Tdl is now considered to be a separate entity, lying on a spectrum between multiple sclerosis and postinfectious demyelination. Paraneoplastic tumefactive demyelination with underlying. Tumefactive demyelinating lesions can present with features similar, clinically and radiologically, to those of brain tumours. In this retrospective study january 2005 till dec 2011, six patients with a histopathological diagnosis of tdl were selected. Whether tumefactive demyelination is another manifestation of ms or represents a distinct entity is apparently a matter of contention.
Spectroscopic magnetic resonance imaging of a tumefactive. Lasala, jerome graber glioblastoma multiforme presenting with an open ring pattern of enhancement on mr imaging. Based on this profile, possibility of inflammatory demyelinating pseudotumour tumefactive demyelination was suspected. Tumefactive demyelinating lesion is defined as large solitary demyelinating lesion with imaging characteristics mimicking neoplasm. This damage impairs the conduction of signals in the affected nerves. Tumefactive multiple sclerosis ms is a rare form of demyelinating disease. Metabolic assessment of monofocal acute inflammatory demyelination using mr spectroscopy and 11 cmethionine, 11 c. To determine retrospectively whether unenhanced computed tomographic ct images of the brain have added value in distinguishing tumefactive demyelinating lesions tdls from primary glioma or central nervous system cns lymphoma, compared with conventional contrast materialenhanced magnetic resonance mr images only. Diseases affecting central nervous system cns myelin can be classified on the basis of. We describe a 60yearold man with tumefactive demyelination who had an underlying retroperitoneal germ cell cancer.
The mri appearance of tumefactive demyelinating lesions. Magnetic resonance imaging characteristics of tumefactive. Mass effect and contrast enhancement on neuroimaging make it difficult to distinguish this type of lesion from highgrade gliomas. View or download all content the institution has subscribed to. Clinically patients left sided weakness is progressive and worsening, now associated with complete left hemi neglect. Mr spectroscopy typically demonstrates an increased. On both occasions, the magnetic resonance mr scan showed a giant demyelinating, peripherally enhancing lesion with mass effect. Tumefactive multiple sclerosis requiring emergent biopsy. Pdf proton mr spectroscopy of tumefactive demyelinating lesions. Inflammatory demyelination occasionally forms a solitary mass lesion clinically and radiographically indistinguishable from. Tumefactive multiple sclerosis ms is an uncommon manifestation of multiple sclerosis.
Proton magnetic resonance spectroscopy differentiates tumefactive demyelinating lesions from gliomas ryotaro ikeguchi et al. Balo concentric sclerosis bcs is a rare, atypical demyelinating disease. Tumefactive demyelinating lesion differentiated from a brain tumor using a combination of magnetic resonance imaging and 11 cmethionine positron emission tomography. The patient was noninvasively diagnosed with tumefactive multiple sclerosis and treated with corticosteroids, and her neurological symptoms and mri findings improved with treatment. However, demyelinating lesions seen in multiple sclerosis or tumefactive demyelination during the active phase of inflammation can also enhance, though the enhancement pattern of these lesions can be faint, with minimal perilesional vasogenic edema, and the enhancing rim may be incomplete. This is a good example of tumefactive demyelination with the typical advancing edge of enhancement.
Proton mr spectroscopy of tumefactive demyelinating. Radiology of demyelinating diseases linkedin slideshare. Download pdf 6145k download meta ris compatible with endnote, reference manager, procite, refworks. Proton magnetic resonance spectroscopy differentiates. Secondary demyelination, where the aetiology is known, includes infectious, metabolic and toxic disease processes. By susan gauthier, do, mph, assistant professor of neurology, weill cornell medical college. Imaging evaluation of demyelinating processes of the.
Atypical inflammatory demyelinating syndromes of the cns. Multiple large tumefactive ms plaques in a young man. Magnetic resonance spectroscopy can also help identify these lesions. Proton mr spectroscopy has been applied in assessing various intracranial diseases and is increasingly used in diagnosis and clinical management.
