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Mutations in the ALS2 gene in this disorder disrupt the instructions for producing alsin. As a result, alsin is unstable and decays rapidly, or it is disabled and cannot function properly. It is currently unknown how the loss of functional alsin protein causes the death of motor neurons and the symptoms of juvenile primary lateral sclerosis. [1]
The lateral corticospinal tract is a descending motor pathway that begins in the cerebral cortex, decussates in the pyramids of the lower medulla [1] (also known as the medulla oblongata or the cervicomedullary junction, which is the most posterior division of the brain [2]) and proceeds down the contralateral side of the spinal cord.
UMNs are motor neurons that project from the cortex down to the brainstem or spinal cord. [18] LMNs originate in the anterior horns of the spinal cord and synapse on peripheral muscles. [18] Both motor neurons are necessary for the strong contraction of a muscle, but damage to an UMN can be distinguished from damage to a LMN by physical exam. [19]
Prolonged deficiency of vitamin B 12 leads to irreversible nervous system damage. HIV-associated vacuolar myelopathy can present with a similar pattern of dorsal column and corticospinal tract demyelination. [citation needed] It has been thought that if someone is deficient in vitamin B 12 and folic acid, the vitamin B 12 deficiency must
Of all the circuits, the motor circuit is the most studied due its importance to motor disorders. The direct pathway of the motor circuit is one in which projections from the cortex travel to the putamen directly to the internal segment of the globus pallidus (GPi also known as GP-Medial) or the substantia nigra, pars reticulata (SNr) and are then directed toward the ventral anterior nucleus ...
The motor pathway is the corticospinal (pyramidal) tract and the medial and lateral vestibular tracts. The first stage of the test (standing with the eyes open with hands on hips), demonstrates that at least two of the three sensory pathways are intact, and that sensorimotor integration and the motor pathway are functioning.
The dorsal column–medial lemniscus pathway (DCML) (also known as the posterior column-medial lemniscus pathway (PCML) is the major sensory pathway of the central nervous system that conveys sensations of fine touch, vibration, two-point discrimination, and proprioception (body position) from the skin and joints.
A frequently occurring motor deficit is left-sided hemiparesis (in strokes affecting the motor cortex). A less common motor deficit in this population is dysphagia. [4] Patients with right hemisphere brain damage often display sensory deficits such as left neglect, in which they ignore everything in the left visual field. [5]