Pathology of roots, spinal cord and brainstem in syringomyelia-like syndrome of Tangier disease
JC Antoine, M Tommasi, S Boucheron… - Journal of the …, 1991 - Elsevier
We report here a post-mortem examination of a 46-year-old patient who died after a 23-year-
long syringomyelia-like syndrome of Tangier disease. The L5 dorsal root and the superficial
peroneal nerve showed fiber loss and lipid vacuole accumulation in Schwann cell
cytoplasm. The L5 ventral root had moderate fiber loss without lipid vacuoles. In the cervical
roots, fiber loss was intense and there were no foamy Schwann cells. Motor neuron loss was
severe in the cervical spinal cord and the facial nerve nucleus and slight at the lumbar level …
long syringomyelia-like syndrome of Tangier disease. The L5 dorsal root and the superficial
peroneal nerve showed fiber loss and lipid vacuole accumulation in Schwann cell
cytoplasm. The L5 ventral root had moderate fiber loss without lipid vacuoles. In the cervical
roots, fiber loss was intense and there were no foamy Schwann cells. Motor neuron loss was
severe in the cervical spinal cord and the facial nerve nucleus and slight at the lumbar level …