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Demyelinating butterfly pseudo glioma

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Downloaded from http://jnnp.bmj.com/ on July 18, 2016 - Published by group.bmj.com Neurological picture Demyelinating butterfly pseudo-glioma A 27-year-old woman had a month history of progressive headache, nausea, vomiting and deterioration in memory, mood and affect She was months post partum Prior to her admission to hospital, her symptoms were attributed to post-partum depression She had no significant medical history Her only medication at admission was citalopram, which was recently started by her family physician because of her deteriorating mood and labile affect On examination, her vital signs were normal She was alert and oriented Formal cognitive testing revealed difficulty with attention, concentration and short term memory Constructional and ideomotor apraxias were evident Bilateral grasp reflexes were present There was bilateral papilloedema Motor examination found a right upper motor neuron weakness of the face, and weakness of the right upper and lower extremity graded at 4+/5 based on Medical Research Council grading Reflexes were bilaterally brisk and asymmetrical with the right upper and lower extremity hyperreflexic compared with the left Plantar reflexes were down-going bilaterally The remainder of the examination was normal Brain MRI showed a large heterogeneously enhancing bifrontal mass, crossing the corpus callosum, which raised concern of a high grade astrocytoma or lymphoma (fig 1) A stereotactic guided biopsy was performed and revealed a demyelinating lesion consistent with ‘‘demyelinating pseudotumour’’ (fig 2) The patient was treated with high dose corticosteroids and slowly improved over several months Acute tumour-like demyelination or ‘‘demyelinating pseudotumour’’ is a variant presentation of demyelinating diseases, such as multiple sclerosis It usually presents as a large solitary lesion within one hemisphere of the brain or less frequently within the spinal cord On rare occasions, a single lesion can involve both cerebral hemispheres as well as the corpus callosum.1 Mass effect can occur secondary to significant oedema and appear as hyperintensity on T2 sequence MRI imaging The oedema may respond rapidly to corticosteroids, and studies have reported good prognosis with corticosteroid treatment.2 Other white matter diseases can occasionally present with rapid clinical deterioration and mass-like features on neuroimaging In particular, progressive multifocal leukoencephalopathy can present with bilateral hemispheric 12 white matter lesions Progressive multifocal leukoencephalopathy is the result of JC virus reactivation and is associated with a significant immunocompromised state, as in the case of HIV infection and the advanced stages of AIDS Our patient had no history or evidence of immunocompromise Furthermore, she had clinical and radiological improvement following one course of steroid treatment and has remained stable with continued Figure MRI of the head (A) Fluid attenuated inversion recovery (FLAIR): bifrontal hyperintense lesion involving the cortex, white matter and corpus callosum (B) T1 weighted post-gadolinium: ring enhancement surrounding central hypointensity Figure Pathology (A) Widespread white matter destruction: pockets of macrophages (*), reactive hypertrophic astrocytes (arrowheads) and axonal sparing (B) Perivascular inflammatory cells: predominantly CD4+ T lymphocytes are shown by their CD45RO immunoreactivity (C) and a few expressing CD8 immunoreactivity (D) Downloaded from http://jnnp.bmj.com/ on July 18, 2016 - Published by group.bmj.com Neurological picture improvement for more than year of follow-up Bihemispheric lesions involving the corpus callosum are often thought to be high grade gliomas or lymphomas As shown by this case, demyelinating lesions can have the same MRI appearance, associated oedema and significant response to corticosteroid treatment, and therefore need to be considered in the diagnosis.3 Biopsy and tissue pathology may be required to distinguish the pathology J Scozzafava,1 E S Johnson,2 G Blevins3 J Neurol Neurosurg Psychiatry 2008;79:12–13 doi:10.1136/jnnp.2007.118182 Department of Critical Care Medicine, University of Calgary, Calgary, Alberta, Canada; Division of Anatomic Pathology, University of Alberta, Edmonton, Alberta, Canada; 3Division of Neurology, University of Alberta, Edmonton, Alberta, Canada REFERENCES Correspondence to: Dr J Scozzafava, Foothills Medical Centre, 1403-29th Street NW, Calgary, Alberta, Canada T2N 2T9; James.Scozzafava@calgaryhealthregion.ca Competing interests: None Kalyan-Raman UP, Garwacki