Aggressive multiple sclerosis

Case contributed by RMH Neuropathology

Presentation

Confusion, agitation and abulia.

Patient Data

Age: 30 years
Gender: Male

MRI brain

Modality: MRI

Extensive predominantly periventricular white matter lesions are present which demonstrate enhancement and restricted diffusion. There is involvement of the corpus callosum. 

Three lesions are also seen in the thoracic spine (not shown) involving the dorsal columns. 

The patient was treated with plasma exchange and steroids and made some clinical and radiological progress (not shown). When the steroids were stopped the patient deteriorated and was re-scanned. 

MRI brain 3 months later

Modality: MRI

A number of very large peripherally enhancing lesions have developed within the white matter. Many of these demonstrate open ring enhancement. There is restricted diffusion at the margin of the lesions. The lesions seen on the previous study have improved and no longer enhance or restrict. 

Brain biopsy

Modality: Pathology

MACROSCOPIC DESCRIPTION:

"Right parietal brain biopsy":  Multiple pieces of soft tan tissue up to 5mm 

MICROSCOPIC DESCRIPTION:

Paraffin sections show fragments of cerebral cortex and white matter. Cortex is unremarkable. The white matter is densely infiltrated by foamy macrophages containing granular PAS+/Luxol Fast Blue positive material consistent with myelin breakdown products. Prominent reactive astrocytes are scattered among the macrophage population. T lymphocytes, predominantly CD8+, cells are scattered among the macrophage cell polulation. Smaller numbers of CD4+ T lymphocytes are also present. Macrophages and activated microglia show strong surface expression of MHC Class I antigen. Luxol Fast Blue staining shows loss of myelin and Bodian silver impregnation shows axonal fragmentation and formation of axonal spheroids indicating recent axonal injury. The overall features are of inflammatory demyelination.  No viral inclusion bodies are identified There is no evidence of tumour.

Immunostaining for SV-40/JC-virus is NEGATIVE

FINAL DIAGNOSIS: 

"Right parietal brain biopsy":  Inflammatory demyelination with features favouring plaque demyelination rather than ADEM. 

Case Discussion

This case illustrates how aggressive multiple sclerosis can be. The appearances on the second scan, taken out of clinical context, would be much more suggestive of ADEM if the presence of old lesions was not appreciated. 

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Case Information

rID: 24397
Case created: 13th Aug 2013
Last edited: 28th Feb 2016
Inclusion in quiz mode: Included

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