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PANAYIOTOPOULOS SYNDROME
A Case Presentation by Zakaria Mukalla

Introduction
Epilepsy syndrome is a form of epilepsy, a set of signs and symptoms that occur together in an epileptic attack.

Over the past two decades, new epilepsy syndromes in children has been found, one of which is Panayiotopoulos syndrome (PS)

PS can be defined as an idiopathic seizure in children, with an EEG feature the spike in the occipital or extraoccipital, and the main clinical manifestation is autonomic seizures.

Introduction
Incident of PS 13% in children aged 3-6 years who had one or more seizures without fever (peak age : 4-5 year), and 6% of the age group 1-15 years

Autonomic seizures are characteristic of PS.

Two thirds of seizures occur during sleep at night or at nap time. At bedtime, the child woke up with headaches, vomiting, confusion, or being unresponsive.

Introduction
It is important for clinicians to understand the syndrome, because it can be very similar to the non-epileptic disease

The purpose of presenting this case is to share about one & rare epilepsy syndrome so that we can quickly identify, diagnose and give appropriately treatment.

Identification

Patient

Father

Mother

DC/ 4 y.o Admitted Feb 22nd 2013

Mr. S 43 y.o High school Employee

Mrs. D 40 y.o High School House wife

Anamnesis
Vomiting

CC

AC Headache

Anamnesis
Present Illness History
1 days b.a Suddenly nausea, vomiting , projectile 7x, what he ate & drank, no pale, no cyanosis, no abdominal pain, no fever, no cough, no diarrhea, no seizure. Headache (+), no migrain, no blurred vision fall asleep Emergency RSMH admitted

Anamnesis
No history of seizure

Past I.H

History of head injury 4 y b.a. (vomit +) Treated in hospital Often wake up in the night : complain headache & vomit

FH

History of seizure : denied

No history of migrain

Anamnesis
History of Pregnancy and labor
Normal

History of immunization
Basic : Complete, no booster

History of nutrition
Good in quality and quantity

Anamnesis

Developmental

Normal

Socio-economic

Middle low income

Physical Examination
General Findings

Alert Pulse 110 b/m (v&p equal) Temperature 37,9o Resp. Rate 24 t/m (regular)

Good Nutritional status

Physical Examination
Spesific Findings Spesific Findings

Head: Normocephal, no icteric, pupil: round, isocor, positive reflex, sunken eyes, no nasal flare. Thoracic: symmetrical, no retraction Cor: normal heart sound, no murmur.

Pulmo: vesicular, no ronchi, no wheezing Abdominal: flat, tender, liver & spleen unpalpable, tymphani, normal bowel sound. Extremities: no cyanosis

Normal Neurologic status

Initial Analysis
Projectile No complain in ENT, teeth

GI Tract

VOMITING Intra Cranial

No history of epigastric pain No diarrhea

HEADACHE

Initial Problem
P1

Cephalgia ec Susp SOL

Problem (P)

CT Scan Abdomen Paracetamol 3x 180mg Consult eye dept Education to parents

P2 Profuse Vomiting with mildmoderate dehydration

Rehydration with IVFD NaCl 0,9% gtt 15 macrodrip in 4 h maintenance Ondansentron 3x1,5mg Ranitidin 2x15mg Education to parents

Progress Note
Day care Day Day

1
Vomiting (+) Headache (+)

2
Headache, vomiting Pulse 102 b/m RR 30 t/m T 36,2O 100/70 Same treatment

3
Headache, vomiting (-) Pulse 98 RR 26 t/m T 36,2O EEG result : spike in occipital, autonomic symptom, 5 year old Panayiotopoulos syndrome Carbamazepin 3x15mg Dexametason stop

Alert Pulse 115 b/m RR 32 t/m T 32O 100/60 Normal laboratory finding CT Scan : no SOL, edema serebri P3 Re-anamnesa: often wake in night with headache& vomit fall asleep susp ec epilepsy Plan to EEG Add dexametason 3x3mg

Progress Note
Day care Day

4
Vomiting (-) Headache (-) Alert Pulse 98 b/m RR 26 t/m T 36,2O 100/70 Better condition Carbamazepin 3x15mg Plan to discharge

Literature Review

PANAYIOTOPOULOS SYNDROME

Definition
a childhood-related idiopathic benign susceptibility to focal, mainly autonomic, seizures and autonomic status epilepticus.

Normal physical and neuropsychological development.

Autonomic manifestations are the cardinal seizure symptoms in PS,

Age at onset : 114 years (peak : 45 years)

Boys = girls, all races

Demographic

Prevalence in children with 1/more non-febrile seizure: 3-6year13% 1-15year6%

In the general population, 23/1000 children may be affected.

Clinical Manifestations
Autonomic symptoms (mainly emetic) fully conscious looks pale

Other Autonomic Symptoms

Cyanosis Incontinence of urine and faeces Mydriasis or miosis Hypersalivation Cephalic aura
Coughing Thermoregulatory Changes Abnormalities of Intestinal Motility Breathing and cardiac irregularities Cardiorespiratory arrest

Conventional Seizure-Symptoms

Impairment of consciousness (94%) Deviation of the eyes (6080%)

Hemiconvulsions (26%)

Generalised convulsions (20%) Speech arrest (8%)

Two-thirds of seizures start in sleep; conscious vomiting, Unresponsive

Etiology
probably genetically determined no family history high prevalence of febrile seizures (about 17%)

Pathophysiology
Diffuse cortical hyperexcitability
Maturation related Unequally distributed Posterior predominant

triggering low-threshold emetic centres and hypothalamus

In vulnerable children

Activate autonomic centre

Autonomic seizure

Electroencephalography
Multi-focal, high amplitude sharp-slow wave complexes Spikes Occur at various posterior locations and, less often anterior locations may appear as cloned-like, repetitive, multifocal spike-wave complexes

Differential Diagnosis

Encephalitis
deteriorating level of consciousness convulsions

Atypical migrain

Gastroenteritis

Differential Diagnosis

Motion sickness

Cardiogenic syncope

etc

Differential Diagnosis from EEG

Rolandic epilepsy Panayiotopoulos syndrome Gastaut Syndrome

29

Prognosis
Benign condition

Outcome is favourable Remission within 12 years from onset

No risk for epilepsy in adult

Case Analysis
Case

Literature

Aged : 5 y.o.

Aged : range 1-14 y.o peak 4-5 y.o

Case Analysis
Case

Literature

Past Illness History: no febrile

13% with 1/more non-febrile seizure

Case Analysis
Case

Literature

Chief Complain: Vomiting

Mainly emetic symptom

Case Analysis
Case

Literature

No Neurological defisit

Normal physical and neuropsychological development.

Case Analysis
Case

Literature
Vomit, pallor, cyanosis, mydriasis, myosis, cardiorespiratory and thermoregulatory alterations, coughing, incontinence of urine / faeces, modifications of intestinal motility. Headache and more.

Autonomic symptomp: Vomiting, Cephalic aura, sub-febrile

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Zakaria Mukalla