Panayiotopoulos syndrome

Abstract

Colin Ferrie and Richard Grünewald, in their March 17 commentary,1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar highlight a common and benign childhood epileptic syndrome and emphasise the predominant autonomic manifestations, which have immense clinical implications.These autonomic seizures and autonomic status epilepticus are peculiar to childhood.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar They do not occur in adults; ictus emeticus (not in the same sequence as in children) is exceptional (18 cases only) in large neurosurgical series.3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar In children, autonomic seizures and autonomic status epilepticus, are common, occurring in 6–7% of childhood epilepsy.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar They largely affect otherwise healthy children, but 10–20% might be the result of documented brain pathology.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google ScholarIrrespective of cause, emetic processes (nausea and vomiting) predominate; 70% have ictus emeticus.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Others include pallor, sometimes flushing or cyanosis, mydriasis, and, less often, miosis, cardiorespiratory changes, incontinence of urine and faeces, thermoregulatory alterations, and modifications of intestinal motility.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Hypersalivation (probably a concurrent Rolandic symptom) can occur.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Headache, and more frequently, cephalic auras may also be autonomic manifestations.4Burgess RC Autonomic signs associated with seizures.in: Luders HO Noachtar S Epileptic seizures: pathophysiology and clinical semiology. Churchill Livingstone, New York2000: 631-641Google ScholarIn a typical presentation, autonomic symptoms appear first while the child is fully conscious and able to speak.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar They generally predominate at any phase of the seizure. Other more traditional seizure-symptoms ensue. Consciousness becomes impaired, eyes initially may gaze but often deviate to one side. Eyelid blinking, hemifacial or more generalised jerking may occur. Visual hallucinations are rare. The child commonly becomes unresponsive and flaccid (ictal syncope) before or without convulsions. A third develop unilateral or generalised convulsions. Even in the worse and lengthier seizures, patients are normal after a few hours' sleep.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google ScholarThe interictal electroencephalo-graphic variations of the benign seizures are illustrated by Ferrie and Grünewald. The electroencephalo-graphy is mostly different in cases with cerebral lesions (figure).Experimentally, autonomic seizures can be elicited by electrical stimulation of the limbic system, the insula, the operculum, the basal frontal regions, and the cingulate gyrus.4Burgess RC Autonomic signs associated with seizures.in: Luders HO Noachtar S Epileptic seizures: pathophysiology and clinical semiology. Churchill Livingstone, New York2000: 631-641Google Scholar Ictal vomiting from intracerebral recordings is attributed to seizure discharges from the non-dominant temporal lobe and insular.2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 4Burgess RC Autonomic signs associated with seizures.in: Luders HO Noachtar S Epileptic seizures: pathophysiology and clinical semiology. Churchill Livingstone, New York2000: 631-641Google Scholar These may not be applicable in children, who are especially susceptible to emesis, as also indicated by cyclic vomiting syndrome—a non-epileptic disorder also peculiar to childhood. Vomiting is coordinated by the brain stem in concert with certain higher-brain centres; the sensation of nausea involves the cerebral cortex. The features described by Ferrie and Grünewald imply diffuse cortical hyperexcitability, which is related to maturation. This diffuse epilepto-genicity may be unequally distributed, predominating in one area, which is generally posterior. The preferential involvement of the autonomic system may be due to epileptic discharges generated at various cortical locations and influencing the hypothalamus, the principal regulatory centre of the autonomic nervous system.Despite striking stereotypical features and diagnostic importance, these autonomic seizures and autonomic status epilepticus, are practically unknown; they are commonly erroneously dismissed or misdiagnosed as non-seizure events such as encephalitis or migraine. Even in the latest proposal on the classification of seizures and status these are ignored.5Engel Jr, J A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology.Epilepsia. 2001; 42: 796-803Crossref PubMed Scopus (1895) Google Scholar Increased awareness, practice parameter guidelines, and systematic studies are overdue. Colin Ferrie and Richard Grünewald, in their March 17 commentary,1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar highlight a common and benign childhood epileptic syndrome and emphasise the predominant autonomic manifestations, which have immense clinical implications. These autonomic seizures and autonomic status epilepticus are peculiar to childhood.