Disorders of Basal Ganglia



Wilson disease is an inherited disorder characterized by excess of copper in the body tissues. It is also known as progressive hepatolenticular degeneration. This disease develops due to damage of the lenticular nucleus particularly, putamen. In Wilson disease, copper is deposited in the liver, brain, kidneys and eyes. Copper deposits cause damage of tissues. And the affected organs stop functioning. In addition to symptoms of Parkinson disease, liver failure and damage to the central nervous system are the most predominant effects of this disorder. Wilson disease is fatal if not treated early.


Chorea is an abnormal involuntary movement. Chorea means rapid jerky movements. It mostly involves the limbs. Chorea is due to the lesion in caudate nucleus and putamen.


Athetosis is another type of abnormal involuntary movement, which refers to slow rhythmic and twisting

movements. It is because of the lesion in caudate nucleus and putamen.


Choreoathetosis is the condition characterized by aimless involuntary muscular movements. It is due to combined effects of chorea and athetosis.


Huntington disease is an inherited progressive neural disorder due to the degeneration of neurons secreting GABA in corpus striatum and substantia nigra. This disease starts mostly in middle age. It is characterized by chorea, hypotonia and dementia. In severe cases bilateral wasting of muscles occurs. It is otherwise called Huntington disease, chronic progressive chorea, degenerative chorea or hereditary chorea.


Hemiballismus is a disorder characterized by violent involuntary abnormal movements on one side of the

body involving mostly the arm. While walking, the arm swings widely. These movements are called the flinging movements. These movements are due to the release phenomenon because of the absence of inhibitory influence on movements. Hemiballismus occurs due to degeneration of subthalamic nucleus of Luys.


Kernicterus is a form of brain damage in infants caused by severe jaundice. Basal ganglia are the mainly

affected parts of brain.

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