Conventional MRI and magnetisation transfer imaging of tumour-like multiple sclerosis in a child.
ABSTRACT Tumefactive multiple sclerosis is a rare entity in children. Differential diagnosis includes other mass lesions such as neoplasm and abscess. A case of tumefactive multiple sclerosis in a child is presented. The open-ring pattern of enhancement on conventional MRI and magnetisation transfer imaging was important for the initial diagnosis and the evaluation of the course of the disease.
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ABSTRACT: Tumefactive demyelinating disease (TDD) is a rare demyelinating disease—probably a variant of multiple sclerosis (MS)—presenting as a focal cerebral mass. Clinically and radiographically, TDD is usually difficult to differentiate from tumor and abscess. Magnetic resonance imaging (MRI) characteristically shows a large lesion—contrast enhancement often like an incomplete ring—with relatively limited mass effect and surrounding edema. Findings on “non-conventional” MRI, multifocality on MRI of the whole central nervous system (CNS), monitoring of visual evoked potentials, and cerebrospinal fluid analysis may all contribute to the differential diagnosis. In analogy with the recommendations for acute MS exacerbations, acute disseminated encephalomyelitis and other forms of acute conditions with severe inflammatory CNS demyelination, high-dose corticosteroid treatment is regarded as first-line therapy. A rapid and pronounced clinical and radiological response to the administration of high-dose corticosteroids is often observed and may alleviate the need for cerebral biopsy. In agreement with the recommendations for other conditions characterized by acute CNS demyelination, plasma exchange is considered to be second-line therapy. In patients who do not respond to non-surgical therapy and who exhibit a pronounced cerebral mass lesion and clinical signs of increased intracranial pressure (ICP), decompressive craniectomy including wide opening of the dura is recommended.European Neurological Journal. 01/2010;
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ABSTRACT: Atypical imaging features of multiple sclerosis lesions include size >2 cm, mass effect, oedema and/or ring enhancement. This constellation is often referred to as 'tumefactive multiple sclerosis'. Previous series emphasize their unifocal and clinically isolated nature, however, evolution of these lesions is not well defined. Biopsy may be required for diagnosis. We describe clinical and radiographic features in 168 patients with biopsy confirmed CNS inflammatory demyelinating disease (IDD). Lesions were analysed on pre- and post-biopsy magnetic resonance imaging (MRI) for location, size, mass effect/oedema, enhancement, multifocality and fulfilment of Barkhof criteria. Clinical data were correlated to MRI. Female to male ratio was 1.2 : 1, median age at onset, 37 years, duration between symptom onset and biopsy, 7.1 weeks and total disease duration, 3.9 years. Clinical course prior to biopsy was a first neurological event in 61%, relapsing-remitting in 29% and progressive in 4%. Presentations were typically polysymptomatic, with motor, cognitive and sensory symptoms predominating. Aphasia, agnosia, seizures and visual field defects were observed. At follow-up, 70% developed definite multiple sclerosis, and 14% had an isolated demyelinating syndrome. Median time to second attack was 4.8 years, and median EDSS at follow-up was 3.0. Multiple lesions were present in 70% on pre-biopsy MRI, and in 83% by last MRI, with Barkhof criteria fulfilled in 46% prior to biopsy and 55% by follow-up. Only 17% of cases remained unifocal. Median largest lesion size on T2-weighted images was 4 cm (range 0.5-12), with a discernible size of 2.1 cm (range 0.5-7.5). Biopsied lesions demonstrated mass effect in 45% and oedema in 77%. A strong association was found between lesion size, and presence of mass effect and/or oedema (P < 0.001). Ring enhancement was frequent. Most tumefactive features did not correlate with gender, course or diagnosis. Although lesion size >5 cm was associated with a slightly higher EDSS at last follow-up, long-term prognosis in patients with disease duration >10 years was better (EDSS 1.5) compared with a population-based multiple sclerosis cohort matched for disease duration (EDSS 3.5; P < 0.001). Given the retrospective nature of the study, the precise reason for biopsy could not always be determined. This study underscores the diagnostically challenging nature of CNS IDDs that present with atypical clinical or radiographic features. Most have multifocal disease at onset, and develop RRMS by follow-up. Although increased awareness of this broad spectrum may obviate need for biopsy in many circumstances, an important role for diagnostic brain biopsy may be required in some cases.Brain 07/2008; 131(Pt 7):1759-75. · 9.92 Impact Factor
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ABSTRACT: Tumefactive demyelinating lesions (TDLs) are misdiagnosed frequently. To investigate the characteristics of TDLs, clinical and radiological data from nine cases with TDLs were analyzed after admission. All cases underwent surgery and pathological examination; some received postoperative steroid therapy. Onsets were mostly within 3 weeks and main presentation included intracranial hypertension, extremity weakness, epilepsy, and visual disturbance. Symptoms in children were acute and severe, frequently including headache, vomiting, and visual disturbance. Most intracephalic lesions were in cerebral hemispheres. All intraspinal lesions were in cervical segments. Radiological features included mass effect, perifocal edema and enhancement (of which open-ring enhancement was diagnostic), and decreased relative cerebral blood volume. Intraoperative frozen section did not confirm the diagnosis, while postoperative paraffin section did confirm it (by evidence of macrophage infiltration). The patients responded well to steroid therapy and no relapse was found during following up. Thus, intensive analysis of both clinical and radiological data may provide some clues for diagnosis. For suspected cases, it is advisable to take steroid therapy or undergo advanced radiological examinations, such as serial magnetic resonance spectroscopy. However, in difficult cases, pathological evidence is beneficial to a final diagnosis.Neurosurgical Review 02/2009; 32(2):171-9; discussion 179. · 1.97 Impact Factor