Overview
Bilateral parasagittal parieto-occipital polymicrogyria (BPP) is a rare brain malformation characterized by an excessive number of abnormally small folds (gyri) on the surface of the brain, specifically affecting the parieto-occipital regions on both sides near the midline (parasagittal area). This condition falls within the broader group of polymicrogyria syndromes, which are cortical malformation disorders classified under ICD-10 code Q04.3 (other reduction deformities of brain). The abnormal cortical development primarily affects the posterior regions of the cerebral hemispheres, which are involved in visual processing, spatial awareness, and sensorimotor integration. Clinically, individuals with bilateral parasagittal parieto-occipital polymicrogyria may present with seizures, intellectual disability of variable severity, and specific cognitive deficits. Visual processing difficulties and visuospatial impairments may be prominent given the posterior brain regions involved. Motor difficulties, including spasticity or motor developmental delays, can also occur. The severity of symptoms can vary considerably between affected individuals, ranging from mild learning difficulties to more significant neurological impairment. Seizures, when present, may begin in childhood and can be of various types. The diagnosis is typically established through brain magnetic resonance imaging (MRI), which reveals the characteristic pattern of polymicrogyria in the parasagittal parieto-occipital regions bilaterally. There is currently no cure for this condition, and treatment is symptomatic and supportive. Management may include antiepileptic medications for seizure control, physical therapy, occupational therapy, speech therapy, and educational support tailored to the individual's specific cognitive and developmental needs. The underlying genetic causes remain incompletely understood, and both genetic and non-genetic (such as prenatal vascular or infectious) etiologies have been proposed for polymicrogyria syndromes in general.
Clinical phenotype terms— hover any for plain English:
Variable
Can be inherited in different ways depending on the underlying gene
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Bilateral parasagittal parieto-occipital polymicrogyria.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
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Common questions about Bilateral parasagittal parieto-occipital polymicrogyria
What is Bilateral parasagittal parieto-occipital polymicrogyria?
Bilateral parasagittal parieto-occipital polymicrogyria (BPP) is a rare brain malformation characterized by an excessive number of abnormally small folds (gyri) on the surface of the brain, specifically affecting the parieto-occipital regions on both sides near the midline (parasagittal area). This condition falls within the broader group of polymicrogyria syndromes, which are cortical malformation disorders classified under ICD-10 code Q04.3 (other reduction deformities of brain). The abnormal cortical development primarily affects the posterior regions of the cerebral hemispheres, which are
At what age does Bilateral parasagittal parieto-occipital polymicrogyria typically begin?
Typical onset of Bilateral parasagittal parieto-occipital polymicrogyria is childhood. Age of onset can vary across affected individuals.