Studies of non-HSP populations have shown FI is rarely disclosed to caregivers due to its’ sensitive nature and the stigma attached, and that FI can severely affect quality of life. Despite lack of optimal treatment solutions for FI, some identified causal factors may be reversed by simple means such as diet, bowel habits, and toilet access. The emphasis on FI symptoms was the result of close co-operation with representatives from the HSP patient user group in Norway, who experience reluctance among their members to discuss FI issues with health care providers. This is unfortunate, and in our experience, HSP patients regard FI as an important issue to address. It is less known to what extent fecal incontinence (FI), defined by unintentional loss of solid or liquid stool, is present in the HSP population. Increased voiding frequency, stress, and urge incontinence linked to neurogenic detrusor over activity has been described in adults with HSP. In Norway, the site of the current study, the estimated combined prevalence of pure and complex HSP has been reported to be 7.4:100000. At least 79 loci and 60 spastic paraplegia genes are currently identified. Complicated forms of HSP are mostly inherited in an autosomal recessive manner, while pure HSP is commonly inherited in an autosomal dominant manner. Several modes of inheritance are described, i.e., autosomal recessive, autosomal dominant, and more rarely, X-linked or maternal. Late onset HSP more often leads to rapid progression of symptoms, while early disease debut often heralds slower progression. Complex HSP is not limited to the lower extremities, and may include additional neurological symptoms such as ataxia, extrapyramidal signs, epilepsy, mental retardation, dementia, and peripheral nerve involvement. Pure forms of HSP affect the lower limbs and lead to slowly progressing spastic weakness, and micturition dysfunction. The term hereditary spastic paraparesis (HSP) is used to describe a genetically and clinically heterogeneous group of neurodegenerative diseases.
0 Comments
Leave a Reply. |
Details
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |