The Role of Dexmedetomidine in Paroxysmal Sympathetic Hyperactivity: A Systematic Review.

OBJECTIVE: The objective was to evaluate the efficacy and safety of dexmedetomidine in the treatment and prophylaxis of paroxysmal sympathetic hyperactivity (PSH). DATA SOURCES: A review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria and queried Embase, MEDLINE (PubMed), Cochrane CENTRAL, Web of Science, SciELO, Korean Journal Index (Clarivate), Global Index Medicus, and CINAHL Plus for results through June 2023. STUDY SELECTION AND DATA EXTRACTION: Studies providing efficacy or safety data associated with dexmedetomidine with a reported diagnosis of PSH were included. Exclusion of studies in pediatric populations, without quantitative and qualitative outcome data, and not readily translatable to English was adhered to. DATA SYNTHESIS: Thirteen observational studies of 178 patients were included in the qualitative analysis. Reductions in PSH frequency or symptom severity were reported in 44 of 48 patients who received dexmedetomidine for acute treatment. Prophylactic use of dexmedetomidine was associated with reductions in PSH-Assessment Measure (PSH-AM) scores in postsurgical patients with traumatic brain injuries (TBIs). Adverse events associated with dexmedetomidine were either absent or reported as none. RELEVANCE TO PATIENT CARE AND CLINICAL PRACTICE: This review supports the safe and effective use of dexmedetomidine in the treatment and prophylaxis of PSH. Further investigation is required to determine optimal dosing strategies and the extent to which PSH etiology correlated to the efficacy of dexmedetomidine. CONCLUSIONS: The use of dexmedetomidine appears to be both efficacious and safe for the treatment and prevention of PSH in patients experiencing a TBI. Additional research is needed to elucidate dosing strategies, titration parameters, and duration of therapy.

Successful cerebral intravascular thrombectomy stops the heart stopping.

A 65-year-old man presented with an acute ischaemic stroke due to right posterior cerebral artery occlusion, complicated by episodes of sinus arrest in the absence of intrinsic cardiac disease. His neurological deficits and sinus node dysfunction resolved following mechanical thrombectomy. We believe this to be a novel case where thrombectomy resulted in successful treatment of cerebral ischaemia mediated cardiac autonomic dysfunction.

Paralytic ileus as the presenting symptom for Guillain-Barré syndrome: a case report.

Guillain-Barré syndrome (GBS) is an acute neuroimmunological disorder characterized by rapidly ascending symmetrical limb weakness, areflexia, and sensory deficits. Approximately 65% of patients with GBS present with autonomic dysfunction, which commonly occurs in advanced stages. However, paralytic ileus, a sign of gastrointestinal dysautonomia, is rare as the presenting feature in GBS before motor weakness becomes evident. We report the case of a 54-year-old man admitted to the Emergency Department with paralytic ileus as the prodromal feature in early-stage GBS. Total parenteral feeding and prokinetic use were initiated, but no clinical improvement was observed. The patient showed rapid progression to quadriplegia, which was ultimately determined to be respiratory muscle failure requiring mechanical ventilation and intensive care unit admission. He underwent 5 days of intravenous immunoglobulin therapy and muscle strength was partially improved thereafter. However, the patient's enteral nutritional support was undesirable because of persistent poor gastric emptying complicated by fungemia and profound sepsis throughout the hospital course. Finally, he died 1 month after admission. Ignorance of this unusual prodrome to GBS could result in delayed treatment, along with potential progression to life-threatening events. Early recognition of GBS and prompt immunotherapy are critical for reducing morbidity and mortality.

Anesthetic experience of frontotemporal dementia patient with severe autonomic dysfunction: a case report.

Anesthetic experience in frontotemporal dementia (FTD) with severe hypotension associated autonomic dysfunction has not yet been reported. Here in case, we report on the case of treatment with vasopressin to refractory hypotension in FTD patient. A 54-year-old male presented with a ten-year history of FTD with frequent syncope. The patient was scheduled to undergo subtotal gastrectomy for resection of stomach cancer. During the operation, sudden hypotension occurred and it was refractory to fluid and 1 unit of blood resuscitation and did not respond to catecholamine. Transesophageal echocardiography showed normal heart with adequate volume state. After intravenous administration of arginine vasopressin, the patient's vital signs returned to baseline values. Arginine vasopressin might be considered as a valuable alternative for treatment of severe refractory hypotension in autonomic dysfunction patients with FTD.

Dominant Transmission Observed in Adolescents and Families With Orthostatic Intolerance.

