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