68 Physiotherapy Research International, 4( 1), 1999 © Whurr Publishers Ltd CASE REPORT Management of spasticity in hereditary spastic paraplegia DAVINIA RICHARDSON The National Hospital for Neurology and Neurosurgery, London, UK ALAN J THOMPSON Institute of Neurology, London, UK INTRODUCTION Hereditary spastic paraparesis (HSP) or Strumpell's disease is a genetically determined neurodegenerative disorder in which the signs and symptoms are predominant in the legs (Bruyn, 1992). The disorder is very slowly progressive and is extremely variable in terms of severity (Harding, 1981; Kolodny et al., 1989). It has been divided into two subgroups according to the age of onset. Type I onset tends to be below 35 years and Type II over 35 years. Type II presents with more marked muscle weakness, urinary and sensory symptoms and tends to progress more rapidly than Type I (Harding, 1981). The clinical signs comprise spastic paraparesis, hyperreflexia of the legs and, to a lesser degree, the arms, and Babinski signs. Sometimes, slight sensory disturances are found, such as diminished vibration sense at the feet and urinary urgency (Bryun, 1992). Pathologically, the abnormal findings in HSP are virtually confined to the spinal cord (Behan and Maia, 1974). There is degeneration of the crossed pyramidal tracts which increases caudally and is least severe in the cervical region. Atrophy of the cord is a characteristic feature (Schwarz and Lui, 1956). The dorsal root ganglia and posterior roots and peripheral nerves are normal. In all cases the disease process is unremitting and continually progressive. Patients with HSP can become progressively more disabled due largely to spastic- ity (Harding, 1984). Treatment for the condition is symptomatic, there is no defini- tive therapy. In view of the slow progression of symptoms an active programme of treatment needs to be available to meet the needs of the patient at varying stages of the disease. Supportive treatment consists of antispastic agents, such as baclofen, dantrolene sodium and tizanidine, along with regular physiotherapy. Orthotics and other assistive divices may prove useful at various stages through the disease. In some cases the use of intrathecal baclofen has been shown to be of benefit (Meythaler et al., 1992).