Hemicraniectomy in Tumefactive Demyelinating Disease

Review Article


Petra Nilsson
Affiliation: Department of Neurology, Clinical Sciences Lund, Lund University, Sweden


ABSTRACT

Tumefactive demyelinating disease (TDD) is a rare demyelinating disease—probably a variant of multiple sclerosis (MS)—presenting as a focal cerebral mass. Clinically and radiographically, TDD is usually difficult to differentiate from tumor and abscess. Magnetic resonance imaging (MRI) characteristically shows a large lesion—contrast enhancement often like an incomplete ring—with relatively limited mass effect and surrounding edema. Findings on “non‐conventional” MRI, multifocality on MRI of the whole central nervous system (CNS), monitoring of visual evoked potentials, and cerebrospinal fluid analysis may all contribute to the differential diagnosis. In analogy with the recommendations for acute MS exacerbations, acute disseminated encephalomyelitis and other forms of acute conditions with severe inflammatory CNS demyelination, high‐dose corticosteroid treatment is regarded as first‐line therapy. A rapid and pronounced clinical and radiological response to the administration of high‐dose corticosteroids is often observed and may alleviate the need for cerebral biopsy. In agreement with the recommendations for other conditions characterized by acute CNS demyelination, plasma exchange is considered to be second‐line therapy. In patients who do not respond to non‐surgical therapy and who exhibit a pronounced cerebral mass lesion and clinical signs of increased intracranial pressure (ICP), decompressive craniectomy including wide opening of the dura is recommended.

Keywords: tumefactive demyelinating disease, multiple sclerosis, hemicraniectomy, magnetic resonance imaging, cerebrospinal fluid, corticosteroids, plasma exchange
Correspondence: Petra Nilsson, Department of Neurology, Lund University Hospital, S‐221 85 Lund, Sweden. Tel: (46)‐46‐17‐14‐27; Fax: (46)‐46‐15‐89‐19; e‐mail: petra.nilsson@med.lu.se