Related Experiment Videos
Bilateral frontal polymicrogyria: a newly recognized brain malformation syndrome
R Guerrini1, A J Barkovich, L Sztriha
1Division of Neurology, King's College, University of London, UK. R.Guerrini@iop.kcl.ac.uk
Neurology
|February 26, 2000
Summary
This study identifies bilateral frontal polymicrogyria (BFP), a rare brain malformation affecting both frontal lobes. BFP presents with developmental delay and spasticity, expanding the known spectrum of polymicrogyria syndromes.
Area of Science:
- Neuroscience
- Developmental Biology
- Genetics
Background:
- Polymicrogyria is a brain malformation with abnormal cortical development.
- Bilateral perisylvian and parasagittal parieto-occipital polymicrogyria are known subtypes.
- This study focuses on a distinct bilateral frontal polymicrogyria (BFP).
Purpose of the Study:
- To describe the clinical and neuroimaging features of 13 patients with bilateral frontal polymicrogyria (BFP).
- To characterize the spectrum of BFP and its associated neurological deficits.
- To expand the understanding of regional polymicrogyria syndromes.
Main Methods:
- Retrospective review of clinical data, brain MRI, and EEG for 13 patients.
- Correlation of BFP findings with other regional polymicrogyria presentations.
- Analysis of clinical presentation, including developmental delay, motor, language, cognitive, and seizure disorders.
Main Results:
- Identified 13 patients with symmetric polymicrogyria affecting the frontal lobes.
- All patients exhibited developmental delay and mild spastic quadriparesis.
- Commonly associated features included impaired language (12/13), mental retardation (11/13), and epilepsy (5/13).
- BFP was sporadic in all cases, with two families reporting consanguinity.
Conclusions:
- Bilateral frontal polymicrogyria (BFP) represents a distinct clinical entity.
- BFP expands the spectrum of recognized bilateral symmetric regional polymicrogyria syndromes.
- Further research into the genetic and etiological factors of BFP is warranted.