Mri spectroscopy within the lesion showed an naacholine ratio of 0. A tumefactive demyelinating lesion tdl is an inflammatory demyelinating disorder of. Glioblastoma multiforme presenting with an open ring pattern. Illustrate the role of multimodal mri with a focus on spectroscopy, diffusion and perfusion imaging to increase diagnostic confidence. Ct brain revealed bulky hyperdense lesion in corpus callosum and subsequently mri of the brain revealed t2hyperintense lesions involving genu and body of corpus callosum with restricted diffusion, and mr spectroscopy revealed reduced uptake of choline. Tumefactive multiple sclerosis is a condition in which the central nervous system of a person has multiple demyelinating lesions with atypical characteristics for those of standard multiple sclerosis ms. Tumefactive demyelinating lesions tdls are often misdiagnosed as brain tumors. Axial mri and mr spectroscopy data acquired at 3 tesla in a 30 year old woman with tumefactive ms. Mri spectroscopy in neurological disorders book chapter. Tumefactive demyelinating lesion differentiated from a brain.
Magnetic spectroscopy findings showed mild increase in choline with decrease in naa with no other significant findings. Paraneoplastic recurrent tumefactive demyelination in a 62. Mr imaging of the brain was obtained in 4 patients who presented clinically with focal neurologic deficits. Tumefactive multiple sclerosis tms is a rare variant of multiple sclerosis ms with atypical features that pose a diagnostic challenge. A tumefactive multiple sclerosis lesion in the brain. Demyelination causes slowing of conduction or complete failure. Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is necessary to avoid the inadvertent intervention.
It is not intended to be and should not be interpreted as medical advice or a diagnosis of any health or fitness problem, condition or disease. Imagingwise diagnosis suggested is tumefactive demyelination and is on steroids now. Tumefactive demyelination the neurosurgical atlas, by aaron. We present a case of a large solitary demyelinating plaque in the brain masquerading as tumour. A challenging diagnosis of lateonset tumefactive multiple. Certain features on standard as well as advanced mri can potentially promote the diagnosis of tumefactive demyelination and obviate the need for biopsy. Glioblastoma multiforme presenting with an open ring.
It is called tumefactive as the lesions are tumorlike and they mimic tumors clinically, radiologically and sometimes pathologically. Cureus tumefactive multiple sclerosis of the cervical. Tumefactive demyelinating lesion radiology reference. Download complete pdf book, the epub book or the kindle book. Seeing demyelination on mri dr garys multiple sclerosis. Advanced mr imaging techniques in the diagnosis of intraaxial. Characterization of tumefactive demyelinating lesions using mr. Proton mr spectroscopy of tumefactive demyelinating lesions. Jan 10, 2016 whether tumefactive demyelination is another manifestation of ms or represents a distinct entity is apparently a matter of contention. Advanced neuroimaging in balos concentric sclerosis. The clinical, neuroimaging and management modalities in these six cases are summarized in. Pitfalls in the diagnosis of a tumefactive demyelinating. A yearold female presented with two episodes of hemiplegia and hemianopia involving opposite sides, each time associated with seizures. Often, it is difficult to distinguish a tumefactive demyelinating lesion from neoplastic lesions at mr spectroscopy,, fig 9d, 9f, 31, 32.
We report a case of brain tdl as the initial manifestation of lateonset ms. A case of pediatric tumefactive demyelinating lesion. His mri was interpreted as a glioma but stereotactic biopsy showed active demyelination. To identify imaging characteristics of tdls for their proper diagnosis, their differences from malignant gliomas, etc. The differential diagnosis however included cns lymphoma and progressive multifocal leucoencephalopathy. T1 hypointense top row left oval, subcortical lesion with corresponding flair hyperintense signal top row right and faint rim of incomplete horseshoe enhancement bottom row left. Tumefactive demyelinationto cracks the nut without. A provisional diagnosis of an intraaxial neoplasm or abscess was given in each case. Recurrent tumefactive demyelination in a child sciencedirect. Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is essential to avoid inadvertent surgical or toxic.
We describe clinical and radiographic features in 168 patients with biopsy confirmed cns inflammatory demyelinating disease idd. As the underlying pathophysiology of neoplasms is different from that of demyelinating disease, one may expect the metabolic composition of neoplasms to be significantly different. In this study, we report a case of cervical ms in a 19yearold patient that was diagnosed based on the mri findings and cerebrospinal fluid analysis. Tumefactive multiple sclerosis radiology reference. Tumefactive demyelinating lesions are generally thought of as solitary lesions, greater than 2. Tdls are defined as demyelinating lesions larger than 2 cm that often have conventional mr imaging. Background and objectivesdiagnosis of tumefactive demyelinating. Distinguishing tumefactive demyelinating lesions from. Combining perfusion mri and mr spectroscopy can help in patients with brain masses in who the preoperative. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. We report a patient with a spaceoccupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as balo concentric sclerosis and a glioma. Advanced mr imaging techniques in the diagnosis of. I, alonso, j, serial diffusionweighted mr imaging and proton mr spectroscopy of acute large demyelinating brain lesions. References pdf pdf plus add to favorites permissions download citation.
