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Panayiotopoulos Syndrome

Benign Occipital Epilepsy


Presentation

  • Here we present a 3½-year-old male child with Panayiotopoulos syndrome who presented with status epilepticus and ictal cardiorespiratory arrest requiring cardiopulmonary resuscitation for revival.[ncbi.nlm.nih.gov]
  • We present a 9 year old boy with this condition, who presented with episodes of severe vomiting, fever, deviation of eyes and altered sensorium, characteristic EEG in the form of multiple occipital spikes and normal neuroradiologic and metabolic investigations[ncbi.nlm.nih.gov]
  • Abstract To present a retrospective study of nine children with Panayiotopoulos syndrome associated with diffuse spikes and waves as the sole EEG manifestation at onset.[ncbi.nlm.nih.gov]
  • A neurologically normal 13-month-old boy presented on 2 occasions with complex partial status epilepticus that included significant ictal priapism.[ncbi.nlm.nih.gov]
  • The AIM of the present study was to introduce and investigate clinical criteria for Panayiotopoulos syndrome that lack a mandatory symptom.[ncbi.nlm.nih.gov]
Collapse
  • A 7-year-old boy had from 5 years of age approximately 12 episodes of collapse at school. All episodes were stereotyped but of variable duration from 2 to 35 minutes.[en.wikipedia.org]
Sighing
  • The clinical symptoms (sighing, arousal with eyes opening, eye-deviation, and emesis with possible alteration of consciousness) started two minutes after the electrographic onset.[ncbi.nlm.nih.gov]
Respiratory Abnormalities
  • This case also illustrates the features of syncope-like epileptic seizures together with other variable autonomic symptoms (emesis, respiratory abnormalities, pallor, mydriasis) in Panayiotopoulos syndrome.[en.wikipedia.org]
Recurrent Vomiting
  • At age 4 years, two episodes of recurrent vomiting, tonic eye deviation and consciousness impairment lasting for about 30 min occurred.[ncbi.nlm.nih.gov]
Tachycardia
  • In the literature, there is little information on the ictal EEG characteristics of Panayiotopoulos syndrome and, in particular, on certain autonomic manifestations, such as tachycardia, as the sole ictal phenomena at the onset of seizures.[ncbi.nlm.nih.gov]
  • Occasionally, these seizures can present with prominent autonomic features such as ictal vomiting, pallor, flushing/cyanosis, and tachycardia with prolonged thermoregulatory changes lasting for hours, constituting autonomic status epilepticus.[ncbi.nlm.nih.gov]
  • Hypertension, tachycardia, and the release of vasopressin suggest activation of the central autonomic network during seizures in familial Panayiotopoulos syndrome.[ncbi.nlm.nih.gov]
  • On many occasions, tachycardia was the first objective sign when ECG was recorded. Vomiting was a common ictal symptom occurring at any stage of the seizures but not as the first clinical manifestation.[en.wikipedia.org]
Flushing
  • Occasionally, these seizures can present with prominent autonomic features such as ictal vomiting, pallor, flushing/cyanosis, and tachycardia with prolonged thermoregulatory changes lasting for hours, constituting autonomic status epilepticus.[ncbi.nlm.nih.gov]
  • Patient 2, a 10-year-old boy, had episodic headaches with pinpoint pupils, skin flushing of the face, trunk, and extremities, purple discoloration of hands and feet, diaphoresis, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • Emesis, pallor, or flushing was almost always among the first symptoms that usually culminated in vomiting (77.4% of patients).[ncbi.nlm.nih.gov]
  • Other autonomic manifestations include pallor (or, less often, flushing or cyanosis), mydriasis (or, less often, miosis), cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal[ncbi.nlm.nih.gov]
Miosis
  • Concurrent autonomic symptoms during AS SLES included emesis, incontinence, mydriasis, miosis, and cardiorespiratory abnormalities.[ncbi.nlm.nih.gov]
  • Other autonomic manifestations include pallor (or, less often, flushing or cyanosis), mydriasis (or, less often, miosis), cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal[ncbi.nlm.nih.gov]
Lacrimation
  • Patient 1, a 12-year-old girl, had a history of involuntary lacrimation, abdominal pain, and recurrent episodes of loss of muscle tone and unresponsiveness followed by somnolence. Her EEG revealed bilateral frontotemporal spikes.[ncbi.nlm.nih.gov]
Episodic Headache
  • Patient 2, a 10-year-old boy, had episodic headaches with pinpoint pupils, skin flushing of the face, trunk, and extremities, purple discoloration of hands and feet, diaphoresis, nausea, and vomiting.[ncbi.nlm.nih.gov]
Mydriasis
  • Concurrent autonomic symptoms during AS SLES included emesis, incontinence, mydriasis, miosis, and cardiorespiratory abnormalities.[ncbi.nlm.nih.gov]
  • The seizures included mainly autonomic symptoms such as nausea, vomiting, pallor, mydriasis, urinary and fecal incontinence, and rarely hypersalivation. Autonomic partial status was detected in one patient.[ncbi.nlm.nih.gov]
  • Other autonomic manifestations include pallor (or, less often, flushing or cyanosis), mydriasis (or, less often, miosis), cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal[ncbi.nlm.nih.gov]
Night Terrors
  • Patient 3, an 8-year-old boy, had a history of restless legs at night, enuresis, night terrors, visual hallucinations, cyclic abdominal pain, and nausea. His EEG showed bitemporal spikes.[ncbi.nlm.nih.gov]
Involuntary Movements
  • The latter occurred in 7 children suddenly and without premonition or obvious triggers while standing, sitting, lying down, or asleep, did not resolve in the horizontal position, and were not associated with stiffening or any involuntary movements, even[ncbi.nlm.nih.gov]
Flaccid Paralysis
  • Paralysis: A case report Ito K,Fujita T,Watanabe E,Ihara Y,Ideguchi H,Morishita T, Inoue T,Inoue T, Hirose S, AOCCN2017 14th Asian and Oceanian Congress of Child Neurology, 2017年5月 The effectiveness of steroid pulse therapy and prognosis in frontal lobe[resweb2.jhk.adm.fukuoka-u.ac.jp]

