2. • 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.
3. • 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.
4. • She was already on several AED’s (TPM, CLB,
LEV, OXCBZ) and was diagnosed as Refractory
focal Epilepsy.
• We added LACOSAMIDE.
5. • She had developed mild cognitive decline mainly
in language skills.
• Mild rt. Hemiperesis grade IV Power.
• Fairly good rt. hand function.
• No hemianopia
6. • In wards, she was noted to have EPC, even in
sleep.
• All Biochemistry WNL.
• CSF studies WNL
7.
8.
9. 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.
10. 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.
11. 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.
12. 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 EPC
2. EEG – Unihemispheric slowing with or without
epileptiform activity
3. Unilateral focal cortical atrophy
a) Grey or white matter T2/FLAIR hyperintense
signal.
b) Hyperintense signal or atrophy of the ipsilateral
caudate head.
13. Diagnostic Criteria of R.E. – by
Christian Bien
Part B
1. Clinical: EPC
2. MRI: Progressive focal cortical atrophy
3. Histopath: T-cell dominated encephalitis
14. • 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.
15. 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
16. Progress over first 6 months showed deterioration
in
– Congnitive function esp. speech.
– Hemiparesis became more significant.
– EPC more pronounced.
17. Treatment changed to
– IV IgG 1 gm every 15 days.
– Tab. Tacrolimus 1 mg bd continued.
– No steroids.
– AEDs as previously.
18. Follow up in Dec. 2012:
– Very subtle seizures.
– Rt. Hemiparesis remains status quo.
– Cognitive improvement significant.
19. 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 –
