NI01
A Case of Balo's Concentric Sclerosis with Typical MRI Findings and Complete Resolution of the Lesion Following Treatment

Thursday, May 31, 2018
Exhibit Hall A (Nashville Music City Center)
Shitiz K Sriwastava, M.D. , Neurology, Wayne State University School of Medicine, Detroit, MI
Aaron Desai, M.D. , Neurology, Wayne state university, Detroit, MI
Evanthia Bernitsas, MD , Neurology, Wayne State University School of Medicine, Detroit, MI
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Background:

Balo’s Concentric Sclerosis (BCS) is a rare and severe demyelinating disease of the central nervous system (CNS), and it is often viewed as a variant of multiple sclerosis (MS).  The typical lesion in BCS is characterized by concentric layers of demyelination alternating with layers of preserved myelin and is known as the “onion bulb” appearance.  It was thought that BCS represents an aggressive MS variant with poor prognosis. However, recent literature contradicts prior observations. We present a case of BCS with typical MRI findings and complete resolution of the lesion following treatment.

Objectives:

To report a case of Balo's concentric sclerosis which resolved completely after treatment with steroid and plasmapharesis.

Methods:

A 46-year-old man noticed numbness and tingling in his right upper extremity and left hand, followed by left eye visual impairment. MRI scan of the brain showed a lesion in left peritrigonal region, ovoid in shape, measuring 7-8 mm. Multiple patchy T2 lesions were also found in the cervical spinal cord at C5 with somewhat patchy distribution and no abnormal enhancement. CSF analysis was significant for more than 3 unique oligoclonal bands. CT of the chest was negative for hilar or mediastinal lymphadenopathy. The diagnosis of relapsing remitting multiple sclerosis (RRMS) was made.

A year later, following an MS relapse, repeat MRI scan of the brain showed a small enhancing focus at the posterior level of the Sylvian fissure on the right side, otherwise it was unchanged.

Three years later, the patient experienced a severe relapse with new left-sided weakness and hemiparetic gait.  MRI scan showed classic BCS appearance with alternating hypo and hyperintense rings. The patient was treated with IV methylprednisolone with some improvement, followed by 6 sessions of plasmapheresis. 

Results:

After treatment with steroids and plasma exchange, the patient’s disease course has been benign with only residual left hemiparesis and total resolution of the original Balo-like lesion.  A follow-up MRI scan showed total resolution of the original BCS lesion. 

Conclusions:

We present this case of BCS to shed light on this rare subtype of MS. Our case adds more evidence that new BCS can arise in the background of typical MS and highlights its potential benign course after treatment with corticosteroids and plasma exchange.