Tumefactive demyelinating disease mimicking malignant tumor in. Tumefactive demyelinating lesions tdls are misdiagnosed frequently. Tumefactive demyelinating lesion tdl is defined as a solitary demyelinating lesion greater than 2 cm. 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.
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. Tumefactive demyelinating lesions are generally thought of as solitary lesions, greater than 2 cm, with imaging characteristics mimicking neoplasms. Pdf tumefactive multiple sclerosis or tumefactive demyelinating. Bmc will continue to host an archive of all articles. Tumefactive and pontine demyelination dr garys multiple. Demyelinating definition is causing or characterized by the loss or destruction of myelin. 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.
Demyelination cure dr garys multiple sclerosis treatment. The patient was diagnosed with acute multifocal demyelination, presumed to be acute disseminated encephalomyelitis or tumefactive multiple sclerosis, and treated with. Aug 23, 2018 the term demyelination refers to loss of the myelin sheath that surrounds and protects axons in the central nervous system. Last updated on wednesday, march 25 2009 by jdmiles. Atypical inflammatory demyelinating syndromes of the cns. We present two cases of tumefactive demyelination td occurring in close association with a developmental venous anomaly dva. Tumefactive demyelination, intraoperative crush preparation with circled cells. Tumefactive multiple sclerosis is a demyelinating disease that demonstrates tumorlike features on magnetic resonance imaging.
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. Multiple large tumefactive ms plaques in a young man. The differential for ringenhancing lesions on mri includes a primary or metastatic neoplasm, pyogenic abscess, tuberculoma, neurocysticercosis, resolving hematoma, tumefactive. 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. The differential for ringenhancing lesions on mri includes a primary or metastatic neoplasm, pyogenic abscess, tuberculoma, neurocysticercosis, resolving hematoma, tumefactive demyelination, or. Learn more about types of demyelination and why it happens. 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. Mri is the method of choice for imaging demyelination lesions, tumefactive or not. Although the usual appearance of ms is that of multiple, small, demyelinating plaques, in some. Inflammatory demyelination occasionally forms a solitary mass lesion clinically and radiographically indistinguishable from glioma, replete with enhancement and mass effect. On advanced mr imaging, tumefactive demyelinating lesions showed a. Oct 01, 2018 overview of common imaging features found with tumefactive demyelination, characteristic pathologic findings, and relevant clinical features. Accurate diagnosis of tumefactive demyelinating lesions is clinically important to. Tumefactive demyelinating lesions in multiple sclerosis and.
To investigate the characteristics of tdls, clinical and radiological data from nine cases with tdls were analyzed after admission. It is called tumefactive as the lesions are tumorlike and they mimic tumors clinically, radiologically and sometimes pathologically. Overview of demyelinating disorders neurologic disorders. Often, it requires a brain biopsy to arrive at the right diagnosis. Tumefactive demyelinating lesions tdls can simulate intracranial neoplasms in clinical presentation and mr imaging appearance, and. Jan 27, 2009 tumefactive demyelinating lesions tdls are misdiagnosed frequently. A demyelinating disease originates in the brain and is often degenerative. Mass effect and contrast enhancement on neuroimaging make it difficult to distinguish. Tumefactive multiple sclerosis requiring emergent biopsy and. One month after, a ct scan showed involvement also of the left. They manifest as solitary lesions or a few separate lesions that result in a mass effect, edema on the surrounding tissue and enhancement 4,5. The central nervous system is made up of the brain, spinal cord, and optic nerve. Jul 10, 2015 tumefactive demyelinating lesions tdls, also referred to as demyelinating pseudo tumors, are a rare type of demyelinating disease.
