Tuberous Sclerosis is a genetically inherited neurocutaneous syndrome can affect families in an autosomal dominant. in this presentaion i will try and give u a review to the case and its management. it will help u get a n outllook to diagnose a case and manage a refractory seizure with help of epilepsy surgery. when i wsa working at hinduja hospital, with Dr. Vrajesh Udani, this was the case which was operated for refractory epilepsy and had seizure freedom.

1. Dr. Amit Vatkar
MBBS, DCH, DNB Pediatrics
Fellow in Pediatric Neurology, Mumbai
Trained in Neurophysiology & Epilepsy, USA
Contact No. : +91-8767844488
Email: vatkaramit@yahoo.com
Epilepsy surgery in
tuberous sclerosis

2. HISTORY
• 2 year old boy with tuberous sclerosis / PDD
• Seizures started at 4 months of age
• Frequency 15-20 /day
• Semiology
– No aura
– Behavioural arrest with occ clenching of
teeth/grimacing
– Sometimes responds to name
– Brief seizures
• AEDs failed- CLB / VGB / OXZ / LEV / PHB at optimal
doses

3. Other evaluations
• Ash leaf macules on back
• Right sided retinal hamartoma noted in
fundus examination
• USG – Bil simple renal cortical cysts
• 2D ECHO- Normal

4. Inter-ictal EEG
RIGHT FRONTAL
INTERICTALS DISCHARGES

5. Seizure onset
• RIGHT FRONTAL FP2 SEIZURE ONSET

7. Seizure termination
RIGHT FRONTAL SLOWING

8. Seizure termination 2

9. MULTIPLE TUBERS IN BILATEREAL FRONTAL LOBES

10. Ictal and Interictal SPECT 1

11. Ictal and Interictal SPECT 2

12. Neuroimaging
• MRI- diffuse subcortical tubers in bilateral
cerebral hemispherees. Right medial frontal
cortical thickening with subcortical
calcifications.
• Enhancing nodule at foramen of Monro
• Ictal SPECT – Right frontal and subfrontal ictal
focus

13. Surgery
• Right frontal craniotomy
• Tuberous cortex identified with scalp EEG and
MRI.
• Extent of lesion decided with the help of ECoG

14. RIGHT FRONTAL LOBE EXCISED

16. ECoG

17. Post -op
• Immediate post-op period : few seizures
• Then seizure free till date (20 months post-op
• CT: partial right frontal lobectomy noted,
minimal extra axial collection seen
• Tapering of AED started

18. Epilepsy in TS
• 70-80 % patients with TS have epilepsy
– 30% are refractory
• Surgical decisions pose dilemmas
– Most children with TS have multiple potentially
epileptogenic tubers
– Localization is challenging
• New strategies have evolved to aid conventional
scalp VEEG and MRI

19. New modalities
• SPECT/PET
• AMT PET
• Multi-stage surgery with invasive EEG monitoring
• MEG

20. Multi-stage surgery
• Retrospective analysis of 25
– 22 underwent 3-stage and 3 underwent 2-stage surgery
• 3 stage involves electrode placement after initial resection
to look for 2nd
epileptogenic region, useful if:
– Multifocal bi-hemispheric involvement
– Involvement of eloquent cortex seen
• 28 mths fup-
– 17 (68%) seizure free
– 6 (24%) class II
– 2 (8%) class III
Paediatrics 2006 Weiner et al

21. Calcified subependymal nodule

22. Subependymal giant cell astrocytoma

23. Radial glial fibres

24. Cortical tuber and sub ependymal nodules

25. Retinal tuber

26. Conclusion
• Earlier surgery has undoubtedly significant benefits
with respect to improved cognition and development
• AMT PET / MEG are new strategies used to further
localization in TS with promising results
• In our scenario what is best?
– identification of TS patients with epilepsy
– Evaluation for early surgery
– ECoG guided surgery

27. About Dr. Amit Vatkar
Dr. Amit Vatkar is a Pediatric Neurologist from Navi Mumbai, India. He has completed his
fellowship in Pediatric Neurology with specialising in Epilepsy surgery workup from Hinduja
hospital under the guidance of Vrajesh Udani, top neurologist in India. He has also been trained
in Epilepsy & neurophysiology at Case Western Reserve University at Cleveland under the
guidance of Dr. Hans Luders.
He specialises in Clinical Neurophysiology (EEG, EMG and NCV). He also provides portable EEG
services in Navi Mumbai.
Currently, He is supporting many schools for children with special needs. He is attached to all
major hospitals in Navi Mumbai where he consults pediatric neurological cases. His areas of
expertise are
1. Epilepsy, Seizure disorders
2. Developmental Disorders including delayed speech, motor milestones, and coordination issues
3. Autism and other Behavioural disorders, including attention-deficit/hyperactivity disorder
(ADHD), school failure and sleep problems
4. Movement Disorders,
5. Cerebral palsy, muscular dystrophy, and nerve muscle disorders
6. Headaches, including migraines

28. Dr. Amit Vatkar
Pediatric Neurologist, Navi Mumbai
MBBS, DNB
Email: vatkaramit@yahoo.com
Contact No.: +91-8767844488
Visit us at: http://pediatricneurology.in/
THANK YOU
!