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Vincenzo Donadio - One of the best experts on this subject based on the ideXlab platform.
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75 absent cardiac and muscle sympathetic nerve activities involvement in Ross Syndrome a follow up study
Clinical Neurophysiology, 2017Co-Authors: Vincenzo Donadio, Pietro Cortelli, Enrico Fileccia, Rocco LiguoriAbstract:Ross Syndrome (RS) is a rare disorder characterized by selective involvement of post-ganglionic skin sympathetic nerve fibres with absent cholinergic sudomotor fibres as hallmark of the disease. It is still not well defined whether autonomic dysfunction remain localized to skin fibres or it affects also cardiovascular autonomic system. To clarify this aspect, we report a cardiovascular autonomic system follow-up study in 4 patients affected by Ross Syndrome. We studied 4 patients affected by RS for a follow-up mean period of 5 years (range 1–10 years). All patients complained of anhydrosis with heat intolerance and showed areflexia and tonic pupil on neurological examination and absent cholinergic sudomotor fibres on skin biopsy. All patients underwent cardiovascular reflexes (CVR, head-up tilt test, Valsalva maneuver, deep breathing, isometric handgrip) and microneurography recording of muscle sympathetic nerve activity (MSNA) from common peroneal nerve. CVR and MSNA resulted normal at baseline and unchanged over the follow-up in all patients although anhydrosis was usually widespread on patient’s clinical re-evaluation. Our preliminary study shows that cardiovascular autonomic system is not affected in Ross Syndrome differently from skin autonomic activity dysfunction which tend to progress over time.
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absent cardiac and muscle sympathetic nerve activities involvement in Ross Syndrome a follow up study
Autonomic Neuroscience: Basic and Clinical, 2017Co-Authors: Enrico Fileccia, Pietro Cortelli, Rocco Liguori, Vincenzo DonadioAbstract:Abstract Purpose Ross Syndrome (RS) is characterized by selective involvement of post-ganglionic skin sympathetic nerve fibres. We report a follow-up study in 4 patients to clarify whether in RS autonomic dysfunction spreads affecting also cardiovascular system. Methods The patients underwent cardiovascular reflexes (CVR) and microneurography recording of muscle sympathetic nerve activity (MSNA) for a follow-up mean period of 5 years. Results CVR and MSNA were normal at baseline and unchanged over the follow-up. Conclusions Cardiovascular autonomic system is spared in RS differently from skin autonomic activity dysfunction which progress over time. However, before drawing any definite conclusion, a large cohort of patients needs to be studied.
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Ross Syndrome a lesson from a monozygotic twin pair
Neurology, 2013Co-Authors: Maria Nolano, Vincenzo Provitera, A.m. Saltalamacchia, Vincenzo Donadio, A Stancanelli, Giuseppe Caporaso, Lucio SantoroAbstract:Ross Syndrome is a rare autonomic disorder described by Ross in 19581 and characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. A postganglionic cholinergic nerve degeneration of unknown cause underlies this condition2 although erratic association with Sjogren3 and antinuclear antibody4 positivity has led some authors to hypothesize immunologic causes. Familial cases have not been reported.
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muscle and skin sympathetic activities in Ross Syndrome
Clinical Neurophysiology, 2012Co-Authors: Vincenzo Donadio, Maria Nolano, Pietro Cortelli, Maria Pia Giannoccaro, V Di Stasi, A Baruzzi, Rocco LiguoriAbstract:Abstract Objectives Ross Syndrome (RS) is a rare degenerative disorder characterized by tonic pupil, areflexia and anhydrosis. The underlying lesion affects postganglionic skin sympathetic nerve fibers whereas the postganglionic muscle sympathetic branch is thought to be spared. Microneurography explores both skin and muscle peripheral sympathetic branches and it does not usually detect peripheral sympathetic outflow in either branch in chronic autonomic failure Syndromes. The aim of this study was to record sympathetic activity by microneurography for the first time in RS patients to confirm the selective involvement of skin sympathetic nerve activity (SSNA) with spared muscle sympathetic nerve activity (MSNA). Methods We studied seven patients (49 ± 14 years, four males) with a typical clinical picture and skin biopsy findings. Patients underwent cardiovascular reflexes and microneurography from the peroneal nerve (anhydrotic skin) to record MSNA, SSNA and the corresponding organ effector responses (skin sympathetic response-SSR and skin vasomotor response-SVR) in the same innervation field. The absence of sympathetic bursts was established after exploring at least three different corresponding nerve fascicles. Twenty age-matched healthy subjects served as controls. Results RS patients complained of diffuse anhydrosis and they showed tonic pupil and areflexia. Cardiovascular reflexes were normal. All patients displayed absent SSNA, SSR and SVR whereas MSNA was always recorded showing normal characteristics. Conclusion Microneurographic study of sympathetic activity from affected skin confirmed the selective involvement of skin sympathetic activity with spared muscle sympathetic activity and it may represent the neurophysiological hallmark of the disease. Significance Microneurography together with clinical and skin biopsy findings may contribute to RS diagnosis. Our data also suggest that autonomic damage in RS does not involve cardiovascular activity.
