Bilateral middle cerebellar peduncles involvement a malnourished man with Marchiafava-Bignami disease

Shen, Yali; Cheng, Zhi-Juan; Dai, Tingmin; Nie, Hongbing

doi:10.1007/s10072-018-3608-7

Cited by 5 publications

(12 citation statements)

References 8 publications

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“…Type B is therefore believed to have a better prognosis as compared with type A. [7][8][9][10][11] Diagnosis nowadays is mainly made using MRI, characteristically demonstrating symmetrical, confluent lesions of the corpus callosum. The lesions typically start in the body, later spreading to the genu and the splenium, and show diffusion restriction and appear hyperintense on T2 and FLAIR images.…”

Section: Discussionmentioning

confidence: 99%

From Mild Gait Difficulties to a Sudden Coma: A Rare Case of Marchiafava-Bignami Disease

Ryck

Cauter²,

Bekelaar³

2023

Topics in Magnetic Resonance Imaging

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In this case report we describe the case of a 66-year old man with subacute gait difficulties, with a progression to confusion coma with multiple generalised epileptic seizures during the following days. Biochemical analysis showed hyperglycaemia, cerebrospinal fluid (CSF) testing showed a mild lymphocytic pleocytosis and an elevated protein and lactate. Broad-spectrum antibiotics and antiviral therapy where initiated. However, all other CSF testing remained negative. Magnetic resonance imaging of the brain showed remarkably symmetric hyperintense T2 white matter lesions most noticable in the corpus callosum. The lesion pattern was suggestive of a metabolic or toxic encephalopathy, the preponderance for the corpus callosum was furthermore suggestive for Marchiafava-Bignami disease (MDB), as was the clinical course since admission of the patient. A high dose IV substitution of vitamin B1, B6 and B12 was started and antibiotic and antiviral therapy was discontinued. After one day the patient showed progressive regaining of consciousness and he returned to premorbid functioning in a matter of 1-2 weeks. MRI of the brain after 1 week showed notable improvement of the white matter lesions. At routine follow-up two weeks later he presented with icterus and a diagnosis of Epstein-Barr virus (EBV) hepatitis was made, lymph node biopsies showed an EBV positive diffuse large cell B-cell lymphoma (DLCBL). MDB is mostly associated with severe alcoholism, with malnourishment being the second leading cause, however there are case reports describing MDB in patients with chronically poorly controlled diabetes mellitus. We hypothesize that his condition may have been precipitated by his poorly controlled diabetes mellitus. However it is also possible that weight loss (probably related to the DLCBL diagnosis) might have contributed to a state of malnourishment and therefore played a role in the aetiology as well.

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Section: Discussionmentioning

confidence: 99%

From Mild Gait Difficulties to a Sudden Coma: A Rare Case of Marchiafava-Bignami Disease

Ryck

Cauter²,

Bekelaar³

2023

Topics in Magnetic Resonance Imaging

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“…Extra-callosal regions, such as SCWM or frontal cortex, have been infrequently described; other extra-callosal lesions including internal capsules, cerebral peduncles, MCPs, and hippocampus have also been rarely documented. [3,4] In this study, the incidence of extra-callosal involvement was estimated to be 53.3%. Among eight patients with extra-callosal involvement, four had cortical gray matter lesions.…”

mentioning

confidence: 83%

Clinical and neuroradiological features of 15 patients diagnosed with Marchiafava-Bignami disease

Shen

Zhou

Han

et al. 2019

Chinese Medical Journal

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“…Hiperintensity in Middle Cerebellar Peduncles in T2 MRI (MCP-sign) is an infrequent nding, usually observed in the context of neurodegenerative pathologies, considered a major diagnostic criteria of fragile X-associated tremor ataxia syndrome 1 . It has rarely been described in other entities like Multiple System Atrophy (MSA), Wilson Disease, etc.…”

Section: Main Articlementioning

confidence: 99%

“…MBD is an infrequent pathology (although probably underdiagnosed), affecting most frequently middleaged male (40-60 years of age) 1 . Its pathophysiology is unknown, it is associated with chronic alcoholism and malnutrition (including vitamin de ciencies, especially thiamine) 2 producing demyelinating lesions involving the corpus callosum 1,3 . The few cases described demonstrate a variable clinical presentation 4 , including ataxia, impairment of mental status, etc.…”

Section: Main Articlementioning

confidence: 99%

“…Given the non-speci c symptomatology, the diagnosis in the past was based on postmortem pathological examination, and currently is based on the characteristic neuroimaging ndings (symmetric lesions of the Corpus Callosum). Extracallosal lesions such as cortical/subcortical white matter lesions are well documented, but the MCP-sign is rare, with only a few cases reported 1,3,5 . Treatment includes vitamin reconstitution and supportive care 4,6 .…”

Section: Main Articlementioning

confidence: 99%

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Reversible Hyperintensity in Middle Cerebellar Peduncles, an infrequent finding in Marchiafava-Bignami Disease

Domínguez

Casadevall

Bravo

2023

Preprint

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Middle Cerebellar Peduncle sign (MCP-sign) is an infrequent finding in brain MRI, usually in the context of neurodegenerative pathologies, considered a classic sign of fragile X-associated tremor ataxia syndrome. Its description in other entities is anecdotal. We present a case of Marchiafava-Bignami Disease (MBD), where reversible hyperintensity in MCP-sign is observed. A 59-year-old male, with history of alcoholism, presented subacute gait disturbance and cognitive deterioration, with progressive worsening over 3 months. On examination, we observed somnolence, dysarthria, cerebellar and sensory ataxia, and cognitive impairment (recovery-memory deficit, executive-attentional dysfunction). Brain MRI showed global thinning of the corpus callosum, T2 hyperintensity at the splenium and MCP-sign; findings described in MBD. The analytical study showed deficiency of folic acid and thiamine. Genetic study ruled out FMR1 premutation. Final diagnosis was probable MBD type B. Treatment with vitamin supplementation showed progressive clinical improvement. A follow-up brain MRI, 2 months after hospital discharge, highlights the resolution of MCP sign, but persistence of the findings in the corpus callosum. MBD is an infrequent pathology, associated with alcoholism and malnutrition, characterized by demyelinating lesions involving the corpus callosum. The few cases described demonstrate a highly variable clinical presentation. The MCP-sign has been described only in a few reports. In our case, good clinical and radiological response stands out after vitamin supplementation. The presence of MCP-sign should also suggest the possibility of MBD, and early vitamin and nutritional assessment should be performed, being an infrequent but treatable entity, with a potentially fatal course in the absence of treatment.

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Bilateral middle cerebellar peduncles involvement a malnourished man with Marchiafava-Bignami disease

Cited by 5 publications

References 8 publications

From Mild Gait Difficulties to a Sudden Coma: A Rare Case of Marchiafava-Bignami Disease

From Mild Gait Difficulties to a Sudden Coma: A Rare Case of Marchiafava-Bignami Disease

Clinical and neuroradiological features of 15 patients diagnosed with Marchiafava-Bignami disease

Reversible Hyperintensity in Middle Cerebellar Peduncles, an infrequent finding in Marchiafava-Bignami Disease

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