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Ataxia (from Greek α- [a negative prefix] + -τάξις [order] = "lack of order") is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in eye movements, that indicates dysfunction of parts of the nervous system that coordinate movement, such as the cerebellum.
Many individuals with Friedreich's ataxia develop scoliosis, diabetes, and hypertrophic cardiomyopathy, a serious heart condition that is a leading cause of mortality in patients. Friedreich's ataxia is caused by mutations in the FXN gene, which result in reduced production of frataxin , a protein essential for mitochondrial function ...
As a stand-alone condition, "ataxia is a degenerative disease of the nervous system," explains Andrew Rosen, chief executive officer of the National Ataxia Foundation. There are many types ...
As a result of this gait impairment, falling is a concern in patients with ataxia. [3] Truncal ataxia affects the muscles closer to the body such as the trunk, shoulder girdle and hip girdle. It is involved in gait stability. [3] Truncal ataxia is different from appendicular ataxia. Appendicular ataxia affects the movements of the arms and legs.
Machado–Joseph disease (MJD), also known as Machado–Joseph Azorean disease, Machado's disease, Joseph's disease or spinocerebellar ataxia type 3 (SCA3), is a rare autosomal dominantly inherited neurodegenerative disease that causes progressive cerebellar ataxia, [1] [2] which results in a lack of muscle control and coordination of the upper and lower extremities. [3]
Hemorrhage into the basal ganglia or thalamus causes contralateral hemiplegia due to damage to the internal capsule. [7] Other possible symptoms include gaze palsies or hemisensory loss. [7] Intracerebral hemorrhage into the cerebellum may cause ataxia, vertigo, incoordination of limbs and vomiting. [7]
Ataxia usually goes away without any treatment. In cases where an underlying cause is identified, medical treatment may be needed. In extremely rare cases, patients can have continuing and disabling symptoms. Treatment includes corticosteroids, intravenous immunoglobulin, or plasma exchange therapy. Drug treatment to improve muscle coordination ...
It is associated with cerebellar ataxia, [1] [2] when the cerebellum has been damaged and does not function to its fullest ability. Lesions to the cerebellum can cause dyssynergia, dysmetria, dysdiadochokinesia, dysarthria, and ataxia of stance and gait. [3] Dyschronometria can result from autosomal dominant cerebellar ataxia (ADCA). [4]