Physiotherapie bei Hereditärer Spastischer Spinalparalyse (HSP) Ein Systematischer Review der Literatur

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1 Physiotherapie bei Hereditärer Spastischer Spinalparalyse (HSP) Ein Systematischer Review der Literatur Luder Gere, Verra Martin Institut für Physiotherapie

2 Hereditary Spastic Paraplegia (HSP) Heterogene Gruppe neurologischer Störungen autosomal-dominant, autosomal-recessive or X-linked pure and complicated forms früher oder später Beginn Trotta et al, 2004, Curr Biol 14(13): Fortini et al, 2003, Funct Neurol 18(1):43-9 Fink, 2006, Curr Neurol Neurosci Rep 6(1):65-76

3 Hereditary Spastic Paraplegia (HSP) Hereditary genetisch bedingt Spastic Hauptsymptom Paraplegia primär Beine

4 Hereditary Spastic Paraplegia (HSP) Sogenannt seltene Krankheit In Europa leiden 30 Mio. an 8000 seltenen Krankheiten wenig Forschung, wenig Geld Rare Diseases Europe, Zugriff 19. April 2010 Komplexe Genetik Mehrere Gene und verschiedene Mutationen Sartucci et al, 2007, Brain Res Bull 28;74(4):243-9; Trotta et al, 2004, Curr Biol 14(13): Komplexe Neurophysiologie Ursache von Spastik Sartucci et al, 2007, Brain Res Bull 28;74(4):243-9; Fortini et al, 2003, Funct Neurol 18(1):43-9

5 Zwei Vorbemerkungen Selber nur wenig klinische neurologische Erfahrung Aussagen basieren primär auf der Literatur Externe Evidenz Kein echter Systematic Review kaum klinische Studien, keine physiotherapeutischen kein zweiter Reviewer, keine einheitliche Beurteilung Übersicht der Literatur

6 Relevanz Exemplarisch für externe Evidenz von Physiotherapie- Interventionen Beispiel für Studiensuche und Bewertung Mögliches Modell für Spastikbehandlung Bestmögliche Behandlung auch für seltene Krankheiten

7 Fragestellung Wie ist die aktuelle Evidenz für die physiotherapeutische Behandlung von Patienten mit HSP? Extensive Literatursuche, Review

8 Suchstrategie: Begriffe 1. Definition von Suchbegriffen Finden von Synonymen 2. Planung der Strategie 3. Suche in PubMed/MedLine Suche in PEDro Suche in Cochrane

9 hereditary spastic paraplegia Suchstrategie Spastic Paraplegia, Hereditary [MeSH] Strumpell disease Strumpell-lorraine Syndrome Strumpell-lorrain familial spasmodic paraplegia Spastic spinal familial paralysis Familial spastic paraplegia

10 Suchstrategie strength Exercise Muscle Strength [MeSH] [MeSH] Exercise Muscle stretching exercise [MeSH] Gait disorders, Neurologic [MeSH] spasticity gait Muscle Spasticity [MeSH] Therapeutics [MeSH] hereditary spastic paraplegia (HSP)

11 Suchresultate, PubMed, strength Exercise Muscle Strength [MeSH] [MeSH] Exercise hits Muscle stretching exercise [MeSH] Gait disorders, Neurologic [MeSH] spasticity hits gait Muscle Spasticity [MeSH] Therapeutics [MeSH] hits hereditary spastic paraplegia (HSP) hits hits

12 Suchresultate, PubMed, strength Exercise Muscle Strength [MeSH] [MeSH] Exercise hits Muscle stretching exercise [MeSH] Gait disorders, Neurologic [MeSH] spasticity hits gait Muscle Spasticity [MeSH] Therapeutics [MeSH] hits hereditary spastic paraplegia (HSP) hits hits

13 Suchresultate, PubMed, strength Exercise Muscle Strength [MeSH] [MeSH] Exercise hits Muscle stretching exercise [MeSH] Gait disorders, Neurologic [MeSH] spasticity hits gait Muscle Spasticity [MeSH] Totally 50 articles! Therapeutics [MeSH] hits hereditary spastic paraplegia (HSP) hits hits

14 Suchresultate und Auswahl im Überblick Suchergebnisse: 50 Artikel Auswahl durch Ausschluss: Genetische Studien, HSP nur Nebenthema, älter als 1985, Sprache 29 Artikel Durchgehen Referenzlisten zusätzlich 60 Artikel, Total 110 Ergänzte Auswahl 32 Artikel

