Email updates

Keep up to date with the latest news and content from BMC Neurology and BioMed Central.

Open Access Case report

A rare presentation of atypical demyelination: tumefactive multiple sclerosis causing Gerstmann’s syndrome

Sharmilee Gnanapavan12, Zane Jaunmuktane3, Kelly Pegoretti Baruteau4, Sakthivel Gnanasambandam4 and Klaus Schmierer12*

Author Affiliations

1 Blizard Institute, Barts and The London School of Medicine & Dentistry, Queen Mary, University of London, London, UK

2 Department of Neurology, Barts Health NHS Trust, The Royal London Hospital, London, UK

3 Division of Neuropathology, UCLH Foundation Trust, The National Hospital for Neurology & Neurosurgery, Queen Square, London, UK

4 Department of Neuroradiology, Barts Health NHS Trust, The Royal London Hospital, London, UK

For all author emails, please log on.

BMC Neurology 2014, 14:68  doi:10.1186/1471-2377-14-68

Published: 2 April 2014

Abstract

Background

Tumefactive demyelinating lesions are a rare manifestation of multiple sclerosis (MS). Differential diagnosis of such space occupying lesions may not be straightforward and sometimes necessitate brain biopsy. Impaired cognition is the second most common clinical manifestation of tumefactive MS; however complex cognitive syndromes are unusual.

Case presentation

We report the case of a 30 year old woman who presented with Gerstmann’s syndrome. MRI revealed a large heterogeneous contrast enhancing lesion in the left cerebral hemisphere. Intravenous corticosteroids did not stop disease progression. A tumour or cerebral lymphoma was suspected, however brain biopsy confirmed inflammatory demyelination. Following diagnosis of tumefactive MS treatment with natalizumab effectively suppressed disease activity.

Conclusions

The case highlights the need for clinicians, radiologists and surgeons to appreciate the heterogeneous presentation of tumefactive MS. Early brain biopsy facilitates rapid diagnosis and management. Treatment with natalizumab may be useful in cases of tumefactive demyelination where additional evidence supports a diagnosis of relapsing MS.

Keywords:
Gerstmann’s syndrome; Tumefactive MS; Demyelination; Aphasia; Agnosia; Left-right disorientation; Dyscalculia; Natalizumab