Startle epilepsy

Startle epilepsy is a rare neurologic disease characterized by frequent and spontaneous epileptic seizures (frequently with symmetrical or asymmetrical tonic features) triggered by a normal startle in response to a sudden and unexpected somatosensory (most frequently auditory) stimulus. Falls are common and can be traumatic. In most cases, the disease is associated with spastic hemi-, di-, or tetraplegia and intellectual disability.

Hemiplegia

Paralysis (complete loss of muscle function) in the arm, leg, and in some cases the face on one side of the body.


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PMID (PMCID)
19766495
 MALE Child
A hemispherotomy for intractable startle epilepsy characterized by infantile hemiplegia and drop attacks.
Anan M, Kamida T, Abe E, Kubo T, Abe T, Hikawa T, Fujiki M, Kobayashi H, Baba H.
J Clin Neurosci. 2009;16(12):1652-5.
A hemispherotomy for intractable startle epilepsy characterized by infantile hemiplegia and drop attacks.
19766495
 MALE Child
A hemispherotomy for intractable startle epilepsy characterized by infantile hemiplegia and drop attacks.
Anan M, Kamida T, Abe E, Kubo T, Abe T, Hikawa T, Fujiki M, Kobayashi H, Baba H.
J Clin Neurosci. 2009;16(12):1652-5.
We present a case of intractable startle epilepsy with infantile hemiplegia and discuss the indications for a hemispherotomy.
19809941
 FEMALE
Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome.
d'Orsi G, Demaio V, Operto F, Auricchio G, Minervini MG, Coppola G.
Neuropediatrics. 2009;40(2):97-100.
Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome.
19809941
 FEMALE
Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome.
d'Orsi G, Demaio V, Operto F, Auricchio G, Minervini MG, Coppola G.
Neuropediatrics. 2009;40(2):97-100.
The aim of this study was to investigate the video-polygraphic features and the long-term outcome of epilepsy in two patients with startle epilepsy associated with infantile hemiplegia (SEIH).
15324827
 MALE Child
Intractable reflex audiogenic epilepsy successfully treated by peri-insular hemispherotomy.
Martinez-Manas R, Daniel RT, Debatisse D, Maeder-Ingvar M, Meagher-Villemure K, Villemure JG, Deonna T.
Seizure. 2004;13(7):486-90.
We report a case of an infantile hemiplegia seizure syndrome (IHSS) that presented with intractable reflex audiogenic startle epilepsy which in itself is an uncommon form of seizure disorder.
9578019
 MALE Adult
Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery.
Oguni H, Hayashi K, Usui N, Osawa M, Shimizu H.
Epilepsia. 1998;39(1):93-8.
Startle epilepsy with infantile hemiplegia is a distinct epileptic syndrome characterized by structural brain damage restricted primarily to one hemisphere, large ipsilateral epileptogenic lesions involving the perisensorimotor area, refractory startle-provoked drop attacks, and a good response to epilepsy surgery.
9578019
 MALE Adult
Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery.
Oguni H, Hayashi K, Usui N, Osawa M, Shimizu H.
Epilepsia. 1998;39(1):93-8.
Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery.