The patient was treated with highdose steroid and five sessions of plasma exchange with significant. Interestingly, edss in biopsied multiple sclerosis cases with a disease duration 10 years was significantly less compared with the prevalence multiple sclerosis cohort matched for disease duration 10 years, 1. Clinical and radiographic spectrum of pathologically. Tumefactive multiple sclerosis is a rare form of multiple sclerosis ms. Management of recurrent tumefactive multiple sclerosis. It predominantly affects individuals in their early adult life, and has a huge impact functionally, financially, and on quality of life. On magnetic resonance imaging mri scans, the condition appears as tumorlike lesions larger than two centimeters. Tumefactive multiple sclerosis ms is a rare form of demyelinating disease. Choosing when to biopsy a tumefactive lesion to exclude alternative pathology can be dif. Neurologia, ospedale san biagio, piazza vittime dei lager nazifascisti 1, 28845, domodossola vb, italy 2 uoc neurology, san giovanniaddolorata hospital, via dellamba aradam 9, 00184, rome, italy 3 department of neurology and. Mr imaging of the brain was obtained in 4 patients who presented clinically with focal neurologic deficits.
Tumefactive multiple sclerosis tms is an unusual variant of demyelinating disease characterized by lesions with pseudotumoral appearance on radiological imaging. Tumefactive demyelination is a rare neurological disorder presenting as tumor with demyelinating etiology. Cureus tumefactive multiple sclerosis of the cervical. Join facebook to connect with tumefactive multiple sclerosis and others you may know. Airasutilization of pet imaging in differential diagnostics between a tumefactive multiple sclerosis lesion and lowgrade glioma mult. Characterization of tumefactive demyelinating lesions using mr imaging and invivo proton mr spectroscopy hs malhotra, kk jain, a agarwal, mk singh, sk yadav, m husain, n krishnani, and rk gupta multiple sclerosis journal 2008 15.
Tumefactive multiple sclerosis tms is a demyelinating disease. To release radiological findings of tumefactive multiple sclerosis lesions. A 34 year old healthy woman presented with sudden onset of left hemiparesis. They constitute a rare inflammatory demyelinating disorder of the central nervous system. Tumefactive demyelinating lesions tdls can simulate intracranial neoplasms in clinical presentation and mr imaging appearance, and surgical biopsy is often performed in suspected tumors. Subsequent mri of the brain showed a single large 2. Tumefactive multiple sclerosis ms, also known as pseudotumoral ms, it is characterized by the presence of a single ms plaque in the brain. Use of serial proton magnetic resonance spectroscopy to. Jun 04, 2008 some studies suggest patients with tumefactive multiple sclerosis have a comparatively benign course compared with other forms of multiple sclerosis kepes, 1993. Its prevalence is reported to be 1 to 2 cases per cases of multiple sclerosis ms, more often in patients aged 10 to 30 years, without a clear predominance of gender. Tms can mimic clinical and radiological features of a neoplasm, infarction, or abscess and therefore can be diagnostically challenging for clinicians.
Silent tumefactive demyelinating lesions and radiologically. We present a case of a large solitary demyelinating plaque in the brain masquerading as tumour. Metabolite findings in tumefactive demyelinating lesions. Multiple sclerosis ms is the most common cause of progressive neurologic disability in adults aged 20 to 40 years in the developed world. We report on the use of serial proton mr spectroscopy 1 h mrs to differentiate between glioma and tumefactive plaque in a known multiple sclerosis ms patient who developed a symptomatic cerebral space occupying lesion. It is called tumefactive as the lesions are tumorlike and they mimic tumors clinically, radiologically and sometimes pathologically. Tumefactive multiple sclerosis is a demyelinating disease that demonstrates tumorlike features on magnetic resonance imaging. To diference these lesions from other spaceoccupying lesions in the central nervous system. Ms is a disabling and progressive disease that affects the central nervous system. Approximately half of tumefactive demyelinating lesions have pathologic contrast enhancement, usually in the form of ring enhancement 2, 4 figs. 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. Proton magnetic resonance spectroscopy differentiates tumefactive demyelinating lesions from gliomas.
