Clinicoradiologic subtypes of Marchiafava-Bignami disease

Heinrich, Annette; Runge, Uwe; Khaw, Alexander V.

doi:10.1007/s00415-004-0566-1

Cited by 179 publications

(145 citation statements)

References 31 publications

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“…In the acute stage, the patient often has nonspecific neurologic changes, such as confusion, coma, seizure, dysarthria, and hemiparesis. 2,3 Classic interhemispheric disconnection syndrome is often obscure in patients with confusional state, and it has often been misdiagnosed as Wernicke encephalopathy, alcohol withdrawal syndrome, or encephalitis. The early diagnosis depends on MR imaging.…”

Section: Discussionmentioning

confidence: 99%

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Marchiafava-Bignami Disease with Widespread Lesions and Complete Recovery

Tung¹,

Wu²,

Tsou³

et al. 2009

AJNR Am J Neuroradiol

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SUMMARY: MBD is a rare disorder strongly associated with alcoholism. It is characterized pathologically by demyelination and necrosis of the corpus callosum. MBD presents with severe neurologic deficits and significant sequelae developing in most survivors. We report a patient with total clinical recovery. Serial MR imaging demonstrated typical lesions with restricted diffusion in the acute stage and total resolution without atrophy or cystic change.ABBREVIATIONS: ADC ϭ apparent diffusion coefficient; DWI ϭ diffusion-weighted imaging; MBD ϭ Marchiafava-Bignami disease; T1WI ϭ T1-weighted imaging; T2WI ϭ T2-weighted imaging M BD is a rare disorder in individuals with chronic alcoholism and is characterized by severe neurologic symptoms and symmetric lesions in the corpus callosum. Cases with a favorable outcome have been reported in recent years.1,2 Nevertheless, most patients with MBD who survive have had significant sequelae. We present a patient with MBD showing complete resolution of the MR imaging abnormalities and clinical recovery, despite severe initial presentation and widespread lesions. Case ReportA 48-year-old man with a history of type II diabetes mellitus and chronic excessive alcohol consumption for Ͼ20 years had vertigo, slurred speech, and progressive gait disturbance. He was referred to us due to a rapidly deteriorating level of consciousness 6 days later. On admission, he was comatose, and findings of neurologic examinations showed tetraparesis, with generalized muscular hypertonia. There was mild hyperglycemia and hypokalemia. Blood test results, including biochemistry, electrolytes, nutrition, and endocrine function, were all within normal limits. Tests of urinary amphetamine and morphine levels were negative. The CSF analysis showed isolated mild hyperproteinorachia.Findings of brain CT performed 3 days after onset were normal. MR imaging on day 5 (Fig 1) demonstrated hyperintensity at the corpus callosum, hemispheric white matter, internal capsules, cerebral peduncles, and middle cerebellar peduncles on T2WI and DWI. The lesion in the corpus callosum appeared hypointense on T1WI. No abnormal enhancement was noted after gadolinium injection.On the basis of clinical and imaging features, MBD was diagnosed. Intravenous vitamin B complex and methylprednisolone therapy (1000 mg/day for 3 days) were started on day 7. On day 17, he regained normal attention, and a follow-up MR imaging demonstrated marked resolution of the lesions in the hemispheric white matter and middle cerebellar peduncles. There were residual hyperintense lesions at the splenium of the corpus callosum, internal capsules, and cerebral peduncles on T2WI and DWI. ADC mapping showed relative hypointensity in the aforementioned lesions (Fig 2AϪC).Approximately 4 weeks after the onset, he had a total recovery.Follow-up MR imaging performed 3 months after onset showed disappearance of signal-intensity abnormalities on T1WI, T2WI, and DWI (Fig 2DϪF), without atrophy or cystic change of the corpus callosum. Four months later, he return...

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Section: Discussionmentioning

confidence: 99%

“…Cases with a favorable outcome have been reported in recent years. 1,2 Nevertheless, most patients with MBD who survive have had significant sequelae. We present a patient with MBD showing complete resolution of the MR imaging abnormalities and clinical recovery, despite severe initial presentation and widespread lesions.…”

mentioning

confidence: 99%

Marchiafava-Bignami Disease with Widespread Lesions and Complete Recovery

Tung¹,

Wu²,

Tsou³

et al. 2009

AJNR Am J Neuroradiol

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“…With regard to the concepts of spirituality and religiosity and their measurement, we agree that these are distinct constructs, sometimes difficult to distinguish. According to Koenig et al, 4 spirituality is ''the personal quest for understanding answers to ultimate questions about life, about meaning and about relationship to the sacred or transcendent, which may (or may not) lead to or arise from the development of religious rituals and the formation of community.'' Several authors have examined relationships between spirituality, religiosity, and mental health, with varying results.…”

Section: Letters To the Editormentioning

confidence: 99%

“…In the acute phase, there can be peripheral enhancement on postcontrast studies, and lesions exhibit restricted diffusion. 3 Heinrich et al 4 proposed an imaging-based classification in which the type A corresponds to diffuse callosal involvement, whereas type B, which carries a better prognosis, includes only partial lesions.…”

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confidence: 99%

Marchiafava-Bignami disease as a cause of visual hallucinations

Augusto

Figueiredo

Costa

et al. 2015

Rev. Bras. Psiquiatr.

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“…Type A, with impairment of consciousness, seizures, hemiparesis, poor prognosis and T2W hyperintense swelling of the entire corpus callosum, sometimes with extracallosal lesions; Type B, with slight impairment of consciousness, progressive dementia, pyramidal and cerebellar dysfunctions, better outcome and partial callosal lesions on MRI [6,7].…”

Section: Mbd Can Present In Two Main Clinical Forms According To Heinmentioning

confidence: 99%

Marchiafava-Bignami Disease: Uncertain MRI Predictors of Outcome

Marchi¹,

Tondo²,

Varrasi³

et al. 2017

J Neurol Neurosci

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Marchiafava-Bignami disease (MBD) is a rare, often fatal, toxic neurological disease mainly characterized by demyelination of the corpus callosum, with or without associated lesions of hemispheric white matter. The clinical picture is severe, and includes both neuropsychiatric features, and signs of interhemispheric disconnection. The disease can have an acute or chronic course. Neuroimaging provides the most useful information for diagnosis and prognosis. We present a 44-year-old patient suffering from subacute onset of MBD, in who diffusion-weighted MRI revealed symmetrical hyperintense lesions in the frontal region of cerebral cortex and in the body/splenium of corpus callosum. Involvement of the corpus callosum was partial; the diagnosis was rather prompt, and the treatment was appropriated. The patient, survived to the acute phase, developed a chronic dementia.

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Clinicoradiologic subtypes of Marchiafava-Bignami disease

Cited by 179 publications

References 31 publications

Marchiafava-Bignami Disease with Widespread Lesions and Complete Recovery

Marchiafava-Bignami Disease with Widespread Lesions and Complete Recovery

Marchiafava-Bignami disease as a cause of visual hallucinations

Marchiafava-Bignami Disease: Uncertain MRI Predictors of Outcome

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