Severe Posterior Reversible Encephalopathy in Pheochromocytoma: Importance of Susceptibility-Weighted MRI

Serter, Asli; Alkan, Alpay; Aralaşmak, Ayşe; Kocakoç, Ercan

doi:10.3348/kjr.2013.14.5.849

Cited by 3 publications

(1 citation statement)

References 10 publications

(16 reference statements)

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“…PRES is a recently recognised clinical-radiological entity, previously called hypertensive encephalopathy. In fact, arterial hypertension could be regarded as the most well-known risk factor for this condition, and therefore, several children with PRES caused by pheochromocytoma or paraganglioma have been reported in the literature [ 109 – 111 ].…”

Section: Disorders Of the Adrenal Medulla And The Paediatric Brainmentioning

confidence: 99%

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: Disorders Of the Adrenal Medulla And The Paediatric Brainmentioning

confidence: 99%

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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Autonomic nervous system and cancer

et al. 2018

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The autonomic nervous system (ANS) is the main homeostatic regulatory system of the body. However, this widely distributed neural network can be easily affected by cancer and by the adverse events induced by cancer treatments. In this review, we have classified the ANS complications of cancer into two categories. The first includes direct cancer-related complications, such as primary ANS tumors (pheochromocytoma, paraganglioma or neuroblastoma), as well as autonomic manifestations induced by non-primary ANS tumors (primary brain tumors and metastases). The second comprises indirect ANS complications, which include autonomic features related to cancer therapy (chemotherapy, radiotherapy and/or surgery) and those not related to cancer therapy, such as paraneoplastic autonomic syndromes. We also review the molecular relationship and modulation between the ANS and the cancer cells and their microenvironment.

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Catecholamine-induced cerebral vasospasm and multifocal infarctions in pheochromocytoma

Madhok¹,

Kloosterboer²,

Venkatasubramanian

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary We report the case of a 76-year-old male with a remote history of papillary thyroid cancer who developed severe paroxysmal headaches in the setting of episodic hypertension. Brain imaging revealed multiple lesions, initially of inconclusive etiology, but suspicious for metastatic foci. A search for the primary malignancy revealed an adrenal tumor, and biochemical testing confirmed the diagnosis of a norepinephrine-secreting pheochromocytoma. Serial imaging demonstrated multiple cerebral infarctions of varying ages, evidence of vessel narrowing and irregularities in the anterior and posterior circulations, and hypoperfusion in watershed areas. An exhaustive work-up for other etiologies of stroke including thromboembolic causes or vasculitis was unremarkable. There was resolution of symptoms, absence of new infarctions, and improvement in vessel caliber after adequate alpha-adrenergic receptor blockade for the management of pheochromocytoma. This clinicoradiologic constellation of findings suggested that the etiology of the multiple infarctions was reversible cerebral vasoconstriction syndrome (RCVS). Pheochromocytoma remains a poorly recognized cause of RCVS. Unexplained multifocal cerebral infarctions in the setting of severe hypertension should prompt the consideration of a vasoactive tumor as the driver of cerebrovascular dysfunction. A missed or delayed diagnosis has the potential for serious neurologic morbidity for an otherwise treatable condition. Learning points: The constellation of multifocal watershed cerebral infarctions of uncertain etiology in a patient with malignant hypertension should trigger the consideration of undiagnosed catecholamine secreting tumors, such as pheochromocytomas and paragangliomas. Reversible cerebral vasoconstriction syndrome is a serious but reversible cerebrovascular manifestation of pheochromocytomas that may lead to strokes (ischemic and hemorrhagic), seizures, and cerebral edema. Alpha-adrenergic receptor blockade can reverse cerebral vasoconstriction and prevent further cerebral ischemia and infarctions. Early diagnosis of catecholamine secreting tumors has the potential for reducing neurologic morbidity and mortality in patients presenting with cerebrovascular complications.

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Severe Posterior Reversible Encephalopathy in Pheochromocytoma: Importance of Susceptibility-Weighted MRI

Cited by 3 publications

References 10 publications

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

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

Autonomic nervous system and cancer

Catecholamine-induced cerebral vasospasm and multifocal infarctions in pheochromocytoma

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