Cushings Syndrome Cushings Disease

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Hypertension, diabetes, hyperphagia, central obesity moon facies, dorsocervical fat deposition (buffalo hump; may cause Mental status changes, idiopathic myelopathy), hirsutism, acne, menstrual disorders evidence of protein cataboism (eg, easy bruising myophathy intracaranial hypertension, visual field osteopenia), cutaneous lesions (hyperpigmentation; wide, purple striae), psychiatric manifistations defects, optic neumpathy

Data from Lazer RB: Neuromuscular manifestations of endocrine disease. In Becker KL, Bilezikian JP Bremmer WJ et al (eds): Principles and Practice of Endocrinology and Metaboism, 2nd ed Philadelphia,,J.B. Lippincott,1995, pp 1762-1770; and Schteingart DE: Cushing syndrome. In Becker KL, Bilezikian JP, Bremmer WJ,et al (eds): Principles and Practice of Endocrinoogly and Metaboism 2nd ed. Philadelphia,J.B. Lippincott, l995, pp 667-682

The treatment of endogenous Cushing's syndrome depends on the underlying etiology. Growth Hormone Disorders

Growth hormone (GH; somatotropin) is secreted by the anterior pituitary in response to hypothalamic GH-releasing hormone (GHRH). GH promotes linear growth and has both anabolic and catabolic effects. Hypersecretion of GH causes gigantism when it occurs before epiphyseal closure and acromegaly when it begins afterward. Hyposecretion causes short stature in childhood and possibly a chronic fatigue-like syndrome in adults. GH acts indirectly through insulin-like growth factors (IGFs, somatomedins), with IGF-1 (formerly somatomedin C) being the most important for growth. Prolonged exposure to elevated levels of GH and IGF-1 results in the insidious onset of skeletal and soft tissue overgrowth, the latter of which is most pronounced in tissues containing large amounts of cartilage proteoglycans. '109] Cortical bone density is increased and trabecular bone (e.g., vertebral) density is decreased, probably because of coexistent hypogonadism. '109] Soft tissue hypertrophy may result in compression neuropathies, radiculopathies, and rarely, myelopathy. '76

More than 98 percent of cases of acromegaly are due to GH hypersecretion by a pituitary adenoma. '110 Rare etiologies include increased hypothalamic production of GHRH, ectopic pharyngeal pituitary tissue, and ectopic hormone (GH, GHRH)-secreting tumors (e.g., hypothalamic gangliocytoma; breast, stomach, and lung tumors).'109] GH-secreting pituitary adenomas are relatively rare, with an incidence of 3 to 4 per million and a prevalence of 50 to 70 per million. '110] There is no sexual predilection, and the majority of patients are diagnosed in their fourth or fifth decade, after a mean delay of 8.7 years. '111]

If it is present, an enlarging pituitary adenoma may produce headaches, visual changes (e.g., bitemporal hemianopia due to optic chiasm compression), and hypopituitarism (e.g., decreased ACTH or TSH levels due to anterior pituitary destruction), as well as DI (posterior pituitary involvement) and diplopia (due to cavernous sinus invasion). The lack of correlation between headache and tumor size reported in some series '111 suggests that headache may also be a feature of GH hypersecretion.

The hypersecretion of GH produces various forms of disfigurement and other physical changes (, Tab.leii.SSzl.I). Central sleep apnea has been reported in one third of acromegalic patients with sleep apnea. W Importantly, the presence of sleep apnea increases the risk for hypertension, myocardial infarction, and stroke, as well as accident susceptibility due to daytime sleepiness. '110] Mononeuropathies, especially compression neuropathies such as carpal tunnel syndrome (CTS), may be noted. CTS occurs in 50 percent of patients and is noted in 75 percent when EMG testing is performed. y Objective weakness, in a myopathic pattern, is observed in about 40 percent of acromegalic patients.y The weakness typically has an insidious onset and is a late manifestation, correlating best with the duration of acromegaly. y Polyneuropathies, nerve root and spinal cord compression, headaches, and visual changes have also been described. y


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