Translation and validation into Brazilian Portuguese of the Spastic Paraplegia Rating Scale (SPRS)

Servelhere, Katiane Raisa; Faber, Ingrid; Coan, Ana Carolina; França, Marcondes C.

doi:10.1590/0004-282x20160047

Cited by 19 publications

(18 citation statements)

References 13 publications

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“…Clinical and neurological evaluations were performed for SPG4‐HSP patients and controls. The severity of the disease and autonomic symptoms were measured with the Brazilian Portuguese validated versions of the Spastic Paraplegia Rating Scale (SPRS‐BR) and the Scales for Outcomes in Parkinson's Disease – Autonomic Questionnaire (SCOPA‐AUT) , respectively. The last questionnaire was chosen because it is the only autonomic instrument validated for our language.…”

Section: Methodsmentioning

confidence: 99%

Autonomic dysfunction in hereditary spastic paraplegia type 4

González-Salazar

Takazaki

Martínez

et al. 2019

Euro J of Neurology

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Background and purpose SPAST mutations are the most common cause of hereditary spastic paraplegia (SPG4‐HSP), which is characterized by progressive lower limb weakness, spasticity and hyperreflexia. There are few studies about non‐motor manifestations in this disease and none about autonomic involvement. Therefore, the aim was to determine the frequency and pattern of autonomic complaints in patients with SPG4‐HSP, as well as to determine the clinical relevance and the possible factors associated with these manifestations. Methods Thirty‐four molecularly confirmed SPG4 patients were recruited in a multicenter cross‐sectional study, of whom 26 underwent detailed neurophysiological testing (heart rate variability, sympathetic skin response and the Quantitative Sudomotor Axonal Reflex Test). The Scales for Outcomes in Parkinson's Disease – Autonomic Questionnaire (SCOPA‐AUT) was applied to quantify the severity of autonomic symptoms. Results were compared with 44 age‐ and gender‐matched healthy controls using non‐parametric tests. P values <0.05 were considered significant. Results In the SPG4‐HSP group, there were 18 men with a mean age of 47.7 ± 12.6 years. SCOPA‐AUT scores were similar between patients and controls (P = 0.238). Only the urinary domain subscore was significantly higher amongst patients (4 vs. 2.5, P = 0.05). Absent sympathetic skin response in the hands and feet was more frequent amongst patients (20% vs. 0%, P < 0.001, and 64% vs. 0%, P = 0.006, respectively). Quantitative Sudomotor Axonal Reflex Test responses were also smaller throughout all recording regions in the SPG4‐HSP group. Conclusion Our results indicate that SPG4‐HSP patients have sudomotor dysfunction caused by damaged small post‐ganglionic cholinergic fibers. Damage in SPG4‐HSP extends to the peripheral nervous system.

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Section: Methodsmentioning

confidence: 99%

Autonomic dysfunction in hereditary spastic paraplegia type 4

González-Salazar

Takazaki

Martínez

et al. 2019

Euro J of Neurology

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“…Patients underwent comprehensive neurological consultation assessing: age and type of first symptom; cognitive and behavioral complaints; and medical and family history. Disease severity was quantified through the Spastic Paraplegia Rating Scale (SPRS) . SPRS ranges from 0 (no abnormalities) to 52 and comprises motor and nonmotor signs and symptoms.…”

Section: Methodsmentioning

confidence: 99%

SPG11‐related parkinsonism: Clinical profile, molecular imaging and l‐dopa response

et al. 2018

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Background: Molecular imaging has proven to be a powerful tool to elucidate degenerated paths in a wide variety of neurological diseases and has not been systematically studied in hereditary spastic paraplegias. Objectives: To investigate dopaminergic degeneration in a cohort of 22 patients with hereditary spastic paraplegia attributed to SPG11 mutations and evaluate treatment response to l‐dopa. Methods: Patients and controls underwent single‐photon emission computed tomography imaging utilizing 99mTc‐TRODAT‐1 tracer. A single‐blind trial with 600 mg of l‐dopa was performed comparing UPDRS scores. Results: Reduced dopamine transporter density was universal among patients. Nigral degeneration was symmetrical and correlated with disease duration and motor and cognitive handicap. No statistically significant benefit could be demonstrated with l‐dopa intake during the trial. Conclusion: Disruption of presynaptic dopaminergic pathways is a widespread phenomenon in patients with SPG11 mutations, even in the absence of parkinsonism. Unresponsiveness to treatment could be related to postsynaptic damage that needs to be further investigated.

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“…Neurological severity was assessed by the Brazilian Portuguese version of the Spastic Paraplegia Rating Scale (SPRS). SPRS scores ranges from 0 to 52 and are crescent in severity (20). Disease stage was classified as: (0) asymptomatic; (1) no functional handicap, but signs at examination (slight gait stiffness); (2) mild gait stiffness, walking unlimited, and running still possible; (3) moderate gait stiffness, limited walking without aid, and running impossible; (4) moderate to severe gait stiffness, walking possible with aid; and (5) walking impossible, wheelchair bound.…”

Section: Methodsmentioning

confidence: 99%

Are Cognitive Changes in Hereditary Spastic Paraplegias Restricted to Complicated Forms?

et al. 2019

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Background: Little is known about the cognitive profile of Hereditary Spastic Paraplegias (HSP), where most scientific attention has been given to motor features related to corticospinal tract degeneration. Objectives: We aimed to perform a broad characterization of the cognitive functions of patients with pure and complicated HSP as well as to determine the frequency of abnormal cognitive performances in the studied subtypes. Methods: A two-center cross-sectional case-control study was performed. All individuals underwent cognitive assessment through screening tests (Mini Mental State Examination—MEEM and Montreal Cognitive Assessment—MOCA) and tests to assess specific cognitive functions (Verbal fluency with phonological restriction—FAS; Verbal categorical fluency—FAS-cat and Rey's Verbal Auditory Learning Test -RAVLT). Results: Fifty four patients with genetically confirmed HSP diagnosis, 36 with spastic paraplegia type 4 (SPG4), 5 SPG11, 4 SPG5, 4 cerebrotendinous xanthomatosis (CTX), 3 SPG7 and 2 SPG3A, and 10 healthy, unrelated control subjects, with similar age, sex, and education participated in the study. SPG4 patients had worse performances in MOCA, FAS, FAS-cat, and RAVLT when compared to controls. Most SPG4 patients presented cognitive changes not compatible with dementia, performing poorly in memory, attention and executive functions. SPG5 patients scored lower in executive functions and memory, and SPG7 patients performed poorly on memory tasks. All evaluated cognitive functions were markedly altered in CTX and SPG11 patients. Conclusions: Cognitive abnormalities are frequent in HSP, being more severe in complicated forms. However, cognitive impairments of pure HSPs might impact patients' lives, decreasing families' socioeconomic status and contributing to the overall disease burden.

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Translation and validation into Brazilian Portuguese of the Spastic Paraplegia Rating Scale (SPRS)

Cited by 19 publications

References 13 publications

Autonomic dysfunction in hereditary spastic paraplegia type 4

Autonomic dysfunction in hereditary spastic paraplegia type 4

SPG11‐related parkinsonism: Clinical profile, molecular imaging and l‐dopa response

Are Cognitive Changes in Hereditary Spastic Paraplegias Restricted to Complicated Forms?

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