Dandy-Walker syndrome presenting as opisthotonus: proposed pathophysiology.
A patient with radiographically confirmed Dandy-Walker syndrome who presented with opisthotonus, a rarely reported clinical manifestation, is reported. From four separate pharmacologic trials (baclofen, diazepam, levodopa/carbidopa, and trihexyphenidyl), combination baclofen and diazepam therapy was determined to be most efficacious. Opisthotonus and extensor posturing remain only rudimentarily understood. We review the subject and propose a specific mechanism relating our patient's anatomic and physiologic conditions.
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