Abstract
Pure autonomic failure (PAF) is a progressive autonomic neurodegenerative disorder.
Cold induced sweating occurred in syndromes with mutations in CRLF1 and CLCF1 genes
and in a case of cervical dissection.
A patient with PAF developed sweating induced by cool ambient temperatures. He had
severe orthostatic hypotension, abnormal cardiovagal reflexes, and paradoxical sweating
in the upper trunk at a room temperature of 18 °C. Skin biopsy showed involvement of somatic epidermal unmyelinated nerve fibers.
Quantitative sensory testing showed abnormal thresholds to all thermal modalities.
Possible mechanisms include cold induced noradrenaline release in remaining autonomic
innervation and a supersensitive sudomotor response.
Keywords
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References
- Differences in skin sympathetic involvements between two chronic autonomic disorders: multiple system atrophy and pure autonomic failure.Parkinsonism Relat. Disord. 2009; 15: 347-350
- Sudomotor function in autonomic failure.Neurology. 1991; 41: 1564-1566
- Somatic and autonomic function in progressive autonomic failure and multiple system atrophy.Ann. Neurol. 1987; 22: 692-699
- Autonomic innervation in multiple system atrophy and pure autonomic failure.J. Neurol. Neurosurg. Psychiatry. 2010; 81: 1327-1335
- Progression of cardiovascular autonomic dysfunction in Holmes–Adie syndrome.J. Neurol. Neurosurg. Psychiatry. 2011; 82: 1046-1049
- Cold-induced sweating syndrome: CISS1 and CISS2: manifestations from infancy to adulthood. Four new cases.J. Neurol. Sci. 2010; 293: 68-75
- Successful treatment of cold-induced sweating in Crisponi syndrome and its possible mechanism of action.Dev. Med. Child Neurol. 2010; 52: 494-497
- Paradoxical focal hyperhidrosis in response to cold after faulty regeneration.Eur. Neurol. 2004; 51: 241-243
- Pure Autonomic Failure. Bradbury Eggleston Syndrome.Rev. Med. Chil. 2005; 133: 215-218
- Pure autonomic failure: a restricted Lewy body synucleinopathy or early Parkinson disease?.Neurology. 2000; 74: 536-537
- Chronic cough in the Holmes–Adie syndrome: association in five cases with autonomic dysfunction.J. Neurol. Neurosurg. Psychiatry. 1998; 65: 583-586
- Cold-induced sweating syndrome is caused by mutations in the CRLF1 gene.Am. J. Hum. Genet. 2003; 72: 375-383
- EFNS guidelines on the use of skin biopsy in the diagnosis of peripheral neuropathy.Eur. J. Neurol. 2005; 12: 747-758
- Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy.J. Neurol. Neurosurg. Psychiatry. 2005; 76: 947-952
- The sympathetic skin response in peripheral autonomic failure-evaluation in pure failure, pure cholinergicdysautonomia and dopamine-beta-hydroxylase deficiency.Clin. Auton. Res. 1998; 8: 133-138
- Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects.Brain. 2006; 129: 2119-2131
- Inactivation of cardiotrophin-like cytokine, a second ligand for ciliary neurotrophic factor receptor, leads to cold induced sweating syndrome in a patient.PNAS. 2006; 103: 1068-1073
- Quantitative somatosensory thermotest. A key method for functional evaluation of small calibre afferent channels.Brain. 1992; 115: 893-913
Article info
Publication history
Published online: March 18, 2013
Accepted:
February 20,
2013
Received in revised form:
January 7,
2013
Received:
August 26,
2012
Identification
Copyright
© 2013 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
