Vincenzo Provitera

Vincenzo Provitera
Istituti Clinici Scientifici Maugeri IRCCS | Fondazione Maugeri · Istituto Scientifico di Riabilitazione di Telese - Benevento

About

127
Publications
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2,577
Citations
Citations since 2016
40 Research Items
1454 Citations
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2016201720182019202020212022050100150200250
2016201720182019202020212022050100150200250
2016201720182019202020212022050100150200250

Publications

Publications (127)
Article
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Backgrounds Hereditary transthyretin amyloidosis (ATTRv) presymptomatic subjects undergo multidisciplinary evaluation to detect, as early as possible, a subclinical involvement of multisystem disease. Quantitative sensory testing (QST) that investigates and discriminates the function of C, Aδ and Aβ fibers is included as an instrumental test to mon...
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Background: The role of peripheral phosphorylated-α-Synuclein (p-α-syn) deposition on nerve degeneration in synucleinopathies is still unknown. Objective: To assess the cutaneous neural distribution of p-α-Syn deposits and its correlation with clinical data and with morphology and function of cutaneous sensory and autonomic nerves in early Parki...
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Introduction Cardiovascular autonomic failure and neurogenic orthostatic hypotension (nOH) are common and disabling features of Parkinson’s disease (PD) and multiple system atrophy (MSA). Compared to PD, MSA is considered a primarily central alpha-synucleinopathy. Aim To characterise the relationship between cardiovascular autonomic failure and cu...
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Long-COVID-19 refers to the signs and symptoms that continue or develop after the “acute COVID-19” phase. These patients have an increased risk of multiorgan dysfunction, readmission, and mortality. In Long-COVID-19 patients, it is possible to detect a persistent increase in D-Dimer, NT-ProBNP, and autonomic nervous system dysfunction. To verify th...
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The serpinins are relatively novel peptides generated by proteolytic processing of chro-mogranin A and they are comprised of free serpinin, serpinin-RRG and pGlu-serpinin. In this study, the presence and source of these peptides were studied in the skin. By Western blot analysis, a 40 kDa and a 50 kDa protein containing the sequence of serpinin wer...
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Background and Objectives Sudomotor impairment has been recognized as a key feature in differentiating Parkinson disease (PD) and multiple system atrophy-parkinsonian type (MSA-P) with the latter been characterized by diffuse anhidrosis in prospective study including patients in late stage of disease. We aimed to evaluate morphological and function...
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Objective To evaluate patients with ganglionic acetylcholine receptor antibody (gAChR‐Ab) positive autoimmune autonomic ganglionopathy using a multi‐modal testing protocol to characterise their full clinical phenotype and explore biomarkers to quantify immunotherapy response. Methods Cohort study of thirteen individuals (seven female; 21–69 years)...
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Aim Progressive Supranuclear Palsy (PSP) is a progressive neurodegenerative tauopathy characterized by motor, behavioural and cognitive dysfunction. While in the last decade, sensory and autonomic disturbances as well as peripheral nerve involvement are well recognized in Parkinson’s Disease (PD), little is known in this regard for PSP. Herein, we...
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In the last three decades the study of cutaneous innervation through 3 mm-punch-biopsy has provided an important contribution to the knowledge of small fiber somatic and autonomic neuropathies but also of large fiber neuropathies. Skin biopsy is a minimally invasive technique with the advantage, compared to sural nerve biopsy, of being suitable to...
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Aims: to examine biomechanical and neuroautonomic adaptation to blood volume displacement induced by tilt-test in patients with previous inferoapical/inferolateral (IA-IL) or basal/apical septal (BS-AS) myocardial infarction (MI). Methods: Twenty-four patients with heart failure (HF) and previous IA-IL MI and thirty patients with HF and previous...
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Diabetic polyneuropathy (DPN) is a common complication to diabetes and is often associated with neuropathic pain. The mechanisms underlying development and maintenance of painful DPN are largely unknown and quantification of intraepidermal nerve fiber density (IENFD) from skin biopsy, one of the neuropathological gold standard when diagnosing DPN,...
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Aim Small fibre neuropathy (SFN) diagnosis represents a challenge for neurologists. The diagnostic gold standard is intraepidermal nerve fibre (IENF) density, but in about 10‐20 % of patients with symptoms/signs and abnormalities on functional tests, it remains within normal range. We propose an adjunctive parameter to improve the efficiency of ski...
