aDepartment of Neurology, Gyeongsang National University School of Medicine, Jinju, Korea
bDepartment of Neurology, Seoul National University, College of Medicine, Seoul, Korea
Copyright © 2009 The Korean Movement Disorder Society
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Study | Age/gender | Onset age, y | Neurologic findings | Adrenal involvement | Neuroimaging |
---|---|---|---|---|---|
Kusaka and Imai2 | 28/M | 21 | Dysarthria, gait disturbance and postural instability, ataxia | Elevated ACTH level | MRI: brainstem and cerebellar atrophy, hyperintense lesions in the central nucleus of the cerebellum bilaterally, posterior limbs of the IC |
Miyai, et al.3 | 29/M | 26 | Dysarthria, ataxic gait, impotent, urinary incontinence, euphoria | Elevated ACTH level, mild hyperpigmentation | MRI: brainstem and cerebellar atrophy, hyperintense lesions in both CR, IC, cerebral peduncles, white matter around the dentate nuclei of the cerebellum |
Kobayashi, et al.4 | 27/M | 27 | Progressive dysarthria, unsteadiness walking | Hyperpigmentation, but normal ACTH level | CT: atrophy of the pons and cerebellum |
27/M | 22 | Spastic-ataxic gait, scanning speech, limb and truncal ataxia | Normal ACTH level | CT: atrophy of the pons and cerebellum | |
Tan, et al.5 | 24/M | 22.5 | Progressive slurred speech, unsteady gait, memory deterioration | Addison’s disease, hyperpigmentation | MRI: cerebellar atrophy, hyperintense lesions in the dentate nuclei and adjacent white matter, MCP and pons |
Mishra, et al.6 | 29/M | 20 | Urinary incontinence, fecal incontinence, erectile dysfunction, ataxic gait, slurred speech | Addison’s disease, hyperpigmentation | MRI: hyperintensities in the bilateral posterior parieto-occipital white matter, splenium, cerebellar white matter, IC, MCP, midbrain, and pons |
Ohno, et al.7 | 54/M | 52 | Gait disturbance, dysarthria, bladder disturbance | Addison’s disease, hyperpigmentation | CT: atrophy of the pons and cerebellum |
Vianello, et al.8 | 29/M | 21 | Progressive ataxic gait, mental retardation, dysarthria, uninhibited behavior | Addison’s disease | MRI: cerebellar, brainstem, and cervical cord atrophy |
Waragai, et al.9 | 30/M | 29 | Difficulty walking, postural instability, dysarthria | Mild adrenal insufficiency | MRI: atrophy of the cerebellum and brainstem, high-signal lesions in the bilateral dentate nuclei, corticospinal tracts |
Suda, et al.10 | 29/M | 6MA | Progressive gait disturbance and dysarthria | Addison’s disease, hyperpigmentation | MRI: hyperintense lesions in both dentate nuclei and red nuclei, pontine portion of the left corticospinal tract |
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