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. Metabolite findings in tumefactive demyelinating lesions. Other questions include how best to treat an acute attack as well as the optimal timing of therapy to prevent relapse. Demyelination in the central nervous system sometimes presents with large pseudotumoral lesions mimicking brain neoplasm. Tumefactive multiple sclerosis is a rare form of multiple sclerosis ms. Ms is a disabling and progressive disease that affects the central nervous system. Tumefactive demyelinating lesions tdls are often misdiagnosed as brain tumors. Multiple large tumefactive ms plaques in a young man. Background multiple sclerosis ms is a chronic inflammatory disease of the central nervous system in which there is focal demyelination. Whether tumefactive demyelination is another manifestation of ms or represents a distinct entity is apparently a matter of contention. Multiple sclerosis ms is the most common demyelinating disease. A tumefactive demyelinating lesion tdl is defined as a solitary demyelinating lesion greater than 2 cm.
Mr imaging and multiple sclerosis differential diagnosis book. Tumefactive demyelinating lesions tdls are atypical presentations of various. 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. When a single large or multiple small tumefactive demyelinating lesions are present, the correct presurgical diagnosis is often difficult, as the mri appearance, comprising enhanced t1weighted images, t2weighted images, and even proton mr spectroscopy, is not specific for these lesions. Section 1 describes the physical principles underlying each technique and their associated artefacts and pitfalls. To use mr spectroscopy to aid in the diagnosis of demyelinating disease and to help differentiate tumefactive demyelinating lesions from neoplastic processes.
Certain features on standard as well as advanced mri can potentially promote the diagnosis of tumefactive demyelination and obviate the need for biopsy. 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. Clinical and magnetic resonance imaging mri distinctions between tumefactive demyelination and brain tumors in children. Chinese guidelines for the diagnosis and management of. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Tumefactive multiple sclerosis requiring emergent biopsy and. The mri appearance of these lesions can aid in preoperative diagnosis and assist with the final pathologic interpretation, potentially sparing the patient unnecessary and possibly debilitating procedures and therapies. Positron emission to mography, magnetic resonance imaging and proton nmr spectroscopy of white matter in multiple sclerosis. Magnetic resonance imaging mri is a medical imaging technique used in radiology to visualize internal structures of the body in detail.
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. Tumefactive multiple sclerosis is a demyelinating disease that demonstrates tumorlike features on magnetic resonance imaging. Metabolite findings in tumefactive demyelinating lesions utilizing. Mri showed a mass lesion with prolonged t1 and t2 values and gadolinium enhancement in all cases. Oct 01, 2019 demyelination is damage to the myelin sheath around nerves. R neelima 1, k krishnakumar 2, md nair 3, c kesavadas 4, divyata rajendra hingwala 4, vv radhakrishnan 1, sruthi s nair 3 1 department of pathology, sree chitra tirunal institute of medical sciences and technology, trivandrum, kerala, india 2 department of.
Tumefactive demyelination mri a 75 y o male with left side weakness since 3 months. The clinical presentation and mri appearance of tdm lesions caused by primary hiv infection can simulate classical demyelinating disease such as multiple sclerosis or an intracranial neoplasm, e. Most patients with lowgrade gliomas had an absence of contrast enhancement on mri and lower uptake of. Tumefactive demyelinating lesion is defined as large solitary demyelinating lesion with imaging characteristics mimicking neoplasm. Progression of tumefactive demyelinating lesion in a child. Characterization of tumefactive demyelinating lesions using mr. Request pdf characterization of tumefactive demyelinating lesions using mr imaging and invivo proton mr spectroscopy diagnosis of tumefactive. Lumbar puncture displayed positive oligoclonal bands. 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. Proton mr spectroscopy of tumefactive demyelinating lesions article pdf available in american journal of neuroradiology 238.
Even the advanced mri techniques, such as mr spectroscopy, led to equivocal results because both normalized choline increase and nacetyl. As the underlying pathophysiology of neoplasms is different from that of demyelinating disease, one may expect the metabolic composition of neoplasms to be. Clinical proton mr spectroscopy in central nervous system. Imaging of demyelinating diseases final linkedin slideshare. As with tumefactive demyelination, balos sclerosis lesions are often mistaken for primary brain. Proton mr spectroscopy has been increasingly used to characterize intracranial pathology. It is often difficult to accurately differentiate tumefactive demyelinating lesions tdls. Saini j, chatterjee s, thomas b, kesavadas c 2011 conventional and advanced magnetic resonance imaging in tumefactive demyelination. Jan 10, 2016 whether tumefactive demyelination is another manifestation of ms or represents a distinct entity is apparently a matter of contention. Myelin and white matter the gray and white matter of the central nervous system cns differ not only in gross morphology but also in water content and macromolecular components, notably membrane lipids.
