High Response Rate to Antidepressants in the Management of Paroxysmal Hypertension (Pseudopheochromocytoma)

There is no widely recognized preventive treatment for patients with paroxysmal hypertension (“pseudopheochromocytoma”) who suffer recurrent and often severe paroxysmal elevation of blood pressure, repeated emergency room visits and hospitalizations. In this case series we assessed the effectiveness of treatment with an antidepressant in preventing recurrent paroxysms. A secondary exploratory objective was to examine the psychological profile of patients and its relationship to response to treatment. The charts of 52 patients who reported having experienced at least 3 symptomatic episodes were selected; and and for whom treatment data were included. Treatment with an antidepressant was offered to, and follow-up data were available in 37..Two patients refused treatment, 6 were unable to tolerate an effective dose, 3 were lost to follow-up, and 1 was non-compliant with the medication. Of the remaining 25 evaluable patients, 92% (23/25) responded, with 80% (21/25) achieving complete and persisting cessation of paroxysms, and 8% (2/25), a reduction in frequency. Importantly, an antidepressant was effective in nearly all patients who reported that they were not suffering from anxiety or depression. The data were insufficient to determine superiority of any one antidepressant versus another. We conclude that treatment with an antidepressant is effective in preventing hypertensive paroxysms in a high proportion of patients with paroxysmal hypertension. Given the absence of any other pharmacologic intervention capable of preventing recurrent paroxysms, the similarly high response rate observed in previous reports, and the relatively safe profile of antidepressant agents, the findings support more widespread use of an antidepressant in patients with this disorder to prevent years of continued hypertensive paroxysms and their consequences.

Perioperative control of paroxysmal hypertension using esmolol with alpha-blockade in a child with a germline mutated paraganglioma

Summary The use of antihypertensive medications in patients with pheochromocytomas and paragangliomas (PCC/PG) is usually a challenge. We report a case of familial paraganglioma that was successfully treated by esmolol and other antihypertensive medications without associated perioperative complications. Our patient was an 11-year-old girl who presented with classic symptoms and signs of PCC/PG and a CT scan of the abdomen that showed a right-sided paravertebral mass. Her father was diagnosed with paraganglioma a few years ago. Prazosin had been started but she continued to experience uncontrolled paroxysms of blood pressure (BP). She was known to have asthma; hence, she developed serious bronchospasm with atenolol. She was, therefore, switched to esmolol that successfully controlled her BP in addition to prazosin and intermittent doses of hydralazine prior to laparoscopic surgery with no side effects of medications or postoperative complications. Esmolol could be a good alternative to routinely used beta-blockers in children with PCC/PG with labile hypertension and related symptoms in the pre and intra-operative periods. It is titrable, effective, and can be weaned rapidly helping to avoid postoperative complications. Further larger studies on the use of esmolol in children with PCC/PG are needed to confirm our observation. Learning points In addition to alpha-blockers, esmolol could be a good alternative for routinely used beta-blockers to control paroxysmal hypertension and tachycardia in the pre- and intra-operative periods. Esmolol is titrable and an effective beta-blocker. It can be weaned rapidly helping to avoid postoperative complications in children with PCC/PG. Children with PCC/PG and other comorbidity like asthma may particularly benefit from the use of esmolol due to no or less side effects on airway resistance and the advantage of rapid titration of the medication compared to other beta-blockers.

Autonomic dysreflexia associated with cervical spinal cord gliofibroma: case report

Background Autonomic dysreflexia (AD) is an abnormal reflex of the autonomic nervous system normally observed in patients with spinal cord injury from the sixth thoracic vertebra and above. AD causes various symptoms including paroxysmal hypertension due to stimulus. Here, we report a case of recurrent AD associated with cervical spinal cord tumor. Case presentation The patient was a 57-year-old man. Magnetic resonance imaging revealed an intramedullary lesion in the C2, C6, and high Th12 levels. During the course of treatment, sudden loss of consciousness occurred together with abnormal paroxysmal hypertension, marked facial sweating, left upward conjugate gaze deviation, ankylosis of both upper and lower extremities, and mydriasis. Seizures repeatedly occurred, with symptoms disappearing after approximately 30 min. AD associated with cervical spinal cord tumor was diagnosed. Histological examination by tumor biopsy confirmed the diagnosis of gliofibroma. Radiotherapy was performed targeting the entire brain and spinal cord. The patient died approximately 3 months after treatment was started. Conclusions AD is rarely associated with spinal cord tumor, and this is the first case associated with cervical spinal cord gliofibroma. AD is important to recognize, since immediate and appropriate response is required.

