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The goal of this interventional non-pharmacological study is to evaluate, using a multimodal approach, the progression of autonomic and sensory involvement in in amyotrophic lateral sclerosis (ALS) patients enrolled within 18 months from motor onset and its relationship with the progression of overall clinical disability.
The main questions it aims to answer are:
Researchers will compare results from ALS patients with data obtained from a population of age and sex matched healthy subjects.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Amyotrophic Lateral Sclerosis patients | Experimental | Amyotrophic Lateral Sclerosis (ALS) patients within 18 months from symptoms onset will be recruited |
|
| Healthy controls | Active Comparator | A population of healthy controls matched for sex and age will be enrolled |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Skin biopsy | Diagnostic Test | A punch skin biopsy of 3mm will be used to analyze cutaneous innervation |
|
| Measure | Description | Time Frame |
|---|---|---|
| Sensory peripheral innervation (IENF) | Quantification Intraepidermal Nerve Fibers (IENF ff/mm) in skin biopsy from fingertip, thigh and leg. | At recruitment |
| Autonomic peripheral innervation | Quantification of nerves in sweat gland (fiber lenght/um3) in skin biopsy from fingertip, thigh and leg. Quantification of nerves in arrector pili muscle (ff/mm) in skin biopsy from thigh and leg. | At recruitment |
| Autonomic peripheral innervation | Quantification sweat gland (fiber lenght/um3) and arrector pili muscle (ff/mm) innervation in skin biopsy from thigh. | At follow-up, an average of 6 months |
| Sensory peripheral innervation (IENF) | Quantification Intraepidermal Nerve Fibers (IENF ff/mm) in skin biopsy from thigh. | At follow-up, an average of 6 months |
| Measure | Description | Time Frame |
|---|---|---|
| Sensory and autonomic symptoms evaluated by clinical scales | Data of sensory and autonomic symptoms (Small fiber neuropathy Symptoms inventory questionnaire (SFN-SIQ) and Scale for Outcomes in Parkinson's disease for Autonomic Symptoms (SCOPA AUT)) will be collected | At the recruitment |
| Sensory and autonomic symptoms evaluated by clinical scales |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Maria Nolano, MD, PhD | Contact | +390824909257 | maria.nolano@icsmaugeri.it | |
| Giuseppe Caporaso | Contact | +390824909645 | giuseppe.caporaso@icsmaugeri.it |
| Name | Affiliation | Role |
|---|---|---|
| Maria Nolano, MD, PhD | Istituti Clinici Scientifici Maugeri SpA | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| ICS Maugeri - IRCCS of Telese Terme | Recruiting | Telese Terme | Benevento | 82037 | Italy |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 33993457 | Background | Mahoney CJ, Ahmed RM, Huynh W, Tu S, Rohrer JD, Bedlack RS, Hardiman O, Kiernan MC. Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis. CNS Drugs. 2021 May;35(5):483-505. doi: 10.1007/s40263-021-00820-1. Epub 2021 May 15. | |
| 31293369 | Background | Gentile F, Scarlino S, Falzone YM, Lunetta C, Tremolizzo L, Quattrini A, Riva N. The Peripheral Nervous System in Amyotrophic Lateral Sclerosis: Opportunities for Translational Research. Front Neurosci. 2019 Jun 25;13:601. doi: 10.3389/fnins.2019.00601. eCollection 2019. |
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There are not masking participants
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| Cardiovascular Reflexes testing | Diagnostic Test | Cardiovascular reflex tests including deep breathing, head-up Tilt, standing, isometric exercises, mental arithmetic and Valsalva maneuver. |
|
| Administration of clinical scales evaluating autonomic symptoms, pain small fiber neuropathy symptoms | Diagnostic Test | We'll characterize patients' symptoms through the administration of clinical scales such as: SCOPA-AUT autonomic symptoms scale; Brief Pain Inventory questionnaire |
