Saturday, 19 January 2019

2018 HSP Research Overview

I had a quick look at the PubMed papers with HSP in them published in 2018. My highlights are:

Spinal electrical stimulation reduces spasticity
A study in Italy tested Transcranial Direct-Current Stimulation delivered delivered over the thoracic spinal cord in a clinical trial for 11 people with HSP with treatment over 5 days and assessment over 2 months. Several measures were made, some showing improvement and others showing no change.
Spasticity was shown to be reduced, and it is suggested that this might be a complementary measure.

Botox injections shown to reduce fatigue
A study in Brazil examined the effects of Botox in 33 HSP patients. The Botox injections were shown to reduce spasticity in the adductors, although the paper notes that there was no significant functional improvement. The papers also examined non-motor symptoms and it was shown that fatigue was improved after the Botox treatment.

Future Treaments
Work in Germany has investigated the use of tideglusib in SPG11 stem cells and it was shown to rescue neurodegneration. It is identified as a candidate compound for future clinical applications for SPG11 and other complex HSPs :

Work in Germany has also trialled atorvastatin in a short term trial of 14 people with SPG5. This study didnt show effects on HSP as expected, but did control levels of 27-hydroxycholesterol which has been shown to correlate with disease severity and disease duration, thereby demonstrating a potential treatment strategy:

More data on Bowel and Urinary Problems
Three studies have undertaken more work on quantifying bladder effects of HSP.
A study in Germany examined 71 HSP patients showing 75% having one or more urinary problems and reduced quality of life:

A study in Japan looked at 12 HSP patients and similarly showed 75% having urinary problems:

A study in Norway looked at 108 HSP patients and reported data on both bowel and urinary problems. For bowels they show 15% are affected by constipation, 8% are affected by alternating constipation and diarrhoea, 48% have uncontrollable flatulence, 12% have fecal incontinence, and 39% have an inability to hold back stools. Urinary complaints included 27% needing to urinate more than 8 times a day, 52% having sudden urgency and 31% having urinary incontinence at least daily/nightly:

Worth a read:
There are three other papers which I think are worth a read:

A study in the USA notes that the more recent genes for HSP are rare and there is likely to be a diagnostic gap.

HSP is specifically included in this estimate of the global burden of disease for motor neuron diseases. The study doesnt report any specifics for HSP, but reports that the burden from motor nuron diseases on health systems is likely to increase substantially in coming decades:

A study from the USA reviewed the cases of 174 children referred to a neurology clinic for toe walking. 62% were found to have a neurological condition. Of these the most common diagnosis was cerebral palsy (37%). HSP was shown in 14% of cases, with peripheral neuropathy and autistic spectrum disorder ranking between CP and HSP.

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