Hyperhidrosis in sleep disorders - A narrative review of mechanisms and clinical significance.

Hyperhidrosis is characterized by excessive sweating beyond thermoregulatory needs that affects patients' quality of life. It results from an excessive stimulation of eccrine sweat glands in the skin by the sympathetic nervous system. Hyperhidrosis may be primary or secondary to an underlying cause. Nocturnal hyperhidrosis is associated with different sleep disorders, such as obstructive sleep apnea, insomnia, restless legs syndrome/periodic limb movement during sleep and narcolepsy. The major cause of the hyperhidrosis is sympathetic overactivity and, in the case of narcolepsy type 1, orexin deficiency may also contribute. In this narrative review, we will provide an outline of the possible mechanisms underlying sudomotor dysfunction and the resulting nocturnal hyperhidrosis in these different sleep disorders and explore its clinical relevance.

Epidemiology of Parkinson's Disease in Chile.

INTRODUCTION: Parkinson's disease (PD) is one of the most common neurodegenerative disorders. There is no epidemiological description of PD in Chile and not many descriptions in Latin America. This study aims to describe the incidence and prevalence of PD in Chile. METHODS: The study group was the population on the public health system in Chile between 2010 and 2018 that were registered in the GES system as having PD. Crude and standardized prevalence and incidence were calculated with a 95% confidence interval. RESULTS: 33,345 patients were found in the register as confirmed cases with PD. The crude incidence in 2018 was 23.7/100,000; the crude prevalence in 2018 was 160.7/100,000. The male-to-female ratio was 1.03. CONCLUSION: The prevalence and incidence observed in the Chilean population are consistent with studies from other countries.

Neurogenic Orthostatic Hypotension. Lessons From Synucleinopathies.

Maintenance of upright blood pressure critically depends on the autonomic nervous system and its failure leads to neurogenic orthostatic hypotension (NOH). The most severe cases are seen in neurodegenerative disorders caused by abnormal α-synuclein deposits: multiple system atrophy (MSA), Parkinson's disease, Lewy body dementia, and pure autonomic failure (PAF). The development of novel treatments for NOH derives from research in these disorders. We provide a brief review of their underlying pathophysiology relevant to understand the rationale behind treatment options for NOH. The goal of treatment is not to normalize blood pressure but rather to improve quality of life and prevent syncope and falls by reducing symptoms of cerebral hypoperfusion. Patients not able to recognize NOH symptoms are at a higher risk for falls. The first step in the management of NOH is to educate patients on how to avoid high-risk situations and providers to identify medications that trigger or worsen NOH. Conservative countermeasures, including diet and compression garments, should always precede pharmacologic therapies. Volume expanders (fludrocortisone and desmopressin) should be used with caution. Drugs that enhance residual sympathetic tone (pyridostigmine and atomoxetine) are more effective in patients with mild disease and in MSA patients with spared postganglionic fibers. Norepinephrine replacement therapy (midodrine and droxidopa) is more effective in patients with neurodegeneration of peripheral noradrenergic fibers like PAF. NOH is often associated with other cardiovascular diseases, most notably supine hypertension, and treatment should be adapted to their presence.

Epidemiology of chronic inflammatory demyelinating polyneuropathy in the South-Eastern area of Santiago, Chile.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy. It usually has an insidious onset, progressive course and heterogeneous clinical features. As far as we know, there is no epidemiological information on CIDP in South America and the Caribbean. Our aim was to estimate the frequency of CIDP in the South-Eastern region of Santiago, where our hospital is based and the population number assigned is officially reported every year by the health authorities. Records of 581 patients registered with the diagnosis of neuropathy were found and all patients meeting the diagnostic criteria of the EFNS/PNS for definitive and possible CIDP were included. Data were collected using a data extraction protocol designed by the authors and which included demographic, clinical, laboratory and electrophysiological information. The estimated prevalence and incidence of CIDP were 2.95/100,000 and 0.46/100,000 respectively. Fifteen patients (8 men, 7 women) were classified as definitive or possible CIDP. Nine patients had typical CIDP and three also had diabetes mellitus. The prevalence and incidence rates were similar to those reported in other regions of the world.

Descripción electrofisiològica del síndrome de túnel carpiano según edad en pacientes adultos / Electrophysiological severity of carpal tunnel syndrome according to age in adult patients

Background: Carpal tunnel syndrome (CTS) represents 90% of entrapment neuropathies. Severity may be greater in older patients. Aim: To describe the electrophysiological findings in adult patients with CTS and determine if severity is related to age. Material and Methods: Descriptive and retrospective study of electrophysiological findings in patients over 18 years of age with clinical suspicion of CTS, studied between January 2011 and December 2015. Neurophysiological severity was classified in 3 grades, comparing them by age, gender and laterality. Results: Of 1156 patients subjected to electrophysiological studies due to a clinical suspicion of CTS, 690 (60%) had electrophysiological features of the disease. In 274 patients (24%) the compromise was mild, in 162 (14%) it was moderate and in 254 (22%) it was severe. There was a positive association between age and CTS severity (p < 0.01). Severity was significantly greater in males than females (p < 0.01). Bilateral CTS was present in 471 patients (68%), which was associated with increased age and severity (p < 0.01). Conclusions: Electrophysiological severity in CTS increases with age. Other factors associated with higher severity are male gender and bilateral disease.