DJ, Elwood PW Demyelinating disease of the corpus callosum presenting as glioma on magnetic resonance scan: a case documented with pathological findings Neurosurgery 1987;21:247–50 Kepes JJ Large focal tumor-like demyelinating lesions of the brain: Intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis? A study of 31 patients Ann Neurol 1993;33:18–27 Reith KG, Di Chiro G, Cromwell LD, et al Primary demyelinating disease simulating glioma of a corpus callosum J Neurosurg 1981;55:620–4 FUNDING AVAILABLE FOR RESEARCH PROJECTS The Committee on Publication Ethics (COPE) has established a Grant Scheme to fund research in the field of publication ethics The Scheme is designed to provide financial support to any member of COPE for a defined research project that is in the broad area of the organisation’s interests, and specifically in the area of ethical standards and practice in biomedical publishing The project should have a specific goal and be intended to form the kernel of a future publication A maximum sum of £5000 will be allocated to any one project, but applications for smaller sums are welcomed The terms and conditions of the Grant are as follows: c At least one of the applicants must be a member of COPE c Calls for applications will be made twice a year with closing dates of December and June An electronic version of the application form must be sent to the Administrator no later than 12 pm (noon GMT) on the closing date for consideration by COPE Council c The application must contain a lay summary of the project, a definition of the question to be posed, sufficient methodological detail to allow assessment of the viability of the project, a clear timeline and a definition of the likely deliverables A full justification for the sum requested must accompany the application c A report on the progress of the research should be presented within one year of the award and at the end of the project The grant must be used within two years from the date of award, and balance sheets must be forwarded annually These should be sent to the Administrator Any remaining funds after two years must be returned c It is anticipated that the work stemming from the project will be presented at one of COPE’s annual seminar meetings within 2–3 years of the award Such data may also be published in peer-reviewed journals Any publications or related presentations at meetings by the recipient emanating in part or whole from COPE’s support should be duly acknowledged and copies sent to the Administrator Applications are reviewed by a COPE sub-committee Applicants will be advised of a decision as soon as practicable after the deadline date An application form can be obtained by contacting Linda Gough, COPE administrator, at LGough@ bmj.com or 020 7383 6602 For more information on COPE, see http://www.publicationethics.org.uk/ The closing date for receipt of applications is December 2007 or June 2008 13 Downloaded from http://jnnp.bmj.com/ on July 18, 2016 - Published by group.bmj.com Demyelinating butterfly pseudo-glioma J Scozzafava, E S Johnson and G Blevins J Neurol Neurosurg Psychiatry 2008 79: 12-13 doi: 10.1136/jnnp.2007.118182 Updated information and services can be found at: http://jnnp.bmj.com/content/79/1/12 These include: References Email alerting service Topic Collections This article cites articles, of which you can access for free at: http://jnnp.bmj.com/content/79/1/12#BIBL Receive free email alerts when new articles cite this article Sign up in the box at the top right corner of the online article Articles on similar topics can be found in the following collections CNS cancer (177) Neurooncology (230) Immunology (including allergy) (1844) Radiology (1684) Surgical diagnostic tests (378) Cranial nerves (501) Headache (including migraine) (427) HIV/AIDS (104) Infection (neurology) (467) Mood disorders (including depression) (215) Multiple sclerosis (886) Ophthalmology (804) Pain (neurology) (711) Drugs: CNS (not psychiatric) (1871) Neuroimaging (375) Notes To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/ ... Downloaded from http://jnnp.bmj.com/ on July 18, 2016 - Published by group.bmj.com Demyelinating butterfly pseudo-glioma J Scozzafava, E S Johnson and G Blevins J Neurol Neurosurg Psychiatry... James.Scozzafava@calgaryhealthregion.ca Competing interests: None Kalyan-Raman UP, Garwacki DJ, Elwood PW Demyelinating disease of the corpus callosum presenting as glioma on magnetic resonance scan: a... documented with pathological findings Neurosurgery 1987;21:247–50 Kepes JJ Large focal tumor-like demyelinating lesions of the brain: Intermediate entity between multiple sclerosis and acute disseminated

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