Saindane am, soonmee c, meng l, xiaonan x, knopp ea, zagzag d. Tumefactive multiple sclerosis tms is a rare variant of multiple sclerosis ms with atypical features that pose a diagnostic challenge. Although altered mental status andor encephalopathy favor a diagnosis of adem, these may also occur in a first attack of multiple sclerosis. To identify imaging characteristics of tdls for their proper diagnosis, their differences from malignant gliomas, etc. Demyelinating lesions larger than 2 cm in diameter, with or without edema, are known as tumefactive demyelinating lesions tdls. Tms can mimic clinical and radiological features of a neoplasm, infarction, or. Tumefactive multiple sclerosis is a rare form of multiple sclerosis ms with symptoms similar to those of a brain tumor. Tumefactive demyelination associated with bilateral optic. Multiple sclerosis and related disorders volume 26, november 2018, pages 7784 proton magnetic resonance spectroscopy differentiates tumefactive demyelinating lesions from gliomas. Symptoms of tumefactive ms often differ from other ms cases and may include, headaches, changes in thinking, confusion, speech. Recently, without biopsy, some tms cases were diagnosed by magnetic resonance spectroscopy. Please remove adblock adverts are the main source of revenue for dovemed.
Tumefactive multiple sclerosis plaque journal of neurology. May 19, 2016 disease modifying therapies for example, interferon. The mri appearance of tumefactive demyelinating lesions. Tumefactive multiple sclerosis is a form of demyelinating disease which patient can present with acute stroke. Tumefactive demyelinating lesion radiology reference. Additionally, there may be histopathological differences between a tdl and a tumefactive multiple sclerosis plaque, namely the presence of more pronounced axonal damage in the latter 1.
The clinical presentations of tumefactive multiple sclerosis and tumefactive adem exhibit overlap and include focal neurologic deficits, seizures, and symptoms attributable to cortical dysfunction. A challenging diagnosis of lateonset tumefactive multiple s. Relapsingremitting tumefactive multiple sclerosis stephen. A tumefactive multiple sclerosis lesion in the brain. When reading about demyelination, one must be wary of older publications that do not draw a distinction between multiple sclerosis with larger tumefactivelike. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis.
Tumefactive multiple sclerosis ms is an uncommon manifestation of multiple sclerosis. Additionally, although patients with multiple sclerosis can develop large tumefactive demyelinating plaques which have very similar appearances see tumefactive multiple sclerosis, patients who present with a solitary tumefactive demyelinating lesion infrequently go on to develop multiple sclerosis ms 3. Proton mr spectroscopy of tumefactive demyelinating lesions. To use mr spectroscopy to aid in the diagnosis of demyelinating disease and to help differentiate tumefactive demyelinating lesions from neoplastic processes. Proton mr spectroscopy of tumefactive demyelinating lesions article pdf available in american journal of neuroradiology 238. Case report open access tumefactive multiple sclerosis. Demyelination causes slowing of conduction or complete failure. We previously reported biopsied multiple sclerosis patients have a similar clinical course and prognosis compared with a populationbased multiple sclerosis. Tumefactive multiple sclerosis genetic and rare diseases. 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. Some studies suggest patients with tumefactive multiple sclerosis have a comparatively benign course compared with other forms of multiple sclerosis kepes, 1993. May 18, 2016 tumefactive multiple sclerosis is characterized by a tumor like lesion larger than two centimeters and signs and symptoms similar to those of a brain tumor.
Tumefactive demyelinating lesions tdls are often misdiagnosed as brain tumors. Multiple sclerosis is a complex condition but some of the fundamental questions relating to causation and susceptibility have been answered. We present a clinical scenario of a patient presenting with left homonymous hemianopia with atypical radiological features. Tumefactive multiple sclerosis is characterized by a tumor like lesion larger than two centimeters and signs and symptoms similar to those of a brain tumor. Tumefactive multiple sclerosis tms is a rare variant with 1 per cases of ms and 3 per million cases per year. We reported a case of a 49yearold woman with well controlled hypertension, who presented with right hemiplegia 15 hours prior to admission. Proton mr spectroscopy has been applied in assessing various intracranial diseases and is increasingly used in diagnosis and clinical management. Characterization of tumefactive demyelinating lesions using. Tumefactive demyelinating lesions and pregnancy plantone d. Other questions include how best to treat an acute attack as well as the optimal timing of therapy to prevent relapse. The lesion mimics intracerebral neoplasms due to its large size, mass effect, and enhancement patterns. Proton magnetic resonance spectroscopy differentiates. Tumefactive acute disseminated encephalomyelitis z ghali mg. Thus far, no consensus exists about definition and management of tumefactive lesions since.
