Tumefactive demyelinating lesion tdl, also sometimes referred to as monofocal acute inflammatory demyelination maid, is a locally aggressive form of demyelination, usually manifesting as a solitary lesion or sometimes a couple of lesions greater than 2 cm that may mimic a neoplasm on imaging. Symptoms of tumefactive ms often differ from other ms cases and may include, headaches, changes in thinking, confusion, speech. Choosing when to biopsy a tumefactive lesion to exclude alternative pathology can be difficult. To investigate the characteristics of tdls, clinical and radiological data from nine cases with tdls were analyzed after. All cases underwent surgery and pathological examination.
The immunosuppressant tacrolimus fk 506 is widely used in transplantation medicine, although it has neurotoxic side effects. The association of tumefactive demyelination and sle has not been previously reported in the literature. Distinguishing tumefactive demyelinating lesions from glioma. Tumefactive demyelinating lesion radiology reference. Tumefactive demyelinating tdl lesions are focal zones of demyelination in the central nervous system and they often mimic the neuroimaging features of an intraxial neoplasm. Patients with central nervous system demyelinating disease were identified from our database over the last 10 years. Mri demonstrated a 3 cm enhancing mass in the right posterior frontal white matter with several smaller lesions in the white matter of the contralateral hemisphere and brainstem. Demyelinating diseases journal of clinical pathology. Tumefactive demyelinating lesions are a known but uncommon complication of multiple sclerosis, a disease rarely reported in children. By susan gauthier, do, mph, assistant professor of neurology, weill cornell medical college. Whether tumefactive demyelination constitutes a disease variant within. Transtentorial herniation from tumefactive multiple sclerosis.
Mri is the method of choice for imaging demyelination lesions, tumefactive or not. 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. On the contrary, a glioblastoma multiforme is rarely misdiagnosed as a td unless the two coexist. Metabolite findings in tumefactive demyelinating lesions. Tumefactive demyelination the neurosurgical atlas, by.
To investigate the characteristics of tdls, clinical and radiological data from nine cases with tdls were analyzed after admission. As a result, treatment decisions are largely based on a doctors experience and information. Tumefactive lesions at ms onset not predictive of a more severe or protracted ms course. Tumefactive demyelination in a patient with relapsing. The clinical presentation of the disorder has wide spectra, from an asymptomatic presentation to a severe lifethreatening disease affecting several organs. Mr imaging of the brain was obtained in 4 patients who presented clinically with focal neurologic deficits. Brain signals cant move across scar tissue as quickly, so your nerves dont.
Tumefactive demyelination and a malignant course in an ms patient during and following fingolimod therapy author links open overlay panel m. May 19, 2016 to date there have been no controlled treatment studies for tumefactive multiple sclerosis. Spectrum of disease, diagnosis and treatment article pdf available in current neurobiology 0701 january 2016 with 990 reads how we measure reads. Overview of common imaging features found with tumefactive demyelination, characteristic pathologic findings, and relevant clinical features. We present a case of a 60year old man who was diagnosed as having tumefactive demyelination on a stereotactic biopsy. Tumefactive demyelinationto cracks the nut without cracking. Primary demyelination refers to a disease process in which the myelin sheath is. Tumefactive demyelination is a term created relatively recently to describe the rare occurrence of a large area of demyelination presenting with focal neurology that demonstrates mass. Tumefactive demyelination and a malignant course in an ms. Tumefactive demyelination is a phenomenon involving the radiographic resemblance of an acute demyelinating process in the central nervous system to neoplasia. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Jan 27, 2009 tumefactive demyelinating lesions tdls are misdiagnosed frequently. Tumefactive demyelination associated with systemic lupus. The characteristics of tumefactive demyelination can mimic other diseases and can even lead to wrong diagnosis.
Multiple sclerosis is an inflammatory disease of the central nervous system cns whose aetiology and pathogenesis are still to be clarified. Jun 12, 2007 a previously healthy 31yearold woman presented with a 2week history of progressive left hemiparesis. Large demyelinating lesions with possible mass effect tumefactive multiple sclerosis or tumefactive demyelination can be mistaken for. A high proportion of cases of tumefactive demyelination follow a relapsing course, thus necessitating a long. Ct brain revealed bulky hyperdense lesion in corpus callosum and subsequently mri of the brain revealed t2. Tumefactive multiple sclerosis radiology reference. A 45yearold female recently detected diabetic, admitted with difficulty in walking, irrelevant talk and urinary incontinence for 3 weeks and deviation of angle of mouth to left. This is a good example of tumefactive demyelination with the typical advancing edge of enhancement. Glioblastoma multiforme masquerading as a tumefactive.
