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Cerebrum: Anatomical Overview II01:11

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Each cerebral hemisphere can be divided into three main regions. The outermost region, the cerebral cortex, is a thin layer (2 to 4 millimeters thick) made up of gray matter, consisting of neuron cell bodies, dendrites, glial cells, and blood vessels. The middle region, or white matter, is primarily composed of myelinated nerve fibers organized into three types of large tracts: association fibers, commissures, and projection fibers. Association fibers connect different areas within the same...
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The cerebellum, also known as the "little brain," is located in the posterior cranial fossa, inferior to the tentorium cerebelli and dorsal to the brainstem. It plays a significant role in motor control, coordination, and proprioception.
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The main and largest component of the human brain is the cerebrum. The cerebrum consists of two main parts: the cerebral cortex, an outer layer with wrinkles or folds known as gyri and shallow grooves called sulci, and a deeper region beneath it. The cerebrum divides into two distinct hemispheres and contains five different lobes: the frontal, parietal, temporal, occipital, and insula. The central sulcus separates the frontal and parietal lobes and two functionally important gyri — the...
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The lateral view of the cranium is dominated by temporal, sphenoid, and ethmoid bones.
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Brain morphometry in Pontocerebellar Hypoplasia type 2.

Kaspar Ekert1, Samuel Groeschel1, Iciar Sánchez-Albisua2

  • 1Department of Child Neurology, Children's Hospital, University of Tübingen, Hoppe-Seyler-Str. 1, 72072, Tübingen, Germany.

Orphanet Journal of Rare Diseases
|July 20, 2016
PubMed
Summary

Pontocerebellar hypoplasia type 2 (PCH2) in children causes severe brain underdevelopment, particularly in the cerebellum and pons. This study quanties infratentorial and supratentorial brain growth, revealing significant growth deficits from birth.

Keywords:
Brain morphometryBrain stemCerebellumFrontal lobeMental retardationMicrocephalyPonsPontocerebellar hypoplasia type 2

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Genetics

Background:

  • Pontocerebellar hypoplasia type 2 (PCH2) is a severe genetic disorder.
  • It results from TSEN54-gene defects, causing early brain development disruption, especially affecting the cerebellum and pons.

Purpose of the Study:

  • To quantify infratentorial and supratentorial brain growth in children with PCH2.
  • To compare brain development in PCH2 patients with typically developing children.

Main Methods:

  • Volumetric MRI analysis of 24 children with PCH2 (aged 0.02-17 years) and 24 age/gender-matched controls.
  • Digitalization of film images and semi-automatic segmentation for volume measurements of cerebellum, brain stem, pons, supratentorial brain, and frontal lobe.
  • Validation of methods and assessment of intra/inter-rater variability.

Main Results:

  • Children with PCH2 exhibited reduced volumes in all measured brain structures compared to controls.
  • Severely hypoplastic infratentorial structures showed minimal postnatal growth.
  • Supratentorial brain volume also displayed reduced growth, leading to microcephaly, with differences apparent from birth and increasing with age.

Conclusions:

  • PCH2 is characterized by severely hypoplastic infratentorial structures with limited postnatal growth.
  • Supratentorial structures show reduced neonatal volumes and slower growth, resulting in microcephaly.
  • Findings suggest a primary cerebellar dysfunction disrupting cerebellar-cerebral networks, rather than postnatal neurodegeneration.