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 |