FAMILIAL SPASTIC PARAPLEGIA (ALSO KNOWN AS STRUMPELLS DISEASE)

by Sue Edwards, Superintendent Physiotherapist at the National Hospital, Institute of Neurology, Queen Square, London

F.S.P. constitutes a group of clinically and genetically diverse disorders that share the primary feature of progressive, severe lower extremity spasticity (Fink et al 1995)

The most notable physical feature of this condition is the difficulty in co-ordinating gait. The pattern of spasticity affecting the legs is usually that of flexion, with the hips pulled in and rotated inwards, the knees bent and tending to knock together and the feet pushing downwards. There is difficulty in isolating the movement of one leg from the other and stepping forwards is achieved only with great effort.

These walking difficulties progress gradually over the years with people often complaining of increasing difficulty in cold weather. Some people may become dependent on a wheelchair for mobility. Shortening of the more affected muscle groups, which may lead to contracture, is a constant danger and although pain in itself is not a feature of this condition, many people with F.S.P. often develop pain as a result of stiff and painful joints. Prevention of the secondary complications of contracture is therefore of primary concern to reduce the abnormal stresses imposed on the lower limbs. The main emphasis of treatment is that of stretching of the more affected muscle groups to maintain mobility and preserve independence, Specific stretching exercises should be carried out following consultation with a physiotherapist. These cannot be listed here as each individual will have different problems necessitating a different regime. Strengthening exercises are not generally recommended as these tend to increase spasticity. However, exercise to improve control and therefore strength of the more vulnerable muscles is encouraged.

Splinting may be of value, not only to maintain range of movement at vulnerable joints, but also bio-mechanically to assist in correcting the gait pattern. For example, a below knee cast controlling the position of the foot and ankle at 90 degrees may improve hip and knee control during stance phase of walking. Again, the type of splint and the material from which it is made will differ depending on the presenting signs and symptoms.

With regard to the availability of neuro-physiotherapy within the NHS, this will vary from district to district. Most people with FSP will be referred to a physiotherapist, but often following the initial consultation there is little if any follow up. FSP is a relatively rare condition and therefore many therapists may only see one or two cases in their working lives. Whilst physiotherapy in itself cannot change the pathology of FSP, it is of paramount importance for therapists to monitor progress and instigate treatment as appropriate..