This patients rapid clinical and mri response suggests that plasma. Demyelination in the central nervous system sometimes presents with large pseudotumoral lesions mimicking brain neoplasm. 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. Moreira ferreira, md, david meredith, md, and james m. It plays a part in several chronic conditions, including multiple sclerosis. 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. 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. An older age at onset makes distinguishing tumefactive demyelinating lesion tdl from tumors even more challenging. 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. Tumefactive lesions are an uncommon manifestation of demyelinating disease and can pose a diagnostic challenge in patients without a preexisting diagnosis of multiple sclerosis. Paraneoplastic tumefactive demyelination with underlying. Treatment of steroidunresponsive tumefactive demyelinating. Demyelination is damage to the myelin sheath around nerves. Tumefactive demyelinating disease tdd is a rare demyelinating diseaseprobably a variant of multiple sclerosis mspresenting as a focal cerebral mass.
Systemic lupus erythematosus sle is a chronic inflammatory multisystem disease characterised by varied clinical manifestation and immunological abnormalities. Tumefactive acute disseminated encephalomyelitis z ghali mg. To use mr spectroscopy to aid in the diagnosis of demyelinating disease and to help differentiate tumefactive demyelinating lesions from neoplastic processes. The posttreatment clinical course correlated with decreasing enhancement and lesion size on mri. Inflammatory demyelination occasionally forms a solitary mass lesion clinically and radiographically indistinguishable from glioma, replete with enhancement and mass effect. Examination revealed pallor, delusional thoughts, right upper motor neuron facial weakness, ataxia. Large focal tumorlike demyelinating lesions of the brain. This is the fourth report of paraneoplastic tumefactive demyelination with seminoma, 1, 3 excluding 1 report in which autopsy showed definite additional intravascular seminoma. Tumefactive demyelination and pontine demyelination are types of these lesions. Paraneoplastic demyelination is a rare disorder of the central nervous system.
Tumefactive demyelinating lesion tdl is defined as a solitary demyelinating lesion greater than 2 cm. Tumefactive multiple sclerosis or tumefactive demyelinating lesion tdl is one of the rare variants of multiple sclerosis ms posing a. Distinguishing tumefactive demyelinating lesions from. Tumefactive demyelinating lesions tdls are not an uncommon manifestation of. Background plasma exchange plex is a beneficial rescue therapy for acute, steroidrefractory central nervous system inflammatory demyelinating disease cnsidd. We report a case of brain tdl as the initial manifestation of lateonset ms associated with cervico. In our case, the thin peripheral rim of restricted diffusion may represent an active edge of demyelination. However, even after unambiguous histopathologic classification, tumefactive lesions pose a therapeutic challenge. Tumefactive acute disseminated encephalomyelitis z ghali.
Tumefactive multiple sclerosis lesions in two patients after cessation of fingolimod treatment. Whether tumefactive demyelination constitutes a disease variant within the. Wallerian degeneration in the corticospinal tract following tumefactive demyelination. Tumefactive demyelinating lesions are generally thought of as solitary lesions, greater than 2 cm, with imaging characteristics mimicking neoplasms. Mri, although helpful in diagnosis, does not predict monophasic or.
Ocrelizumab reduced gadoliniumenhancing activity 94% and 95% compared with subcutaneous interferon beta1a in relapsingremitting ms rrms. There was marked clinical improvement with plasma exchange after failure of highdose iv corticosteroids. Demyelination, which means degeneration and loss of myelin already formed, can be divided into primary and secondary types. Tumefactive demyelinating lesions tdls can simulate intracranial neoplasms in clinical presentation and mr imaging appearance, and surgical biopsy is. 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. The neurotoxicity of tacrolimus to the cns consists mainly of. Tumefactive multiple sclerosis genetic and rare diseases. Mass effect and contrast enhancement on neuroimaging make it difficult to distinguish this type of lesion from highgrade gliomas. Here we report a patient with a relapsing unifocal tumefactive demyelinating lesion. Although this has been described and characterized for multiple sclerosis, it has been reported in a few cases in patients with acute disseminated encephalomyelitis adem within the past.
Paraneoplastic recurrent tumefactive demyelination in a 62. Demyelinating disorders are any conditions that damage myelin. Relapsingremitting tumefactive demyelination demyelinating. A previously healthy 26yearold woman was seen with a progressive leftside weakness lasting 3 days. As a result, treatment decisions are largely based on a doctors experience and information from single case reports. Conventional and advanced magnetic resonance imaging volume 43 issue 5 todd a.
Pitfalls in the diagnosis of a tumefactive demyelinating. These are lesions of more than 2 cm in size with the presence of mass effect, edema or ring enhancement. Learn more about types of demyelination and why it. 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. Other questions include how best to treat an acute attack as well as the optimal timing of therapy to prevent relapse. Pdf recurrent tumefactive demyelination in a child.
