J Clin Neurol.  2007 Mar;3(1):45-49. 10.3988/jcn.2007.3.1.45.

Rapidly Progressive Toxic Leukoencephalomyelopathy with Myelodysplastic Syndrome: a Clinicopathological Correlation

• Jung KH
• Chu K
• Kim YA
• Jeon BS

• KMID: 1700686
• DOI: http://doi.org/10.3988/jcn.2007.3.1.45

Abstract

Neurological disorders induced by long-term exposure to organic solvents typically have a slowly progressive clinical course, which may be arrested or even reversed following discontinuation of exposure. We report an unusual case of rapidly progressive toxic leukoencephalomyelopathy in a 29-year-old man who had worked at a chemical factory that used toluene for the manufacture of nylon 66 for 5 years. He presented with progressive weakness of legs, recurrent seizures, and cognitive decline. Widespread white-matter changes in the brain and spinal cord, and myelodysplastic syndrome were noted. He died 6 months after the onset of his symptoms, and autopsy showed discrete multifocal demyelination and necrosis in the central nervous system, and dysplastic cells of erythroid, myeloid, and megakaryotic lineages in blood vessels. The co-occurrence of leukoencephalomyelopathy and myelodysplastic syndrome highlights the vulnerability of the white matter and bone marrow to injury from organic solvents. Intravascular congestion of dysplastic hematopoietic cells might have led to his unusually rapid progression of leukoencephalomyelopathy.

Keyword

Toxic leukoencephalomyelopathy; Organic solvent; Toluene; Myelodysplastic syndrome

MeSH Terms

Adult
Autopsy
Blood Vessels
Bone Marrow
Brain
Central Nervous System
Demyelinating Diseases
Estrogens, Conjugated (USP)
Humans
Leg
Myelodysplastic Syndromes*
Necrosis
Nervous System Diseases
Nylons
Seizures
Solvents
Spinal Cord
Toluene
Estrogens, Conjugated (USP)
Nylons
Solvents
Toluene

Figure

• 

  Figure 1 Cranial axial T2-weighted MRI images show multiple high-intensity signals in the periventricular white matter and symmetric hypointensities in the thalamus (A, C), and a T1-weighted MRI image shows multifocal calcification in the periventricular white matter (B). Follow-up brain MRI performed 3 months later (D-F) shows more extensive, bilateral, and symmetrical high-intensity signals in the white matter. Note that the differentiation between the gray and white matter is preserved. Spinal T1-weighted MRI image shows mild atrophy (G), and T2-weighted MRI reveals extensive high-intensity signals throughout the spine (H).

• 

  Figure 2 Coronal cross section of the brain shows diffuse gray-yellow discoloration and softening of the white matter (A). The white matter shows a diffuse area of necrosis (white arrowheads) with dystrophic calcification (black arrowheads; B), and the high-magnification view of the necrotic white matter shows rarefaction and occasional reactive astrocytes (white arrow; C). The cerebral blood vessel was filled with dysplastic hematopoietic cells. (D) Black arrow indicates a foam cell in the necrotic white matter.

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