Clinical Autonomic Research

Induced pluripotent stem cells in multiple system atrophy: recent developments and scientific challenges Abstract Multiple system atrophy (MSA) is a rare and fatal neurodegenerative disease, with no known genetic cause to date. Oligodendroglial α-synuclein accumulation, neuroinflammation, and early myelin dysfunction are hallmark features of the disease and have been modeled in part in various preclinical models of MSA, yet the pathophysiology of MSA remains elusive. Here, we review the role and scientific challenges of induced pluripotent stem cells in the detection of novel biomarkers and druggable targets in MSA.

Relapsing paralytic ileus in multiple sclerosis requiring surgery: a video case report

Post-stroke short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA): response to lamotrigine and sphenopalatine ganglion block

Autonomic nervous system dysfunction and fatigue in multiple sclerosis: common pathophysiology or spurious association?

Does clip removal help for compensatory hyperhidrosis complicating thoracic sympathetic clipping?

Do astronauts get postural tachycardia syndrome? And other updates on recent autonomic research

Autoimmune autonomic neuropathies and ganglionopathies: epidemiology, pathophysiology, and therapeutic advances Abstract Autonomic disorders can be the result of autoimmunity. The classic, well-characterized example is autoimmune autonomic ganglionopathy (AAG), in which antibodies against the ganglionic nicotinic acetylcholine receptor impair autonomic transmission, causing autonomic failure, which responds to immunotherapy. However, a number of other autoimmune disorders cause autonomic failure through a variety of mechanisms. In this article, we review autoimmune disorders causing impairment of the peripheral autonomic nervous system (ganglia and nerves), including AAG, other autoimmune autonomic neuropathies, paraneoplastic autonomic neuropathies, and neuromuscular and rheumatologic diseases with autonomic symptomatology. Awareness of primary autoimmune autonomic disorders and the autonomic manifestations of other autoimmune diseases promotes timely diagnosis and appropriate management, including supportive care for unpleasant or dangerous autonomic dysfunction, a search for underlying malignancy when indicated, and the use of immunotherapy when appropriate. A better understanding of the underlying pathophysiology aids in the judicious use and selection of immunotherapy.

Heart rate variability and baroreflex sensitivity abnormalities in Guillain–Barré syndrome: a pilot study Abstract Objective The current study aimed to investigate autonomic dysfunction in Guillain–Barré syndrome (GBS) patients and describe the results of computational heart rate variability (HRV)/baroreflex sensitivity (BRS) and autonomic challenge tests. Methods GBS patients were consecutively recruited and the results were compared to age- and gender-matched healthy controls. A series of autonomic function tests including computation-dependent tests (power spectrum analysis of HRV and BRS at rest) and challenge maneuvers (deep breathing, eyeball compression, active standing, the Valsalva maneuver, sustained handgrip, and the cold pressor test) were performed. Results Ten GBS patients (six men; mean age = 40.1 ± 13.9 years) and ten gender- and age-matched healthy controls were recruited. The mean GBS functional grading scale at disease plateau was 3.4 ± 1.0. No patients required intensive care unit admission or mechanical ventilation. Low-frequency HRV (p = 0.027), high-frequency HRV (p = 0.008), and the total power spectral density of HRV (p = 0.015) were significantly reduced in patients compared to controls. The mean up slope (p = 0.034), down slope (p = 0.011), and total slope (p = 0.024) BRS were significantly lower in GBS patients. The diastolic rise in blood pressure in the cold pressor test was significantly lower in GBS patients compared to controls (p = 0.008). Interpretation Computation-dependent tests (HRV and BRS) were more useful for detecting autonomic dysfunction in GBS patients, whereas the cold pressor test was the only reliable challenge test, making it useful as a bedside measure of autonomic function in GBS patients.

Autonomic dysfunction in the neurological intensive care unit Abstract Autonomic dysfunction is common in neuro-critical care patients and may compromise the function of various organs. Among the many diseases causing or being associated with autonomic dysfunction are traumatic brain injury, cerebrovascular diseases, epilepsy, Guillain–Barré syndrome (GBS), alcohol withdrawal syndrome, botulism and tetanus, among many others. Autonomic dysfunction may afflict various organs and may involve hyper- or hypo-activity of the sympathetic or parasympathetic system. In this short overview, we address only a small number of neuro-intensive care diseases with autonomic dysfunction. In GBS, autonomic dysfunction is frequent and may account for increased mortality rates; rapid changes between sympathetic and parasympathetic hypo- or hyper-activity may cause life-threatening cardiovascular complications. Paroxysmal sympathetic hyperactivity occurs after brain injury, hypoxia and cerebrovascular and other events, causes paroxysmal tachycardia, hypertension, tachypnoea and hyperthermia and is associated with a poorer prognosis and prolonged intensive care treatment. Other, at times life-threatening autonomic complications with exaggerated sympathetic activity and compromised baroreflex sensitivity arise during the alcohol withdrawal syndrome triggered by abrupt cessation of alcohol consumption. Botulism and tetanus are examples of life-threatening autonomic dysfunction caused by bacterial neurotoxins. Common neurological diseases, such as epilepsy, stroke or subarachnoid haemorrhage, are also associated with autonomic dysfunction that can on occasion cause critical deterioration of disease severity and prognosis.

Neurogenic lower urinary tract dysfunction in multiple sclerosis, neuromyelitis optica, and related disorders Abstract Purpose Multiple sclerosis (MS), neuromyelitis optica (NMO), and related disorders are major immune-mediated central nervous system diseases affecting the spinal cord. We reviewed the occurrence of neurogenic lower urinary tract dysfunction (NLUTD) in these categories of diseases. Methods We systematically reviewed the literature regarding bladder dysfunction in MS, NMO spectrum disorder (NMOSD), and related disorders (acute disseminated encephalomyelitis, acute immune-mediated myelopathy, and meningitis-retention syndrome). Results The literature, although somewhat limited for diseases other than MS, suggests that bladder dysfunction is not uncommon in these diseases, presumably reflecting lesions in the spinal descending and ascending pathways. The pattern of bladder dysfunction is a combination of overactive bladder and large post-void residuals/urinary retention. Post-void residual is measured by portable ultrasound devices. Conclusions Because of the complexity of bladder behavior, careful consideration of bladder management is necessary in MS, NMO, and related disorders: e.g., antimuscarinics, etc., for overactive bladder and clean, intermittent self-catheterization for urinary retention. These management practices should help improve patients' quality of life.

Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182

6948891480

alsfakia@gmail.com