What is stiff baby syndrome?

Updated: Jun 14, 2018
  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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The clinical presentation of stiff baby syndrome is somewhat different. Babies and young children are less rigid between attacks. Involvement of the distal muscles is often more evident, particularly during paroxysms. Opisthotonic posturing is more prominent. Startle or stress is a frequent and prominent precipitant of the attacks.

Its clinical characteristics are within a broader descriptive category known as hyperekplexia. Differentiation of a particular case as stiff baby syndrome sometimes is considered dependent upon the presence of anti-GAD antibodies. In addition, stiff baby syndrome may be more persistent or more frequently recurrent, although this is not invariable.

Diagnosis can also be more complex because other etiologies (eg, other neuromuscular disorders, seizures, withdrawal or intoxication from maternal drug abuse) need to be excluded.

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