|
| Dinamic Sweat Test | Diagnostic Test | Test for the functional assessment of postganglionic sudomotor pathway |
|
Data of sensory and autonomic symptoms (Small fiber neuropathy Symptoms inventory questionnaire (SFN-SIQ) and Scale for Outcomes in Parkinson's disease for Autonomic Symptoms (SCOPA AUT)) will be collected |
| At follow-up, an average of 6 months |
| Assessment of Cardiovascular function | Data from cardiovascular reflex test will be analyzed and compared with morphological data and clinical motor severity | baseline |
| Sudomotor function | Data from dinamic sweat test will be analyzed and compared with morphological data and clinical motor severity | baseline |
| Department of Neurosciences, Reproductive Sciences and Odontostomatology, University of Naples Federico II | Recruiting | Naples | 80131 | Italy |
|
| 26908600 | Background | Sassone J, Taiana M, Lombardi R, Porretta-Serapiglia C, Freschi M, Bonanno S, Marcuzzo S, Caravello F, Bendotti C, Lauria G. ALS mouse model SOD1G93A displays early pathology of sensory small fibers associated to accumulation of a neurotoxic splice variant of peripherin. Hum Mol Genet. 2016 Apr 15;25(8):1588-99. doi: 10.1093/hmg/ddw035. Epub 2016 Feb 9. |
| 21646630 | Background | Weis J, Katona I, Muller-Newen G, Sommer C, Necula G, Hendrich C, Ludolph AC, Sperfeld AD. Small-fiber neuropathy in patients with ALS. Neurology. 2011 Jun 7;76(23):2024-9. doi: 10.1212/WNL.0b013e31821e553a. |
| 25683764 | Background | Truini A, Biasiotta A, Onesti E, Di Stefano G, Ceccanti M, La Cesa S, Pepe A, Giordano C, Cruccu G, Inghilleri M. Small-fibre neuropathy related to bulbar and spinal-onset in patients with ALS. J Neurol. 2015;262(4):1014-8. doi: 10.1007/s00415-015-7672-0. Epub 2015 Feb 17. |
| 27288647 | Background | Nolano M, Provitera V, Manganelli F, Iodice R, Caporaso G, Stancanelli A, Marinou K, Lanzillo B, Santoro L, Mora G. Non-motor involvement in amyotrophic lateral sclerosis: new insight from nerve and vessel analysis in skin biopsy. Neuropathol Appl Neurobiol. 2017 Feb;43(2):119-132. doi: 10.1111/nan.12332. Epub 2016 Jul 7. |
| 33372053 | Background | deCarvalho M, Gromicho M, Andersen P, Grosskreutz J, Kuzma-Kozakiewicz M, Petri S, Uysal H, Pinto S. Peripheral neuropathy in ALS: phenotype association. J Neurol Neurosurg Psychiatry. 2021 Oct;92(10):1133-1134. doi: 10.1136/jnnp-2020-325164. Epub 2020 Dec 28. No abstract available. |
| 27964824 | Background | Chio A, Mora G, Lauria G. Pain in amyotrophic lateral sclerosis. Lancet Neurol. 2017 Feb;16(2):144-157. doi: 10.1016/S1474-4422(16)30358-1. Epub 2016 Dec 8. |
| 32897040 | Background | Fasolino A, Di Stefano G, Leone C, Galosi E, Gioia C, Lucchino B, Terracciano A, Di Franco M, Cruccu G, Truini A. Small-fibre pathology has no impact on somatosensory system function in patients with fibromyalgia. Pain. 2020 Oct;161(10):2385-2393. doi: 10.1097/j.pain.0000000000001920. |
| 27115670 | Background | Finnerup NB, Haroutounian S, Kamerman P, Baron R, Bennett DLH, Bouhassira D, Cruccu G, Freeman R, Hansson P, Nurmikko T, Raja SN, Rice ASC, Serra J, Smith BH, Treede RD, Jensen TS. Neuropathic pain: an updated grading system for research and clinical practice. Pain. 2016 Aug;157(8):1599-1606. doi: 10.1097/j.pain.0000000000000492. |
| 22236582 | Background | Damon-Perriere N, Foubert-Samier A, De Cock VC, Gerdelat-Mas A, Debs R, Pavy-Le Traon A, Senard JM, Rascol O, Tison F, Meissner WG. Assessment of the Scopa-Aut questionnaire in multiple system atrophy: relation to UMSARS scores and progression over time. Parkinsonism Relat Disord. 2012 Jun;18(5):612-5. doi: 10.1016/j.parkreldis.2011.12.009. Epub 2012 Jan 9. |
| 11464847 | Background | Brooks BR, Miller RG, Swash M, Munsat TL; World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Dec;1(5):293-9. doi: 10.1080/146608200300079536. No abstract available. |
| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
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