Journal of Pediatric Epilepsy 2016; 05(04): 182-185
DOI: 10.1055/s-0036-1584913
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Symptomatic Case of Startle Epilepsy with Infantile Brain Damage Treated with Hemispherotomy: A Case Report and Review of Literature

Yeeshu Singh
1   Department of Neurosciences, Medanta – The Medicity, Gurgaon, Haryana, India
Atma Ram Bansal
1   Department of Neurosciences, Medanta – The Medicity, Gurgaon, Haryana, India
Aditya Gupta
1   Department of Neurosciences, Medanta – The Medicity, Gurgaon, Haryana, India
Arun Garg
1   Department of Neurosciences, Medanta – The Medicity, Gurgaon, Haryana, India
› Author Affiliations
Further Information

Publication History

02 March 2015

15 February 2016

Publication Date:
11 July 2016 (online)


We are reporting a case of rare reflex epilepsy—“startle” epilepsy effectively treated with hemispherotomy. A 6-year-old boy, a child of nonconsanguineous parentage with infantile hemiparesis presented to us with recurrent right focal seizures along with secondary generalization provoked by sudden and unexpected auditory stimuli for the last 1 year. The boy had a minimum of four episodes per day despite maximal medical management with multiple antiepileptic drugs. Magnetic resonance imaging of the brain showed severe atrophy of the whole of the left hemisphere. Interictal electroencelphographic (EEG) data showed predominantly left central epileptiform discharges. Long-term video-EEG monitoring record showed seizures of the left hemispheric origin. He underwent a left functional hemispherotomy and has been totally seizure free in the 12-month follow-up period with an improved quality of life both for him and for his caregivers. We feel that surgical management should be considered in startle epilepsy with infantile hemiparesis when seizures are refractory to the optimal medical treatment.


Written informed consent was taken from the parents.

  • References

  • 1 Gowers WR. Epilepsy and other chronic convulsions. London: Churchill Livingstone; 1901
  • 2 Alajouanine T, Gastaut H. La syncinhie-sursaut et l'epilepsie- sursaut a dkclanchement sensoriel ou sensity inopin6. Rev Neurol 1955; 93: 29-41
  • 3 Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989; 30 (4) 389-399
  • 4 Panayiotopoulos CP. Reflex seizures and related epileptic syndromes: startle seizures. In: Panayiotopoulos CP. ed. Clinical Guide to Epileptic Syndromes and Their Treatment. 2nd ed. Berlin: Springer; 2010: 524-526
  • 5 Yang Z, Liu X, Qin J , et al. Clinical and electrophysiological characteristics of startle epilepsy in childhood. Clin Neurophysiol 2010; 121 (5) 658-664
  • 6 Tibussek D, Wohlrab G, Boltshauser E, Schmitt B. Proven startle-provoked epileptic seizures in childhood: semiologic and electrophysiologic variability. Epilepsia 2006; 47 (6) 1050-1058
  • 7 Guerrini R, Genton P, Bureau M, Dravet C, Roger J. Reflex seizures are frequent in patients with Down syndrome and epilepsy. Epilepsia 1990; 31 (4) 406-417
  • 8 Chauvel P, Trottier S, Vignal JP, Bancaud J. Somatomotor seizures of frontal lobe origin. Adv Neurol 1992; 57: 185-232
  • 9 Vignal JP, Biraben A, Chauvel PY, Reutens DC. Reflex partial seizures of sensorimotor cortex (including cortical reflex myoclonus and startle epilepsy). In: Zifkin BG, Andermann F, Beaumanoir A, Rowan AJ, eds. Reflex Epilepsies and Reflex Seizures: Advances in Neurology. Vol. 75. Philadelphia, PA: Lippincott-Raven; 1998: 207-226
  • 10 Manford MR, Fish DR, Shorvon SD. Startle provoked epileptic seizures: features in 19 patients. J Neurol Neurosurg Psychiatry 1996; 61 (2) 151-156
  • 11 Oguni H, Hayashi K, Usui N, Osawa M, Shimizu H. Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery. Epilepsia 1998; 39 (1) 93-98
  • 12 Halgren E, Baudena P, Clarke JM , et al. Intracerebral potentials to rare target and distractor auditory and visual stimuli. I. Superior temporal plane and parietal lobe. Electroencephalogr Clin Neurophysiol 1995; 94 (3) 191-220
  • 13 Caraballo R, Semprino M, Cersósimo R, Sologuestua A, Arroyo HA, Fejerman N. Hemiparetic cerebral palsy and startle epilepsy [in Spanish]. Rev Neurol 2004; 38 (2) 123-127
  • 14 Martinez-Mañas R, Daniel RT, Debatisse D , et al. Intractable reflex audiogenic epilepsy successfully treated by peri-insular hemispherotomy. Seizure 2004; 13 (7) 486-490
  • 15 Tinuper P, Andermann F, Villemure JG, Rasmussen TB, Quesney LF. Functional hemispherectomy for treatment of epilepsy associated with hemiplegia: rationale, indications, results, and comparison with callosotomy. Ann Neurol 1988; 24 (1) 27-34
  • 16 Gómez NG, Hamad AP, Marinho M , et al. Corpus callosotomy in a patient with startle epilepsy. Epileptic Disord 2013; 15 (1) 76-79
  • 17 Job AS, De Palma L, Principe A , et al. The pivotal role of the supplementary motor area in startle epilepsy as demonstrated by SEEG epileptogenicity maps. Epilepsia 2014; 55 (8) e85-e88
  • 18 Klinkenberg S, Ubbink S, Vles J , et al. Noninvasive treatment alternative for intractable startle epilepsy. Epilepsy Behav Case Rep 2014; 2: 49-53