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Isolated generalised anhidrosis induced by postganglionic sympathetic skin nerve fibre degeneration: an incomplete Ross Syndrome?
Journal of neurology neurosurgery and psychiatry, 2008Co-Authors: Vincenzo Donadio, Pietro Cortelli, Francesca Maria Antonella Falzone, Enrico Bugiardini, Alessandro Giuliani, Cosimo Misciali, Pasquale Montagna, Laura CalzàAbstract:Ross Syndrome is characterised by tonic pupil, areflexia and anhidrosis, and the underlying lesion affects postganglionic skin sympathetic nerve fibres. We describe a 51-year-old man who had complained of anhidrosis since adolescence, at which time this problem was limited to the lower arms. The thermoregulatory sweating test disclosed generalised anhidrosis (GA) except for two small skin areas that were located in the right palm and left neck. Immunofluorescence analysis disclosed no cholinergic sudomotor fibres around the sweat glands of non-sweating skin areas, which were evident although sparse and deranged in the sweating site. In our patient, GA was induced by degeneration of postganglionic sympathetic skin nerve fibres, as found in Ross Syndrome, although his clinical picture was incomplete as it lacked tonic pupil and areflexia. Isolated GA induced by degeneration of postganglionic sympathetic nerve fibers, directly evaluated by skin biopsy, has not previously been described.
Maria Nolano - One of the best experts on this subject based on the ideXlab platform.
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Ross Syndrome a lesson from a monozygotic twin pair
Neurology, 2013Co-Authors: Maria Nolano, Vincenzo Provitera, A.m. Saltalamacchia, Vincenzo Donadio, A Stancanelli, Giuseppe Caporaso, Lucio SantoroAbstract:Ross Syndrome is a rare autonomic disorder described by Ross in 19581 and characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. A postganglionic cholinergic nerve degeneration of unknown cause underlies this condition2 although erratic association with Sjogren3 and antinuclear antibody4 positivity has led some authors to hypothesize immunologic causes. Familial cases have not been reported.
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muscle and skin sympathetic activities in Ross Syndrome
Clinical Neurophysiology, 2012Co-Authors: Vincenzo Donadio, Maria Nolano, Pietro Cortelli, Maria Pia Giannoccaro, V Di Stasi, A Baruzzi, Rocco LiguoriAbstract:Abstract Objectives Ross Syndrome (RS) is a rare degenerative disorder characterized by tonic pupil, areflexia and anhydrosis. The underlying lesion affects postganglionic skin sympathetic nerve fibers whereas the postganglionic muscle sympathetic branch is thought to be spared. Microneurography explores both skin and muscle peripheral sympathetic branches and it does not usually detect peripheral sympathetic outflow in either branch in chronic autonomic failure Syndromes. The aim of this study was to record sympathetic activity by microneurography for the first time in RS patients to confirm the selective involvement of skin sympathetic nerve activity (SSNA) with spared muscle sympathetic nerve activity (MSNA). Methods We studied seven patients (49 ± 14 years, four males) with a typical clinical picture and skin biopsy findings. Patients underwent cardiovascular reflexes and microneurography from the peroneal nerve (anhydrotic skin) to record MSNA, SSNA and the corresponding organ effector responses (skin sympathetic response-SSR and skin vasomotor response-SVR) in the same innervation field. The absence of sympathetic bursts was established after exploring at least three different corresponding nerve fascicles. Twenty age-matched healthy subjects served as controls. Results RS patients complained of diffuse anhydrosis and they showed tonic pupil and areflexia. Cardiovascular reflexes were normal. All patients displayed absent SSNA, SSR and SVR whereas MSNA was always recorded showing normal characteristics. Conclusion Microneurographic study of sympathetic activity from affected skin confirmed the selective involvement of skin sympathetic activity with spared muscle sympathetic activity and it may represent the neurophysiological hallmark of the disease. Significance Microneurography together with clinical and skin biopsy findings may contribute to RS diagnosis. Our data also suggest that autonomic damage in RS does not involve cardiovascular activity.
