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Diseases

Genetic and Rare Diseases Information Center (GARD)

Other Names for this Disease
  • GSM 1
  • Hereditary chin tremor/myoclonus
  • Hereditary geniospasm
  • Trembling chin
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Your Question

Myself and both my children suffer from geniospasms. Is it common for a parent and all their children to have geniospasms? What is the most effective treatment for geniospasms?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How is hereditary geniospasm inherited?

Hereditary geniospasm is inherited in an autosomal dominant manner.[1] This means that having only one mutated copy of the causative gene in each body cell is sufficient to cause signs and symptoms of the condition. When an individual with an autosomal dominant condition has children, each child has a 50% (1 in 2) chance to inherit the mutated copy of the gene and also be affected. Because there is a 50% chance for each child, it is possible for all of the children of an affected individual to be affected, or likewise, for all of the children to be unaffected.
Last updated: 6/5/2013

How might hereditary geniospasm be treated?

Hereditary geniospasm, which may also be referred to as hereditary essential chin myoclonus, is generally considered a benign disorder although in some cases it can cause anxiety and social embarrassment.[2] Significant improvement with age has been reported.[1] Several drugs are used to treat myoclonus, such as benzodiazepines and anticonvulsants. However, individuals may not respond to a single medication and may experience significant side effects if a combination of drugs is used. It has also been suggested that botulinum toxin be considered as a primary treatment because it has been shown to be effective and well tolerated.[2]
Last updated: 6/5/2013

References
  • Hereditary geniospasm. Orphanet. April 2009; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=53372. Accessed 6/5/2013.
  • Devetag Chalaupka F, Bartholini F, Mandich G, Turro M. Two new families with hereditary essential chin myoclonus: clinical features, neurophysiological findings and treatment. Neurol Sci. June 2006; 27(2):97-103.