Anhidrosis as the first sign of Ross Syndrome

Xavier, Marcus H. deS.B; Porto, Fábio Henrique de Gobbi; Pereira, Gabrilela B.; Quevedo, Luiz Peres; Freitas, Paula Martins; Nascimento, Osvaldo Jm; Freitas, Marcos R. G. de

doi:10.1590/s0004-282x2009000300025

Cited by 4 publications

(3 citation statements)

References 10 publications

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“…Another cholinergic neuropathy is Ross syndrome, which is characterized by the clinical triad of progressive segmental anhidrosis, Adie's tonic pupils, and areflexia (Macefield, 2012;Xavier et al, 2009;Ross, 1958). The anhidrosis, which may be extensive enough to cause heat intolerance, often occurs asymmetrically adjacent to areas of preserved or compensatory sweating (Xavier et al, 2009;Weller et al, 1992). Pharmacologic and histopathological studies have indicated a postganglionic cholinergic neuronal deficit (Perretti et al, 2003;Sommer et al, 2002;Wolfe et al, 1995).…”

Section: Disorders Of the Response To Heatmentioning

confidence: 99%

Thermoregulatory disorders and illness related to heat and cold stress

Cheshire

2016

Autonomic Neuroscience

239

165

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Thermoregulation is a vital function of the autonomic nervous system in response to cold and heat stress. Thermoregulatory physiology sustains health by keeping body core temperature within a degree or two of 37°C, which enables normal cellular function. Heat production and dissipation are dependent on a coordinated set of autonomic responses. The clinical detection of thermoregulatory impairment provides important diagnostic and localizing information in the evaluation of disorders that impair thermoregulatory pathways, including autonomic neuropathies and ganglionopathies. Failure of neural thermoregulatory mechanisms or exposure to extreme or sustained temperatures that overwhelm the body's thermoregulatory capacity can also result in potentially life-threatening departures from normothermia. Hypothermia, defined as a core temperature of <35.0°C, may present with shivering, respiratory depression, cardiac dysrhythmias, impaired mental function, mydriasis, hypotension, and muscle dysfunction, which can progress to cardiac arrest or coma. Management includes warming measures, hydration, and cardiovascular support. Deaths from hypothermia are twice as frequent as deaths from hyperthermia. Hyperthermia, defined as a core temperature of >40.5°C, may present with sweating, flushing, tachycardia, fatigue, lightheadedness, headache, and paresthesia, progressing to weakness, muscle cramps, oliguria, nausea, agitation, hypotension, syncope, confusion, delirium, seizures, and coma. Mental status changes and core temperature distinguish potentially fatal heat stroke from heat exhaustion. Management requires the immediate reduction of core temperature. Ice water immersion has been shown to be superior to alternative cooling measures. Avoidance of thermal risk and early recognition of cold or heat stress are the cornerstones of preventive therapy.

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Section: Disorders Of the Response To Heatmentioning

confidence: 99%

Thermoregulatory disorders and illness related to heat and cold stress

Cheshire

2016

Autonomic Neuroscience

239

165

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“… 1 , 3 , 4 , 5 In cases where there is extensive anhidrosis, the remaining eutrophic areas with functioning eccrine sweat glands may present compensatory hyperhidrosis, the symptom that most distresses the patients, who also become uncomfortable with heat intolerance. 4 , 5 , 6 The hyperchromia observed seems to be pigmentation similar to notalgia paresthetica, in face of the histologic findings.…”

Section: Discussionmentioning

confidence: 76%

Syndrome in question

Dalapicola

Veasey

Lellis

2016

An. Bras. Dermatol.

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Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years.

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“…1 As for the pathophysiology of the alterations, they are based on modifications of the nerve fibers belonging to the autonomic nervous system; in the case of Adie's pupil, there is damage to cholinergic postganglionic fibers between the ciliary ganglion and the muscular sphincter of the iris, which can be unilateral and progress to bilateral. 1 Hyporeflexia is explained by degeneration of dorsal root fibers and loss of medullary interneurons, 8 with the most affected reflexes being the patellar, Achilles, brachioradialis, tricipital, bicipital, and supinator reflexes. 3 Hypohidrosis results from damage to postganglionic sympathetic fibers innervating the sweat glands, which eventually leads to anhidrosis.…”

Section: Discussionmentioning

confidence: 99%