Neurologic complications of disorders of the adrenal glands

Bertorini, Tulio E.; Pérez, Aníbal

doi:10.1016/b978-0-7020-4087-0.00050-4

Cited by 13 publications

(20 citation statements)

References 149 publications

(112 reference statements)

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“…In children under 7 years, Cushing disease is less frequent, and the adrenal causes of Cushing syndrome (i.e., adenoma, carcinoma, or bilateral hyperplasia) represent the most common causes of the condition in infants [8]. Adrenal cortical neoplasms in paediatric patients are more difficult to diagnose, and it is also more difficult to separate benign from malignant tumours in this age group [9, 10]. …”

Section: Hypercortisolism and The Paediatric Brainmentioning

confidence: 99%

“…CNC1 complex is a multiple endocrine neoplasia (MEN) syndrome that affects endocrine glands such as the adrenal cortex (causing Cushing's syndrome), the pituitary, and the thyroid. PPNAD is associated with germ line inactivating mutations of the PRKAR1A gene, which is located at 17q22-24 and encodes protein kinase A [10]. …”

Section: Hypercortisolism and The Paediatric Brainmentioning

confidence: 99%

“…Adrenal insufficiency (or even Addisonian crisis) can sometimes be the presenting symptom of X-ALD in boys and men, years or even decades before the onset of neurological symptoms [10, 36, 62]. The most aggressive and devastating phenotype is cerebral ALD, consisting of a rapidly progressive demyelinating condition that affects the cerebral white matter.…”

Section: Adrenal Insufficiency and The Paediatric Brainmentioning

confidence: 99%

“…It progresses to visual symptoms, auditory processing difficulties, and motor incoordination. Later neurological manifestations can include moderate dystonia, pyramidal signs, gait disturbances with features of cerebellar and pyramidal tract involvement, hemiparesis, or spastic tetraparesis [10, 61, 62]. Typical MRI white matter changes include signal hyper intensities on T2-weighed and FLAIR sequences in the parietooccipital region and the splenium of the corpus callosum [61, 62].…”

Section: Adrenal Insufficiency and The Paediatric Brainmentioning

confidence: 99%

“…It is likely that if they survive into adulthood, all patients with X-ALD eventually develop adrenomyeloneuropathy (AMN), a condition characterised by paraparesis, spasticity, and signs of neuropathy, with onset usually after 20 years of age [10, 63]. …”

Section: Adrenal Insufficiency and The Paediatric Brainmentioning

confidence: 99%

See 4 more Smart Citations

Adrenal Disorders and the Paediatric Brain: Pathophysiological Considerations and Clinical Implications

Salpietro

Polizzi

Rosa

et al. 2014

International Journal of Endocrinology

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Various neurological and psychiatric manifestations have been recorded in children with adrenal disorders. Based on literature review and on personal case-studies and case-series we focused on the pathophysiological and clinical implications of glucocorticoid-related, mineralcorticoid-related, and catecholamine-related paediatric nervous system involvement. Childhood Cushing syndrome can be associated with long-lasting cognitive deficits and abnormal behaviour, even after resolution of the hypercortisolism. Exposure to excessive replacement of exogenous glucocorticoids in the paediatric age group (e.g., during treatments for adrenal insufficiency) has been reported with neurological and magnetic resonance imaging (MRI) abnormalities (e.g., delayed myelination and brain atrophy) due to potential corticosteroid-related myelin damage in the developing brain and the possible impairment of limbic system ontogenesis. Idiopathic intracranial hypertension (IIH), a disorder of unclear pathophysiology characterised by increased cerebrospinal fluid (CSF) pressure, has been described in children with hypercortisolism, adrenal insufficiency, and hyperaldosteronism, reflecting the potential underlying involvement of the adrenal-brain axis in the regulation of CSF pressure homeostasis. Arterial hypertension caused by paediatric adenomas or tumours of the adrenal cortex or medulla has been associated with various hypertension-related neurological manifestations. The development and maturation of the central nervous system (CNS) through childhood is tightly regulated by intrinsic, paracrine, endocrine, and external modulators, and perturbations in any of these factors, including those related to adrenal hormone imbalance, could result in consequences that affect the structure and function of the paediatric brain. Animal experiments and clinical studies demonstrated that the developing (i.e., paediatric) CNS seems to be particularly vulnerable to alterations induced by adrenal disorders and/or supraphysiological doses of corticosteroids. Physicians should be aware of potential neurological manifestations in children with adrenal dysfunction to achieve better prevention and timely diagnosis and treatment of these disorders. Further studies are needed to explore the potential neurological, cognitive, and psychiatric long-term consequences of high doses of prolonged corticosteroid administration in childhood.

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Section: Hypercortisolism and The Paediatric Brainmentioning

confidence: 99%

Section: Hypercortisolism and The Paediatric Brainmentioning

confidence: 99%