Tumefactive demyelinating lesions and pregnancy domenico plantone 1, carlo piantadosi 2, serena ruggieri 3, vincenzo anelli 4, tatiana koudriavtseva 5 1 s. Pdf proton mr spectroscopy of tumefactive demyelinating lesions. Proton magnetic resonance spectroscopy differentiates tumefactive demyelinating lesions from gliomas ryotaro ikeguchi et al. Multiple sclerosis in 79% and an isolated demyelinating syndrome in 14% lucchinetti cf et al. Mr imaging initially revealed parenchymal mass lesions.
Tumefactive multiple sclerosis requiring emergent biopsy and. Commonly the enhancement patterns will be in the form of an open ring, with the incomplete portion of the ring on the gray matter side of the lesion. Because of its tumorlike features on magnetic resonance imaging mri 1,2, histological investigation had played an important role for definite diagnosis of tms 3,4. When reading about demyelination, one must be wary of older publications that do not draw a distinction between multiple sclerosis with larger tumefactive like.
Costs are considerable and rise with increasing disability. Tdls are typically characterized by headaches, cortical symptoms such as aphasia, hemiparesis, hemisensory deficits, seizures, and changes in consciousness. Magnetic resonance imaging characteristics of tumefactive. 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. Tumefactive multiple sclerosis radiology reference article. 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. The natural course of the disease has been characterized as presenting with a masslike demyelinating lesion converting to typical relapsingremitting disease with future exacerbations. To understand clinical and radiological appearance of bale concetric sclerosis. The demyelinating lesion can mimic infections, vascular lesions, and inflammatory. Proton mr spectroscopy of tumefactive demyelinating. Gliomas and acute ms plaques may have indistinguishable chemical resonance spectra, whereas that of chronic plaque is distinct.
Thus far, no consensus exists about definition and management of tumefactive lesions since the only available evidence comes from small case series and. 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. Previously it was designated as schilders disease or marburgs variant of multiple sclerosis. Illustrate the role of multimodal mri with a focus on spectroscopy, diffusion and perfusion imaging to increase diagnostic confidence. Airasutilization of pet imaging in differential diagnostics between a tumefactive multiple sclerosis lesion and lowgrade glioma.
Singlevoxel proton spectroscopy image obtained using pointresolved sequence press, trte, 1,5005 placed along medial border of lesion at area of enhancement reveals elevated choline cho and suppressed nacetylaspartate naa relative to creatine cr and. Traditionally, balos concentric sclerosis has been grouped as one of the atypical forms of multiple sclerosis, with marburgs disease, tumefactive demyelination, schilders disease, and acute haemorrhagic leukoencephalitis, although the contemporary status and usefulness of these categorisationsapart from tumefactive demyelinationis contentious. Tumefactive multiple sclerosis tms is a rare entity which can be difficult to diagnose unless definitive diagnostic measures are taken. On magnetic resonance imaging mri scans, the condition appears as tumorlike lesions larger than two centimeters multiple sclerosis, is a disease of the central nervous system cns, which is comprised of the brain, spinal cord and optic nerve.
Tumefactive multiple sclerosis clinically mimicking acute. Recognise the difficulties associated with diagnosing tumefactive multiple sclerosis from clinical presentation and conventional imaging. Pdf proton mr spectroscopy of tumefactive demyelinating. Cureus tumefactive multiple sclerosis, a rare variant. Central nervous system cns inflammatory demyelinating diseases idds, such as multiple sclerosis, can be easily diagnosed based on imaging examinations, such as mri, and assessment of the clinical course of disease. Learn indepth information on tumefactive multiple sclerosis, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis.
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