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Startle-induced seizure

MedGen UID:: 1709409
•Concept ID:: C5397592
•: Disease or Syndrome

HPO:	HP:0020214

Definition

Startle-induced seizures are triggered by multiple and non-specific stimuli (auditory, somatosensory, and rarely visual) and are characterized by their sudden unexpected nature. Sudden noise rather than pure sound is the most effective acoustic stimulus. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVStartle-induced seizure

Fetal anomaly
- Congenital Systemic Disorder
  - Abnormality of the nervous system
    - Abnormal nervous system physiology
      - Seizure
        Reflex seizure
        Startle-induced seizure

Professional guidelines

PubMed

The startle syndromes: physiology and treatment.

Dreissen YE, Tijssen MA
Epilepsia 2012 Dec;53 Suppl 7:3-11. doi: 10.1111/j.1528-1167.2012.03709.x. PMID: 23153204

Benzodiazepines in the treatment of children with epilepsy.

Farrell K
Epilepsia 1986;27 Suppl 1:S45-52. doi: 10.1111/j.1528-1157.1986.tb05733.x. PMID: 3743524

See all (2)

Recent clinical studies

Etiology

The startle syndromes: physiology and treatment.

Dreissen YE, Tijssen MA
Epilepsia 2012 Dec;53 Suppl 7:3-11. doi: 10.1111/j.1528-1167.2012.03709.x. PMID: 23153204

Lamotrigine is favourable for startle-induced seizures.

Ikeda H, Imai K, Ikeda H, Shigematsu H, Shishido T, Takayama R, Fujiwara T, Takahashi Y, Inoue Y
Epileptic Disord 2011 Sep;13(3):277-83. doi: 10.1684/epd.2011.0458. PMID: 21896426

The movement disorders of Coffin-Lowry syndrome.

Stephenson JB, Hoffman MC, Russell AJ, Falconer J, Beach RC, Tolmie JL, McWilliam RC, Zuberi SM
Brain Dev 2005 Mar;27(2):108-13. doi: 10.1016/j.braindev.2003.11.010. PMID: 15668049

Reflex seizures are frequent in patients with Down syndrome and epilepsy.

Guerrini R, Genton P, Bureau M, Dravet C, Roger J
Epilepsia 1990 Jul-Aug;31(4):406-17. doi: 10.1111/j.1528-1157.1990.tb05496.x. PMID: 2142449

See all (4)

Diagnosis

Interstitial 6q deletion in a patient presenting with drug-resistant epilepsy and Prader-Willi like phenotype: An electroclinical description with literature review.

Cutillo G, Bonacchi R, Cecchetti G, Bellini A, Vabanesi M, Zambon A, Natali Sora MG, Baldoli C, Del Carro U, Minicucci F, Fanelli GF, Filippi M
Seizure 2023 Jul;109:45-49. Epub 2023 May 14 doi: 10.1016/j.seizure.2023.05.011. PMID: 37210930

Reflex epileptic features in patients with focal epilepsy of unknown cause.

Atalar AÇ, Vanlı-Yavuz EN, Yılmaz E, Bebek N, Baykan B
Clin Neurol Neurosurg 2020 Mar;190:105633. Epub 2019 Dec 9 doi: 10.1016/j.clineuro.2019.105633. PMID: 31865219

The startle syndromes: physiology and treatment.

Dreissen YE, Tijssen MA
Epilepsia 2012 Dec;53 Suppl 7:3-11. doi: 10.1111/j.1528-1167.2012.03709.x. PMID: 23153204

Startle epilepsy associated with gait-induced seizures: Pathomechanism analysis using EEG, MEG, and PET studies.

Saeki K, Saito Y, Sugai K, Nakagawa E, Komaki H, Sakuma H, Sasaki M, Kaneko Y
Epilepsia 2009 May;50(5):1274-9. Epub 2008 Dec 20 doi: 10.1111/j.1528-1167.2008.01890.x. PMID: 19400869

Startle epilepsy: a clinical study.

Sáenz-Lope E, Herranz FJ, Masdeu JC
Ann Neurol 1984 Jul;16(1):78-81. doi: 10.1002/ana.410160115. PMID: 6465864

See all (9)

Therapy

Reflex epileptic features in patients with focal epilepsy of unknown cause.

Atalar AÇ, Vanlı-Yavuz EN, Yılmaz E, Bebek N, Baykan B
Clin Neurol Neurosurg 2020 Mar;190:105633. Epub 2019 Dec 9 doi: 10.1016/j.clineuro.2019.105633. PMID: 31865219

Lamotrigine is favourable for startle-induced seizures.

Ikeda H, Imai K, Ikeda H, Shigematsu H, Shishido T, Takayama R, Fujiwara T, Takahashi Y, Inoue Y
Epileptic Disord 2011 Sep;13(3):277-83. doi: 10.1684/epd.2011.0458. PMID: 21896426

Startle-induced epileptic seizures.

Aguglia U, Tinuper P, Gastaut H
Epilepsia 1984 Dec;25(6):712-20. doi: 10.1111/j.1528-1157.1984.tb03482.x. PMID: 6510379

Startle epilepsy: a clinical study.

Sáenz-Lope E, Herranz FJ, Masdeu JC
Ann Neurol 1984 Jul;16(1):78-81. doi: 10.1002/ana.410160115. PMID: 6465864

Effectiveness of clonazepan in startle-induced seizures.

Giménez-Roldán S, Martín M
Epilepsia 1979 Oct;20(5):555-61. doi: 10.1111/j.1528-1157.1979.tb04838.x. PMID: 477644

See all (11)

Prognosis

Lamotrigine is favourable for startle-induced seizures.

Ikeda H, Imai K, Ikeda H, Shigematsu H, Shishido T, Takayama R, Fujiwara T, Takahashi Y, Inoue Y
Epileptic Disord 2011 Sep;13(3):277-83. doi: 10.1684/epd.2011.0458. PMID: 21896426

See all (1)

Clinical prediction guides

Lamotrigine is favourable for startle-induced seizures.

Ikeda H, Imai K, Ikeda H, Shigematsu H, Shishido T, Takayama R, Fujiwara T, Takahashi Y, Inoue Y
Epileptic Disord 2011 Sep;13(3):277-83. doi: 10.1684/epd.2011.0458. PMID: 21896426

Lamotrigine for startle-induced seizures.

Faught E
Seizure 1999 Sep;8(6):361-3. doi: 10.1053/seiz.1999.0297. PMID: 10512780

Startle-induced epilepsy in a patient with epidermal nevus syndrome.

Sasaki M, Matsuda H, Arai Y, Hashimoto T
Pediatr Neurol 1998 Apr;18(4):346-9. doi: 10.1016/s0887-8994(97)00191-4. PMID: 9588533

Benzodiazepines in the treatment of children with epilepsy.

Farrell K
Epilepsia 1986;27 Suppl 1:S45-52. doi: 10.1111/j.1528-1157.1986.tb05733.x. PMID: 3743524

Use of clobazam in certain forms of status epilepticus and in startle-induced epileptic seizures.

Tinuper P, Aguglia U, Gastaut H
Epilepsia 1986;27 Suppl 1:S18-26. doi: 10.1111/j.1528-1157.1986.tb05729.x. PMID: 3743522

See all (5)