Other Names for this Disease
- Startle disease, familial
- Startle reaction, exaggerated
- Exaggerated startle reaction
- Stiff-baby syndrome
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Hyperekplexia is commonly treated with the anti-anxiety and anti-spastic drug clonazepam. Other drugs which have shown variable results (mostly in case studies) include carbamazepine, phenytoin, diazepam, valproate, 5-hydroxytryptophan, piracetam, phenobarbital, and fluoxetine. In some cases, a combination of treatments (i.e. clobazam and clonazepam) may be needed. Physical and cognitive therapy may help reduce anxiety as a supportive measure, which may translate into improved ability to walk.
Last updated: 11/22/2015
- Haruno L. Hyperekplexia. National Organization for Rare Disorders (NORD). 2013; http://rarediseases.org/rare-diseases/hyperekplexia/. Accessed 11/22/2015.
- Tijssen MAJ, Rees MI. Hyperekplexia. GeneReviews. October 2012; http://www.ncbi.nlm.nih.gov/books/NBK1260/. Accessed 11/22/2015.
- Mineyko A, Whiting S, Graham GE. Hyperekplexia: treatment of a severe phenotype and review of the literature. Can J Neurol Sci. 2011 May; 38(3):411-6. http://www.ncbi.nlm.nih.gov/pubmed/21515498. Accessed 11/22/2015.
- McAbee GN. Clobazam-clonazepam combination effective for stimulus-induced falling in hyperekplexia. J Child Neurol. 2015 Jan; 30(1):91-2. http://www.ncbi.nlm.nih.gov/pubmed/24453146. Accessed 11/22/2015.
- Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.
- GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
- ClinicalTrials.gov lists trials that are studying or have studied Hereditary hyperekplexia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.