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Clinical and radiological features of Marchiafava-Bignami disease.

ABSTRACT: Marchiafava-Bignami disease (MBD) is a rare neurological disease usually associated with chronic alcoholism and characterized by demyelination and necrosis. Our aims were to describe the clinicoradiological features and identify factors that may affect the prognosis of patients with MBD.We examined clinical manifestations, laboratory results, and neuroradiological features of 9 patients with MBD. The patients were classified into 2 subgroups (favorable and poor outcome subgroups) based on the Modified Oxford Handicap Scale (MOSH). In addition, we compared the clinical and neuroimaging features between the 2 subgroups.Nine adult male patients (age of onset range 37-62 years, with a mean age of 47.00?±?14.50 years) were included in this study. According to MOSH, 4 patients were placed in the poor outcome subgroup (MOHS???3), 5 patients were placed in the favorable outcome subgroup (MOHS???2). Relatively high score of MAST-C (?6) (P?=?.008), extracallosal lesions (P?=?.048), GCS (P?=?.026), cerebral lobe impairment (P?=?.048) was significantly more common in the poor outcome subgroup.Clinical manifestations of MBD are variable and lack specificity. Early diagnosis by relatively specific performance of bisymmetric lesions in corpus callosum of diffusion-weighted imaging (DWI) may affect the prognosis. The prognosis of patients with severe disturbance of consciousness, heavy alcohol consumption, extracallosal lesions, cerebral lobe impairment is probably unfavorable.

SUBMITTER: Dong X

PROVIDER: S-EPMC5805414 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Clinical and radiological features of Marchiafava-Bignami disease.

Dong Xiaoyu X Bai Chaobo C Nao Jianfei J

Medicine 20180201 5

Marchiafava-Bignami disease (MBD) is a rare neurological disease usually associated with chronic alcoholism and characterized by demyelination and necrosis. Our aims were to describe the clinicoradiological features and identify factors that may affect the prognosis of patients with MBD.We examined clinical manifestations, laboratory results, and neuroradiological features of 9 patients with MBD. The patients were classified into 2 subgroups (favorable and poor outcome subgroups) based on the Mo ...[more]

PMID: 29384842

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Project description:OBJECTIVES: To illustrate the clinical and radiological features of idiopathic interstitial pneumonias (IIPs), according to the American Thoracic Society (ATS)/European Respiratory Society (ERS) classification updated in 2013. METHODS: IIPs include a subset of diffuse and restrictive lung diseases, resulting from damage to the parenchyma characterised by inflammation and fibrosis of the interstitium. Classification into major and rare IIPs is based on the 2013 ATS/ERS committee. RESULTS: The diagnosis of idiopathic pulmonary fibrosis (IPF) needs to exclude other well-known causes of interstitial lung diseases. According to the 2011 evidence-based guidelines, usual interstitial pneumonia (UIP) can be diagnosed by HRCT when all criteria are fulfilled. Non-specific interstitial pneumonia (NSIP) is characterised by patchy ground-glass opacities and irregular linear/reticular opacities. The imaging patterns of respiratory bronchiolitis associated-interstitial lung disease (RB-ILD) and desquamative interstitial pneumonia (DIP) show centrolobular nodules and ground-glass opacities. Cryptogenic organising pneumonia (COP) consists of patchy peripheral or peribronchial consolidations, while ground-glass opacities are typically associated with diffuse lung consolidation, evolving to fibrosis, in acute interstitial pneumonia (AIP). Rare IIPs include lymphoid interstitial pneumonia and idiopathic pleuro-parenchymal fibroelastosis (IPPFE). CONCLUSIONS: The knowledge of IIP imaging features on HRCT images help radiologists in diagnosis. Moreover, the overlap of imaging features needs a multidisciplinary approach. TEACHING POINTS: • UIP findings are reticulations, bronchiectasis, honeycombing and absence of inconsistent features. • Bilateral patchy ground-glass areas represent the most encountered features in NSIP. • Poorly defined centrilobular nodules are typical of RB-ILD, whereas a ground-glass appearance is typical of DIP. • HRCT features of COP include characteristic peripheral or peribronchial patchy consolidations. • Rare IIPs include idiopathic LIP and idiopathic pleuro-parenchymal fibroelastosis (PPFE).

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Clinical and radiological features of Marchiafava-Bignami disease.

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Clinical and radiological features of Marchiafava-Bignami disease.

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