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Basal ganglia disease information


Basal ganglia disease
Basal ganglia and related structures
SpecialtyNeurology
Types8

Basal ganglia disease is a group of physical problems that occur when the group of nuclei in the brain known as the basal ganglia fail to properly suppress unwanted movements or to properly prime upper motor neuron circuits to initiate motor function.[1] Research indicates that increased output of the basal ganglia inhibits thalamocortical projection neurons. Proper activation or deactivation of these neurons is an integral component for proper movement. If something causes too much basal ganglia output, then the ventral anterior (VA) and ventral lateral (VL) thalamocortical projection neurons become too inhibited, and one cannot initiate voluntary movement. These disorders are known as hypokinetic disorders. However, a disorder leading to abnormally low output of the basal ganglia leads to reduced inhibition, and thus excitation, of the thalamocortical projection neurons (VA and VL) which synapse onto the cortex. This situation leads to an inability to suppress unwanted movements. These disorders are known as hyperkinetic disorders.[2]

Reasons for abnormal increases or decreases of basal ganglia output are not yet well understood. One possible factor could be the natural accumulation of iron in the basal ganglia, causing neurodegeneration due to its involvement in toxic, free-radical reactions.[3] Though motor disorders are the most common associated with the basal ganglia, recent research shows that basal ganglia disorders can lead to other dysfunctions such as obsessive–compulsive disorder (OCD) and Tourette syndrome.[4]

  1. ^ Purves, D.; Augustine, G.; Fitzpatrick, D.; Hall, W.; LaManita, A.-S.; McNamara, J.; et al. (2008). Neuroscience (4th ed.). Sunderland MA: Sinauer Associates.
  2. ^ Wichmann T, DeLong MR (December 1996). "Functional and pathophysiological models of the basal ganglia". Curr. Opin. Neurobiol. 6 (6): 751–8. doi:10.1016/S0959-4388(96)80024-9. PMID 9000030. S2CID 30435859.
  3. ^ Curtis AR, Fey C, Morris CM, et al. (August 2001). "Mutation in the gene encoding ferritin light polypeptide causes dominant adult-onset basal ganglia disease". Nat. Genet. 28 (4): 350–4. doi:10.1038/ng571. PMID 11438811. S2CID 25483839.
  4. ^ DeLong MR, Wichmann T (January 2007). "Circuits and circuit disorders of the basal ganglia". Arch. Neurol. 64 (1): 20–4. doi:10.1001/archneur.64.1.20. PMID 17210805.

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The basal ganglia (BG) or basal nuclei are a group of subcortical nuclei found in the brains of vertebrates. In humans and other primates, differences...

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neurodegenerative disorder characterized by the accumulation of iron in the basal ganglia, cerebellum, and motor cortex of the human brain. Symptoms, which are...

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calcification (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, is a rare, genetically dominant or recessive, inherited...

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hyperkinetic movements are the result of improper regulation of the basal ganglia–thalamocortical circuitry. Overactivity of a direct pathway combined...

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Leigh syndrome

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oxidative phosphorylation. In the case of Leigh disease, crucial cells in the brain stem and basal ganglia are affected. This causes a chronic lack of energy...

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Molecular neuroscience

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Parkinson's disease is the second most common neurodegenerative disease after Alzheimer's disease. It is a hypokinetic movement basal ganglia disease caused...

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the basal ganglia and the neurophysiology of habit formation, implicit learning, and her work is relevant to Parkinson's disease, Huntington's disease,...

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Direct pathway

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neural pathway within the central nervous system (CNS) through the basal ganglia which facilitates the initiation and execution of voluntary movement...

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brain, the basal forebrain structures are located in the forebrain to the front of and below the striatum. They include the ventral basal ganglia (including...

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External globus pallidus

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target for deep brain stimulation as a treatment for Parkinson's disease. The basal ganglia functions to tonically inhibit movement, mainly in the absence...

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the SLC19A3 gene have been linked to biotin-thiamine responsive basal ganglia disease, which is treated with pharmacological doses of thiamine and biotin...

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Athymhormic syndrome

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Hemiballismus

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Hemiballismus or hemiballism is a basal ganglia syndrome resulting from damage to the subthalamic nucleus in the basal ganglia. Hemiballismus is a rare hyperkinetic...

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Putamen

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Subthalamic nucleus

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the brain where it is, from a functional point of view, part of the basal ganglia system. In terms of anatomy, it is the major part of the subthalamus...

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