Susanne A Schneider1,2, Viktoria E Beckinger3, Bettina Möller3, Stephanie Knüpfer4, Moritz Hamann4, Günther Deuschl3. 1. Department of Neurology, Christian-Albrechts-University Kiel, Kiel, Germany. susanne.schneider@med.uni-muenchen.de. 2. Department of Neurology, Ludwig-Maximilians-University München, Feodor-Lynen-Straße 19, 81377, Munich, Germany. susanne.schneider@med.uni-muenchen.de. 3. Department of Neurology, Christian-Albrechts-University Kiel, Kiel, Germany. 4. Department of Urology, Christian-Albrechts-University Kiel, Kiel, Germany.
Abstract
BACKGROUND: Urinary involvement is common in hereditary spastic paraplegias (HSPs), but has rarely been assessed systematically. METHODS: We characterized urinary complaints in 71 German HSP patients (mean age 55.4 ± 13.9 years; mean disease duration 20.7 ± 14.3 years; 48% SPG4-positive) using validated clinical rating scales (SCOPA-AUT, ICIQ-SF, ICIQ-LUTSqol). Treatment history and satisfaction with medical care was also assessed. RESULTS: 74.6% of patients had one or more urological problems, most commonly nocturia and urgency. Incontinence was more severe in women, correlating with SCOPA-AUT. Female gender and SPG4 mutations were associated with higher urinary frequency and severity of urological involvement. QoL was overall reduced, more in women and in SPG4 mutation carriers. Almost 90% consulted a medical specialist; more than half were largely satisfied. 43.4% received oral medication and 5.7% received intravesical botulinum toxin. However, more than one-third of patients remained untreated. CONCLUSION: Urinary complaints are common in HSP and should be addressed and treated.
BACKGROUND: Urinary involvement is common in hereditary spastic paraplegias (HSPs), but has rarely been assessed systematically. METHODS: We characterized urinary complaints in 71 German HSPpatients (mean age 55.4 ± 13.9 years; mean disease duration 20.7 ± 14.3 years; 48% SPG4-positive) using validated clinical rating scales (SCOPA-AUT, ICIQ-SF, ICIQ-LUTSqol). Treatment history and satisfaction with medical care was also assessed. RESULTS: 74.6% of patients had one or more urological problems, most commonly nocturia and urgency. Incontinence was more severe in women, correlating with SCOPA-AUT. Female gender and SPG4 mutations were associated with higher urinary frequency and severity of urological involvement. QoL was overall reduced, more in women and in SPG4 mutation carriers. Almost 90% consulted a medical specialist; more than half were largely satisfied. 43.4% received oral medication and 5.7% received intravesical botulinum toxin. However, more than one-third of patients remained untreated. CONCLUSION: Urinary complaints are common in HSP and should be addressed and treated.
Entities:
Keywords:
Bladder; Hereditary spastic paraplegia; International Consultation on Incontinence Questionnaire; Nocturia; Quality of life; SCOPA-AUT; SPG4; Satisfaction with care; Urgency; Urinary frequency; Urinary hesitancy; Urinary incontinence
Authors: Bas J H van Lith; Hans C J W Kerstens; Laura A C van den Bemd; Maria W G Nijhuis-van der Sanden; Vivian Weerdesteyn; Rob J E M Smeets; Klemens Fheodoroff; Bart P C van de Warrenburg; Alexander C H Geurts Journal: Orphanet J Rare Dis Date: 2020-03-04 Impact factor: 4.123