Recessive dystrophic epidermolysis bullosa results in painful small fibre neuropathy.

Small fibres in the skin are vulnerable to damage in metabolic or toxic conditions such as diabetes mellitus or chemotherapy resulting in small fibre neuropathy and associated neuropathic pain. Whether injury to the most distal portion of sensory small fibres due to a primary dermatological disorder can cause neuropathic pain is still unclear. Recessive dystrophic epidermolysis bullosa (RDEB) is a rare condition in which mutations of proteins of the dermo-epidermal junction lead to cycles of blistering followed by regeneration of the skin. Damage is exclusive to the skin and mucous membranes, with no known direct compromise of the nervous system. It is increasingly recognized that most RDEB patients experience daily pain, the aetiology of which is unclear but may include inflammation (in the wounds), musculoskeletal (due to atrophy and retraction scars limiting movement) or neuropathic pain. In this study we investigated the incidence of neuropathic pain and examined the presence of nerve dysfunction in RDEB patients. Around three quarters of patients presented with pain of neuropathic characteristics, which had a length-dependent distribution. Quantitative sensory testing of the foot revealed striking impairments in thermal detection thresholds combined with an increased mechanical pain sensitivity and wind up ratio (temporal summation of noxious mechanical stimuli). Nerve conduction studies showed normal large fibre sensory and motor nerve conduction; however, skin biopsy showed a significant decrease in intraepidermal nerve fibre density. Autonomic nervous system testing revealed no abnormalities in heart rate and blood pressure variability however the sympathetic skin response of the foot was impaired and sweat gland innervation was reduced. We conclude that chronic cutaneous injury can lead to injury and dysfunction of the most distal part of small sensory fibres in a length-dependent distribution resulting in disabling neuropathic pain. These findings also support the use of neuropathic pain screening tools in these patients and treatment algorithms designed to target neuropathic pain.

[Usefulness of the activities of daily living questionnaire (T-ADLQ) in patients with minor stroke]. / Cuestionario de actividades de la vida diaria (T-ADLQ): utilidad en pacientes con accidente cerebrovascular menor.

BACKGROUND: The inability to carry out activities of daily living (ADL) is prevalent in elderly people and it is associated with hypertension and stroke. AIM: To evaluate ADLs using the T-ADLQ in hypertensive patients with minor stroke. SUBJECTS AND METHODS: T-ADLQ, Cognitive tests (Minimental and Addenbrooke), and Hamilton depression test were applied to 100 hypertensive ambulatory patients (55 without symptomatic stroke and 45 with ischemic stroke, Rankin ≤ 2). RESULTS: In stroke patients the ability to perform ADL was significantly reduced compared with hypertensive patients without stroke. Cognitive dysfunction and depressive symptoms were associated with a lower ADL performance. CONCLUSIONS: The T-ADLQ is useful to evaluate ADL in hypertensive ambulatory patients with ischemic stroke.

Cuestionario de actividades de la vida diaria (T-ADLQ): utilidad en pacientes con accidente cerebrovascular menor / Usefulness of the activities of daily living questionnaire (T-ADLQ) in patients with minor stroke

Background: The inability to carry out activities of daily living (ADL) is prevalent in elderly people and it is associated with hypertension and stroke. Aim: To evaluate ADLs using the T-ADLQ in hypertensive patients with minor stroke. Subjects and Methods: T-ADLQ, Cognitive tests (Minimental and Addenbrooke), and Hamilton depression test were applied to 100 hypertensive ambulatory patients (55 without symptomatic stroke and 45 with ischemic stroke, Rankin ≤ 2). Results: In stroke patients the ability to perform ADL was significantly reduced compared with hypertensive patients without stroke. Cognitive dysfunction and depressive symptoms were associated with a lower ADL performance. Conclusions: The T-ADLQ is useful to evaluate ADL in hypertensive ambulatory patients with ischemic stroke.

Carotid body chemoreceptors, sympathetic neural activation, and cardiometabolic disease.

