Dr. Amit VatkarMBBS, DCH, DNB Pediatrics

Fellow in Pediatric Neurology, MumbaiTrained in Neurophysiology & Epilepsy, USA

Contact No. : +91-8767844488Email: vatkaramit@yahoo.com

Epilepsy surgery in tuberous sclerosis

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

Other evaluations

• Ash leaf macules on back• Right sided retinal hamartoma noted in

fundus examination• USG – Bil simple renal cortical cysts• 2D ECHO- Normal

Inter-ictal EEG

RIGHT FRONTAL INTERICTALS DISCHARGES

Seizure onset

• RIGHT FRONTAL FP2 SEIZURE ONSET

Seizure termination

RIGHT FRONTAL SLOWING

Seizure termination 2

MULTIPLE TUBERS IN BILATEREAL FRONTAL LOBES

Ictal and Interictal SPECT 1

Ictal and Interictal SPECT 2

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

Surgery

• Right frontal craniotomy • Tuberous cortex identified with scalp EEG and

MRI.• Extent of lesion decided with the help of ECoG

RIGHT FRONTAL LOBE EXCISED

ECoG

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

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

New modalities

• SPECT/PET• AMT PET• Multi-stage surgery with invasive EEG monitoring • MEG

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

Calcified subependymal nodule

Subependymal giant cell astrocytoma

Radial glial fibres

Cortical tuber and sub ependymal nodules

Retinal tuber

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

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 disorders2. Developmental Disorders including delayed speech, motor milestones, and coordination issues3. Autism and other Behavioural disorders, including attention-deficit/hyperactivity disorder (ADHD), school failure and sleep problems4. Movement Disorders, 5. Cerebral palsy, muscular dystrophy, and nerve muscle disorders 6. Headaches, including migraines

Dr. Amit VatkarPediatric Neurologist, Navi Mumbai

MBBS, DNB

Email: vatkaramit@yahoo.comContact No.: +91-8767844488

Visit us at: http://pediatricneurology.in/

THANK YOU !