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Ross Syndrome a rare or a misknown disorder of thermoregulation a skin innervation study on 12 subjects
Brain, 2006Co-Authors: Maria Nolano, Vincenzo Provitera, A.m. Saltalamacchia, Vincenzo Donadio, Fiore Manganelli, A Perretti, A Stancanelli, B Lanzillo, Lucio SantoroAbstract:Ross Syndrome is described as a rare disorder of sweating associated with areflexia and tonic pupil. Since Ross's first description in 1958, ∼40 cases have been described. We assessed the involvement of cutaneous innervation in 12 subjects with Ross Syndrome using quantitative sensory testing, sweating assessment and immunohistochemical study of anhidrotic and hyperhidrotic skin. This evaluation was repeated over time in 4 out of 12 subjects. In addition, we enrolled four subjects with Holmes–Adie Syndrome (areflexia and tonic pupil) to investigate similarities between the two conditions. We found in Ross patients a complex and progressive involvement of cutaneous sensory and autonomic innervation underlying the impairment of heat production and heat dissipation through both loss of sweating and loss of cutaneous blood flow regulation. In Holmes–Adie subjects we found a mild impairment of sweating without thermoregulatory problems. The persistence of a sudomotor vasoactive intestinal peptide-immunoreactive (VIP-ir) innervation, although deranged and poor, definitely differentiated Holmes–Adie from Ross patients. Ross Syndrome is a progressive and complex disorder of thermoregulation difficult to differentiate from the probably pathogenetically related Holmes–Adie Syndrome. Sweating assessment and skin biopsy are suitable tools to define a boundary between them. Owing to the large number of Ross patients observed in only 5 years, and to the long and complex medical history of most of them, doubts arise on the effective rarity of this condition, and we warn family doctors and other specialists, besides neurologists, to become aware of this complex disorder.
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Cutaneous innervation in Ross Syndrome: a functional and morphological study in 8 patients
Journal of the Peripheral Nervous System, 2004Co-Authors: Maria Nolano, Vincenzo Provitera, Anna Perretti, A.m. Saltalamacchia, Valeria Tugnoli, Vincenzo Donadio, Fiore Manganelli, Luigi SantoroAbstract:Ross Syndrome is described as a very rare disorder characterized by segmental anhydrosis, hyporeflexia and tonic pupils. We observed 8 subjects (6 males and 2 females; age range 31–57 years) affected by Ross Syndrome in the last 4 years. They had suffered heat intolerance for at least 7 years with severe impairment of thermoregulation before diagnosis of Ross Syndrome was defined. All patients underwent neurological examination, electrophysiological study, quantitative sensory testing, Minor test, silastic imprint test, sympathetic skin response, cardiovascular reflexes and 3 mm punch biopsies from fingertip, thigh, leg and from residual hyperhydrotic areas. Skin samples were cut in 80-micron sections and incubated with a panel of primary antibodies and then with secondary antibodies conjugated with fluorophores Cy2, Cy3, Cy5 to visualize neural and vascular structures. Different primary antibodies were used to distinguish myelinated and sensory and autonomic unmyelinated fibers. Digital images were obtained using a non-laser confocal microscope (CARV, Atto Biosciences, Rockville MD, USA) and analyzed by dedicated image analysis software (Neurolucida, Microbrightfield, Willistone VT, USA). As we previously described in 3 patients (Perretti et al., Clin Neurophysiol, 2003), besides a lack of cholinergic sudomotor fibers we found a milder involvement of unmyelinated and myelinated sensory fibers. These morphological findings were in keeping with a moderate subclinical impairment of tactile and thermal thresholds and mechanical pain detection. Sensory nerve conduction study was normal indicating a very distal involvement of sensory nerve endings. This study confirms our previous observation that Ross Syndrome is a degenerative process involving progressively, besides cholinergic autonomic fibers, sensory unmyelinated and myelinated nerve fibers.