Tumefactive demyelination, intraoperative crush preparation with circled cells last updated on wednesday, march 25 2009 by jdmiles when a brain biopsy is performed, often the pathologist will do what is called a touch preparation or crush preparation. Our purpose is to describe the association between. To investigate the characteristics of tdls, clinical and radiological data from nine cases with. Multiple sclerosis and demyelinating disorders articles. She improved rapidly but shortly after discharge developed worsening rightsided weakness and dysarthria and returned several weeks later. A case of pediatric tumefactive demyelinating lesion. Miscellaneous demyelinating diseases frontalcortex. The patient clinically improved and in a followup mri 3 months later there was substantial regression of all lesions. 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. Spectrum of disease, diagnosis and treatment article pdf available in current neurobiology 0701 january 2016 with 990 reads how we measure reads. 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. 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. These are lesions of more than 2 cm in size with the presence of mass effect.
Frontal tumefactive demyelinating lesion mimicking. In a study regarding multiple sclerosis is a chronic debilitation special needs trusts are prognosis for tumefactive multiple sclerosis currently used to allow multiple sclerosis will be tough much. May 21, 2016 the patient was diagnosed with acute multifocal demyelination, presumed to be acute disseminated encephalomyelitis or tumefactive multiple sclerosis, and treated with intravenous corticosteroids. Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is essential to avoid inadvertent surgical or toxic. The picture of openring with the open portion joining the grey matter. Tumefactive multiple sclerosis tumefactive ms is an atypical variant of. Tumefactive demyelinating lesions tdls are large usually 2 cm demyelinating brain lesions that mimic primary brain tumors, including primary central nervous system lymphoma pcnsl and highgrade glioma. The associated t2 hyperintensity does not extend significantly beyond the leading edge of enhancement. Tumefactive demyelinating lesion tdl is a rare variation of multiple sclerosis ms, 1 which presents as a profound brain edema or mass due to an acute demyelination in the central nervous system. A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. 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. Pitfalls in the diagnosis of a tumefactive demyelinating.
Jan 10, 2016 tumefactive demyelination, part of the spectrum of demyelinating disease, poses a diagnostic conundrum by mimicking a highgrade neoplasm. Tumefactive demyelinating lesions tdls, also referred to as demyelinating pseudo tumors, are a rare type of demyelinating disease. Tumefactive demyelination, intraoperative crush preparation. Tumefactive demyelination and pontine demyelination are types of these lesions. Multiple sclerosis is an inflammatory disease of the central nervous system cns whose aetiology and pathogenesis are still to be clarified. Tumefactive demyelinating lesions in multiple sclerosis. Demyelination occurs as the result of diseases that damage the myelin.
Tumefactive demyelination associated with developmental. 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 rare form of multiple sclerosis ms. The characteristics of tumefactive demyelination can mimic other diseases and can even lead to wrong diagnosis. Mass effect and contrast enhancement on neuroimaging make it difficult to distinguish this type of lesion from highgrade gliomas. Mri findings in tumefactive demyelinating lesions american. Tumefactive multiple sclerosis requiring emergent biopsy. There is incomplete peripheral enhancement of the lesion. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, balos concentric sclerosis, schilders disease, and marburgs. Demyelination cure relates to any treatment to alleviate this condition. Presentation tdl are very rare in ms and occur in the early stage variable presentation impairment may be very slight even in a patient with a.
Tumefactive multiple sclerosis radiology reference. 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. Tumefactive demyelinating lesion radiology reference. Tumefactive demyelinating lesions are rare consequences of central nervous system cns idiopathic inflammatory demyelinating diseases.
This damage impairs the conduction of signals in the affected nerves. Tumefactive demyelination mri neuroradiology cases. Multiple sclerosis ms is the most common of the demyelinating diseases of the central nervous system and thorough descriptions of its usual. Other questions include how best to treat an acute attack as well as the optimal timing of therapy to prevent relapse. Inflammatory demyelination occasionally forms a solitary mass lesion. By susan gauthier, do, mph, assistant professor of neurology, weill cornell medical college. These are lesions of more than 2 cm in size with the presence of mass effect, edema or ring enhancement. 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. A previous mri and ct report mentions a recent ischemic lesion in right parietal white. Biopsy of the frontal lesion revealed inflammation with perivascular cd20 positive cells which was compatible with demyelination. Axial t1 prepost contrast and t2 fatsat images demonstrate an ovoid lesion in the right centrum semiovale that is not associated with mass effect. Demyelination is often secondary to an infectious, an ischemic, a metabolic, or a hereditary disorder or to a toxin eg, alcohol, ethambutol. Demyelinating definition of demyelinating by merriamwebster. 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.
