Lower motor neuron involvement in perisylvian polymicrogyria
Maria Clark1, Matthew Pitt, Brian G R Neville
1Neurosciences Unit, University College London Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.
Developmental Medicine and Child Neurology
|September 19, 2006
Summary
Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) typically affects the brain
Area of Science:
- Neurology
- Genetics
- Developmental Biology
Background:
- Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) is a brain malformation.
- Its phenotype was traditionally considered exclusively central nervous system-related.
- This limited the understanding of its full clinical spectrum.
Purpose of the Study:
- To investigate potential non-central nervous system manifestations in CBPS.
- To explore the association between CBPS and motor neuron involvement.
- To challenge the traditional classification of cerebral palsies.
Main Methods:
- Electrophysiological studies were conducted on seven children with CBPS.
- Clinical assessments focused on neurological signs and congenital contractures.
- Patient data included age, sex, and clinical history.
Main Results:
- Six of seven children with CBPS showed electrophysiological evidence of lower motor neuron involvement.
- This involvement was associated with congenital contractures of the limbs or jaw.
- The findings suggest a broader phenotype than previously recognized.
Conclusions:
- CBPS may involve both upper and lower motor neuron pathways.
- This dual involvement is not typically expected in CBPS.
- The findings suggest a potential genetic or pathogenic sequence previously unrecognized in CBPS.
More Related Videos
Related Concept Videos
01:14Motor and Sensory Areas of the Cortex
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.
Motor Areas
The motor areas located in the frontal lobe are central to controlling voluntary movements. This region is further subdivided into the primary motor cortex and the premotor cortex.
Motor Areas
The motor areas located in the frontal lobe are central to controlling voluntary movements. This region is further subdivided into the primary motor cortex and the premotor cortex.
01:23Somatosensory, Motor, and Association Cortex
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 the...
01:11Direct Motor Pathways
The direct motor pathways, also known as the pyramidal tracts, are a group of neural pathways that originate in the brain and descend through the spinal cord. They control the voluntary movement of the body. There are two major direct motor pathways: the corticospinal and the corticobulbar tracts.
The corticospinal tract is responsible for the voluntary movement of the limbs and trunk. It originates in the cerebral cortex of the brain and descends through the cerebrum's internal capsule and the...
The corticospinal tract is responsible for the voluntary movement of the limbs and trunk. It originates in the cerebral cortex of the brain and descends through the cerebrum's internal capsule and the...
01:19Brainstem
The brainstem, located inferior to the brain and superior to the spinal cord, serves as a bridge between the cerebrum and the spinal cord. It plays a vital role in relaying information and controlling critical life functions. It comprises three primary regions: the midbrain, pons, and medulla oblongata.
The Midbrain
The midbrain is located beneath the diencephalon and connects the cerebrum with the lower parts of the brain. The cerebral peduncles are prominent midbrain structures that house the...
The Midbrain
The midbrain is located beneath the diencephalon and connects the cerebrum with the lower parts of the brain. The cerebral peduncles are prominent midbrain structures that house the...