Acute tumefactive demyelinating lesions in a pediatric. To date there have been no controlled treatment studies for tumefactive multiple sclerosis. 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. Although the usual appearance of ms is that of multiple, small, demyelinating plaques, in some cases, it can simulate a mass lesion, which it would be hard to distinguish from a brain tumor. Beneficial plasma exchange response in central nervous. This article includes discussion of tumefactive demyelinating lesions, tdls, tumefactive demyelinating lesions in multiple sclerosis, tumefactive multiple sclerosis, tumorlike multiple sclerosis. 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.
Acute tumefactive demyelinating lesions in a pediatric patient with known diagnosis of multiple sclerosis. A brain magnetic resonance imaging mri scan showed a rightsided mass lesion with. Ocrelizumab is an intravenously infused humanized monoclonal antibody that induces antibodydependent cellular cytolysis and complementmediated lysis of cd20expressing b cells. Tumefactive demyelination the neurosurgical atlas, by aaron. Tumefactive demyelinationto cracks the nut without. Serum antimyelin oligodendrocyte glycoprotein antibodies are present in up to half of.
Faissner s, hoepner r, lukas c, chan a, gold r, ellrichmann g. Although the usual appearance of ms is that of multiple, small, demyelinating plaques, in some cases. Clinicalscientific notes open access tumefactive demyelination in a patient with relapsingremitting ms on ocrelizumab vanessa f. Antimog associated encephalomyelitis is an inflammatory demyelinating disease of the central nervous system. Pdf tumefactive multiple sclerosis or tumefactive demyelinating lesion tdl is one of the rare. The sole manifestation of lupus erythematosus could be neurological syndrome.
Accurate diagnosis of tumefactive demyelinating lesions is clinically important to avoid unnecessary invasive biopsy or inappropriate treatment. Tumefactive multiple sclerosis radiology reference article. Pdf hemicraniectomy in tumefactive demyelinating disease. It is called tumefactive as the lesions are tumorlike and they mimic tumors clinically, radiologically and sometimes pathologically. Mass effect and contrast enhancement on neuroimaging make it difficult to distinguish this type of. 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. Whether tumefactive demyelination is another manifestation of ms or represents a distinct entity is apparently a matter of contention. Tumefactive multiple sclerosis is a rare form of multiple sclerosis ms. Until now, there have been no guidelines on how to treat patients with these. Oct 01, 2018 overview of common imaging features found with tumefactive demyelination, characteristic pathologic findings, and relevant clinical features. We aimed to evaluate conventional and advanced mr imaging findings of tumefactive demyelinating lesions and determine the diagnostic performance of mr imaging for differentiating tumefactive demyelinating lesions from primary. Given the age of the patient, the diagnosis was confirmed with a biopsy.
We describe a 60yearold man with tumefactive demyelination who had an underlying retroperitoneal. Tumefactive demyelinating lesions tdls are misdiagnosed frequently. Tumefactive and pontine demyelination dr garys multiple. Tumefactive demyelinating lesions tdls, are a rare demyelinating. Ms is a disabling and progressive disease that affects the central nervous system. Tdl is now considered to be a separate entity, lying on a spectrum between multiple sclerosis and postinfectious demyelination acute. Jan 10, 2016 tumefactive demyelination, part of the spectrum of demyelinating disease, poses a diagnostic conundrum by mimicking a highgrade neoplasm. Demyelinating diseases of the cns can be classified according to their pathogenesis into several categories. Until now, there have been no guidelines on how to treat patients with these rare and extreme lesion phenotypes. Mr imaging initially revealed parenchymal mass lesions. A challenging diagnosis of lateonset tumefactive multiple s. In a small number of cases, people with tumefactive multiple sclerosis have minimal to no symptoms. Tumefactive demyelination is defined as a demyelinating plaque of more than 2 cm in diameter and is considered as intermediate between multiplesclerosis and acute demyelinating encephalomyelitis.
Proton mr spectroscopy of tumefactive demyelinating. The authors describe a patient with an isolated, gadoliniumenhancing, biopsyproven focus of tumefactive demyelination. The mri appearance of tumefactive demyelinating lesions. Tumefactive demyelinating lesions medlink neurology. On imaging, they usually present with relatively little mass effect or surrounding edema.
Active foci of demyelination may demonstrate restricted diffusion. Cerebral tumefactive demyelinating lesions spandidos publications. Pdf demyelination in the central nervous system sometimes presents with large pseudotumoral lesions mimicking brain neoplasm. At autopsy, however, the lesion revealed a grade iv glioblastoma.
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