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A case of tumefactive demyelination

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Introduction and objectives

To describe a case of tumefactive demyelinating disease and discuss the difficulty in differentiation from tumor.  

Case presentation

A 50 y/o male from Qatar presented for worsening R sided weakness.

18 years prior to presentation the patient developed diffuse weakness of upper and lower extremities symmetrically. Had EMGs, diagnosed with CIDP. Treated with regular IV steroids for four years then switched to azathioprine. His symptoms had resolved and he was clinically stable for many years.  10 months prior to presentation he agreed with MD in Kuwait to decrease the dose in azathioprine from 150mg daily to 100mg daily for 2 mos. 2 months after that they did EMG showing "a good result". Dose was then further decreased to 75mg daily.

Two months prior to presentation he developed heaviness of his RLE affecting his gait. This was initially attributed to the CIDP so azathioprine dose was increased. However, the weakness worsened and by one month prior to presentation the weakness had spread to RUE as well. 

MRI of the brain showed a large, L parietal periventiruclar lesion with patchy enhancement and very little edema. There were also small, non-enhancing lesions to the L pons and L corona radiate.  MRI of the C spine was nl. CSF showed nl glucose and protein, no pleocytosis, no OCBs, negative viral and infectious studies, and negative cytology.  

A course of IV steroids was given without signficant improvement.  

 

Clinical presentation

 

On initial presentation, the patient's exam was notable for mildly decreased sensation to the face in the VI-V3 distribution, trace spasticity to his RLE, trace weakness to the R deltoid, R iliopsoas, and R hamstring, mild decrease in sensation to pin throughout the R side, and decreased reflexes throughout.  He walked with mild circumduction of his R leg, and had difficulty with heel and toe walking on the R, and mild difficulty with tandem walking. 

 

Imaging

Reimaging showed progression of the size and extent of enhancement of the left parietal lobe lesion. 

Laboratory studies

Repeat CSF testing showed no pleocytois and mildly elevated protein (56).  Infectious studies were negative.  No oligoclonal bands were found.  Cytology showed many small and few medium sized lymphocytes, and few plasma cells and monocytes.  Flow cytometry did not show evidence of lymphoma.   

Brain biopsy showed small, well demarcated area of an inflammatory demyelinating process with abundant macrophages, myelin loss, relatively spared axons and T cell response (only few B cells present). In the sampled specimens, there is no evidence of lymphoma. Stainting for JC virus was negative.

 

Diagnosis

Tumefactive demyelination.  

Initial treatment

Clinically the patient’s R sided weakness continued to worsen.

With results consistent with a demyelinating process, the patient was given 5 days of IV solumedrol, followed by two doses of rituximab.   When last seen the patient's R sided weakness had improved significantly (he estimated 70% improvement) and he said he was continuing to improve on a daily basis.

Outstanding questions

Initial concern for CNS lymphoma was very high, especially given many years of immunosuppression.  Ultimately biopsy revealed a demyelinating process.  Whether this patient will continue to do well with immunomodulatory therapy remains to be seen.  

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Comments

Very interesting case but caution is warranted. This could still represent PCNSL and sentinel demyelination could be misleading and mask an underlying lymphoma. A lymphoma could also present as there are reports that 'histological conversion' can occur. ADEM and tumefactive demyelination are reasonable diagnoses but PCNSL is still a strong consideration. Lastly, PML should be also be in mix and must be tested for, in CSF, via JCV PCR, as the authors know.