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


METHODS
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.
Sharmilee
S
Gnanapavan
Zane
Z
Jaunmuktane
Kelly Pegoretti
KP
Baruteau
Sakthivel
S
Gnanasambandam
Blizard Institute, Barts and The London School of Medicine & Dentistry, Queen Mary, University of London, London, UK. k.schmierer@qmul.ac.uk.
Klaus
K
Schmierer
D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001921:Brain / Q000473:pathology*; D005260:Female; D005862:Gerstmann Syndrome / Q000209:etiology*; D006801:Humans; D009103:Multiple Sclerosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D000069442:Natalizumab
D061067:Antibodies, Monoclonal, Humanized; D000069442:Natalizumab