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 ......
Images in Neurology |

Thalamic Changes in Tay-Sachs' Disease

Suvasini Sharma, MD; Naveen Sankhyan, MD; Veena Kalra, MD; Ajay Garg, MD, DM
Arch Neurol. 2008;65(12):1669. doi:10.1001/archneur.65.12.1669.
Text Size: A A A
Published online


A 1-year-old girl was brought in for evaluation of her developmental delay and regression of achieved milestones. Born full-term, she was the first child of a nonconsanguineous couple. The antenatal and perinatal history was unremarkable. She achieved social smile at 4 months and neck control at 6 months. She had just begun to sit with support at 9 months of age, when her parents noticed an exaggerated startle response to noise. Subsequently, she became listless and irritable and lost her sitting ability and neck control. She also developed reduced eye contact. Examination revealed a well, thriving infant with a weight of 9 kg, a length of 75 cm, and a head circumference of 47.5 cm. Facies were normal. Central nervous system examination revealed reduced alertness; absent menace response; reduced tone in all 4 limbs; brisk, deep tendon reflexes; and extensor plantar response bilaterally. Fundus examination showed bilateral cherry-red macular spots. Results of a systemic examination were normal.

Figures in this Article

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption

Thalamic changes in Tay-Sachs' disease. Axial T1-weighted (A) and T2-weighted (B) images at the level of the thalami show diffuse dysmyelination of hemispheric white matter with bilaterally symmetric thalamic signal changes, which appear hyperintense on T1-weighted and hypointense on T2-weighted images. Globus pallidi and caudate nuclei appear swollen and have a mixed low and high signal intensity on the T1- weighted image.

Graphic Jump Location




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.


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

1 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

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

Articles Related By Topic
PubMed Articles