Paraganglioma Not Only Secrete Catecholamines but Also Inflammatory Cytokines: A Case Report

Background: Paraganglioma not only can secrete catecholamines with vasoconstriction activity resulting in hypertension and metabolic disorders,but also cytokines which can induce inflammatory response syndrome.Case presentation: We report a 19-year-old girl with a paraganglioma that presented with fever and thrombocytosis, leukocytosis, and elevated high sensitivity C-reactive protein. After paraganglioma’s resection, the little girl had no fever and the platelet, white blood cell and high sensitivity C-reactive protein recover to nomal level. Conclusion: We propose in the future we should check cytokine in patients with paroxysmal hypertension complicated with inflammatory response syndrome and thrombocytosis, and think of that it may be caused by pheochromocytoma or paraganglioma.

Abstract P108: The Cause and Treatment of Paroxysmal Hypertension

Introduction: The cause and treatment of paroxysmal hypertension (PH) (“pseudopheochromocytoma”) in the 98-99% of patients who do not have a pheochromocytoma has long been a mystery. It has been linked to a psychosomatic origin based on the finding in nearly all patients of repression of emotions associated with either a past history of overwhelming stress or trauma or a repressive coping style. The purpose of this study is to convey further experience in understanding and treating PH. Methods: We reviewed the medical records of patients with a diagnosis of PH. Patients were considered to have PH if episodes were sudden in onset, were unprovoked (not precipitated by stress or anxiety) and were symptomatic (e.g., headache, flushing, tachycardia and/or others). Patient characteristics, psychosocial history and response to treatment were assessed. Based on the previous study, the psychosocial characteristics examined most closely were the presence of a past history of trauma or overwhelming stress, the emotional response to that trauma, the presence or absence of a history of depression or anxiety, and the presence or absence of a repressive coping style. Results: Sixty-eight percent of patients (24 of 35) reported a history of trauma with repression of related emotions; a repressive coping style was evident in 26% (9 of 35). Neither characteristic was evident in only 2 patients (6%). Most patients were prescribed clonidine and/or alprazolam for acute management of paroxysms; this intervention was considered sufficient by 10 (28%). Twenty-three of the remaining 25 were offered treatment with an antidepressant. Two refused and two could not tolerate an effective dose. Of the remaining 19, 17 (89%) responded, including 10 (53%) with complete cessation and 7 (37%) with reduction in frequency of paroxysms. The disorder resolved promptly without medication in 2 patients who gained awareness of emotions long held from awareness. Conclusions: The psychosocial history, the marked efficacy of antidepressant agents, and the rapid cure associated with gaining of awareness of previously repressed emotions strongly support the origin of PH in repressed emotions. To date, no other cause or effective treatment has been reported.

Acute Heart Failure as a First Presentation of Pheochromocytoma Complicated with “Inverted” Takotsubo Syndrome

Takotsubo syndrome is a rare but emerging form of acute reversible myocardial injury characterized by transient systolic LV dysfunction, often related to emotional or physical stress. Pheochromocytoma is increasingly recognised as another possible trigger. Pheochromocytoma is a rare catecholamine-secreting tumour arising from chromaffin cells within the adrenal medulla or extra-adrenal paraganglia. The pathognomonic quartet of paroxysmal hypertension, palpitations, headache, and diaphoresis is rarely present, and diagnosis is often delayed. We describe a 43-year-old formerly healthy patient with an adrenal pheochromocytoma, presenting as an “inverted” takotsubo syndrome complicated with acute heart failure and pulmonary oedema.