Central Pontine Myelinolysis

Central pontine myelinolysis has been reported in malnourished chronic alcoholics. It also occurs in liver dis eases, liver transplants, severe burns, and hyponatremia. A rapid correction of hyponatremia is suggested to be a major cause of the disease. The onset is acute, with para- or quadriparesis, dysarthria, and dysphagia resembling the locked-in syndrome. The prognosis is poor. Death may occur within a few weeks; a small number of patients may recover partially or completely. The diagnosis is supported by CT scan and MRI.

Pathology: Grossly, a triangular or butterfly-shaped grayish, soft, slightly granular lesion is situated in the center of the basis of the pons. The histology is distinguished by demyelination of the fiber tracts, most commonly the transverse pontine fibers. The axons are relatively preserved except in severe and chronic cases, and the neurons are spared (Fig. 10.11). Lipid-laden macrophages are numerous in the acute stage, and reactive astrocytes predominate in the chronic stage. Similar lesions may occur outside the pons, in the striatum, thalamus, lateral geniculate bodies, cerebral white matter, and cerebellum.

figure 10.10

Alcoholic cerebellar atrophy. A. MRI of a chronic alcoholic man shows atrophy of the vermis, particularly the superior folia. B. Sagittal section of the vermis shows prominent atrophy of the superior folia in a 58-year-old alcoholic woman. Superior vermis shows (C) loss of the Purkinje cells and a prominent Bergmann astrocytosis (HE). D. Some basket cell fibers are preserved, and axonal torpedoes are present in the granular layer (Bodian stain).

figure 10.10

Alcoholic cerebellar atrophy. A. MRI of a chronic alcoholic man shows atrophy of the vermis, particularly the superior folia. B. Sagittal section of the vermis shows prominent atrophy of the superior folia in a 58-year-old alcoholic woman. Superior vermis shows (C) loss of the Purkinje cells and a prominent Bergmann astrocytosis (HE). D. Some basket cell fibers are preserved, and axonal torpedoes are present in the granular layer (Bodian stain).

figure 10.11

Central pontine myelinosis. An incidental autopsy finding in a 74-year-old man with Alzheimer's disease. A. Macrosection shows demyelination in the center of the pontine basis. Higher-magnification view shows in the demy-elinated area: (B) partial preservation of nerve fibers (Holmes stain).

figure 10.11

Central pontine myelinosis. An incidental autopsy finding in a 74-year-old man with Alzheimer's disease. A. Macrosection shows demyelination in the center of the pontine basis. Higher-magnification view shows in the demy-elinated area: (B) partial preservation of nerve fibers (Holmes stain).

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