Troyer-Syndrom

Hereditary
spastic paraplegia • Ears of the lynx sign of hereditary spastic paraplegia - Ganzer Fall bei Radiopaedia
Coulthard, A. Ears of the lynx sign of hereditary spastic paraplegia. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-72453 • CC by-nc-sa 3.0 (modified)
Hereditary
spastic paraplegia • Hereditary spastic paraplegia with thin corpus callosum - Ganzer Fall bei Radiopaedia
Alsmair, A. Hereditary spastic paraplegia with thin corpus callosum. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-90083 • CC by-nc-sa 3.0 (modified)
Radiopaedia • CC-by-nc-sa 3.0 • de

Hereditary spastic paraplegia (HSP) refers to a heterogeneous group of neurodegenerative conditions characterized by progressive degeneration of the corticospinal tracts and posterior column of the spinal cord.

Clinical presentation

Patients often tend to have progressive lower extremity weakness and spasticity.

Pathology

Genetics

Most carry an autosomal recessive inheritance.

Subtypes
  • Troyer syndrome 

Radiographic features

MRI

Often non-specific and variable but can include:

  • corpus callosum atrophy
  • increased T2 signal intensity in the posterior limb of the internal capsule
  • spinal cord atrophy
  • ears of the lynx sign: high T2/FLAIR signal intensity at the tips of the frontal horns of the lateral ventricles
Siehe auch:
und weiter: