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Spinocerebellar ataxia (SCA) is a progressive, degenerative, [1] genetic disease with multiple types, each of which could be considered a neurological condition in its own right. An estimated 150,000 people in the United States have a diagnosis of spinocerebellar ataxia at any given time. SCA is hereditary, progressive, degenerative, and often ...
Genetic testing is available for many SCA types, including the relatively common types SCA1, 2, 3, 6, and 7; and the less common SCA8, 10, 12, 14, and 17. [39] However, genetic testing is high in cost and has a low diagnostic yield, with positive diagnoses being found in only 24% of tests ordered by a subspecialist and 10% overall.
The Boston Diagnostic Aphasia Examination provides a comprehensive exploration of a range of communicative abilities. Its results are used to classify patient's language profiles into one of the localization based classifications of aphasia: Broca's, Wernicke's, anomic, conduction, transcortical, transcortical motor, transcortical sensory, and global aphasia syndromes, although the test does ...
Spinocerebellar ataxia type 13 (SCA13) is a rare autosomal dominant disorder, which, like other types of SCA, is characterized by dysarthria, nystagmus, and ataxia of gait, stance and the limbs due to cerebellar dysfunction.
The Boston Naming Test (BNT), introduced in 1983 by Edith Kaplan, Harold Goodglass and Sandra Weintraub, is a widely used neuropsychological assessment tool to measure confrontational word retrieval in individuals with aphasia or other language disturbance caused by stroke, Alzheimer's disease, or other dementing disorder. [1]
Spinocerebellar ataxia type 6 (SCA6) is a rare, late-onset, autosomal dominant disorder, which, like other types of SCA, is characterized by dysarthria, oculomotor disorders, peripheral neuropathy, and ataxia of the gait, stance, and limbs due to cerebellar dysfunction. Unlike other types, SCA 6 is not fatal.
Landau–Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia, [1] or aphasia with convulsive disorder, is a rare neurological syndrome that develops during childhood. [2] It is named after William Landau and Frank Kleffner, who characterized it in 1957 with a diagnosis of six children. [3] [4]
The scale was originally introduced in 1957 by Dr. John Rankin of Stobhill Hospital, Glasgow, Scotland as a 5-level scale ranging from 1 to 5. [ 3 ] [ 4 ] It was then modified by either van Swieten et al. [ 5 ] or perhaps Prof. C. Warlow's group at Western General Hospital in Edinburgh for use in the UK-TIA study in the late 1980s to include ...