Epilepsy at the Gastroenterology consultation

Larrosa Espinosa, Cristina; García Romero, Ruth; López-Pisón, Javier

doi:10.1016/j.medcle.2019.07.021

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PS, described in 1975 by C P Panayiotopoulos, is included within benign focal idiopathic childhood-related epilepsies. It is an entity that, despite a non-negligible prevalence, characteristic symptoms and EEG results, it is frequently underdiagnosed, and is confused with surgical, neurological, metabolic, renal and digestive entities. The result is avoidable morbidity, erroneous treatment, and costly hospital admissions. PS is a childhood-related susceptibility to focal seizures which are sometimes generalised, prolonged and with an autonomic component. It is benign in terms of its evolution, however the impact on the patient's quality of life can be significant.<a class="elsevierStyleCrossRef" href="#bib0030">1</a>A patient aged 2 years and 11 months, with no relevant history except a transient lactose-intolerance, with adequate weight gain, and for the past 6 months weekly episodes of nocturnal vomiting of almost daily frequency (range: 2–6episodes/week), one hour and a half after getting into bed, preceded by vegetative symptoms (sweating, pale skin, nausea and abdominal pain), with a sustained level of consciousness and subsequent amnesia of no more than 5min. A 12 year old boy, no history, with a condition for the past 2 years of monthly nocturnal vomiting (range: 3–5episodes/month) during sleep, also associated with profuse sweating, pale skin and nausea. In both cases the physical examination, blood test (thyroid hormones, celiac disease, liver profile, blood count, ESR, and ions), abdominal ultrasound, hydrogen test, urease test, and pH-metry were normal. Given the paroxysmal episodes and in the absence of other digestive symptoms, PS was suspected. Nocturnal polysomnograms were performed, which demonstrated epileptiform activity in the electroencephalographic recording. The first case received antiepileptic treatment due to the recurrence of the seizures and the impact on the quality of life (significant daytime fatigue), with partial control of the seizures initially and total control at one year after increasing the dose (levetiracetam 40–80mg/kg/day). The second case achieved spontaneous remission without treatment at 6 months after diagnosis. Brain MRI was normal in both cases. Both patients are currently under follow-up after 2.5 and 4 years, respectively, from the onset of symptoms.The estimated prevalence of PS is 6 to 8% in patients aged 1 to 14 years who present at least one seizure, and up to 13% in the subgroup 3 to 6 years.<a class="elsevierStyleCrossRef" href="#bib0030">1</a> It presents as prolonged epileptic seizures (>5min) of nocturnal onset associating autonomic symptoms (vomiting: 70–85%).<a class="elsevierStyleCrossRef" href="#bib0030">1</a> The electroencephalographic record of nocturnal sleep can support the diagnosis, based largely on the clinical information.Often PS is underdiagnosed because autonomic symptoms are not recognised as seizures, especially if they are brief episodes that simulate conditions such as gastroenteritis, syncopes, sleep disorders, or surgical (malrotation, volvulus), metabolic and renal entities.<a class="elsevierStyleCrossRefs" href="#bib0035">2–4</a> Longer episodes can be mistaken for cerebrovascular accidents, encephalitis, or poisoning.<a class="elsevierStyleCrossRef" href="#bib0035">2</a> They can also be evocative of neurological processes, migraines and occipital epilepsy due to the presence of visual symptoms, although in PS they are of shorter duration (<3min), periodic (often daily) and may progress to tonic ocular deviation or loss of consciousness with/without seizures.<a class="elsevierStyleCrossRef" href="#bib0035">2</a> Up to 11%<a class="elsevierStyleCrossRef" href="#bib0045">4</a> of cases are diagnosed as digestive disorders, as focus is placed on the cyclical vomiting syndrome and gastroesophageal reflux. GER can be very nonspecific, and CVS also presents with paroxysmal vomiting, with sudden onset at night or at dawn. However, none of these entities present electroencephalographic expressions.<a class="elsevierStyleCrossRefs" href="#bib0030">1–3</a>The natural evolution of the disease is benign. 44% of patients have a single seizure, 41% present 2–5 seizures, and 14% present repeated seizures, and the cumulative probability of recurrence is higher during the first 2years.<a class="elsevierStyleCrossRef" href="#bib0050">5</a> Subsequent neurological disorders attributable to the seizures have not been described.<a class="elsevierStyleCrossRef" href="#bib0035">2</a> The need to prescribe antiepileptic treatment is disputed, because in addition to its benign course, the seizures are frequently refractory to treatment. Treatment is mainly an answer to the frequency, intensity and duration of the seizures, the impact on the patient's quality of life and family anguish.<a class="elsevierStyleCrossRef" href="#bib0035">2</a>PS is frequently underdiagnosed and classified as another condition, especially a digestive condition. The presence of repeated vomiting is resonant with entities such as GER and cyclical vomiting. It is essential that all paediatricians know and understand this entity so as to provide an early diagnosis and proper management, avoiding unnecessary diagnostic and therapeutic procedures. Although specialisation is essential to achieve excellence in the knowledge and care of paediatric patients, we must retain an integrated vision of the child that will enable us to broaden our diagnostic horizons." 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Fejerman" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1528-1167.2007.01087.x" "Revista" => array:6 [ "tituloSerie" => "Epilepsia" "fecha" => "2007" "volumen" => "48" "paginaInicial" => "1165" "paginaFinal" => "1172" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17442005" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0035" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "A. Covanis" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Pediatrics" "fecha" => "2006" "volumen" => "118" "paginaInicial" => "1237" "paginaFinal" => "1243" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0040" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Panayiotopoulos syndrome: an important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non-epileptic disorders" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M. Michael" 1 => "K. Tsatsou" 2 => "C.D. Ferrie" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.braindev.2009.03.002" "Revista" => array:6 [ "tituloSerie" => "Brain Dev" "fecha" => "2010" "volumen" => "32" "paginaInicial" => "4" "paginaFinal" => "9" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19386455" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pediatric epilepsies misdiagnosed as gastrointestinal disorders" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "G. Carbonari" 1 => "G. Tonti" 2 => "V. di Pisa" 3 => "E. Franzoni" 4 => "D.M. Cordelli" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.yebeh.2018.03.034" "Revista" => array:7 [ "tituloSerie" => "Epilepsy Behav" "fecha" => "2018" "volumen" => "83" "paginaInicial" => "137" "paginaFinal" => "139" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29705623" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S1935861X13001654" "estado" => "S300" "issn" => "1935861X" ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Panayiotopoulos syndrome: a common and benign childhood epileptic syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "C.P. Panayiotopoulos" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Libro" => array:3 [ "fecha" => "2002" "editorial" => "John Libbey & Company" "editorialLocalizacion" => "London, United Kingdom" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015500000010/v1_202011260800/S2387020620304903/v1_202011260800/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015500000010/v1_202011260800/S2387020620304903/v1_202011260800/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020620304903?idApp=UINPBA00004N"]

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Vol. 155. Issue 10.

Pages 468-469 (November 2020)

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