The carotid body (CB) is the main peripheral chemoreceptor that senses the arterial PO2, PCO2 and pH. In response to hypoxemia, hypercapnia and acidosis, carotid chemosensory discharge elicits reflex respiratory, autonomic and cardiovascular adjustments. The classical construct considers the CB as the main peripheral oxygen sensor, triggering reflex physiological responses to acute hypoxemia and facilitating the ventilatory acclimation to chronic hypoxemia at high altitude. However, a growing body of experimental evidence supports the novel concept that an abnormally enhanced CB chemosensory input to the brainstem contributes to overactivation of the sympathetic nervous system, and consequent pathology. Indeed, the CB has been implicated in several diseases associated with increases in central sympathetic outflow. These include hypertension, heart failure, sleep apnea, chronic obstructive pulmonary disease and metabolic syndrome. Indeed, ablation of the CB has been proposed for the treatment of severe and resistant hypertension in humans. In this review, we will analyze and discuss new evidence supporting an important role for the CB chemoreceptor in the progression of autonomic and cardiorespiratory alterations induced by heart failure, obstructive sleep apnea, chronic obstructive pulmonary disease and metabolic syndrome.

Carotid body Thermoreceptors, sympathetic neural activation, and cardiometabolic disease

The carotid body (CB) is the main peripheral chemoreceptor that senses the arterial PO2, PCO2 and pH. In response to hypoxemia, hypercapnia and acidosis, carotid chemosensory discharge elicits reflex respiratory, autonomic and cardiovascular adjustments. The classical construct considers the CB as the main peripheral oxygen sensor, triggering reflex physiological responses to acute hypoxemia and facilitating the ventilatory acclimation to chronic hypoxemia at high altitude. However, a growing body of experimental evidence supports the novel concept that an abnormally enhanced CB chemosensory input to the brainstem contributes to overactivation of the sympathetic nervous system, and consequent pathology. Indeed, the CB has been implicated in several diseases associated with increases in central sympathetic outflow. These include hypertension, heart failure, sleep apnea, chronic obstructive pulmonary disease and metabolic syndrome. Indeed, ablation of the CB has been proposed for the treatment of severe and resistant hypertension in humans. In this review, we will analyze and discuss new evidence supporting an important role for the CB chemoreceptor in the progression of autonomic and cardiorespiratory alterations induced by heart failure, obstructive sleep apnea, chronic obstructive pulmonary disease and metabolic syndrome.

Autonomic dysfunction in neurodegenerative dementias.

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.

Transient orthostatic hypertension after partial cerebellar resection.

An effective baroreflex and autonomic pathways normally ensure that blood pressure (BP) is satisfactorily maintained, despite various stimuli in daily life that include postural changes. We describe a 20-year-old man with a cerebellar hematoma and acute hydrocephalus, who had a vermian and partial right cerebellar hemisphere resection followed by orthostatic hypertension (OHT) and mutism. On standing his systolic BP rose over 60 mmHg with a fivefold increase in plasma noradrenaline. After a period of 8 weeks, postural BP regulation improved along with his ability to communicate. We conclude that transient impairment of cerebellar autonomic modulation or dysfunction of the baroreflex medullary circuit, may have resulted in OHT.

[Primary vasculitis of the central nervous system: report of one case]. / Vasculitis primaria del sistema nervioso central, diagnóstico diferencial de demencia subaguda: Caso clínico.

A primary central nervous system vasculitis is an uncommon and invalidating disease, which has a fatal course if left untreated. We report a 63 year-old woman presenting with a history of two months of cognitive impairment, dysarthria, gait instability and tremor. After four months of evolution a right hemianopsia and a flaccid paresis of upper right limb appeared. A brain biopsy was performed and the histological findings confirmed the suspicion of primary cerebral vasculitis. The patient was treated with cyclophosphamide and prednisone, observing a partial recovery of cognitive and motor function.

Autonomic and cognitive dysfunction in Parkinson's disease.

OBJECTIVE: To investigate whether there is an association between autonomic failure and cognitive impairment in patients with idiopathic Parkinson's disease (PD) METHODS: 40 PD patients and 30 age matched controls were assessed for cognitive and behavioral manifestations using the Mini-Mental State Examination (MMSE), the Frontal Assessment Battery (FAB), the Blessed scale and Cornell scale for depression. The subjects were also assessed for orthostatic hypotension (OH), postprandial hypotension (PPH), heart rate responses to deep breathing (HR(DB)) and autonomic symptoms using the Scale for Outcomes in PD for autonomic symptoms (SCOPA AUT). RESULTS: There was a correlation between the severity of motor symptoms and cognitive impairment in our PD patients. Eleven of the 40 PD patients fulfilled the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) criteria of dementia. The presence of OH or PPH did not correlate with the severity of cognitive impairment in our PD cases. However, PD patients with dementia reported more cardiovascular symptoms than PD patients without dementia. There was no correlation between gastrointestinal or urologic symptoms and cognitive impairment in our PD cases. CONCLUSION: The results of this limited study indicate that despite the higher incidence of cardiovascular symptoms in PD patients with dementia than in those without dementia, there is no consistent association between OH or PPH and cognitive deficits in PD. The lack of correlation between OH, gastrointestinal and urinary symptoms with cognitive impairment suggests that cognitive and autonomic involvement progresses independently from each other and variably among PD patients.