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The present literature review aims to present the physiology of paroxysmal sympathetic hyperactivity (PSH) as well as its clinical course, conceptualizing them, and establishing its diagnosis and treatment. Paroxysmal sympathetic hyperactivity is a rare syndrome, which often presents after an acute traumatic brain injury. Characterized by a hyperactivity of the sympathetic nervous system, when diagnosed in its pure form, its symptomatologic presentation is through tachycardia, tachypnea, hyperthermia, hypertension, dystonia, and sialorrhea. The treatment of PSH is basically pharmacological, using central nervous system suppressors; however, the nonmedication approach is closely associated with a reduction in external stimuli, such as visual and auditory stimuli. Mismanagement can lead to the development of serious cardiovascular and diencephalic complications, and the need for neurosurgeons and neurointensivists to know about PSH is evident in order to provide a fast and accurate treatment of this syndrome.

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Acute-onset and severe sensory and autonomic deficits with no motor dysfunction, typically preceded by a febrile illness, with poor recovery, and often fatal outcome are the hallmark features of acute sensory and autonomic neuronopathy (ASANN). Pathologically and electrophysiologically, ASANN is characterized by an extensive ganglionopathy affecting sensory and autonomic ganglia with preservation of motor neurons. Consequently, patients, usually children or young adult, develop acute-onset profound widespread loss of all sensory modalities resulting in automutilations, as well as autonomic failure causing neurogenic orthostatic hypotension, neurogenic underactive bladder, and gastroparesis and constipation. The diagnosis is clinical with support of nerve conduction studies and autonomic testing, as well as spinal cord magnetic resonance imaging showing characteristic posterior cord hyperintensities. Although the presumed etiology is immune-mediated, further studies are required to clarify the physiopathology of the disease. We here performed a systematic review of the epidemiology, pathophysiology, diagnosis, and management of ASANN, with three representative cases that recently presented at our clinic. All three patients had the typical clinical manifestations of ASANN but in different combinations, illustrating the variable phenotype of the disorder. Immunosuppression is seldom effective. Management options are limited to supportive and symptomatic care with the goal of minimizing complications and preventing death.

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OBJECTIVE: To evaluate the effects of physical exercise on autonomic dysfunction in patients with fibromyalgia syndrome (FM). LITERATURE SURVEY AND METHODOLOGY: A systematic review of experimental studies published until December 2017 that analyzed the effect of physical exercise on autonomic dysfunction in patients with FM was performed using the PubMed, Pedro, Scopus, ScienceDirect, and Web of Science databases. SYNTHESIS: A total of 1105 articles were identified, 12 of which were included in the final analysis. The most analyzed exercise modalities were aerobic and resistance exercises. Overall, the studies demonstrated that aerobic exercise performed twice a week with moderate to high intensity was effective in reducing autonomic dysfunction by increasing heart rate variability. Resistance training was associated with reduced symptoms of anxiety and depression as well as increased muscle strength; however, it did not reduce autonomic dysfunction in these patients in the short or long term. CONCLUSIONS: Preliminary evidence suggests that aerobic exercise reduces autonomic dysfunction in patients with FM, whereas resistance training reduces psychological symptoms such as anxiety and depression. LEVEL OF EVIDENCE: I.

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Diabetes is one of the most common chronic pathologies around the world, involving treatment with general clinicians, endocrinologists, cardiologists, ophthalmologists, nephrologists and a multidisciplinary team. Patients with type 2 Diabetes Mellitus (T2DM) can be affected by cardiac autonomic neuropathy, leading to increased mortality and morbidity. In this review, we will present current concepts, clinical features, diagnosis, prognosis, and possible treatment. New drugs recently developed to reduce glycemic level presented a pleiotropic effect of reducing sudden death, suggesting a potential use in patients at risk.

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SUMMARY Diabetes is one of the most common chronic pathologies around the world, involving treatment with general clinicians, endocrinologists, cardiologists, ophthalmologists, nephrologists and a multidisciplinary team. Patients with type 2 Diabetes Mellitus (T2DM) can be affected by cardiac autonomic neuropathy, leading to increased mortality and morbidity. In this review, we will present current concepts, clinical features, diagnosis, prognosis, and possible treatment. New drugs recently developed to reduce glycemic level presented a pleiotropic effect of reducing sudden death, suggesting a potential use in patients at risk.

