Reversible brain atrophy and cognitive impairment in an adolescent Japanese patient with primary adrenal Cushing’s syndrome

Figures & data

Figure 1 Transverse T1-weighted magnetic resonance images of the brain.

Notes: (A–C) Diffuse atrophy of the brain, including the cerebrum, cerebellum, and hippocampus was found, accompanied by dilatation of ventricles, subarachnoid space, and sulci, with an Evans’ index of 0.25. (D–F) Eight months after surgical removal of cortisol-secreting adrenocortical adenoma, brain atrophy recovered. Dilatation of ventricles, subarachnoid space, and sulci was also resolved, with an Evans’ index of 0.22.

Figure 2 Radiological findings.

Notes: (A) Plain abdominal computed tomography showed a tumor of 2.7 cm diameter with 35 Hounsfield units (arrow) in the left adrenal gland. (B) ¹³¹I-adosterol scan (posterior view) demonstrated radioisotope accumulation in accordance with the left adrenal mass (arrow), while no uptake was detected on the opposite side.

Table 1 Laboratory findings in August 2011

Hematology
White blood cells	10,250/mm^3
Red blood cells	422×10^4/μL
Hemoglobin	13.3 g/dL
Hematocrit	38.8%
Platelets	18.8×10^4/mm^3
Chemistry
Total protein	6.3 g/dL
Albumin	4.1 g/dL
Aspartate aminotransferase	27 IU/L
Alanine aminotransferase	30 IU/L
Gamma-glutamyl transpeptidase	36 IU/L
Urea nitrogen	13 mg/dL
Creatinine	0.73 mg/dL
Sodium	145 mmol/L
Potassium	2.7 mmol/L
Chloride	104 mmol/L
C-reactive protein	0.01 mg/dL
Fasting plasma glucose	4.9 mmol/L
Hemoglobin A1c	5.2%
Urine analysis
Specific gravity	1.009
Glucose	–
Protein	–
Occult blood reaction	–
Ketone body	–
Endocrinological examination
Plasma aldosterone	6.3 ng/dL
Plasma renin activity	2.0 mg/dL/h
Serum DHEA-S	294 ng/mL (240–5,370)#
Plasma cortisol
8 AM	29.1 μg/dL
4 PM	26.2 μg/dL
12 PM	26.4 μg/dL
Plasma ACTH
8 AM	<1.0 pg/mL
4 PM	<1.0 pg/mL
12 PM	<1.0 pg/mL
Urinary free cortisol	1,280 μg/day

Note:

# Reference range.

Abbreviations: ACTH, adrenocorticotropic hormone; DHEA-S, dehydroepiand-rosterone sulfate.

Figure 3 Histological findings of the resected left adrenal gland.

Notes: (A) Tissue sections stained with hematoxylin and eosin (HE) (20×) demonstrated an apparently marginated encapsulated tumor without any capsular or blood vessel invasion. (B) The zona reticularis and fasciculata of the attached non-tumoral adrenal tissue was atrophic (arrow) and showed little expression of dehydroepiandrosterone sulfate (20×). (C) The tumor consisted mainly of compact cells and partially of clear cells (HE, 400×). A slight infiltration of lymphocytes (arrow) was observed in the tumor. (D) Most of the tumor cells indicated positive immunostaining for 17α-hydoroxylase (400×).