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Objective: To describe morphological changes associated with degeneration and regeneration of large fibers in the skin using a model of chronic compression of the median nerve. Methods: We studied cutaneous innervation in 30 patients with chronic compression of the median nerve at the wrist. Before surgery, we assessed the symptom severity and p...
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Background and aims: The development of patient-specific induced pluripotent stem cells (iPSCs) offered interesting insights in modeling the pathogenesis of Charcot-Marie-Tooth (CMT) disease and thus we decided to explore the phenotypes of iPSCs derived from a single CMT patient carrying a mutant ATP1A1 allele (p.Pro600Ala). iPSCs clones generated...
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See Karakaya and Wirth (doi:10.1093/brain/awz273) for a scientific commentary on this article. Neurofascin (NFASC) isoforms are immunoglobulin cell adhesion molecules involved in node of Ranvier assembly. Efthymiou et al. identify biallelic NFASC variants in ten unrelated patients with a neurodevelopmental disorder characterized by variable degrees...
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To improve patient care and help clinical research, the Neuropathic Pain Special Interest Group of the Italian Neurological Society appointed a task force to elaborate a consensus statement on pharmacoresistant neuropathic pain. The task force included 19 experts in neuropathic pain. These experts participated in a Delphi survey consisting of three...
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We reported a sensory cutaneous denervation more pronounced on the more affected side in Parkinson’s disease (PD)¹. Our aim is to evaluate if autonomic function is also asymmetrically affected. We investigated sudomotor function on both legs of ten patients affected by idiopathic PD by the dynamic sweat test (DST)². After pilocarpine by iontophores...
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Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disease characterized by intraneuronal accumulation of hyperphosphorylated Tau. Because PSP is strongly linked biochemically and genetically to tau protein abnormalities there is a growing interest in new tau-directed therapies and in the search of new biomarkers able to monitor disea...
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Objective: The aim of this study was to report a method that quantifies axon reflex sweating from individual sweat glands with nanoliter precision. Measurement of the axon reflex is generally expressed as a single variable (e.g., the flare area or total sweat volume). High-definition videography enables precise measurement of sweating from single,...
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Congenital insensitivity to pain is a rare autosomal recessive disorder presenting with a spectrum of clinical features caused by mutations in different genes. A 10‐year‐old girl with congenital insensitivity to pain, hyposmia and hypogeusia, and her unaffected twin and parents underwent next generation sequencing of SCN9A exons and flanking splice...
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Background: We aimed to test the clinical utility of leg to thigh intraepidermal nerve fiber (IENF) density ratio as a parameter to discriminate between length-dependent small fiber neuropathy (SFN) and small fiber sensory ganglionopathy (SFSG) in subjects with signs and symptoms of small fiber pathology. Methods: We retrospectively evaluated th...
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Small fiber pathology is part of the clinical picture of PD. Cross-sectional studies have shown no correlation between intraepidermal nerve fiber (IENF) density and patient disease duration and severity. However, it is not known whether IENF density changes over time. We aimed to assess the progression of small fiber pathology in PD patients along...
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Degeneration of several brainstem nuclei occurs in Parkinson disease (PD). Vestibular-evoked-myogenic-potentials (VEMPs) represent a valuable tool to assess the function of these structures. VEMPs abnormalities have been reported in PD. We used this tool to assess the asymmetry between the two sides and the difference between anterior and posterior...
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Vestibular-Evoked-Myogenic-Potentials (VEMPs) represent a muscular response following an intense acoustic stimulation. The cVEMPs explore a reflex arc (from saccule to the neck musculature) and usually are only recorded from sternocleidomastoideus (SCM).¹ They are non-invasive, well-tolerated, simple-to-perform and very useful in several diseases....
Article
“The bumps” is a sensitive test to quantify tactile threshold of fingerpads. We tested its ability to detect distal axonal degeneration in peripheral neuropathies. The bumps device is a checkerboard-like surface divided into squares. Each square includes 5 colored circles, one of which contains a round bump (height range 2.5–14 μm) to identify usin...
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Objective: To study small and large fiber pathology in drug-naive and l-dopa-treated patients affected by Parkinson disease (PD) in early phases, before the occurrence of neuropathic electrophysiologic abnormalities. Methods: We enrolled 85 patients with idiopathic PD (male/female 49/36, age 61.3 ± 9.7 years) without electrophysiologic signs of...
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Objective: To describe a second hereditary sensory autonomic neuropathy type VI (HSAN-VI) family harboring 2 novel heterozygous mutations in the dystonin (DST) gene and to evaluate their effect on neurons derived from induced pluripotent stem cells (iPSC). Methods: The family consisted of 3 affected siblings from nonconsanguineous healthy parent...