15 Suchresultate und Auswahl im Überblick Ergänzte Auswahl 32 Artikel Clinical Trials zu PT-Interventionen 0 Artikel Studien mit konkreten Hinweisen zu PT 4 Artikel Studien zur Behandlung von Spastik (bei HSP) 10 Artikel

16 Ausgewählte Studien Authors Title Reference Year Study type Fink JK Hereditary spastic paraplegia Curr Neurol Neurosci Rep. (1): Fink JK Advances in the hereditary Exp Neurology spastic paraplegias 184, S 106-S110 Klebe S, Stolze H Gait analysis of sporadic and J Neurol. et al. hereditary spastic paraplegia 251(5): Pease WS. Therapeutic electrical stimulation Am J Phys Med Rehabil. for spasticity: quantitative gait 77(4): analysis Richardson D, Management of spasticity in Physiother Res Int Thompson AJ. hereditary spastic paraplegia 4(1): Thompson AJ, Jarrett L et al. Clinical management of spasticity J Neurol Neurosurg Psychiatry 76: Review (narrative) 2003 Review (narrative) 2004 Cross-sectional, Gait analysis 1998 Case study (n-of-1-trial) 1999 Case report 2005 Review / Editorial

17 Fink, 2003, Experimental Neurology 184,S 106-S110 Treatment for HSP limited to reducing muscle spasticity through exercise and medication. Lioresal is particularly useful in this regard. Tizanidine, Dantrolene, and Botox have also been useful to reduce muscle spasticity in HSP.

18 Fink, 2003, Experimental Neurology 184,S 106-S110 We advocate that HSP subjects participate in daily physical therapy designed to (1) improve muscle flexibility and range of motion (2) improve muscle strength (3) maintain walking reflexes (4) improve cardiovascular fitness. These recommendations are based not on the results of well-controlled, prospective studies, but on approximately 200 HSP subjects who have (nearly unanimously) reported benefit of daily physical exercise

19 Fink, 2006, Curr Neurol Neurosci Rep. 6(1): Individuals with HSP consistently report the benefits of stretching for 10 minutes twice a day and daily physical exercise designed to improve lower extremity strength and general cardiovascular conditioning. strength and gait improvement through physical therapy

20 Klebe et al, 2004, J Neurol. 251(5): Gait analysis of sporadic and hereditary spastic paraplegia The HSP-patients showed a reduction of the temporal and kinematic gait parameters (velocity, cadence, stride length, range of motion of the knee) due to the spastic paraparesis of the lower extremity.

21 Pease, 1998, Am J Phys Med Rehabil 77(4):351-5 Therapeutic electrical stimulation for spasticity: quantitative gait analysis Functional electrical stimulation for three months 2-3 times per week Quadriceps and ankle flexors Gait analysis before and after stimulation Following this treatment protocol, the patient reported that his coworkers had begun to comment on his improved ambulation, which was quite pleasing to him.

22 Pease, 1998, Am J Phys Med Rehabil 77(4):351-5 Repeat gait analysis demonstrated significant improvements in right hip and knee extension during the middle and late stance phase as well as improvements in symmetry of his gait pattern. An overall 26% increase in gait velocity was seen because of an improved cadence and improved length of step...

23 Richardson & Thompson, 1999, Physiother Res Int 4:68-76 Management of spasticity in hereditary spastic paraplegia. Case report of a single subject with HSP Conclusion Today's attempts at managing tone remain on a somewhat trial and error basis or a process of elimination. It is therefore important to monitor what is done and the patient's response to any intervention.

24 Thompson et al, 2005, J Neurol Neurosurg Psychiatry 76: Clinical management of spasticity (Review) Conclusion There is no agreed evidence based model available for the management of spasticity and much of what is done is based on a logical and pragmatic approach.

25 Schlussfolgerungen Sehr wenige Studien vorhanden Keine Clinical trials, ausser zur Medikation bei Spastik Kein klares physiotherapeutisches Konzept für HSP Offene Fragen Welche und wie viel Therapie brauchen HSP-Patienten? Welche (physiotherapeutischen) Interventionen sind sinnvoll bei Spastik? Wie kann das Management von HSP-Patienten und anderen Patienten mit Spastik verbessert werden?

26 Vielen Dank für die Aufmerksamkeit... Statements und vollständige Literaturliste als pdf-file bei gibt es Fragen?

27 Physiotherapie bei Hereditärer Spastischer Spinalparalyse (HSP) Ein Systematischer Review der Literatur Luder Gere,Verra Martin Institut für Physiotherapie