Tumefactive multiple sclerosis requiring emergent biopsy and. Silent tumefactive demyelinating lesions and radiologically. Tumefactive demyelination associated with systemic lupus. The classic example of primary demyelination is multiple sclerosis. Glioblastoma multiforme presenting with an open ring pattern of. A clinicopathological correlative study r neelima 1, k krishnakumar 2, md nair 3, c kesavadas 4, divyata rajendra hingwala 4, vv radhakrishnan 1, sruthi s nair 3. Tumefactive demyelinating lesion tdl is defined as a solitary demyelinating lesion. However, demyelinating lesions seen in multiple sclerosis or tumefactive. The mri appearance of tumefactive demyelinating lesions ajr. Consulted to a local hospital for 2 weeks of progressive severe headache, nauseavomiting, mild gait impairment, mild right hemiparesis and difficulty with vision focus. An older age at onset makes distinguishing tumefactive demyelinating lesion tdl from tumors even more challenging. One year after initial presentation, she remained symptomfree without any. Request pdf characterization of tumefactive demyelinating lesions using mr imaging and invivo proton mr spectroscopy diagnosis of. Tumefactive multiple sclerosis or tumefactive demyelinating lesion tdl is one of the rare variants of multiple sclerosis ms posing a diagnostic challenge and a therapeutic enigma since it is difficult to distinguish from a true central nervous system cns neoplasm or other cns lesions on magnetic resonance imaging mri.
Magnetic resonance imaging, magnetic resonance spectroscopy mrs. Other questions include how best to treat an acute attack as well as the optimal. He presented with visuospatial problems and memory loss and had a visual field defect. Differentiation of tumefactive demyelinating lesions from high. Although diagnostic challenges without biopsy have been tried by employing radiological studies and cerebrospinal fluid examinations, histological investigation is still necessary for certain diagnosis in some complicated cases. Early reports described ms variants with tumourlike presentation as shilders disease or marburgs variants poser et al. A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged.
Other questions include how best to treat an acute attack as well as the optimal timing of therapy to prevent relapse. Characterization of tumefactive demyelinating lesions using mr imaging and invivo proton mr spectroscopy. Oct 01, 2018 overview of common imaging features found with tumefactive demyelination, characteristic pathologic findings, and relevant clinical features. Gauthier reports she receives research support from emd serono, biogen idec, and novartis pharmaceuticals, and is on the speakers bureau for biogen idec and teva neurosciences. Nov, 20 the term tumefactive demyelination td describes intraaxial space occupying lesions of the brain which histopathologically are characterized by perivascular lymphocytic infiltrates, macrophages, reactive astrocytes and myelin loss. The clinical features of tumefactive ms are variable and polysymptomatic, depending on the. The natural course of the disease has been characterized as presenting with a masslike demyelinating lesion converting to typical relapsingremitting disease with future exacerbations. Tumefactive multiple sclerosis is a term used to describe patients with established multiple sclerosis who develop large aggressive demyelinating lesions, similaridentical in appearance to those seen in sporadic tumefactive demyelinating lesions tdl. Mr imaging initially revealed parenchymal mass lesions. In multiple sclerosis, the spectroscopic abnormalities are not. Tumefactive multiple sclerosis is a demyelinating disease that demonstrates tumorlike features on magnetic resonance imaging. In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors.
Tumefactive demyelinating lesion tdl is defined as a solitary demyelinating lesion greater than 2 cm. Inflammatory demyelination occasionally forms a solitary mass lesion clinically and radiographically indistinguishable from glioma, replete with enhancement and mass effect. Metabolite findings in tumefactive demyelinating lesions. Brain magnetic resonance imaging mri demonstrated an incomplete. Mr spectroscopy provides valuable information about tissue pathophysiology for at least three different metabolic profiles. Normally a tumefactive demyelinating lesion appears together with smaller. It is important for medical professionals and patients to know that seeing demyelination on mri scans is the only way to properly identify it and see what parts of the body it is affecting. Given the age of the patient, the diagnosis was confirmed with a biopsy. Demyelination is something that occurs in several different diseases. Spectroscopic magnetic resonance imaging of a tumefactive demyelinating lesion.
