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The Self-Notion and Perception (SNAP) questionnaire developed at IRCCS E.Medea by Eleonora Diella and Roberta Morganti, arises from the need to quantify the subjective perception of the patient suffering from HSP of the typical symptoms of pathology, such as spasticity, weakness, changes in balance, resistance in walking, pain and fatigue. The purpose of this study is to validate this instrument and test its reliability, looking for correlations with the measurement scales used in the literature for the evaluation of the patient with HSP. The most used for this population are the Spastic Paraplegia Rating Scale (SPRS) and the Six-Minute Walk Test (6MWT) which assess the severity of the disease and the level of endurance (8-9) respectively.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients affected by hereditary spastic paraplegia | Experimental | 40 subjects affected by genetically determined hereditary spastic paraparesis or subjects without defined genetics but who unequivocally show a dominant or recessive familiarity with exclusive involvement of the pyramidal system. |
|
| healthy subjects | Active Comparator | 40 healthy subjects will also be recruited to whom the questionnaire will be submitted to assess the variability of the score within a healthy population. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| SNAP questionnaire | Other | The SNAP questionnaire will be administered 2 times in 2 consecutive days to evaluate its reliability. The questionnaire will be self-filled by the patient. Then the SPRS scale and, where possible, the 6MWT will be administered. |
| Measure | Description | Time Frame |
|---|---|---|
| Test the validity, investigating the correlation with Spastic Paraplegia Rating Scale and with Six-Minute Walk Test, and the reliability (through test-retest mode) of the SNAP questionnaire in the aforementioned population. | the Spastic Paraplegia Rating Scale (SPRS) and the Six-Minute Walk Test (6MWT) which assess the severity of the disease and the level of endurance respectively. | 2 days |
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Inclusion Criteria:
Exclusion Criteria:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Eleonora Diella | Bosisio Parini | Italy |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 38557877 | Derived | Diella E, D'Angelo MG, Stefan C, Girardi G, Morganti R, Martinuzzi A, Biffi E. Validation of the Italian version of a patient-reported outcome measure for Hereditary Spastic Paraplegia. PLoS One. 2024 Apr 1;19(4):e0301452. doi: 10.1371/journal.pone.0301452. eCollection 2024. |
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| ID | Term |
|---|---|
| D015419 | Spastic Paraplegia, Hereditary |
| ID | Term |
|---|---|
| D015417 | Hereditary Sensory and Motor Neuropathy |
| D009421 | Nervous System Malformations |
| D009422 | Nervous System Diseases |
| D020271 | Heredodegenerative Disorders, Nervous System |
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| D019636 | Neurodegenerative Diseases |
| D011115 | Polyneuropathies |
| D010523 | Peripheral Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D030342 | Genetic Diseases, Inborn |