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[Benign paroxysmal tonic upward gaze syndrome]
V L Ruggieri1, I I Yépez, N Fejerman
1Hospital Nacional de Pediatría Prof. Juan P. Garrahan, Buenos Aires, Argentina.
Revista De Neurologia
|July 23, 1998
Summary
Benign paroxysmal tonic upward gaze syndrome (BPTUG) is a rare, non-epileptic condition presenting in infancy. Episodes typically resolve by age four, but neurological follow-up is advised.
Area of Science:
- Neurology
- Ophthalmology
- Pediatrics
Background:
- Benign paroxysmal tonic upward gaze syndrome (BPTUG) is a rare disorder.
- This study details two new pediatric cases, examining clinical, genetic, and therapeutic aspects.
Observation:
- Two young girls presented with recurrent episodes of upward gaze deviation, neck hyperextension, and vertical nystagmus.
- Symptoms were exacerbated by nervousness and fever; one patient experienced falls without loss of consciousness.
- Slow motor development was noted in one case.
Findings:
- Both patients had normal complementary studies and no family history of similar disorders.
- Unlike some conditions, BPTUG is not responsive to L-Dopa treatment.
- BPTUG typically begins in infancy, follows a benign course, and resolves by age four.
Implications:
- Differentiating BPTUG from epileptic phenomena is crucial for accurate diagnosis and management.
- Monitoring for potential later neurological complications is advised, despite the generally benign course.
- BPTUG may represent the clinical manifestation of diverse underlying conditions.