Disease Modifying Therapies – Mechanism of Action Poster Presentation

P0299 - Balo’s concentric sclerosis successfully treated with Alemtuzumab (ID 683)

Speakers
  • D. Paling
Authors
  • D. Paling
  • T. Hardy
  • A. Khan
  • J. Redgrave
  • N. Hoggard
  • S. Coley
  • R. Dyde
  • R. O Malley
  • Z. Yasiry
Presentation Number
P0299
Presentation Topic
Disease Modifying Therapies – Mechanism of Action

Abstract

Background

Balo’s concentric sclerosis is often regarded as a rare variant of multiple sclerosis (MS) and is characterised by concentrically multi-layered ring-like lesions in the cerebral white matter. Despite pathological overlap with MS, the effect of disease modifying therapies is unclear.

The only existing case report of Alemtuzumab in Balo’s concentric sclerosis described a lack of clinical response in a patient with Balo’s concentric sclerosis who had previously not responded to corticosteroids, plasma exchange and cyclophosphamide. The authors did speculate that Alemtuzumab may have been more effective if started earlier in the disease process.

Objectives

We present the imaging and clinical outcomes of a patient with Balo’s concentric sclerosis who was successfully treated with Alemtuzumab over a period of 3 years.

Methods

A 54 year old nurse with no prior history of neurological symptoms presented with a 2 day history of expressive dysphasia and subacute onset of right hemiplegia. MRI of the brain revealed a large enhancing lesion in the white matter of the posterior temporal lobe measuring 29.5mm in maximal diameter with a complex layered enhancement pattern. There were also non-enhancing lesions present in the periventricular and deep white matter with an appearance typical of demyelination due to multiple sclerosis. CSF examination revealed positive oligoclonal bands. Anti-aquaporin-4 IgG and MOG-IgG were negative. A diagnosis of Balo’s concentric sclerosis was made, and she was treated with intravenous and oral steroids, followed by Alemtuzumab.

Results

Clinically her speech and weakness gradually improved and she is now symptom free. She has had no further relapses. Radiologically the Balo’s lesion ceased to enhance and reduced in size and T2 hyperintensity gradually. She has had no new or enlarging T2 lesions.

Conclusions

We present the second case report of the use of Alemtuzumab in Balo’s concentric sclerosis. Our patient has enjoyed a good outcome with resolution of symptoms, and a period of freedom from relapse and radiological activity. Alemtuzumab may be effective in the treatment of Balo’s concentric sclerosis if started early.

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