Workup

  • We describe a young girl with intractable and lengthy vomiting attacks, several admissions to hospitals, and extensive gastroenterological workup for several years from early childhood.[ncbi.nlm.nih.gov]
Multifocal Spikes
  • Interictal EEG shows occipital spikes although multifocal spikes with high amplitude sharp-slow wave complexes at various locations can be present.[ncbi.nlm.nih.gov]
  • Interictal EEG showed multifocal spikes in the centrotemporal and left posterior regions.[ncbi.nlm.nih.gov]
  • The last seizure was captured with electroencephalography and showed a diffuse onset of the ictal discharge, whereas various interictal electroencephalography had infrequent multifocal spikes.[ncbi.nlm.nih.gov]
  • Multifocal spikes were apparent over frontal areas in the EEG and MRI demonstrated an inferior parietal lobe (IPL) lesion. RESULTS: A long-term 35-channel scalp EEG was obtained, which was processed with a Blind Source Separation algorithm.[ncbi.nlm.nih.gov]
  • Abstract Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy, which has a good prognosis despite the fact that it is frequently associated with abundant multifocal spikes on the electroencephalography (EEG).[ncbi.nlm.nih.gov]
Occipital Sharp-and-Slow-Waves
  • The electroencephalogram manifestations are severe with long runs of high amplitude occipital sharp and slow waves.[adc.bmj.com]
  • Both syndromes share similar classical inter-ictal EEG features with runs of occipital sharp and slow wave complexes which attenuate on eye opening (Panayiotopoulos, 1981 ).[academic.oup.com]
Irregular Generalized Spike-and-Slow-Waves
  • An outpatient EEG 6 months later showed abundant multifocal, predominantly posterior, as well as irregular generalized spike-and-slow-wave discharges on normal background consistent with the diagnosis of Panayiotopoulos syndrome.[ncbi.nlm.nih.gov]
Multifocal Discharges
  • Sequential EEGs obtained from 5 to 11 years of age showed both multifocal discharges and generalized spike and wave complexes. With these changes in EEG findings, the patient experienced various types of seizures.[ncbi.nlm.nih.gov]
Multifocal Epileptiform Discharges
  • Multifocal epileptiform discharges on EEG were identified in 84% of the patients. Two or more antiepileptic drugs were required in only 13% of the patients.[ncbi.nlm.nih.gov]

Treatment

  • Considerable international variation in management and controversy about non-treatment indicate the need for high quality randomised controlled trials (RCT).[ncbi.nlm.nih.gov]
  • All three children received LEV monotherapy and remained seizure-free after 3, 3 and 2 years of treatment, respectively. One child, after 2 years seizure free, stopped LEV treatment. Now, he is asymptomatic for 2 years.[ncbi.nlm.nih.gov]
  • All patients except for one received carbamazepine treatment 450-600 mg/day. None of our patients had seizures under antiepileptic treatment.[ncbi.nlm.nih.gov]
  • Prophylactic treatment with antiepileptic medication may not be needed for most patients.[ncbi.nlm.nih.gov]

