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Dandy-Walker syndrome presenting as opisthotonus: proposed pathophysiology
1Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
Pediatric Neurology
|February 1, 1996
Summary
This study reports a rare case of Dandy-Walker syndrome presenting with opisthotonus. Combination therapy with baclofen and diazepam proved most effective in managing this neurological manifestation.
Area of Science:
- Neurology
- Developmental Neuroscience
Background:
- Dandy-Walker syndrome is a congenital brain malformation.
- Opisthotonus, characterized by severe extensor spasms, is a rare manifestation in Dandy-Walker syndrome.
Observation:
- A patient with radiographically confirmed Dandy-Walker syndrome presented with opisthotonus.
- This clinical presentation is infrequently documented in existing literature.
Findings:
- Four pharmacologic trials were conducted: baclofen, diazepam, levodopa/carbidopa, and trihexyphenidyl.
- Combination therapy of baclofen and diazepam demonstrated the highest efficacy in alleviating opisthotonus and extensor posturing.
Implications:
- The findings suggest a potential therapeutic strategy for managing opisthotonus in Dandy-Walker syndrome.
- Further research is needed to elucidate the specific mechanisms underlying opisthotonus in this condition.