Hallervorden-Spatz disease
From WikiCNS
- i. A progressive autosomal recessive neurologic disorder that usually presents in the first decade of life
- ii. Symptoms: extrapyramidal features of dystonia, rigidity, spasticity, abnormal speech along with mental deterioration, and dementia
- iii. MRI shows hypointensity on T2 in the globus pallidus and substantia nigra
- iv. CT shows bilateral low densities in GP and SN
- v. Pathology: neuronal loss in the globus pallidus and substantia nigra from iron deposition (globus pallidus and substantia nigra turn yellow)
- 1. neuraxon spheroids (degenerating neurons) and iron deposition are the key pathologic features