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is Ross Syndrome a dysautonomic disorder only an electrophysiologic and histologic study
Clinical Neurophysiology, 2003Co-Authors: A Perretti, Maria Nolano, Vincenzo Provitera, Valeria Tugnoli, G De Joanna, G D Iannetti, G Cruccu, Lucio SantoroAbstract:Abstract Objective : To define the involvement of peripheral nerve fibers in Ross Syndrome. Methods : Mechanical pain perception, tactile and thermal thresholds on hand, foot dorsum, thigh, median nerve orthodromic sensory conduction velocity (SCV) and motor conduction velocity (MCV), sural nerve antidromic SCV, peroneal nerve MCV, H-reflex, F-wave, median, tibial nerve somatosensory evoked potentials (SSEPs), perioral, hand CO 2 laser late (LEPs) and ultralate evoked potentials, sympathetic skin response (SSRs), cardiovascular, Minor sweat, silastic imprint, histamine, photopletysmographic and pupil pilocarpine tests, cutaneous innervation immunohistochemical techniques were studied in 3 patients with Ross Syndrome. Results : Quantitative sensory testing showed altered results in patients 1 and 2, and patient 3 had a slight impairment of mechanical pain perception. Nerve conduction, except for a median nerve distal reduction of sensory conduction in patient 1, F-wave and SSEP findings were normal; H-reflex was absent at rest in all patients. Hand LEPs were absent in patient 2, ultralate potentials were absent in patients 1 and 2. Skin biopsy showed a disease duration related reduction of unmyelinated and myelinated sensory fibers and a lack of unmyelinated autonomic fibers in all patients. Conclusions : Our data suggest that Ross Syndrome is a degenerative disorder involving progressive sudomotor fibers, and then epidermal sensory unmyelinated and myelinated fibers.
Lucio Santoro - One of the best experts on this subject based on the ideXlab platform.
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Ross Syndrome a lesson from a monozygotic twin pair
Neurology, 2013Co-Authors: Maria Nolano, Vincenzo Provitera, A.m. Saltalamacchia, Vincenzo Donadio, A Stancanelli, Giuseppe Caporaso, Lucio SantoroAbstract:Ross Syndrome is a rare autonomic disorder described by Ross in 19581 and characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. A postganglionic cholinergic nerve degeneration of unknown cause underlies this condition2 although erratic association with Sjogren3 and antinuclear antibody4 positivity has led some authors to hypothesize immunologic causes. Familial cases have not been reported.
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Ross Syndrome a rare or a misknown disorder of thermoregulation a skin innervation study on 12 subjects
Brain, 2006Co-Authors: Maria Nolano, Vincenzo Provitera, A.m. Saltalamacchia, Vincenzo Donadio, Fiore Manganelli, A Perretti, A Stancanelli, B Lanzillo, Lucio SantoroAbstract:Ross Syndrome is described as a rare disorder of sweating associated with areflexia and tonic pupil. Since Ross's first description in 1958, ∼40 cases have been described. We assessed the involvement of cutaneous innervation in 12 subjects with Ross Syndrome using quantitative sensory testing, sweating assessment and immunohistochemical study of anhidrotic and hyperhidrotic skin. This evaluation was repeated over time in 4 out of 12 subjects. In addition, we enrolled four subjects with Holmes–Adie Syndrome (areflexia and tonic pupil) to investigate similarities between the two conditions. We found in Ross patients a complex and progressive involvement of cutaneous sensory and autonomic innervation underlying the impairment of heat production and heat dissipation through both loss of sweating and loss of cutaneous blood flow regulation. In Holmes–Adie subjects we found a mild impairment of sweating without thermoregulatory problems. The persistence of a sudomotor vasoactive intestinal peptide-immunoreactive (VIP-ir) innervation, although deranged and poor, definitely differentiated Holmes–Adie from Ross patients. Ross Syndrome is a progressive and complex disorder of thermoregulation difficult to differentiate from the probably pathogenetically related Holmes–Adie Syndrome. Sweating assessment and skin biopsy are suitable tools to define a boundary between them. Owing to the large number of Ross patients observed in only 5 years, and to the long and complex medical history of most of them, doubts arise on the effective rarity of this condition, and we warn family doctors and other specialists, besides neurologists, to become aware of this complex disorder.