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar They do not occur in adults; ictus emeticus (not in the same sequence as in children) is exceptional (18 cases only) in large neurosurgical series.3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar In children, autonomic seizures and autonomic status epilepticus, are common, occurring in 6–7% of childhood epilepsy.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar They largely affect otherwise healthy children, but 10–20% might be the result of documented brain pathology.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Irrespective of cause, emetic processes (nausea and vomiting) predominate; 70% have ictus emeticus.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Others include pallor, sometimes flushing or cyanosis, mydriasis, and, less often, miosis, cardiorespiratory changes, incontinence of urine and faeces, thermoregulatory alterations, and modifications of intestinal motility.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Hypersalivation (probably a concurrent Rolandic symptom) can occur.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar Headache, and more frequently, cephalic auras may also be autonomic manifestations.4Burgess RC Autonomic signs associated with seizures.in: Luders HO Noachtar S Epileptic seizures: pathophysiology and clinical semiology. Churchill Livingstone, New York2000: 631-641Google Scholar In a typical presentation, autonomic symptoms appear first while the child is fully conscious and able to speak.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar They generally predominate at any phase of the seizure. Other more traditional seizure-symptoms ensue. Consciousness becomes impaired, eyes initially may gaze but often deviate to one side. Eyelid blinking, hemifacial or more generalised jerking may occur. Visual hallucinations are rare. The child commonly becomes unresponsive and flaccid (ictal syncope) before or without convulsions. A third develop unilateral or generalised convulsions. Even in the worse and lengthier seizures, patients are normal after a few hours' sleep.1Ferrie CD Grünewald RA Panayiotopoulos syndrome: a common and benign childhood epilepsy.Lancet. 2001; 357: 821-823Summary Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 3Panayiotopoulos CP Benign childhood partial seizures and related epileptic syndromes. John Libbey, London1999Google Scholar The interictal electroencephalo-graphic variations of the benign seizures are illustrated by Ferrie and Grünewald. The electroencephalo-graphy is mostly different in cases with cerebral lesions (figure). Experimentally, autonomic seizures can be elicited by electrical stimulation of the limbic system, the insula, the operculum, the basal frontal regions, and the cingulate gyrus.4Burgess RC Autonomic signs associated with seizures.in: Luders HO Noachtar S Epileptic seizures: pathophysiology and clinical semiology. Churchill Livingstone, New York2000: 631-641Google Scholar Ictal vomiting from intracerebral recordings is attributed to seizure discharges from the non-dominant temporal lobe and insular.2Panayiotopoulos CP Vomiting as an ictal manifestation of epileptic seizures and syndromes.J Neurol Neurosurg Psychiatry. 1988; 51: 1448-1451Crossref PubMed Scopus (118) Google Scholar, 4Burgess RC Autonomic signs associated with seizures.in: Luders HO Noachtar S Epileptic seizures: pathophysiology and clinical semiology. Churchill Livingstone, New York2000: 631-641Google Scholar These may not be applicable in children, who are especially susceptible to emesis, as also indicated by cyclic vomiting syndrome—a non-epileptic disorder also peculiar to childhood. Vomiting is coordinated by the brain stem in concert with certain higher-brain centres; the sensation of nausea involves the cerebral cortex. The features described by Ferrie and Grünewald imply diffuse cortical hyperexcitability, which is related to maturation. This diffuse epilepto-genicity may be unequally distributed, predominating in one area, which is generally posterior. The preferential involvement of the autonomic system may be due to epileptic discharges generated at various cortical locations and influencing the hypothalamus, the principal regulatory centre of the autonomic nervous system. Despite striking stereotypical features and diagnostic importance, these autonomic seizures and autonomic status epilepticus, are practically unknown; they are commonly erroneously dismissed or misdiagnosed as non-seizure events such as encephalitis or migraine. Even in the latest proposal on the classification of seizures and status these are ignored.5Engel Jr, J A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology.Epilepsia. 2001; 42: 796-803Crossref PubMed Scopus (1895) Google Scholar Increased awareness, practice parameter guidelines, and systematic studies are overdue.