Sporadic hyperekplexia due to self-limiting brainstem encephalopathy

Dilek Yilmaz,1 Bülent Cengiz2 1Department of Neurology, Konya Numune State Hospital, Konya, 2Department of Neurology, Gazi University Faculty of Medicine, Ankara, Turkey Abstract: Hyperekplexia is a rare movement disorder characterized by pathologically exaggerated response to unexpected...

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Autores principales:	Yilmaz D, Cengiz B
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2017
Materias:	Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/89da4510dba846afab6bec60b32e9cda
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spelling	oai:doaj.org-article:89da4510dba846afab6bec60b32e9cda2021-12-02T04:47:22ZSporadic hyperekplexia due to self-limiting brainstem encephalopathy1178-2021https://doaj.org/article/89da4510dba846afab6bec60b32e9cda2017-10-01T00:00:00Zhttps://www.dovepress.com/sporadic-hyperekplexia-due-to-self-limiting-brainstem-encephalopathy-peer-reviewed-article-NDThttps://doaj.org/toc/1178-2021Dilek Yilmaz,1 Bülent Cengiz2 1Department of Neurology, Konya Numune State Hospital, Konya, 2Department of Neurology, Gazi University Faculty of Medicine, Ankara, Turkey Abstract: Hyperekplexia is a rare movement disorder characterized by pathologically exaggerated response to unexpected stimuli. It is differentiated from the normal startle reflex by its lower threshold, higher intensity, and resistance to habituation. Many of the acquired hyperekplexias result from brainstem involvement such as encephalitis, infarct, hemorrhage, pontocerebellar hypoplasia and medullary compression. This case report depicts a rare manifestation of hyperekplexia. The unusual aspect of this case was the vocalization that was reproduced in response to startling stimuli. Startle induced vocalization is not a part of the classical hyperekplexia description. When faced with a patient with pathologically exaggerated response to unexpected stimuli, the physician should consider this rare condition in the differential diagnosis, and also keep in mind that the disease may present with features different from those listed in the textbooks. Keywords: hyperekplexia, startle, brainstem encephalopathyYilmaz DCengiz BDove Medical PressarticleNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571Neurology. Diseases of the nervous systemRC346-429ENNeuropsychiatric Disease and Treatment, Vol Volume 13, Pp 2581-2584 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
spellingShingle	Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429 Yilmaz D Cengiz B Sporadic hyperekplexia due to self-limiting brainstem encephalopathy
description	Dilek Yilmaz,1 Bülent Cengiz2 1Department of Neurology, Konya Numune State Hospital, Konya, 2Department of Neurology, Gazi University Faculty of Medicine, Ankara, Turkey Abstract: Hyperekplexia is a rare movement disorder characterized by pathologically exaggerated response to unexpected stimuli. It is differentiated from the normal startle reflex by its lower threshold, higher intensity, and resistance to habituation. Many of the acquired hyperekplexias result from brainstem involvement such as encephalitis, infarct, hemorrhage, pontocerebellar hypoplasia and medullary compression. This case report depicts a rare manifestation of hyperekplexia. The unusual aspect of this case was the vocalization that was reproduced in response to startling stimuli. Startle induced vocalization is not a part of the classical hyperekplexia description. When faced with a patient with pathologically exaggerated response to unexpected stimuli, the physician should consider this rare condition in the differential diagnosis, and also keep in mind that the disease may present with features different from those listed in the textbooks. Keywords: hyperekplexia, startle, brainstem encephalopathy
format	article
author	Yilmaz D Cengiz B
author_facet	Yilmaz D Cengiz B
author_sort	Yilmaz D
title	Sporadic hyperekplexia due to self-limiting brainstem encephalopathy
title_short	Sporadic hyperekplexia due to self-limiting brainstem encephalopathy
title_full	Sporadic hyperekplexia due to self-limiting brainstem encephalopathy
title_fullStr	Sporadic hyperekplexia due to self-limiting brainstem encephalopathy
title_full_unstemmed	Sporadic hyperekplexia due to self-limiting brainstem encephalopathy
title_sort	sporadic hyperekplexia due to self-limiting brainstem encephalopathy
publisher	Dove Medical Press
publishDate	2017
url	https://doaj.org/article/89da4510dba846afab6bec60b32e9cda
work_keys_str_mv	AT yilmazd sporadichyperekplexiaduetoselflimitingbrainstemencephalopathy AT cengizb sporadichyperekplexiaduetoselflimitingbrainstemencephalopathy
_version_	1718401020256583680

Sporadic hyperekplexia due to self-limiting brainstem encephalopathy

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