RESUMO Diabetes é uma das mais frequentes patologias crônicas em todo o mundo, cujo tratamento envolve uma equipe multidisciplinar, médicos generalistas, endocrinologistas, cardiologistas, nefrologistas e oftalmologistas. Pacientes com diabetes mellitus tipo 2 (DMT2) podem apresentar neuropatia autonômica cardíaca (NAC), levando a aumento de mortalidade e morbidade. Nesta revisão, apresentaremos atuais conceitos, características clínicas, diagnóstico, prognóstico e possíveis tratamentos. Novas drogas recentemente desenvolvidas para redução de níveis glicêmicos apresentaram efeito pleiotrópico de redução de morte súbita, sugerindo um potencial uso neste perfil de pacientes.

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A literatura já demonstrou que a estimulação auditiva por meio de música influencia o sistema cardiovascular. Neste estudo, foi realizada uma revisão da literatura, a fim de investigar a relação entre os mecanismos auditivos e a regulação autonômica cardíaca. Os estudos selecionados indicaram forte correlação entre a intensidade do ruído e o equilíbrio simpatovagal. Além disso, foi relatado que a terapia com música melhorou a variabilidade da frequência cardíaca em pacientes com câncer de mama tratados com antraciclinas. Postula-se que a dopamina liberada no sistema estriatal, induzida por canções alegres, está envolvida na regulação autonômica. Estudos posteriores sãonecessários para adicionar novos elementos na literatura, para melhorar a novas terapias e para o tratar doenças cardiovasculares.

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Presentamos el caso de un hombre de 24 años que, secundario a un accidente de tránsito, presentó un traumatismo encefalocraneano grave con daño axonal difuso. Luego de un mes de evolución en la unidad de cuidados intensivos comenzó a presentar episodios súbitos de hipertensión, taquicardia, diaforesis, hipertermia, descerebración y dilatación pupilar, todo lo anterior con resolución espontánea en el curso de minutos. Estas crisis se repetían varias veces en el día. Se llegó al diagnóstico de un cuadro denominado Tormenta Simpática Paroxística que puede presentarse muy ocasionalmente como consecuencia de una lesión cerebral grave, especialmente el daño axonal postraumático. En este trastorno prima un desbalance simpático/parasimpático, lo que podría deberse a una pérdida del control cortical. El paciente fue tratado con opiáceos y betabloqueo con una respuesta satisfactoria, logrando disminuir significativamente sus episodios de tormentas simpáticas paroxísticas. En suma, nos parece importante comunicar esta experiencia dado la alta prevalencia de pacientes con trauma cerebral en nuestras Unidades de Pacientes Críticos. A pesar de que ni su diagnóstico ni tratamiento mejoran el pronóstico, su reconocimiento ahorra estudios innecesarios y permite iniciar una terapia sencilla que lleva al control precoz de la sintomatología.

A 24-year-old man suffered a traumatic brain injury due to a car accident. After one month of hospitalization in intensive care unit, he experimented episodic crisis of hypertension, tachycardia, hyperhidrosis, hypertermia, extensor posturing and pupil dilatation. This events presented in average 10 times per day. We reached to the diagnosis of Paroxysmal Sympathetic Storm. This is a subtype of dysautonomy which is present occasionally after a brain traumatic injury. The main mechanism of this brain dysfunction is a disassociation between the sympathetic and parasympathetic nervous systems due to cortical control loss. The patient was treated with morphine and labetalol and he experimented an excellent response, reducing his episodes of paroxysmal sympathetic storm by 80 percent. The aim of this review is to communicate this entity because a prompt and accurate diagnosis could minimize unnecessary studies and treatments.

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Syncope associated to orthostatic hypotension (OH), urinary incontinence and constipation is common symptoms in demented patients, mainly in dementia with Lewy bodies (DLB) and in Parkinson's disease dementia (PDD). Alzheimer's disease (AD) and fronto temporal lobar degeneration (FTLD) show less autonomic dysfunction. Urinary symptoms are a prominent component of normal pressure hydrocephalus (NPH). There are non invasive tests including standard cardiovascular tests, 123 I-metaiodobenzylguanide (MIBG) cardiac scintigraphy, urodynamic tests, gastrointestinal motility studies, sweating reflexes and pupillary responses that assess autonomic dysfunction in these patients. The study of autonomic symptoms and abnormal tests in patients with dementia is useful to prevent morbidity due falls, severe constipation and to avoid side effects of drugs that interfere with autonomic function.

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