The startle reflex is a motor response that originates in the lower brainstem.1 Abnormal symptomatic startle can be secondary to lesions in the startle pathway, involving brainstem and spinal cord.2 A 56-year-old woman developed an acute demyelinating lesion of unknown origin in medulla oblongata (figure, A–D), causing dizziness and bilateral sensory impairment with paresthesias. No tongue weakness, myoclonus, or symptoms of restless leg syndrome were present. When the symptoms remitted, she developed a severe symptomatic startle response. Pathologic startle was elicited by sensory—especially acoustic—stimuli (video, see the Neurology Web site at www.neurology.org). Startle was bilateral and the EMG burst duration, recorded with surface deltoid EMG, ranged from 500 to 1,200 msec. Startle was not responsive to pharmacologic treatment (benzodiazepines and carbamazepine) and was disabling for the patient.
Figure. MRI scans. An area of abnormal signal, probably of demyelinating origin, is evident in the upper medulla and pons. No other signal abnormalities are evident within the CNS; in particular, the cervical cord is spared (B).
Footnotes
-
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the February 6 issue to find the title link for this article.
Disclosure: The authors report no conflicts of interest.
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Hastening the Diagnosis of Amyotrophic Lateral Sclerosis
Dr. Brian Callaghan and Dr. Kellen Quigg