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is Ross Syndrome a dysautonomic disorder only an electrophysiologic and histologic study
Clinical Neurophysiology, 2003Co-Authors: A Perretti, Maria Nolano, Vincenzo Provitera, Valeria Tugnoli, G De Joanna, G D Iannetti, G Cruccu, Lucio SantoroAbstract:Abstract Objective : To define the involvement of peripheral nerve fibers in Ross Syndrome. Methods : Mechanical pain perception, tactile and thermal thresholds on hand, foot dorsum, thigh, median nerve orthodromic sensory conduction velocity (SCV) and motor conduction velocity (MCV), sural nerve antidromic SCV, peroneal nerve MCV, H-reflex, F-wave, median, tibial nerve somatosensory evoked potentials (SSEPs), perioral, hand CO 2 laser late (LEPs) and ultralate evoked potentials, sympathetic skin response (SSRs), cardiovascular, Minor sweat, silastic imprint, histamine, photopletysmographic and pupil pilocarpine tests, cutaneous innervation immunohistochemical techniques were studied in 3 patients with Ross Syndrome. Results : Quantitative sensory testing showed altered results in patients 1 and 2, and patient 3 had a slight impairment of mechanical pain perception. Nerve conduction, except for a median nerve distal reduction of sensory conduction in patient 1, F-wave and SSEP findings were normal; H-reflex was absent at rest in all patients. Hand LEPs were absent in patient 2, ultralate potentials were absent in patients 1 and 2. Skin biopsy showed a disease duration related reduction of unmyelinated and myelinated sensory fibers and a lack of unmyelinated autonomic fibers in all patients. Conclusions : Our data suggest that Ross Syndrome is a degenerative disorder involving progressive sudomotor fibers, and then epidermal sensory unmyelinated and myelinated fibers.
Claudia Sommer - One of the best experts on this subject based on the ideXlab platform.
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selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross Syndrome
Annals of Neurology, 2002Co-Authors: Claudia Sommer, Thies Lindenlaub, Detlef Zillikens, Klaus V Toyka, Markus NaumannAbstract:Ross Syndrome consists of segmental hyperhidrosis with widespread anhidrosis, Adie Syndrome, and areflexia. The cause of this disorder is unknown. Selective degeneration of cholinergic fibers or of neural crest-derived structures has been suggested. We present clinical and skin biopsy data of 4 patients, providing evidence of reduced cholinergic sweat gland innervation in hypohidrotic skin by morphometric analysis. These findings indicate a selective degenerative process of the cholinergic sudomotor neurons.
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Selective degeneration of sudomotor fibers in Ross Syndrome and successful treatment of compensatory hyperhidrosis with botulinum toxin
Muscle & nerve, 1998Co-Authors: Ilka Bergmann, Michel Dauphin, Markus Naumann, Peter Flachenecker, Wolfgang Müllges, Martin Koltzenburg, Claudia SommerAbstract:We report a 5-year follow-up of a patient with Ross Syndrome. A biopsy of the anhidrotic skin immunostained with protein gene product 9.5 visualized by confocal microscopy revealed selective loss of sudomotor fibers, whereas epidermal innervation remained intact, providing the first morphologic evidence of selective loss of sudomotor fibers in this Syndrome. Among the different treatment strategies employed for the patient's disabling segmental hyperhidrosis, intracutaneous injection of botulinum toxin A was the most helpful.
Rocco Liguori - One of the best experts on this subject based on the ideXlab platform.
