Abteilung Prächirurgische Epilepsiediagnostik – EpilepsiezentrumKlinik für Neurochirurgie

Treatment approaches for epilepsy due to hypothalamic hamartomas

Hypothalamic hamartomas are the basis for epilepsies manifesting with gelastic, cognitive, tonic and tonic-clonic seizures, often accompanied by an epileptic encephalopathy with behavioral and cognitive deterioration. Epilepsies are mostly unresponsive to currently available antiepileptic drugs.

At the Freiburg Epilepsy Center stereotactic and stimulation approaches to treat epilepsy patients with hypothalamic are studied in close collaboration with the Department of Stereotactic Neurosurgery. Treatments offered include

• interstitial radiosurgery using intrahypothalamic I-Seed implants 
• hypothalamic disconnection using stereotactic thermocoagulation
• deep brain stimulation of the anterior nuclei of the thalamus.

Presently ongoing patient series suggest that complete seizure control can bei achieved in >70% of patients with good tolerability of treatment approaches.

References

Cognition in epilepsy patients with hypothalamic hamartomas.
Wagner K, Wethe JV, Schulze-Bonhage A, Trippel M, Rekate H, Prigatano GP, Kerrigan JF.
Epilepsia. 2017 Jun;58 Suppl 2:85-93. doi: 10.1111/epi.13759.

Reduced glucose metabolism in neocortical network nodes remote from hypothalamic hamartomasreflects cognitive impairment.
Wagner K, Schulze-Bonhage A, Urbach H, Trippel M, Spehl TS, Buschmann F, Metternich B, Ofer I, Meyer PT, Frings L.
Epilepsia. 2017 Jun;58 Suppl 2:41-49. doi: 10.1111/epi.13757.

Ictal tachycardia in patients with hypothalamic hamartoma.
San Antonio-Arce V, Schulze-Bonhage A.
J Neurol Neurosurg Psychiatry. 2015 Oct;86(10):1163-4. doi: 10.1136/jnnp-2014-309681. Epub 2014 Dec 17

Memory outcome one year after stereotactic interstitial radiosurgery in patients with epilepsy due to hypothalamic hamartomas.
Wagner K, Buschmann F, Zentner J, Trippel M, Schulze-Bonhage A.
Epilepsy Behav. 2014 Aug;37:204-9. doi: 10.1016/j.yebeh.2014.06.031. Epub 2014 Jul 23.

Dacrystic seizures: demographic, semiologic, and etiologic insights from a multicenter study in long-term video-EEG monitoring units.
Blumberg J, Fernández IS, Vendrame M, Oehl B, Tatum WO, Schuele S, Alexopoulos AV, Poduri A, Kellinghaus C, Schulze-Bonhage A, Loddenkemper T.
Epilepsia. 2012 Oct;53(10):1810-9. doi: 10.1111/j.1528-1167.2012.03578.x. Epub 2012 Jul 10.

Semiologic aspects of epileptic seizures in 31 patients with hypothalamic hamartoma.
Oehl B, Brandt A, Fauser S, Bast T, Trippel M, Schulze-Bonhage A.
Epilepsia. 2010 Oct;51(10):2116-23. doi: 10.1111/j.1528-1167.2010.02686.x.

Mirthful gelastic seizures with ictal involvement of temporobasal regions.
Oehl B, Biethahn S, Schulze-Bonhage A.
Epileptic Disord. 2009 Mar;11(1):82-6. doi: 10.1684/epd.2009.0245. Epub 2009 Mar 20.

Outcome and predictors of interstitial radiosurgery in the treatment of gelastic epilepsy.
Schulze-Bonhage A, Trippel M, Wagner K, Bast T, Deimling FV, Ebner A, Elger C, Mayer T, Keimer R, Steinhoff BJ, Spreer J, Fauser S, Ostertag C.
Neurology. 2008 Jul 22;71(4):277-82. doi: 10.1212/01.wnl.0000318279.92233.82.

Treatment options for gelastic epilepsy due to hypothalamic hamartoma: interstitial radiosurgery.
Schulze-Bonhage A, Ostertag C.
Semin Pediatr Neurol. 2007 Jun;14(2):80-7.

Assessment of cognitive functions before and after stereotactic interstitial radiosurgery of hypothalamic hamartomas in patients with gelastic seizures.
Quiske A, Unterrainer J, Wagner K, Frings L, Breyer T, Halsband U, Ostertag C, Elger CE, Ebner A, Tuxhorn I, Ernst JP, Steinhoff BJ, Mayer T, Schulze-Bonhage A.
Epilepsy Behav. 2007 Mar;10(2):328-32. Epub 2007 Feb 20.

Cognitive functions in juvenile and adult patients with gelastic epilepsy due to hypothalamic hamartoma. Quiske A, Frings L, Wagner K, Unterrainer J, Schulze-Bonhage A.
Epilepsia. 2006 Jan;47(1):153-8.

Interstitial radiosurgery in the treatment of gelastic epilepsy due to hypothalamic hamartomas.
Schulze-Bonhage A, Homberg V, Trippel M, Keimer R, Elger CE, Warnke PC, Ostertag C.
Neurology. 2004 Feb 24;62(4):644-7.

Effect of interstitial stereotactic radiosurgery on behavior and subjective handicap of epilepsy in patients with gelastic epilepsy.
Schulze-Bonhage A, Quiske A, Homberg V, Trippel M, Wagner K, Frings L, Bast T, Huppertz HJ, Warnke C, Ostertag Ch.
Epilepsy Behav. 2004 Feb;5(1):94-101.