Sunday 17 May 2015

Recent Research Papers

Having recently been alerted to the Ampyra/Dalfampridine story, I wondered if I was missing out on anything else. I used the http://www.ncbi.nlm.nih.gov/pubmed?term=((hereditary%20OR%20familial)%20AND%20%22spastic%20paraplegia%22)%20or%20%22strumpell%20lorrain%22 link to get my results and simply browsed interesting titles. The following is an overview of 10 interesting papers which I found, and why....

1) A treatable mimic of HSP

This paper from the Neurology Unit at the UK University of Sheffield reports a condition that is more rare than HSP - Cerebrotendinous xanthomatosis - which can cause spastic paraplegia. The condition is treatable with chenodeoxycholic acid if diagnosed in the early stages. The researchers hope that genetic sequencing may identify people earlier and allow treatment. It is not clear from the abstract if the person had been diagnosed with HSP, but I infer this from the title. I wonder if this might open up further potential for treatment for HSP?
http://www.ncbi.nlm.nih.gov/pubmed/25862734

2) Ampyra/Dalfampridine

No surprises to find this paper again.  This paper from the neurology unit at CHRU de Besancon in France showed that half of HSP patients in a trial responded favourably to treatment with Dalfampridine with improved walking. Another potential treatment for people.
http://www.ncbi.nlm.nih.gov/pubmed/25808501

3) Intrathecal Baclofen

Two papers report improvements in walking from HSP patients from the use of intrathecal baclofen. The first paper from the University Medical Center Groningen, in The Netherlands reports one patient who had improvements in walking from the use of both a test implant and a baclofen pump. The paper recommends the use of the pump for people who do not respond well to oral tablets. The second paper from the University of Athens Medical School, in Greece reports improvements in all 14 patients in the study which lasted on average for about 2 years. These patients had not responded well to oral tablets. The paper reports that intrathecal baclofen can improve walking/spasticity but that the improvement might be limited by either of the residual motor function or the patient continuing with their rehabilitation programme.
http://www.ncbi.nlm.nih.gov/pubmed/25626112
http://www.ncbi.nlm.nih.gov/pubmed/24973568

4) Robotic Gait Training

Two papers present improvements in gait obtained by robotic training. The first paper, from the University Hospital of Pisa in Italy reports that 13 patients with uncomplicated HSP got improvements in gait/walking and balance after a 6 week robotic gait training programme. The benefits were maintained at a re-test 2 months later. The recommend that robotic gait training is considered in exercise routines. The second paper, from Seoul National University Hospital in Korea reports that one patient improved walking speed and balance after a 6 week programme of robotic gait training and physiotherapy. They note that whilst speed and balance improved the gait itself did not (kinematics and kinetics).
http://www.ncbi.nlm.nih.gov/pubmed/25547770
http://www.ncbi.nlm.nih.gov/pubmed/25255290

5) Pelvic Floor Training

This paper, from the University of São Paulo in Brazil reports one woman with HSP who had bladder and bowel complaints, pelvic pain and pain during intercourse. A course of perineal and pelvic floor stretching was developed which resulted in less pain and improvements in bowel and bladder function.
http://www.ncbi.nlm.nih.gov/pubmed/25478261

6) Botox

This paper, from the Radboud University Medical Centre in The Netherlands reports that 15 patients with HSP were given a course of Botulinum toxin type-A in their calf muscles together with daily calf muscle stretching over 18 weeks improved walking speed and muscle tone. It is not clear from the abstract which was deemed to be more important - the Botox or the stretching.
http://www.ncbi.nlm.nih.gov/pubmed/25325386

7) Gait Variation

This paper, from the Geneva University Hospitals in Switzerland reports a gait analysis of 6 patients with HSP from the same family, with a follow up between 4 and 15 years after. Their analysis shows that there is a large variation in walking ability within one family (the inference is that they all have the same type of HSP). Over the period of the study three of the patients had improved their gait (from childhood) and three had worsened (within adulthood). There was no statistical difference between the sets.
http://www.ncbi.nlm.nih.gov/pubmed/25218933

8) Stem Cell Research

This paper, from Germany & USA, describes that stem cells derived from normal adult cells have been shown to have some relevance to HSP - potentially allowing a model of the neurons.
http://www.ncbi.nlm.nih.gov/pubmed/24821704

9) Temperature Effects

This paper, from the USA, describes that drosophila (fruit flies) with HSP have improved mobility and survive longer when reared in colder temperatures. They suggest that mild hypothermia might hold promise as a therapeutic approach for HSP. I note, however that many people complain that HSP appears worse in the cold.
http://www.ncbi.nlm.nih.gov/pubmed/24906373

10) Japanese Research Group

I note this paper, from Japan, simply because it was produced by the Japan Spastic Paraplegia Research Consortium (JASPAC). I wasn't aware of JASPAC previously, and this simply puts me on a mission to try and find out more about what they are doing. There are also other papers above from areas of the world which I've not seen before in HSP reporting, again opening up further investigation.
http://www.ncbi.nlm.nih.gov/pubmed/25296875

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