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Introduction: There is a need for quantitative, precise assessment of small fiber peripheral nerve function. We tested a customized camera device and protocol designed to quantify secretions of individual sweat glands (SGs). Materials and methods: Testing was performed on 178 healthy controls and 20 neuropathy subjects. Sweating was stimulated o...
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Intraepidermal nerve fiber (IENF) density at distal leg is considered sufficient to confirm the clinical suspect of small fiber neuropathy. Extensive normative datasets demonstrate a reduced nerve density with ageing and in male compared to female subjects at this site. An additional sample from thigh is often analyzed to calculate leg/thigh IENF d...
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Small fiber pathology has been demonstrated in Parkinson Disease (PD). To verify if it is among the early non-motor features of PD, unrelated to Levodopa therapy, we assessed function and morphology of cutaneous sensory and autonomic nerves in 32 consecutive drug-naive PD patients (M/F = 16/16; age 57.3 ± 11.1 years) and 64 age and sex matched cont...
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Aims: To assess cutaneous sensory and autonomic nerves and the vascular bed in amyotrophic lateral sclerosis. Methods: We enrolled 41 patients (M=20, aged 63.5±11.8 years), and 41 age- and sex-matched healthy volunteers (M=20, aged 63.5±11.8 years). Disease severity and sensory and autonomic symptoms were scored using dedicated rating scales. Sk...
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Abnormalities of internodal and nodal length associated with aspects of axonal degeneration have been described in skin samples of Charcot–Marie–Tooth type 1A (CMT1A) patients. It is not clear how early such abnormalities occur and if they change with aging. To elucidate these aspects we assessed myelinated fiber (MF) density and morphometry in ski...
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We created an age and gender adjusted normative dataset for intraepidermal nerve fiber (IENF) density at distal leg in skin samples processed with immunofluorescence. We collected from four experienced laboratories worldwide IENF density data of 528 healthy individuals. Skin samples were collected, processed and analyzed according to standard proce...
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Objective: To assess the involvement of the peripheral nervous system in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) by means of immunofluorescence and confocal analysis of punch skin biopsies. Methods: We recruited 14 unrelated patients with CADASIL (M/F = 9/5; age 53.9 ± 10.5 years) and...
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Background and purpose: Quantification of intraepidermal nerve fibers (IENFs) in skin biopsies is now the tool of choice to diagnose small fiber neuropathies. An adequate normative dataset, necessary to assess normality cutoffs, is available for brightfield microscopy but not for immunofluorescence. Methods: Intraepidermal nerve fiber density da...
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Objective: To evaluate, by skin biopsy, dermal nerve fibers in 31 patients with 3 common Charcot-Marie-Tooth (CMT) genotypes (CMT1A, late-onset CMT1B, and CMTX1), and rarer forms of CMT caused by mutations in RAB7 (CMT2B), TRPV4 (CMT2C), and GDAP1 (AR-CMT2K) genes. Methods: We investigated axonal loss by quantifying Meissner corpuscles and intra...
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We investigated the agreement between simple indirect immunofluorescence (IF) and bright-field immunohistochemistry (BFI) on free-floating sections for intraepidermal nerve fiber density (IENFD) quantification. Fifty-five healthy subjects and 63 patients with probable small fiber neuropathy (SFN) underwent two adjacent skin biopsies at the distal l...
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Postherpetic neuralgia (PHN) is a common and exceptionally drug-resistant neuropathic pain condition. In this cross-sectional skin biopsy study, seeking information on the responsible pathophysiological mechanisms we assessed how ophthalmic PHN affects sensory and autonomic skin innervation. We took 2-mm supraorbital punch skin biopsies from the af...
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Sensory symptoms in CMT1A are generally attributed to large fiber. However, an involvement of small fibers has been recently brought up. We performed an extensive clinical, morphological and functional study of cutaneous somatic and autonomic innervation in 10 patients affected by CMT 1A, in order to assess small fiber involvement in this condition...
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Facial-onset sensory-motor neuronopathy (FOSMN) first manifests with trigeminal sensory loss and pain, then spreads to bulbopontine and spinal motoneurons, sometimes with a fatal outcome.(1,2)
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To assess the involvement of small nerve fibers in Charcot-Marie-Tooth type 1A (CMT1A). We used indirect immunofluorescence and confocal microscopy on punch biopsies from glabrous (fingertip) and hairy (thigh and leg) skin of 20 unrelated patients with CMT1A to quantify somatic and autonomic nerve fibers. In particular, we quantified epidermal nerv...
