Adrenocortical Atrophy and Diffuse Cerebral Sclerosis

Forsyth, Constance C.; Forbes, Mehroo; Cumings, J. N.

doi:10.1136/adc.46.247.273

Cited by 38 publications

(11 citation statements)

References 26 publications

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“…Among other rarer causes are histoplasmosis, coccidioidomyocosis, torulosis, myocosis fungoides, amyloidosis [14], X-linked congenital adrenal hypoplasia in the neonatal period, [1,10,15,25], X-linked adrenoleukodystrophy [3,8,13], and local hemorrhage and dystrophic calcification [9].…”

Section: Discussionmentioning

confidence: 99%

Sudden death due to auto-immune Addison's disease in a 12-year-old girl

Sabri

Smith

Busuttil

1997

International Journal of Legal Medicine

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A 12-year-old female suffering from adrenocortical insufficiency showed symptoms similar to a gastro-enteritis, and severe electrolytic and acid/base disturbances which progressed into cerebral oedema and death. Autopsy findings included depletion of the adrenal cortex, with enlargement and eosinophilia of surviving cells. In ante-mortem blood, anti-adrenal auto-antibodies were found and elevated levels of ACTH and cortisol with a low level of aldosterone.

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Section: Discussionmentioning

confidence: 99%

Sudden death due to auto-immune Addison's disease in a 12-year-old girl

Sabri

Smith

Busuttil

1997

International Journal of Legal Medicine

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“…The pathophysiology of this disease is an accumulation in brain and adre nal glands of lipids composed of an abnor mally large proportion of long chain fatty acids esterified to cholesterol [1,2], Histo logic studies of brain and adrenal cortex have demonstrated similar cytoplasmic inclusions characteristic of this disorder [3][4][5], Although Table II shows the endocrine data on the 7 prepubertal subjects of the study. The normal values quoted are those determined by the Pediatric Endocrine Laboratories of The Johns Hopkins Hospital.…”

Section: Introductionmentioning

confidence: 99%

Adrenal and Testicular Function in 14 Patients with Adrenoleukodystrophy or Adrenomyeloneuropathy

et al. 1986

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Testicular and adrenal function were evaluated in 12 patients with adrenoleukodystrophy and 2 patients with adrenomyeloneuropathy. Although only 5 subjects had clinical symptoms suggesting adrenal insufficiency, an additional 5 showed laboratory evidence of reduced adrenal reserve. 9 of the 14 patients developed neurological deficits prior to the onset of clinical or biological adrenal insufficiency. In the remaining 5 patients, adrenal insufficiency antedated the appearance of neurological symptoms; 2 of these 5 patients had only laboratory evidence of hypoadrenocorticism, and 3 had both clinical and laboratory abnormalities. None of the prepubertal patients had detectable signs of testicular insufficiency, while 3 of the 7 pubertal/adult patients had elevated serum LH or FSH levels. This mild testicular deficiency was seen only in association with clinical adrenal insufficiency and significant neurological impairment.

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“…Though the literature suggests that clinical adrenal insufficiency antedates the neurological disorder (Forsyth, Forbes, and Cumings, 1971), in my experience in a series of cases selected by their presentation as neurological syndromes (mimicking acute encephalopathies, or acute demyelinating diseases) the only evidence of suprarenal dysfunction has been reduced or absent cortisol production after ACTH stimulation.…”

mentioning

confidence: 85%

Investigation of degenerative disease of the central nervous system.

Wilson¹

1972

Archives of Disease in Childhood

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Adrenocortical Atrophy and Diffuse Cerebral Sclerosis

Cited by 38 publications

References 26 publications

Sudden death due to auto-immune Addison's disease in a 12-year-old girl

Sudden death due to auto-immune Addison's disease in a 12-year-old girl

Adrenal and Testicular Function in 14 Patients with Adrenoleukodystrophy or Adrenomyeloneuropathy

Investigation of degenerative disease of the central nervous system.

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