KANIJ FATEMA1, MIZANUR RAHMAN2, SHAHEEN AKHTER3, TAHMINA KARIM4
Introduction:
Hereditary spastic paraplegia (HSP) also called
Familial spastic Paraplagia or Strumpell- Lorrain
disease is a heterogeneous group of inherited
disorders1 in which the main feature is progressive
spasticity in the lower limbs due to pyramidal tract
dysfunction2. The prevalence range from 3 to 10 cases
per 100,000 populations in Europe.3
HSP was first described in 1883 by Adolph Strumpell,
a German neurologist, and was later described more
extensively in 1888 by Maurice Lorrain, a French
physician. 4 It is diagnosed using the criteria proposed
by Fink JK et al (1996) as 1) all alternative disorders
have been excluded 2) the family history supports an
inherited disorder 3) subject reports a progressive
disturbance and 4) neurological examination shows a
frank corticospinal tract deficit in the lower limbs,
including grade 4 hyperreflexia and extensor planter
responses.5