benign occipital epilepsy

2. Jagadeesh, 3 year old male child, born to
parents of non-consanguinous marriage.
Child was of normal development.
Known case of seizure disorder on anti-
epileptic drug Oxcarbazepine was brought
to casualty in unconscious state and child
was managed as per protocol.

4. This child had an episode on dec 2014 was
seen by a paediatric neurologist and
investigated.
EEG done on Dec 2014- abnormal record
suggesting bilateral dysfunction cortical in
nature.

5. Child was started on AED Oxcarbazepine
(10mg/kg).
Child was not responding to AED and
continued to have similar 4 episodes.
Probing the details of the actual nature of
the fit it was brought to light that the child
has never had tonic clonic convulsions.

6. All the episodes were associated with
vomiting, sweating and drooling of saliva
and occurred during sleep.
There was no h/o incontinence of urine or
faeces.
Other alternative diagnosis like syncope,
non-convulsive status were thought of.

7. In consultation with neurologist,
anticonvulsant was stopped.
Child was investigated further, EEG was
done and showed abnormal record due to
high voltage slow wave discharges.
ECHO and ECG were within normal limits.
The child was normal during the stay in the
hospital.

8. The features of
 unconsciousness
 not associated with any tonic clonic
convulsions
 associated with autonomic features like
sweating, vomiting, drooling
a diagnosis of Panayiotopoulos epilepsy or
autonomic epilepsies was made.

9.  Panayiotopoulos syndrome has been
characterized as “early onset benign
childhood occipital epilespy.”
INCIDENCE:
 Age of onset: 1-14 years of age. (peak
age – 3 to 6 years)
 Both sexes are equally affected.
 It is a rare condition

10. CLINICAL FEATURES:
 Nausea and emesis are usually the
heralding symptom.
 Seizure occurrence is more common in
sleep, especially the early part of sleep.
Two-thirds of seizures begin in sleep.

11. Other autonomic symptoms may occur at
seizure onset
• flushing or cyanosis
• pupillary changes
• coughing
• cardiorespiratory changes
• thermoregulatory changes
• fecal or urinary incontinence

12.  A triad of nausea, retching and vomiting
occurs in upto 74% of seizures.
 Headaches and cephalic aura have been
observed.
 Ictal syncope is a described feature. In
atleast 20% of seizures, child becomes
unresponsive and flaccid prior to
convulsions.

13.  It is characterized by transient loss of
responsiveness and postural tone.
 10% of patients will have pure autonomic
seizures – comprise autonomic
symptoms only.
 Impairment of consciousness may be
mild to moderate.

14. Less common ictal symptoms
 speech arrest
 hemifacial spasm
 visual hallucinations
 oropharyngeal movements
 unilateral mouth droop
 eyelid jerks
 myoclonic jerks
 nystagmus, automatisms.
Nearly half of the seizures last for more
than 30 minutes.

15. ETIOLOGY:
 It is genetic in origin.
 PS can be observed in siblings.
 A SCN1A gene mutation has been
reported in few cases and this is
speculated to represent cases with a
more severe phenotype [7,19]

16. DIAGNOSTIC WORKUP:
 Normal neurological examination
 Normal MRI findings
 In 90% EEG will demonstrate multi-focal,
high amplitude, sharp and slow wave
complexes.

17.  Spikes may also be present and shift
between hemispheres. Occipital spikes
are frequent.
 EEG should be acquired during sleep
since interictal epileptiform activity is
more prevalent in sleep.

18. TREATMENT:
 Seizures typically remit with one to 2
years of onset.
 Most patients will not require anti
convulsant therapy.

19.  Autonomic status epilepticus should be
thoroughly evaluated to rule out other
causes.
 Frequent seizures require AED.
Carbamazepine is recommended though
newer drugs with focal-onset coverage
have been used.

20. THANK YOU