Proton mr spectroscopy of tumefactive demyelinating lesions. Tumefactive demyelinating lesions tdls can simulate intracranial neoplasms in clinical presentation and mr imaging appearance, and surgical biopsy is often performed in suspected tumors. In primary demyelinating disorders, cause is unknown, but an autoimmune mechanism is suspected because the disorder sometimes follows a viral infection or viral vaccination. All cases underwent surgery and pathological examination. A previous mri and ct report mentions a recent ischemic lesion in right parietal white matter with restricted diffusion and was on anti coagulant therapy. Myelin and white matter the gray and white matter of the. Ms is a disabling and progressive disease that affects the central nervous system. Tumefactive multiple sclerosis is a term used to describe patients with established multiple sclerosis who develop large aggressive demyelinating lesions, similaridentical in appearance. Whether tumefactive demyelination is another manifestation of ms or represents a distinct entity is apparently a matter of contention.
Combining diffusion tensor metrics and dsc perfusion imaging. Demyelination is damage to the myelin sheath around nerves. Tumefactive lesions are an uncommon manifestation of demyelinating disease and can pose a diagnostic challenge in patients without a preexisting diagnosis of multiple sclerosis. Tumefactive demyelination mri a 75 y o male with left side weakness since 3 months. These atypical features include size more than 2 cm. Tumefactive demyelination the neurosurgical atlas, by aaron. Imaging of demyelinating diseases final slideshare.
Tumefactive lesions are an uncommon manifestation of demyelinating disease and can pose a diagnostic challenge in patients without a preexisting diagnosis of multiple. Abstract tumefactive demyelination is sometimes difficult differentiate from malignant tumors. Tumefactive demyelinating lesion is defined as large solitary demyelinating lesion with imaging characteristics mimicking neoplasm. The mri appearance of tumefactive demyelinating lesions. Oct 01, 2019 demyelination is damage to the myelin sheath around nerves. Axial t1 prepost contrast and t2 fatsat images demonstrate an ovoid lesion in the right centrum semiovale that is not associated with mass.
Methionine pet in the differentiation of tumefactive multiple. Large demyelinating lesions with possible mass effect tumefactive multiple sclerosis or tumefactive demyelination can be mistaken for. In this report we describe the clinical, neuroimaging and neuropathological features of six cases of tdl. In a study regarding multiple sclerosis is a chronic debilitation special needs trusts are prognosis for tumefactive multiple sclerosis currently used to allow multiple sclerosis will be tough much tougher than a joint policy has been established by smith et al am j gastroenterol 2007. Tumefactive multiple sclerosis radiology reference article. Tumefactive demyelination the neurosurgical atlas, by. Overview of common imaging features found with tumefactive demyelination, characteristic pathologic findings, and relevant clinical features.
In advanced ms the multifocal t2 lesions can merge and become confluent and at the same time brain atrophy has been shown to progress. In turn, the reduction in conduction ability causes deficiency in sensation, movement, cognition, or other functions depending on which nerves are involved. Choosing when to biopsy a tumefactive lesion to exclude alternative pathology can be difficult. Though not pathognomonic of tumefactive demyelination, the cells are characteristically seen within tumefactive demyelinating lesions. Proton mr spectroscopy has been applied in assessing various intracranial diseases and is increasingly used in diagnosis and clinical management. Demyelination in the central nervous system sometimes presents with large pseudotumoral lesions mimicking brain neoplasm. Multiple sclerosis and demyelinating disorders will cease to be published by bmc as of 6th november 2019. Neuroradiological evaluation of demyelinating disease. T1 hypointense top row left oval, subcortical lesion with corresponding flair hyperintense. Tumefactive lesions at ms onset not predictive of a more severe or protracted ms course. 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. Objectivelarge demyelinating lesions with possible mass effect tumefactive multiple sclerosis or tumefactive.
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