Bilateral posterior agyria-pachygyria and epilepsy
Roberto Horacio Caraballo1, Ricardo Oscar Cersosimo, Alberto Espeche
1Servicio de Neurologia, Hospital de niñfos Juan P. Garrahan, Combate de los Pozos 1181, CP 1245, Buenos Aires, Argentina. rcaraballo@janssen.eom.ar
Brain & Development
|February 13, 2003
Summary
Bilateral posterior agyria-pachygyria is a rare brain malformation causing intellectual disability, motor deficits, and epilepsy. MRI is crucial for diagnosing this condition, characterized by specific EEG patterns.
Area of Science:
- Neuroscience
- Developmental Neuroscience
- Clinical Neurology
Background:
- Agyria-pachygyria is a severe neuronal migration disorder characterized by a lack of normal cortical folding.
- Posteriorly located agyria-pachygyria is less common and presents unique diagnostic challenges.
Observation:
- Two patients with bilateral posterior agyria-pachygyria exhibited intellectual disability, mild motor deficits, and epilepsy.
- Electroencephalography (EEG) revealed frequent generalized tonic/atonic seizures, occipital spikes, and posterior polyspike-wave paroxysms.
- Ictal EEG showed diffuse 10-11 Hz activity.
Findings:
- Cerebral magnetic resonance imaging (MRI) demonstrated thickened parieto-occipital cortices, reduced white matter volume, enlarged subarachnoid spaces, and dilated occipital horns.
- These imaging findings confirmed bilateral posterior agyria-pachygyria.
Implications:
- Bilateral posterior agyria-pachygyria should be considered in patients with epilepsy, intellectual disability, and specific EEG findings.
- MRI is the definitive imaging modality for diagnosing this cortical development disorder.
- Understanding these electroclinical features aids in early diagnosis and management.
More Related Videos
09:57Electromagnetic Source Imaging in Presurgical Evaluation of Children with Drug-Resistant Epilepsy
Published on: September 20, 2024
06:04Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
Published on: August 16, 2024
Related Concept Videos
01:24Epilepsy and Seizures: Overview
Epilepsy is a chronic neurological disease marked by recurrent, unpredictable seizures. These seizures are caused by abnormal electrical discharges in the brain, leading to behavior, sensation, or consciousness alterations. They can also cause transient impairment of awareness, interfering with daily activities.
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
01:13Seizures: Classification
Epilepsy is primarily characterized by unpredictable seizures, either provoked by an identifiable factor, such as injury or illness, or unprovoked, occurring spontaneously without apparent cause.
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
01:18Antiepileptic Drugs: GABAergic Pathway Potentiators
γ-aminobutyric acid or GABA, plays a pivotal role as an inhibitory neurotransmitter in the brain. GABA pathway potentiators, also known as GABAergic drugs, are a class of pharmaceutical agents designed to enhance the functioning of the GABAergic system. These medications primarily treat epilepsy, a neurological disorder characterized by recurrent seizures.
The key GABA pathway potentiators used in epilepsy management are as follows.
Benzodiazepines are a well-known class of drugs used for their...
The key GABA pathway potentiators used in epilepsy management are as follows.
Benzodiazepines are a well-known class of drugs used for their...
01:20Seizures l: Introduction
Understanding seizures and epilepsy relies on key definitions that help in recognizing, classifying, and managing these disorders. These definitions provide a framework for recognizing, classifying, and managing seizure disorders.DefinitionsA seizure is a sudden, abnormal burst of electrical activity in the brain that can cause changes in awareness, movement, sensation, or behavior, depending on the area involved. Epilepsy is a chronic condition characterized by recurrent, unprovoked seizures,...
01:19Seizures ll: Types
Seizures are sudden bursts of abnormal electrical discharge in the brain that interfere with normal function. They are commonly divided into three groups: focal seizures, generalized seizures, and other types that do not fit neatly into either category.Focal SeizuresFocal seizures begin in a single brain region. When awareness is preserved, they are called focal aware seizures and may cause sensations such as tingling, unusual smells, or flashing lights. When awareness is impaired, they are...
01:22Epilepsy ll: Types
Recurrent seizures, stemming from abnormal electrical activity in the brain, are the defining characteristic of epilepsy, a chronic neurological condition. Because seizure features vary greatly, epilepsy is classified using two systems: by seizure type and by epilepsy syndromes. These classifications enable clinicians to describe seizure patterns and select suitable treatment strategies.I. Classification by Seizure Type1. Focal EpilepsyFocal epilepsy begins in one hemisphere of the brain.