February 1997 Newsletter


I have an on-going need for FSP-related articles to include in the Newsletter. I do know from conversations I have had with several members, that some of you have interesting stories to tell. I am particularly keen to hear of any personal experiences of treatment - so we can build up a fuller picture of pros and cons of such procedures.

Also in this issue is an item on dietary supplements from Sue Padfield, and a new, revised, 'official' definition of FSP from Nick Wood, which forms the substance of our Group entry in the new edition of the Gaf Directory of Rare Conditions. I am indebted to both writers for their contributions.

FSP Survey Results

23 members completed the questionnaire, and gave details of a total of 46 sufferers. 28 of these were male, and 18 were female. 7 of the 23 respondents were the only ones in their families known to have FSP. With a sample of this size it must be stressed that the results can in no way be seen as an objective analysis of the condition - rather they mirror our Group as a whole.

Age of On-Set
Where an accurate age was known, 25 out of 41 (61%) suffered on-set before the age of 16. 14 members (34%) saw on-set after the age of 35. Only 2 people saw on-set between 16 to 35.

Time to Diagnosis
The figures were not statistically significant. However, in the Late On-Set Group (Age over 35) a ten year gap between perceived on-set and diagnosis was not uncommon. In the Early On-Set Group (Age under 16) the period between on-set and diagnosis was extremely varied but meaned out at about 4 years.

What was evident in this section was that for several families where parental on-set was in middle age, for off-spring the on-set came in childhood years - perhaps evidence of the phenomenon as 'anticipation', and explained by Dr Kathy White at our September meeting.

Two members reported that their condition had initially been diagnosed as Multiple Sclerosis, with final diagnosis coming rather by process of elimination.

Areas of Body Affected
100% are affected in their lower limbs. 4 respondents specified spinal problems, and a similar number kidney problems. 3 reported arm/hand problems.

Severity of Handicap
Wheelchairs are regularly used by 13 out of 41 respondents (31%). Walking sticks are used by 14 (34%) respondents. 8 people used no walking aids (20%). Other members used crutches, splints or walking frames.

After walking, the most common difficulty reported was urinary urgency/incontinence. 34% suffered this, albeit many stated they only suffrered mildly. 30% reported cramps as their major complication, with 27% reporting some degree of regular pain. Interestingly in this category, it was apparent that all members of several families were entirely pain free. Problems with the upper body were rare, with only single incidences of chewing/swallowing problems, dexterity or mental problems.

Access to Specialists
Because I had not asked the question whether people were actively seeking assistance, it would be wrong to derive any significant trends from the information given. What was apparent, however, was that far more people currently attend a consultant's clinic than attend a neuro-physiotherapist's clinic.

Family History
11 out of 23 families (48%) had positively identified FSP in their wider family or in earlier generations, whilst 9 families (40%) claimed no previous known history within their families.

Notwithstanding the statistically small sample, the results fell largely in line with current thinking and understanding of the FSP condition. The early and late on-set distinction was very apparent, and the symptoms and difficulties experienced were very much as expected. The Survey reinforced the perception that no two families are alike in the manner in which FSP manifests itself.

Dietary Supplements

This is important in the functioning of both nerves and muscles. Combined with calcium, it is known to help with muscle cramps.

The B Vitamins
These are jointly beneficial for the maintenance of condition of the nervous system. It is therefore prudent to take supplements to ensure there is no deficiency. It should be noted that stress increases the requirement for B vitamins. The MS sufferers report some relief of symptoms if they have regular B vitamin injections.

This is necessary for nerve development and function.

Vitamin E
This has an important neurological role and helps prevent degeneration of the nerves and muscles. This vitamin is abundant in many foods and it is unlikely that anyone is deficient in it. However, in certain circumstances a supplement may be prudent.

'Quest' is one of the better brand names of vitamin and mineral supplements amongst the many available. Some of the popular brands are just too weak to be of any use, or are poorly produced and thus difficult to absorb. The author's husband takes:

  • one Quest "improved Once a Day" tablet.
  • one "Super Mega B+C"
  • 1.5 times the daily recommended does of calcium and magnesium.

He felt real benefit from the calcium and magnesium supplements after the first couple of days of use as they go some way to reducing the tension in his legs.

The author's source document is 'Which Vitamin' by Hasnain Walji.

FSP Group Democratisation

On the 25th January the Committee held a telephone conference to discuss the democratisation process, and to discuss of the Group. A draft constitution was presented and discussed. The purpose of introducing a constition is to make the workings of the Group more transparent, and thus to encourage greater participation. A constitution is a necessary prerequisite in the obtaining of charitable status - our ultimate goal. The outcome of our discussions will be presented to you in the near future.

FSP: A Redefinition

Also known as Strumpell Disease, FSP describes a group of disorders characterised by progressive stiffness (spasticity) of the legs associated with varying degrees of weakness. Our current understanding permits us to classify this into broad types - the pure form in which Spastic Paraplegia exists alone, and the more complicated form in which a variety of neurological symptoms can co-exist. This latter group is much rarer than the former. The pure form can also be sub-classified into Type 1 and Type 2. This is dependent on age on-set; Type 1 has an early age of on-set; whilst Type 2 comes on somewhat later (typically after 35). A number of symptoms can herald the presentation of this condition including stiffness and leg cramps which often precede the emergence of a scissoring spastic gait; significant urinary problems are rare in this condition. The severity of the condition can also vary significantly both between families and within families.

Inheritance Patterns
Both autosomal dominant and recessive inheritances are reported, and very occassionally X linked has been shown. Autosomal dominant inheritance predominates thus giving a 50% chance risk to any infected individual's offspring.

Prenatal Diagnosis
No autosomal genes are as yet identified although linkage to some chromosomal areas has been demonstrated. The X linked gene loci are known but identification of mutations in these genes is complicated and no readily available prenatal diagnosis exists at the present.