We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Article |

Hyperexplexia: Not Hereditary Stiff-Baby Syndrome

Am J Dis Child. 1982;136(6):562. doi:10.1001/archpedi.1982.03970420086029.
Text Size: A A A
Published online

Sir.—In the article "Hereditary Stiff-Baby Syndrome" (Journal 1981; 135:909-911), Lingam et al described a family with an autosomal dominant disorder characterized by infantile stiffening and an abnormal startle response. Lingam and colleagues seem to have reported a previously described condition known as hyperexplexia or Kok's disease.1,2

In hyperexplexia, as in the family described by Lingam and associates, hypertonia in the affected neonate leads to hypokinesis and a "fetal position." The hypertonia lessens or disappears during sleep and with holding of the infant, and it completely disappears by 2 years of age. Sitting, walking, and other gross motor milestones are delayed because of the hypertonia. In addition, affected persons during infancy and throughout life have an exaggerated startle reaction that occasionally may produce a generalized hypertonic response and falling. These persons, while falling, have no loss of consciousness nor do they make any attempt to protect themselves from the


Sign in

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview





Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment


Some tools below are only available to our subscribers or users with an online account.

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.