Tumefactive demyelinating lesion tdl is defined as a solitary demyelinating lesion greater than 2 cm. On imaging, they usually present with relatively little mass effect or surrounding edema. To identify imaging characteristics of tdls for their proper diagnosis, their differences from malignant gliomas, etc. Proton magnetic resonance spectroscopy differentiates tumefactive. Clinical proton mr spectroscopy in central nervous system disorders. Neurological disorders and imaging physics, volume 1 book home. The mri scan of the brain revealed a space occupying lesion within the occipital lobe and mr spectroscopy highlighted this to be inflammatory in nature, most likely a tumefactive demyelinating lesion tdl. It is important to be aware of the differential diagnosis of tumefactive demyelination, in order to direct appropriate investigation. Proton mr spectroscopy of tumefactive demyelinating lesions. Demyelination is something that occurs in several different diseases. This is a good example of tumefactive demyelination with the typical advancing edge of enhancement. When the myelin sheath is damaged, nerve impulses slow or even stop, causing neurological problems. In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. 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.
Proton magnetic resonance spectroscopy differentiates tumefactive demyelinating lesions from gliomas. It is called tumefactive as the lesions are tumorlike and they mimic tumors clinically, radiologically and sometimes pathologically. Given the age of the patient, the diagnosis was confirmed with a biopsy. Illustrate the role of multimodal mri with a focus on spectroscopy, diffusion and perfusion imaging to increase diagnostic confidence. Clinical, imaging, and followup observations in thirtynine patients. This is the best way to tell what the severity of the demyelination is, what the demyelination could be caused by, and what disease the. Metabolic assessment of monofocal acute inflammatory demyelination using mr spectroscopy and 11 cmethionine, 11 c. Mr imaging of the brain was obtained in 4 patients who presented clinically with focal neurologic deficits. 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. Pdf proton mr spectroscopy of tumefactive demyelinating lesions.
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. Tumefactive demyelination tumefactive demyelinating tdm lesions present a considerable diagnostic challenge. Saindane am, soonmee c, meng l, xiaonan x, knopp ea, zagzag d. 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. Proton mr spectroscopy and 201 tl spect showed findings supportive of the diagnosis of malignant glioma in all cases. Part 2, t1weighted imagingbased differential diagnosis christopher j. Tumefactive demyelination does not usually originate as apostinfectious or postvaccination response. Proton mr spectroscopy of tumefactive demyelinating. Characterization of tumefactive demyelinating lesions. 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.
Mass effect and contrast enhancement on neuroimaging make it difficult to distinguish this type of lesion from highgrade gliomas. The incomplete rim of enhancement and low t1 signal is fairly classic for a tumefactive, demyelinating lesion. This is the best way to tell what the severity of the demyelination is, what the demyelination could be caused by, and what disease the demyelination could. From the medical records, the patients initial symptoms and edss at the time of diagnosis of tumefactive demyelination, magnetic resonance imaging mri results contemporaneous with the above diagnosis and up to two subsequent mris during the period of observation, treatments if any initiated after the diagnosis of tumefactive demyelination and later. A large body of published work shows that proton hydrogen 1 1 h magnetic resonance mr spectroscopy has evolved from a research tool into a clinical neuroimaging modality. Primary central nervous system lymphoma pcnsl may rarely be preceded by sentinel demyelination, a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis ms or acute disseminated encephalomyelitis adem. Tumefactive multiple sclerosis requiring emergent biopsy. This damage impairs the conduction of signals in the affected nerves. Glioblastoma multiforme presenting with an open ring.