Proton magnetic resonance spectroscopy differentiates tumefactive demyelinating lesions from gliomas. To use mr spectroscopy to aid in the diagnosis of demyelinating disease and to help differentiate tumefactive demyelinating lesions from neoplastic processes. Tumefactive multiple sclerosis is a rare form of multiple sclerosis ms. There is no consensus however, regarding the definition of tdls, but typically represent lesions larger than 2 cm in. On imaging, they usually present with relatively little mass effect or surrounding edema. Clinical and radiographic spectrum of pathologically. Author links open overlay panel ryotaro ikeguchi a yuko shimizu a. The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. Subsequent mri of the brain showed a single large 2. In turn, the reduction in conduction ability causes deficiency in sensation, movement, cognition, or other functions depending on which nerves are involved. These atypical features include size more than 2 cm, mass effect, edema, andor ring enhancement.
Whether tumefactive demyelination constitutes a disease variant within the broad spectrum of multiple sclerosis or rather depicts a different entity is still matter of debate. A fulminant tumefactive demyelinating lesion may show high choline and low naa signals as well as presence of lactate, 31. Compared to previous mri, lesion is markedly increased in size. Proton mr spectroscopy has been increasingly used to characterize intracranial pathology. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, balos concentric sclerosis, schilders disease, and marburgs. As the underlying pathophysiology of neoplasms is different from that of demyelinating disease, one may expect the metabolic composition of neoplasms.
Tumefactive multiple sclerosis is a condition in which the central nervous system of a person. The characteristic mri findings for this demyelinating disease are a large isolated mass in the white matter with incomplete rim enhancement, slight perilesional edema and a mass effect 2, 3. Tumefactive multiple sclerosis ms is a rare variant of ms characterized by the presence of large demyelinating plaques of more than 2 cm, identified with magnetic resonance imaging mri. Tumefactive demyelinating lesion differentiated from a. Mr spectroscopy was performed and showed an increased cholinetocreatine ratio. Pdf proton mr spectroscopy of tumefactive demyelinating. Tumefactive demyelination mri neuroradiology cases. Tumefactive demyelinating lesions are generally thought of as solitary lesions, greater than 2 cm, with imaging characteristics mimicking neoplasms. Atypical inflammatory demyelinating syndromes are rare disorders that differ from multiple sclerosis owing to unusual clinical or mri findings or poor response to treatments used for multiple sclerosis. Article information, pdf download for characterization of tumefactive. Lesions were analysed on pre and postbiopsy magnetic resonance imaging mri for location, size, mass effectoedema, enhancement, multifocality and fulfilment of barkhof criteria. Background multiple sclerosis ms is a chronic inflammatory disease of the central nervous system in which there is focal demyelination.
Multiple sclerosis ms is the most common cause of progressive neurologic disability in adults aged 20 to 40 years in the developed world. Paraneoplastic demyelination is a rare disorder of the central nervous system. The combination of mri findings and insignificant uptake on metpet is useful for noninvasively differentiating tumefactive demyelinating lesions from brain tumors. Conventional and advanced magnetic resonance imaging in. Clinical proton mr spectroscopy in central nervous system. Proton mr spectroscopy has been applied in assessing various intracranial diseases and is increasingly used in diagnosis and. Demyelinating processes involving the central nervous system have a variety of aetiologies and can be separated into primary and secondary demyelinating processes.
Tumefactive demyelinating lesions tdls can simulate intracranial neoplasms in clinical presentation and mr imaging appearance, and surgical biopsy is often performed in suspected tumors. Tumefactive lesions are an uncommon manifestation of demyelinating disease and can pose a diagnostic challenge in patients without a preexisting diagnosis of multiple sclerosis. Characteristics of individual lesions, individual patients, or a unique disease entity. Ms is a disabling and progressive disease that affects the central nervous system. Introduction demyelinating diseases comprise of diseases of central and peripheral nervous system in which disruption of myelin is a dominant feature.
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