Panayiotopoulos Syndrome.pptx

7/27/2019 Panayiotopoulos Syndrome.pptx

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PANAYIOTOPOULOSSYNDROME

A Case Presentation by Zakaria Mukalla


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In t roduct ion

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

Over the past two decades, new epilepsy syndromes inchildren has been found, one of which is Panayiotopoulos

syndrome (PS)

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


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In t roduct ion

Two thirds of seizures occur during sleep at night or at naptime. At bedtime, the child woke up with headaches,

vomiting, confusion, or being unresponsive.

Autonomic seizures are characteristic of PS.

Incident of PS 13% in children aged 3-6 years who hadone or more seizures without fever (peak age : 4-5 year),

and 6% of the age group 1-15 years


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In t roduct ion

The purpose of presenting this case is toshare about one & rare epilepsy syndrome sothat we can quickly identify, diagnose and give

appropriately treatment.

It is important for clinicians to understand thesyndrome, because it can be very similar to

the non-epileptic disease


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Identi f icat ion

DC/

4 y.oAdmitted Feb

22nd 2013

Mr. S

43 y.oHigh school

Employee

Mrs. D

40 y.oHigh School

House wife

Patient MotherFather


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CC Vomiting

Anamnesis

HeadacheAC


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Anamnesis

1 days b.aSuddenly 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

Present Illness History


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No history of seizure

History of head injury 4 y b.a. (vomit +)Treated in hospital

Often wake up in the night : complain

headache & vomit

Anamnesis

Past I.H

History of seizure : denied No history of migrain

FH


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Anamnesis

Normal

Basic : Complete, no booster

Good in quality and quantity

History of Pregnancy and labor

History of immunization

History of nutrition


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Anamnesis

Normal

Middle low income

Developmental

Socio-economic


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Phys ical Exam inat ion

Alert

Pulse 110 b/m (v&p equal)

Temperature 37,9o

Resp. Rate 24 t/m (regular)

Good Nutritional status

General Findings


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Phys ical Exam inat ion

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 wheezingAbdominal: flat, tender, liver

& spleen unpalpable,

tymphani, normal bowel

sound.

Extremities: no cyanosis

Spesific Findings Spesific Findings

Normal Neurologic status


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In i t ial Analys is

GI Tract

Intra Cranial

ProjectileNo complain in

ENT, teeth

No history of

epigastric pain

No diarrhea

VOMITING

HEADACHE


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In it ial Prob lem

CT Scan Abdomen

Paracetamol 3x 180mg

Consult eye dept

Education to parents

Rehydration with IVFD NaCl 0,9% gtt

15 macrodrip in 4 h maintenanceOndansentron 3x1,5mg

Ranitidin 2x15mgEducation to parents

Problem

(P)

P 1

Cephalgia

ec Susp

SOL

P 2

Profuse

Vomiting withmild-

moderate

dehydration


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Prog ress Note

Day

Day care

Day

1 2 3

Vomiting (+) Headache (+)

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 wakein night with headache&

vomit fall asleep susp

ec epilepsy Plan to EEG

Add dexametason 3x3mg

Headache, vomiting Pulse 102 b/m

RR 30 t/m T 36,2O

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Same treatment

Headache, vomiting (-) Pulse 98 RR 26 t/m

T 36,2O

EEG result : spike in

occipital, autonomic

symptom, 5 year old

Panayiotopoulossyndrome

Carbamazepin 3x15mg

Dexametason stop


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Prog ress Note

Day

Day care

4

Vomiting (-) Headache (-)

Alert Pulse 98 b/m

RR 26 t/m T 36,2O

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Better condition

Carbamazepin 3x15mg Plan to discharge


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L iterature Rev iew

PANAYIOTOPOULOSSYNDROME


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Defini t ion

a childhood-relatedidiopathic benignsusceptibility to

focal,

mainly autonomic,seizures and

autonomic status

epilepticus.

Normal physical

andneuropsychological

development.

Autonomic

manifestations arethe cardinal seizure

symptoms in PS,


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Age at onset : 114years (peak : 45

years)Boys = girls, all races

Prevalence in childrenwith 1/more non-febrileseizure: 3-6year13%

1-15year6%

In the generalpopulation, 23/1000

children may beaffected.

Demographic


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Clin ical Man ifes tat ions

Autonomic symptoms(mainly emetic)

fully conscious

looks pale


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Other Autonom ic Symptoms

Cyanosis

Incontinence of urine and faeces

Mydriasisor miosis

Hypersalivation

Cephalic aura

Coughing

Thermoregulatory Changes

Abnormalities of Intestinal Motility

Breathing and cardiac irregularities

Cardiorespiratory arrest


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Convent ional Seizu re-Symptom s

Deviation of the eyes (6080%)

Hemiconvulsions (26%)

Generalised convulsions (20%)

Speech arrest (8%)

Impairment of consciousness (94%)


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Two-thirds of seizures start in sleep;

conscious

vomiting,

Unresponsive


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Et io logy

probably geneticallydetermined

no family history

high prevalence of febrileseizures (about 17%)


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Pathophysio logy

Autonomic seizure

triggering low-threshold emetic centres andhypothalamus

In vulnerable children Activate autonomic centre

Diffuse cortical hyperexcitability

Maturation relatedUnequally distributedPosterior predominant


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Electroencephalography

Multi-focal, high amplitude sharp-slow wavecomplexes Spikes

Occur at various posterior locations and, lessoften anterior locations

may appear as cloned-like,

repetitive, multifocal spike-wave complexes


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Differen tial Diagnos is

Encephalitis

deteriorating

level ofconsciousness

convulsionsAtypicalmigrain

Gastroenteritis


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Differen tial Diagnos is

Motionsickness

Cardiogenicsyncope

etc


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Differen t ial Diagnos is from EEG

Rolandic epilepsy

Panayiotopoulos syndrome

Gastaut Syndrome

29


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Prognos is

Benign condition

Outcome is favourable

Remission within 12years from onset

No risk for epilepsy inadult


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Case Analys is

Case

Aged :

5 y.o.

Literature

Aged :

range 1-14 y.o

peak 4-5 y.o


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Case Analys is

Case

Past Illness History:

no febrile

Literature

13% with 1/more

non-febrile seizure


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Case Analys is

Case

Chief Complain:

Vomiting

Literature

Mainly emetic symptom


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Case Analys is

Case

No Neurological defisit

Literature

Normal physical and

neuropsychological

development.


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Case Analys is

Case

Autonomic symptomp:

Vomiting, Cephalic aura,

sub-febrile

LiteratureVomit, pallor, cyanosis,

mydriasis, myosis,cardiorespiratory and

thermoregulatory

alterations, coughing,

incontinence of urine /faeces, modifications of

intestinal motility.

Headache and more.

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Zakaria Muk alla

Panayiotopoulos Syndrome.pptx

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