MRCP PACES Cord Syndromes Spastic Paraparesis
  • Clinical picture
    • Walking aids / wheelchair
    • Disuse atrophy
    • Increased tone, usually in the lower limbs first
    • Hyperreflexia
    • Pyramidal weakness below the level of the lesion
    • Sensation usually intact
      • Some forms of hereditary spastic paraplegia may involve mild dorsal column loss
  • Causes
    • Human T cell lymphotropic virus-1 (HTLV1, tropical spastic paraparesis)
      • Retrovirus which infects CD4 T cells
      • Transmission: vertical, sexual, contaminated blood products
      • Often in Africa, South America and southern Japan
      • Also causes adult T cell leukaemia / lymphoma
      • Lifetime risk of haematological or neurological events in carriers: 0.25 – 3%
      • Usual onset 40 – 50, more common in women
      • Onset usually asymmetric and gradual, with gait and urinary disturbance
      • May have brisk upper limb reflexes
    • Hereditary spastic paraplegia
      • Almost 50 gene loci
      • De novo mutations are frequent
      • May be inherited in autosomal dominant, autosomal recessive or X-linked pattern
        • Autosomal dominant is most common
      • Phenotype and penetrance is variable – determining family history may be difficult
      • Usually presents in the fourth decade (range of first till seventh decade)
      • Spastic paraparesis, usually of the lower limbs
        • 30% have dorsal column involvement
        • 30% have neurogenic bladder
    • Primary lateral sclerosis (see Motor Neurone Disease)
  • Investigation
    • Peripheral blood film
      • Atypical T lymphocytes with convoluted nuclei – “flower cells” (HTLV1)
    • HTLV1 antibodies in CSF and serum
    • Genetic testing for hereditary spastic paraparesis (most common is spastic paraplegia gene 4)
  • Management
    • Multidisciplinary team approach
    • HTLV
      • Transient improvement with corticosteroids
      • Combivir (lamivudine + zidovudine) may decrease viral load, no evidence for effect on neurological function
Previous Topic
Back to Section
Next Topic