BACKGROUND: Orthostatic intolerance is typically thought to be sporadic and attributed to cerebral autonomic dysfunction. We sought to identify families with inherited autonomic dysfunction manifest as symptomatic orthostatic intolerance to characterize mode of inheritance and clinical features. METHODS: Sixteen families with two or more first- or second-degree relatives with autonomic dysfunction and orthostatic intolerance were enrolled. A clinical diagnosis of autonomic dysfunction defined by symptomatic orthostatic intolerance diagnosed by head-up tilt table testing was confirmed for each proband. Clinical features and evaluation were obtained from each proband using a standardized intake questionnaire, and family history information was obtained from probands and available relatives. RESULTS: Comprehensive pedigree analysis of 16 families (39 individuals with orthostatic intolerance and 40 individuals suspected of having orthostatic intolerance) demonstrated dominant transmission of autonomic dysfunction with incomplete penetrance. Affected individuals were predominantly female (71.8%, 28/39; F:M, 2.5:1). Male-to-male transmission, although less common, was observed and demonstrated to transmit through unaffected males with an affected parent. Similar to sporadic orthostatic intolerance, probands report a range of symptoms across multiple organ systems, with headaches and neuromuscular features being most common. CONCLUSIONS: Familial occurrence and vertical transmission of autonomic dysfunction in 16 families suggest a novel genetic syndrome with dominant transmission, incomplete penetrance, and skewing of the sex ratio. Elucidation of potential genetic contributions to orthostatic intolerance may inform therapeutic management and identification of individuals at risk. Adolescent evaluation should include identification and treatment of potential at-risk relatives.

The addition of renal sympathetic denervation to pulmonary vein isolation reduces recurrence of paroxysmal atrial fibrillation in chronic kidney disease patients.

BACKGROUND: Atrial fibrillation (AF) frequently complicates chronic kidney disease (CKD). AF treatment is challenging and requires complete pulmonary vein isolation (PVI). Recently, renal sympathetic denervation (RSD) has been reported to reduce AF recurrence when performed alongside PVI. METHODS: A prospective therapeutic study of patients with controlled hypertension and paroxysmal AF was undertaken. Renal function was evaluated using estimated glomerular filtration rate. Outcomes for patients with normal renal function who underwent PVI (n = 101) were compared with those for CKD patients who underwent either PVI alone (n = 96) or PVI + RSD (n = 39). The primary endpoint was recurrence of AF recorded by 24-h Holter monitoring. RESULTS: During the 22.4 ± 12.1 months following intervention, the incidence of AF recurrence was higher in CKD patients treated with PVI alone (61.5 %) than in CKD patients treated with PVI + RSD (38.5 %; HR 1.86, 95 % CI 1.14-3.03, P = 0.0251) or patients without CKD subjected to PVI (35.6 %; hazard ratio (HR) 2.27, 95 % confidence interval (CI) 1.51-3.42, P < 0.0001). In particular, the addition of RSD to PVI significantly reduced AF recurrence in CKD stage 4, but not stage 2 or 3, patients. Ambulatory blood pressure and mean heart rate were not different between groups or time points. No complications of either procedure were observed. CONCLUSIONS: PVI + RSD is a safe treatment that is superior to PVI alone for treatment of paroxysmal AF in CKD patients.

A longitudinal study of a family with adult-onset autosomal dominant leukodystrophy: Clinical, autonomic and neuropsychological findings.

BACKGROUND AND PURPOSE: Adult-onset autosomal dominant leukodystrophy (ADLD) is a rare progressive neurological disorder caused by Lamin B1 duplication (LMNB1). Our aim was to investigate longitudinally the pattern of the autonomic dysfunction and the degree of neuropsychological involvement. METHODS: Three related ADLD patients and one asymptomatic carrier of LMNB1 duplication underwent a standardized evaluation of autonomic nervous system, including cardiovascular reflexes, pharmacological testing, microneurography, skin biopsy, Metaiodobenzylguanidine scintigraphy and a complete neuropsychological battery. RESULTS: An early neurogenic orthostatic hypotension was detected in all patients and confirmed by a low rise in noradrenaline levels on Tilt Test. However infusion of noradrenaline resulted in normal blood pressure rise as well as the infusion of clonidine. At the insulin tolerance test the increase in adrenaline resulted pathological in two out three patients. Microneurography failed to detect muscle sympathetic nerve activity bursts. Skin biopsy revealed a poor adrenergic innervation, while cardiac sympathetic nerves were normal. None of ADLD patients showed a global cognitive deficit but a selective impairment in the executive functions. CONCLUSION: Autonomic disorder in ADLD involves selectively the postganglionic sympathetic system including the sympatho-adrenal response. Cognitive involvement consisting in an early impairment of executive tasks that might precede brain MR abnormalities.

Cost considerations in the treatment of anal fissures.

Anal fissure is a split in the lining of the distal anal canal. Lateral internal sphincterotomy remains the gold standard for treatment of anal fissure. Although technique is simple and effective, a drawback of this surgical procedure is its potential to cause minor but some times permanent alteration in rectal continence. Conservative approaches (such as topical application of ointment or botulinum toxin injections) have been proposed in order to treat this condition without any risk of permanent injury of the internal anal sphincter. These treatments are effective in a large number of patients. Furthermore, with the ready availability of medical therapies to induce healing of anal fissure, the risk of a first-line surgical approach is difficult to justify. The conservative treatments have a lower cost than surgery. Moreover, evaluation of the actual costs of each therapeutic option is important especially in times of economic crisis and downsizing of health spending.