Selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross syndrome

Sommer, Claudia; Lindenlaub, Thies; Zillikens, Detlef; Toyka, Klaus V.; Naumann, Markus

doi:10.1002/ana.10256

Cited by 52 publications

(34 citation statements)

References 14 publications

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“…2 These beliefs are mainly derived from indirect evidence such as a consistent clinical response to modulation of the mesencephalic dopaminergic system, modifications of tic severity after electrical stimulation or lesions of some of these areas, and clinicoanatomical correlations in secondary tourettism. 1,[3][4][5][6][7] Whereas standard anatomical magnetic resonance imaging (MRI) studies are classically unremarkable in TS, volume of interest (VOI)-based volumetric MRI investigations, which mainly tested for the presence of abnormalities in basal ganglia and cortex, found abnormal volume or asymmetry in caudate or lenticular nuclei, pallidum, 8 -11 and cortical gray matter. 12 However, Devinsky's hypothesis has never been tested in these VOI-based morphometric studies.…”

Section: (ϫ40) D␤h-ir Fibers Encircle the Inner Part Of The Collagenmentioning

confidence: 99%

“…1 Autonomic innervation of dermal annexes has been extensively investigated using protein gene product (PGP) as panneuronal marker and vasoactive intestinal peptide (VIP) as autonomic marker in healthy controls 2 and in subjects with sudomotor disorders. [3][4][5][6] However, VIP immunoreactivity does not differentiate sympathetic adrenergic from sympathetic cholinergic fibers, although they differ both functionally 7 and histologically. 8 Tyrosine hydroxylase has been used as a selective marker of noradrenergic fibers in human skin, 9,10 but an extensive description of noradrenergic innervation of dermal annexes is lacking.…”

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confidence: 93%

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Skin sympathetic adrenergic innervation: An immunofluorescence confocal study

Donadio

Nolano

Provitera

et al. 2006

Annals of Neurology

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Section: (ϫ40) D␤h-ir Fibers Encircle the Inner Part Of The Collagenmentioning

confidence: 99%

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confidence: 93%

Skin sympathetic adrenergic innervation: An immunofluorescence confocal study

Donadio

Nolano

Provitera

et al. 2006

Annals of Neurology

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“…In 97 patients with diabetic neuro pathy, innervation of sweat glands correlated with glycated haemoglobin and BMI 139 . Similarly, degeneration of cholinergic fibres that innervate the sweat glands has been identified in the anhidrotic skin areas of patients with Ross syndrome 140 . This change, along with severe heat intolerance owing to the inability to sweat, could be used to distinguish patients with Ross syndrome from those with Holmes-Adie syndrome, who also exhibit sweating abnormalities 141 .…”

Section: Stromamentioning

confidence: 81%

New technologies for the assessment of neuropathies

et al. 2017

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Peripheral neuropathies are among the most common neurological disorders 1 . In routine clinical practice, diagnosis of peripheral neuropathies is typically based on clinical assessment, nerve conduction studies and needle electromyography, which can determine whether symptoms are caused by primary axonal damage or demyelinating damage. This distinction is often relevant to therapeutic choices and to prognosis, as axonal damage typically reflects an inflammatory (for example, vasculitic) or degenerative (for example, hereditary or idiopathic) process that correlates with disability, whereas demyelinating damage can reflect a hereditary neuro pathy, but more often reflects an acquired immunemediated neuropathy. Besides metabolic, inflammatory or primary degenerative neuropathies, peripheral nerves can also be damaged by compression from adjacent tissue structures, such as tendons, cysts or haematomas. In this case, proper identification of the site, nature and degree of the lesion might be challenging, but is relevant to treatment and prognosis. Moreover, some patients present with symptoms that suggest damage to small nerve fibres, which nerve conduction studies cannot be used to detect.In the past 15 years, several techniques have become available to investigate focal and diffuse peripheral neuropathies, and their reliability has widened the spectrum of diagnostic tools for clinicians and improved assessment of neuropathies. In this Review, we provide an overview of these new and emerging technologies. We first discuss peripheral nerve imaging with MRI and ultrasonography, which can provide information about the precise localization of neuro pathies and the pathological processes involved in large-fibre neuropathies, and can complement clinical and electrophysiological evaluation. We then consider techniques that can provide diagnostic information when the pathological process is restricted to small nerve fibres and is consequently not detectable with nerve conduction studies or neuroimaging. These include skin biopsy, which is established in clinical practice, and the emerging techniques of corneal confocal microscopy, laser-evoked potentials and heat-related and pain-related evoked potentials, and microneurography.Nerve imaging technology Advances in imaging techniques have made it possible to gain great insight into nerve pathology with magnetic resonance and ultrasound. Each approach offers distinct advantages, and can provide different information about damage to large myelinated fibres. Abstract | Technical advances are rapidly changing the clinical and instrumental approach to peripheral nerve diseases. Magnetic resonance neurography, diffusion tensor imaging and nerve ultrasonography are increasingly entering the diagnostic workup of peripheral neuropathies as tools that complement neurophysiology and enable investigation of proximal structures, such as plexuses and roots. Progress in the design of magnetic resonance scanners and sequences, and the development of high-frequency ultrasound probes mean th...

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“…Patients usually do not complain about one of the classical symptoms but about segmental hyperhidrosis. Only a minority of patients present with the full picture of all three symptoms [1,3,4], and there is a wide overlap with other autonomic diseases such as Adie or Harlequin syndrome, which has raised doubts whether those disorders are distinct entities [4]. While the anhidrosis appears to be a neurodegenerative process with progressive loss of cholinergic sudomotor fibers [3], the origin of hyperhidrosis is still vague.…”

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confidence: 98%