibility to early onset benign childhood seizures with occipital and. Panayiotopoulos syndrome share this page it is also known as early onset occipital epilepsy, is a common childhood epilepsy syndrome with partial also called focal seizures. Panayiotopoulos syndrome is different to the epilepsy syndrome called lateonset selflimiting occipital epilepsy, which also used to be called benign epilepsy of childhood with occipital paroxysms becop.
Although ps usually accompanies occipital spike discharges, many cases show shift of the location, multiplication and propagation with aging. A study of 43 patients with panayiotopoulos syndrome, a. The seizures usually start with autonomic symptoms nausea, retching, or vomiting and evolve to altered awareness usually only after several minutes. His service provides comprehensive services for children with epilepsy in leeds and the yorkshire region. We describe the clinical features, eeg findings and evolution in three patients affected by fragile. In three, in addition to the diffuse paroxysms, focal spikes appeared later. After this, he told us he was fairly confident that our son had panayiotopoulos syndrome. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms. The prognosis for children with this syndrome is good. The international league against epilepsy ilae diagnostic manuals goal is to assist clinicians who look after people with epilepsy to diagnose the epilepsy syndrome and if possible the etiology of the epilepsy.
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