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Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this...
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The somatosensory cortex in the parietal lobes is crucial for interpreting sensory data such as touch, temperature, and proprioception. The somatosensory cortex, situated in the parietal lobes, plays a vital role in interpreting sensory information like touch, temperature, and proprioception—awareness of body position. This specialized brain region features an organized structure wherein neurons at the top primarily process sensations originating from the lower body. In contrast, those at...
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Association areas are regions of the cerebral cortex that do not have a specific sensory or motor function. Instead, they integrate and interpret information from various sources to enable higher cognitive processes such as memory, learning, and decision-making. Some key association areas include the following:
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Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...
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The cerebral cortex, the brain's outermost layer, is pivotal in processing complex cognitive tasks, emotions, and various sensory inputs and executing voluntary motor activities. This intricate structure is divided into three primary functional areas: the motor areas, sensory areas, and association areas.
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Cerebral cortex involvement in Machado-Joseph disease.

T J R de Rezende1, A D'Abreu, R P Guimarães

  • 1Department of Neurology, University of Campinas (UNICAMP), Campinas, Brazil; Department of Cosmic Rays and Chronology, University of Campinas (UNICAMP), Campinas, Brazil.

European Journal of Neurology
|September 25, 2014
PubMed
Summary
This summary is machine-generated.

Machado-Joseph disease (MJD/SCA3) causes widespread brain atrophy, affecting both the cortex and subcortical areas. These structural changes correlate with the disease's clinical symptoms, impacting motor and cognitive functions.

Keywords:
FreeSurferMachado−Joseph diseasecerebral cortexcortical thicknessneuropsychological tests

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Area of Science:

  • Neuroscience
  • Radiology
  • Genetics

Background:

  • Machado-Joseph disease (MJD/SCA3) is the most common spinocerebellar ataxia, leading to damage in the brainstem, basal ganglia, and cerebellum.
  • Cerebral cortex involvement in MJD/SCA3 is less understood, with limited MRI-based studies.

Purpose of the Study:

  • To investigate cerebral cortex atrophy in MJD/SCA3 patients using MRI.
  • To identify specific cortical regions vulnerable to damage.
  • To explore correlations between brain atrophy and clinical/neuropsychological data.

Main Methods:

  • MRI scans (3T) including 3D T1 images were acquired from 49 MJD/SCA3 patients and 49 controls.
  • Cortical thickness and volume were analyzed using FreeSurfer software.
  • Statistical analyses (ANCOVA, GLM) compared groups and assessed correlations with clinical variables.

Main Results:

  • MJD/SCA3 patients exhibited bilateral paracentral cortex atrophy, alongside damage in the caudal middle frontal, superior/transverse temporal, and lateral occipital gyri (left), and supramarginal gyrus (right).
  • Significant volumetric reduction was observed in basal ganglia and hippocampi.
  • Atrophy in the brainstem and precentral gyrus correlated with the Scale for Assessment and Rating of Ataxia (SARA) score.

Conclusions:

  • Machado-Joseph disease (MJD/SCA3) is associated with extensive cortical and subcortical atrophy.
  • Structural brain damage in MJD/SCA3 is linked to clinical manifestations, supporting a relationship between cognitive/motor impairment and cerebral lesions.