Characterization of tumefactive demyelinating lesions using mr imaging and invivo proton mr spectroscopy article in multiple sclerosis 152. 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. While perfusion can be mildly increased along the periphery of the lesion, it is often much less than would be expected with a high grade glioma. 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. 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 prove to be a diagnostic dilemma to neurosurgeons, radiologists, and pathologists. Mri is often sufficient to make a diagnosis of tumefactive demyelination and the results of biopsy can be inconclusive or misleading. Tumefactive demyelinating lesions tdls can simulate intracranial neoplasms in clinical presentation and mr imaging appearance, and surgical biopsy is often performed in suspected tumors. The clinical use of mr spectroscopy in multiple sclerosis, an acquired demyelinating disease, remains limited despite the various insights into. Mri makes use of the property of nuclear magnetic resonance nmr to image nuclei of atoms inside the body. Inflammatory demyelinating disease mimicking malignant glioma. Mr imaging initially revealed parenchymal mass lesions.
Oct 01, 2018 tumefactive demyelination last updated. Tumefactive demyelinating lesions can present with features similar, clinically and radiologically, to those of brain tumours. Tdls are defined as demyelinating lesions larger than 2 cm that often have. Differentiation of tumefactive demyelinating lesions from high. Additional white matter lesions are present in 85% of patients with td and these may be reassuring if their distribution is typical for ms and if the tumefactive lesions have radiological characteristics in. Spectroscopic magnetic resonance imaging of a tumefactive. In particular, differential diagnosis between tumefactive demyelinating lesions tdls and brain tumours is often challenging lucchinetti et al.
Interpreting the overlapping radiologic and clinical characteristics associated with each of these. A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. A demyelinating disease is any condition that results in damage to the protective covering myelin sheath that surrounds nerve fibers in your brain, optic nerves and spinal cord. Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is essential to avoid inadvertent surgical or toxic chemotherapeutic. Large, atypical demyelinating lesions on mri can be difficult to distinguish between primary central nervous system tumors and are referred to as tumefactive demyelinating lesions. These atypical features include size more than 2 cm, mass effect, edema, andor ring enhancement. In this report we describe the clinical, neuroimaging and neuropathological features of six cases of tdl. Clinical mr neuroimaging, second edition, provides radiologists, neuroscientists and researchers with a clear understanding of each physiological mr methodology and their applications to the major neurological diseases.
Clinical examination was unremarkable except for a rightsided homonymous hemianopia. Approximately half of tumefactive demyelinating lesions have pathologic contrast enhancement, usually in the form of ring enhancement 2, 4 figs. Seeing demyelination on mri dr garys multiple sclerosis. Glioblastoma multiforme presenting with an open ring pattern. This method is reliable because mris assess changes in proton density. It plays a part in several chronic conditions, including multiple sclerosis. Hmrs and conventional magnetic resonance imaging mri. Metabolite findings in tumefactive demyelinating lesions utilizing short echo time proton magnetic resonance spectroscopy. Spectroscopic magnetic resonance imaging of a tumefactive demyelinating lesion article in neuroradiology 4412. Choosing when to biopsy a tumefactive lesion to exclude alternative pathology can be difficult. Oct 09, 2011 tumefactive demyelination mri a 75 y o male with left side weakness since 3 months.
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. 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. Magnetic resonance spectroscopy mrs is a promising technique for the. Nov, 20 saini j, chatterjee s, thomas b, kesavadas c 2011 conventional and advanced magnetic resonance imaging in tumefactive demyelination. Tumefactive multiple sclerosis tms is a demyelinating disease. This case report highlights important diagnostic clues in the differential. Recently, without biopsy, some tms cases were diagnosed by magnetic resonance spectroscopy mrs, positron emission tomography pet, cerebrospinal fluid.
She improved rapidly but shortly after discharge developed worsening rightsided weakness and dysarthria and returned several weeks later. Proton magnetic resonance spectroscopy differentiates. 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 demyelinating lesion radiology reference. Inflammatory demyelination occasionally forms a solitary mass lesion clinically and radiographically indistinguishable from glioma, replete with enhancement and mass effect. However, surgical biopsy revealed inflammatory demyelinating disease. Axial mri and mr spectroscopy data acquired at 3 tesla in a 30 year old woman with tumefactive ms. This is the best way to tell what the severity of the demyelination is, what the. It is very important to differentiate tdl from glioma, because steroid use is a poor prognostic factor in glioblastoma steroids are often used in tdl shields et al. We report a case of tumefactive demyelinating lesion tdl diagnosed using 1hmagnetic resonance spectroscopy.
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