Article
IntroductionIntraepidermal nerve fiber (IENF) linear density is a reliable tool to detect small fiber neuropathies. In immunofluorescence, the IENF count is performed typically by computer-assisted nerve tracing on confocal images. Alternatively, the count can be performed directly through the oculars of a standard epifluorescence microscope. We sp...
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Objective: To evaluate postganglionic autonomic involvement in multiple system atrophy (MSA). Methods: We quantified sudomotor innervation in skin biopsy of 29 patients with MSA (19 male and 10 female; age 60.0 ± 7.7 years) and 29 age- and sex-matched healthy subjects. Samples were obtained from thigh and leg and, in 20 out of the 29 cases, also...
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In fibromyalgia (FM), reduced habituation of laser-evoked potentials (LEPs) suggests a dysfunction of pain processing at a central level. In this study, we aimed to further examine the nociceptive pathways at the peripheral to the central level in a large group of FM patients by means of LEPs and skin biopsy, in light of healthy controls findings a...
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Our aim was to evaluate if autonomic impairment in Parkinson disease (PD) differently affects the primarily compromised side. Fourteen PD patients underwent bilateral sympathetic skin response (SSR) recording. The response was elicited on palms and soles following standard procedures. Patients underwent also sudomotor output evaluation by dynamic s...
Article
Sudomotor function is often impaired in peripheral (PNSd) and central nervous system disorders (CNSd). Mechanisms underlying sudomotor failure in CNSd are not always clear. However, recently an involvement of peripheral nervous system in central neurodegenerative diseases has been observed. Objective of this study is to try of clarifying the involv...
Article
The assessment of autonomic nervous system (ANS) in hereditary sensory autonomic neuropathy (HSAN) type II is still lacking. To this aim we investigated the ANS involvement in a new family with HSAN II phenotype. Three siblings from non consanguineous healthy parents were clinically and electrophysiologically evaluated. Two of them underwent quanti...
Article
The assessment of epidermal nerve fiber density (ENF) in skin biopsy has been accepted as an objective and reliable technique to diagnose SFN. Bright-field microscopy and immunofluorescence with or without confocal microscopy, are currently used to quantify ENF. The objective of this study was to evaluate the agreement between the two techniques of...
Article
Our aim was to study peripheral nerve involvement in CADASIL, a hereditary cerebral microangiopathy due to mutations in the Notch 3 gene. We studied skin innervation in 14 patients with CADASIL (6 women, 8 male, mean age 57.6 ± 5.4) by means of 3 mm-punch biopsy from thigh, distal leg and fingertip. Samples were processed using indirect immunofluor...
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We describe the occurrence of small fiber neuropathy in a patient affected by Chagas disease in the indeterminate phase. After the exclusion of all the possible etiologies of small fiber neuropathy, the disorder was considered related to Trypanosoma cruzi infection. Although a peripheral involvement has been described in Chagas disease, this is the...
Article
Ross syndrome is a rare autonomic disorder described by Ross in 1958(1) and characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. A postganglionic cholinergic nerve degeneration of unknown cause underlies this condition(2) although erratic association with Sjögren(3) and antinuclear antibody(4) positivity has led some authors to hyp...
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The morphology of cutaneous sensory and autonomic innervation in human trigeminal territory is still unknown. The aim of this study is to describe facial cutaneous innervation using skin biopsy. This new tool could be useful in understanding the mechanisms underlying several facial pain conditions. In 30 healthy subjects, we quantified epidermal ne...
Article
We describe the first Italian family affected by CMT2B carrying a Val162Met substitution in the RAB7 gene. The clinical and electrophysiological features of our family are similar to those of previously reported families with RAB7 mutations, also for the higher occurrence of ulcers in males. However, in this family we evaluated the autonomic nervou...
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To devise a rapid, sensitive method to quantify tactile threshold of finger pads for early detection and staging of peripheral neuropathy and for use in clinical trials. Subjects were 166 healthy controls and 103 patients with, or at risk for, peripheral neuropathy. Subjects were screened by questionnaire. The test device, the Bumps, is a checkerbo...
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Full-text available
To evaluate postganglionic autonomic and somatic nerve fiber involvement in a patient with chronic autoimmune autonomic ganglionopathy. Case report. Department of Neurological Sciences, University Federico II of Naples. A patient with a 16-year history of severe autonomic failure and a high nicotinic acetylcholine receptor antibody titer underwent...