Prognosis

  • PURPOSE: To determine prevalence, clinical, EEG features, and prognosis of Panayiotopoulos syndrome and to examine the proposition that clinical manifestations are more important than EEG findings.[ncbi.nlm.nih.gov]
  • PURPOSE: To explore the clinical, electroencephalography (EEG), neuropsychological features, and prognosis of Panayiotopoulos syndrome (PS).[ncbi.nlm.nih.gov]
  • The overall prognosis was excellent. After the first event, 15 subjects (40.54%) experienced at least another typical PS seizure, but all patients were seizure free at the last follow-up.[ncbi.nlm.nih.gov]
  • Poma, Mantova, Italy. pippo.capovilla@ospedalimantova.it Abstract In its first description (1989), Panayiotopoulos syndrome was defined as an idiopathic epilepsy syndrome with an excellent prognosis, characterized by a clinical ictal triad of nocturnal[ncbi.nlm.nih.gov]
  • Prognosis was excellent. Eighty-four (44.2%) had a single seizure, 79 (41.2%) had 2-5 fits, and 28 (14.6%) had frequent seizures.[ncbi.nlm.nih.gov]

Etiology

  • If an infectious or a metabolic etiology is suspected, laboratory tests can also be helpful.[amboss.com]
  • Introduction Occipital epilepsies commonly start in childhood or adolescence and include two etiologic groups: Symptomatic and idiopathic epilepsies.[neupsykey.com]
  • […] return to baseline LOC Refractory status epilepticus – seizure lasting 1h or refractory to 2 appropriate AEDs Engel classification Axis 1 – ictal phenomenology Axis 2 – seizure type, focal origin, precipitating stimuli Axis 3 – epilepsy syndrome Axis 4 – etiology[emilytam.com]
  • The other etiologies to be eliminated are hyperglycemias, Lafora or mitochondrial diseases or occipital calcifications in relation with a c?liac disease (5, 6, 9). COE-G may be misdiagnosed as Panayiatopoulos syndrome (PS).[latunisiemedicale.com]
  • Vadlamudi L, Kjeldsen MJ, Corey LA, Solaas MH, Friis ML, et al. (2006) Analyzing the etiology of benign rolandic epilepsy: a multicenter twin collaboration. Epilepsia 47: 550-555.[omicsonline.org]

Epidemiology

  • Thirty-seven medical records of patients diagnosed with PS were reviewed and the epidemiological and clinical features, results of complementary studies and evolutional data were collected. Mean age at diagnosis was 5.4 years.[ncbi.nlm.nih.gov]
  • PURPOSE: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management.[ncbi.nlm.nih.gov]
  • EPIDEMIOLOGY: Panayiotopoulos syndrome probably affects 13% of children aged 3 to 6 years who have had 1 or more afebrile seizures and 6% of such children in the 1- to 15-year age group.[ncbi.nlm.nih.gov]
  • EPIDEMIOLOGY. Panayiotopoulos syndrome probably affects 13% of children aged 3 to 6 years who have had 1 or more afebrile seizures and 6% of such children in the 1- to 15-year age group. DIAGNOSTIC TESTS.[doi.org]
  • Prospective epidemiological and clinical studies should assess the actual prevalence, delineate the clinical spectrum, and define possible clinical and electroencephalography markers of atypical clinical presentations and complicated evolution that is[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • This is a critical review of the clinical and EEG features of PS, focusing on those characteristics that may shed some light on its so far elusive pathophysiology.[ncbi.nlm.nih.gov]
  • Abstract Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder with significant clinical, pathophysiological and management implications. It affects otherwise normal children with onset at around 3-6 years.[ncbi.nlm.nih.gov]
  • However, many theoretical and practical points, including diagnostic, genetic, and pathophysiologic issues remain still unresolved for PS. [Indexed for MEDLINE] Free full text[ncbi.nlm.nih.gov]
  • This case contributes to the understanding of the pathophysiology of Panayiotopoulos syndrome in favor of a diffuse and multifocal cortical epileptogenicity that triggers an unstable central autonomic nervous system solely or prior to the focal cortical[ncbi.nlm.nih.gov]
  • PURPOSE: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management.[ncbi.nlm.nih.gov]

Prevention

  • Knowing that an autonomic manifestation during fever can be an epileptic seizure could facilitate diagnosis and prevent unnecessary investigations and erroneous treatments.[ncbi.nlm.nih.gov]
  • Treatment management may be required to prevent SE as much as possible to achieve optimal prognosis in PS at least in some patients. Copyright 2015 Elsevier B.V. All rights reserved.[ncbi.nlm.nih.gov]
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  • The decision should also take into account a likely traumatising parental experience as in febrile convulsions. 3 , 4 , 8 Appropriate advice by the family doctor is expected to shape parental attitude and prevent chronic anxiety.[ncbi.nlm.nih.gov]
  • A history of a previous similar seizure is reassuring and may prevent further procedures. Electroencephalography (EEG). EEG is the only investigation with abnormal results, usually showing multiple spikes in various brain locations (Figure).[en.wikipedia.org]

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