DR. DEEPA DIVEKAR

PAEDIATRIC NEUROLOGIST

SAHYADRI SPECIALITY HOSPITAL, PUNE

• 4 year old girl; previously healthy.

• Onset of seizures June 2011.

• Initially 2-4/month.

• Started with Rt. Focal ; then Lt. focal, at times

becoming generalised.

• By Dec 2011, when she presented to us her

seizures had increased to around 20-30 /day,

brief rt. focal jerking or dystonia, with rt. facial

twitchings,drooling and brief unresponsivness

lasting < 30 secs.

• She was already on several AED’s (TPM, CLB,

LEV, OXCBZ) and was diagnosed as Refractory

focal Epilepsy.

• We added LACOSAMIDE.

• She had developed mild cognitive decline mainly

in language skills.

• Mild rt. Hemiperesis grade IV Power.

• Fairly good rt. hand function.

• No hemianopia

• In wards, she was noted to have EPC, even in

sleep.

• All Biochemistry WNL.

• CSF studies WNL

T2 AXIAL

Dec 2011

May 2012

Progressive atrophy of cortex, prominence of sulcal spaces with ipsilateral ventricular dilatation and increase in the white matter signal.

FLAIR Coronal

Dec 2011

May 2012

Progressive atrophy of cortex, prominence of sulcal spaces with ipsilateral ventricular dilatation and increase in the white matter signal.

The GluR 3 antibody test was pursued but it was

not possible to do it in India. So we decided to

treat her vigorously before she has further

cognitive decline.

Diagnostic Criteria of R.E. – by Christian Bien

RE can be diagnosed if either all 3 of Part A or 2 out of 3 of Part B are present.

Part A 1. Clinical Focal or EPC2. EEG – Unihemispheric slowing with or without

epileptiform activity3. Unilateral focal cortical atrophy

a) Grey or white matter T2/FLAIR hyperintense signal.

b) Hyperintense signal or atrophy of the ipsilateral caudate head.

Diagnostic Criteria of R.E. – by Christian Bien

Part B

1. Clinical: EPC2. MRI: Progressive focal cortical atrophy3. Histopath: T-cell dominated encephalitis

• According to Diagnostic Criteria of RE our

patient has 3 criteria of Part A and 2 criteria of

Part B.

• So she fits into a diagnosis of RE.

She was started on

– I/V Methyl Pred. 30mg/Kg/day x 5 days.

– Followed I/V Ig 400mg/Kg/day x 5 days

– Started on Tab Tacrolimus 1mg BD x 6 mths

– IV IG 1gm once a month x 6 months

Pulse dose

Maintenance Therapy

Progress over first 6 months showed deterioration

in

– Congnitive function esp. speech.

– Hemiparesis became more significant.

– EPC more pronounced.

Treatment changed to

– IV IgG 1 gm every 15 days.

– Tab. Tacrolimus 1 mg bd continued.

– No steroids.

– AEDs as previously.

Follow up in Dec. 2012:

– Very subtle seizures.

– Rt. Hemiparesis remains status quo.

– Cognitive improvement significant.

1) How long to continue above treatment?

2) Does surgery have a role in this patient?

3) Is it worthwhile trying Rituximab?

Questions to be answered –

THANK YOU