Miscellaneous Demyelinating Diseases

Last updated on Tuesday, September 9 2008 by jdmiles

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Tumefactive demyelination, H&E stain x 400
Acute demyelinating lesions may demonstrate prominent astrocytic pleomorphism exemplified by a binucleate cells seen here. Note, however, the preservation of low nuclear to cytoplasmic ratio

Central Pontine Myelinolysis, transverse section
Although clearly pontine and central, more than the myelin undergoes lysis secondary to osmotic stress upon over rapid correction of hyponatremia. The presence of even grossly visible cavitation explains the poor prognosis of symptomatic patients with this disorder.

Tumefactive demyelination, H&E x 400
The enlarged cell with the bizarre fragment appearing nucleus was first described by Creutzfeldt and Peters in the early part of the twentieth century. Though not pathognomonic of tumefactive demyelination, the cells are characteristically seen within tumefactive demyelinating lesions

Leigh's disease, coronal section through basal ganglia
Symmetrical discoloration of putamen and thalamic nuclei, accompanied by bilateral cavitation of the substantia nigra

Leigh's disease, transverse section through midbrain
Bilateral cavitary degeneration involving substantia nigra, periventricular gray matter, and brachium conjunctiva

Machiafava-Bignami disease,Coronal sectioned through basal ganglia and cerebrum
Bilateral cavitary lesions within corpus callosum, suggesting pathogenesis similar to central and extra pontine myelinolysis

Tumefactive demyelination, intraoperative crush preparation
Lipid laden macrophagesare readily appreciated on cytologic preparations

Tumefactive demyelination, low power H&E stained section
Sharp demarcation is typical in tumefactive demyelination (if the interface is available).

Tumefactive demyelination, Creutzfeldt-Peters cell
TLDL creutzfeldt cell

Tumefactive demyelination, lesion/brain interface, medium power H&E stain slide
Sharp demarcation is typical in tumefactive demyelination (if the interface is available).

Tumefactive demyelination, high power H&E stained slide demonstrating perivascular inflammation
Although perivascular inflammation may be encountered in a variety of tumors, the combination of this lymphocytic perivascular infiltrate with the surrounding lipid laden macrophages is typical in demyelinating lesions

Tumefactive demyelination, immunohistochemical staining for glial fibrillary acidic protein
GFAP immunohistochemical staining demonstrates evenly spaced reactive astrocytes with abundant processes and relatively small eccentric nuclei. Macrophages stand out as negatively stained cells within the fibrillary brown background.

Stereotactic biopsy of tumefactive demyelinating lesion, H&E stain x 20
The circumscribed nature of tumefactive demyelination is well demonstrated within the stereotactic biopsy core

Tumefactive demyelination, brain/lesion interface, high power H&E stain
Even at high magnification, sharply circumscribed nature of tumefactive demyelination is apparent

Tumefactive demyelination, immunohistochemical staining for CD68
CD68 labels macrophages, which account for much of the hypercellularity seen within these tumefactive lesions

Tumefactive demyelination, frozen section preparation, H&E stain x 100
Discerning macrophages is much more challenging on frozen section band at permanent section or in cytologic preparations

Tumefactive demyelination, immunohistochemical staining for GFAP x 100
This photograph is taken at the edge of the tumefactive demyelinating lesion. Note the intimate admixture of GFAP-positive reactive astrocytes and GFAP nonreactive macrophages.

Tumefactive demyelinating lesion, GFAP immunohistochemical staining x 200
immunohistochemical That staining for GFAP demonstrates classic reactive morphology, and highlights macrophages due to their absence of staining

Tumefactive demyelination, H&E stained slide x 200
Binucleate and even multinucleated astrocytes are common in tumefactive demyelination

Tumefactive demyelination, H&E stain slide x 200
Astrocyte pleomorphism is commonly seen within tumefactive demyelinating lesions

tumefactive demyelination, brain/lesion interface, neurofilament immunostaining x 100
Axons are relatively well preserved within tumefactive demyelinating lesions. macrophages stand out as negatively stained well circumscribed discohesive cells within the neurofilament matrix.

Tumefactive demyelination, intraoperative crush preparation x 200
Macrophages are readily appreciated in cytologic preparations of demyelinating lesions.

Tumefactive demyelination, low power H&E stain slide
Hypercellular astrocytic proliferation may suggest neoplasia. However, cytologic features of malignancy are not identified.

Tumefactive demyelination, H&E stained slide x 200
Tumefactive demyelinating lesions may be confused with oligodendroglial tumors if macrophages are not recognized for what they are.