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75 absent cardiac and muscle sympathetic nerve activities involvement in Ross Syndrome a follow up study
Clinical Neurophysiology, 2017Co-Authors: Vincenzo Donadio, Pietro Cortelli, Enrico Fileccia, Rocco LiguoriAbstract:Ross Syndrome (RS) is a rare disorder characterized by selective involvement of post-ganglionic skin sympathetic nerve fibres with absent cholinergic sudomotor fibres as hallmark of the disease. It is still not well defined whether autonomic dysfunction remain localized to skin fibres or it affects also cardiovascular autonomic system. To clarify this aspect, we report a cardiovascular autonomic system follow-up study in 4 patients affected by Ross Syndrome. We studied 4 patients affected by RS for a follow-up mean period of 5 years (range 1–10 years). All patients complained of anhydrosis with heat intolerance and showed areflexia and tonic pupil on neurological examination and absent cholinergic sudomotor fibres on skin biopsy. All patients underwent cardiovascular reflexes (CVR, head-up tilt test, Valsalva maneuver, deep breathing, isometric handgrip) and microneurography recording of muscle sympathetic nerve activity (MSNA) from common peroneal nerve. CVR and MSNA resulted normal at baseline and unchanged over the follow-up in all patients although anhydrosis was usually widespread on patient’s clinical re-evaluation. Our preliminary study shows that cardiovascular autonomic system is not affected in Ross Syndrome differently from skin autonomic activity dysfunction which tend to progress over time.
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absent cardiac and muscle sympathetic nerve activities involvement in Ross Syndrome a follow up study
Autonomic Neuroscience: Basic and Clinical, 2017Co-Authors: Enrico Fileccia, Pietro Cortelli, Rocco Liguori, Vincenzo DonadioAbstract:Abstract Purpose Ross Syndrome (RS) is characterized by selective involvement of post-ganglionic skin sympathetic nerve fibres. We report a follow-up study in 4 patients to clarify whether in RS autonomic dysfunction spreads affecting also cardiovascular system. Methods The patients underwent cardiovascular reflexes (CVR) and microneurography recording of muscle sympathetic nerve activity (MSNA) for a follow-up mean period of 5 years. Results CVR and MSNA were normal at baseline and unchanged over the follow-up. Conclusions Cardiovascular autonomic system is spared in RS differently from skin autonomic activity dysfunction which progress over time. However, before drawing any definite conclusion, a large cohort of patients needs to be studied.
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muscle and skin sympathetic activities in Ross Syndrome
Clinical Neurophysiology, 2012Co-Authors: Vincenzo Donadio, Maria Nolano, Pietro Cortelli, Maria Pia Giannoccaro, V Di Stasi, A Baruzzi, Rocco LiguoriAbstract:Abstract Objectives Ross Syndrome (RS) is a rare degenerative disorder characterized by tonic pupil, areflexia and anhydrosis. The underlying lesion affects postganglionic skin sympathetic nerve fibers whereas the postganglionic muscle sympathetic branch is thought to be spared. Microneurography explores both skin and muscle peripheral sympathetic branches and it does not usually detect peripheral sympathetic outflow in either branch in chronic autonomic failure Syndromes. The aim of this study was to record sympathetic activity by microneurography for the first time in RS patients to confirm the selective involvement of skin sympathetic nerve activity (SSNA) with spared muscle sympathetic nerve activity (MSNA). Methods We studied seven patients (49 ± 14 years, four males) with a typical clinical picture and skin biopsy findings. Patients underwent cardiovascular reflexes and microneurography from the peroneal nerve (anhydrotic skin) to record MSNA, SSNA and the corresponding organ effector responses (skin sympathetic response-SSR and skin vasomotor response-SVR) in the same innervation field. The absence of sympathetic bursts was established after exploring at least three different corresponding nerve fascicles. Twenty age-matched healthy subjects served as controls. Results RS patients complained of diffuse anhydrosis and they showed tonic pupil and areflexia. Cardiovascular reflexes were normal. All patients displayed absent SSNA, SSR and SVR whereas MSNA was always recorded showing normal characteristics. Conclusion Microneurographic study of sympathetic activity from affected skin confirmed the selective involvement of skin sympathetic activity with spared muscle sympathetic activity and it may represent the neurophysiological hallmark of the disease. Significance Microneurography together with clinical and skin biopsy findings may contribute to RS diagnosis. Our data also suggest that autonomic damage in RS does not involve cardiovascular activity.
