Clinico-radiologic subtypes and therapeutic observation of acute Marchiafava-Bignami disease.

We aimed to investigate the clinico-radiologic features of acute Marchiafava-Bignami disease (MBD) and its evolutionary process after effective treatment through subgroup comparison. The clinical and MRI data of 23 patients with acute MBD were retrospectively analyzed and divided into type A (12 cases, with entire callosal involvement) and type B (11 cases, with focal callosal involvement). The clinical assessments and MRI findings (before and after treatment) were compared between the two subtypes. Compared with type B, type A had lower MoCA (Montreal Cognitive Assessment) scores at admission (16.50 ± 1.73 vs 18.27 ± 1.68, P = 0.021) and were more common with extracallosal involvement (66.67% vs 18.18%, P = 0.036) and longer illness duration (18.3 ± 2.1 days vs 15.6 ± 2.4 days, P = 0.012). During the treatment, the residual lesion in the splenium was more common in type A (58.33% vs 9.09%, P = 0.027). After treatment, the MoCa scores of both subtypes gradually increased (P < 0.001), and the callosal and extracallasal lesions disappeared completely. Clinico-radiologic typing of acute MBD is related to the severity of early symptoms, but not to the prognosis. Complete clinico-radiologic recovery is possible for both subtypes with combined treatment. The clinico-radiologic reversibility is helpful for accurate diagnosis and therapeutic evaluation.

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

ABSTRACT: 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.

Neuroimaging of Multiple Sclerosis Mimics.

Multiple sclerosis (MS) is the most common immune-mediated disease of the central nervous system, characterized by demyelinating lesions of the brain and the spinal cord. Although it is extremely important to diagnose this condition in a timely manner, to initiate and monitor treatment to prevent permanent neurologic damage and disability, it is also necessary that other demyelinating conditions collectively referred to as MS mimics be identified and excluded. This article describes the in-depth neuroimaging characteristics and morphology of the pathologic lesions on the various neuroimaging modalities.

Antemortem histopathology and imaging findings in a case of Marchiafava-Bignami disease.

Marchiafava-Bignami disease (MBD) is a rare condition often associated with chronic alcohol abuse. Clinical presentation is diverse. Characteristic magnetic resonance imaging (MRI) changes in the corpus callosum are the mainstay of radiological diagnosis. We present a case of a 54-year-old man with chronic alcoholism and peripherally enhancing lesion in the body of the corpus callosum on MRI Brain. Open biopsy of the lesion showed necrosis and demyelination. He was diagnosed with Marchiafava-Bignami disease based on clinical, radiology and histopathology findings. Our case represents the only case in the literature with antemortem histopathology findings describing MBD.

Unilateral Apraxic Agraphia without Ideomotor Apraxia from a callosal lesion in a patient with Marchiafava-Bignami disease.

Apraxic agraphia can be caused by left hemispheric cerebral lesions in the area that contains the spatial representations of the movements required to write, from a lesion in, or connections to, the frontal premotor cortex that converts these spatial representations to motor programs (Exner's area).  A right-handed woman with Marchiafava Bignami disease and lesions of the genu and splenium of her corpus callosum had apraxic agraphia without ideomotor apraxia of her left. A disconnection of Exner's area in the left hemisphere from the right hemisphere's premotor and motor areas may have led to her inability to write with her left hand.

Demyelinating diseases as a result of cerebral edema?

Due to the elastic properties of the human organs, tissue edema causes an increased tissue pressure. This phenomenon leads to a reduction of blood circulation or ischemia, and thus leads to the hypothesis that tissue edema can be the cause of demyelinating lesions. Even though brain edema occurs in the whole brain, the authors assume that the characteristically focal appearance of demyelinated lesions, for instance of multiple sclerosis plaques, are attributable to anatomical and structural characteristics of the brain. In an experimental section, a balloon inserted into the brain and other organs removed during autopsies produces an increased tissue pressure. This model shows tissue pressure in the vicinity of the balloon up to 80mmHg. The height of the produced pressure varies in different organs and special regions of the brain. The verified pressures in the pons cerebri show that stretched myelinated fiber bundles in outer regions can induce strong pressures in enclosed edematous tissue, as seen in central pontine myelinolysis. The presented experimental results support the hypothesis that demyelinated lesions, as seen in multiple sclerosis, may be caused by increased tissue pressure, or respectively, brain edema.

Marchiafava-Bignami Disease with frontal cortex involvement and late onset, long-lasting psychiatric symptoms: a case report.

AIMS: To describe the case and management of a patient with Marchiafava-Bignami Disease (MBD) with frontal cortical lesions, no specific symptom at first referral to the Emergency Room, and late onset of atypical psychiatric symptoms. METHODS: We report the case of a 44-year-old patient with a history of chronic alcohol abuse, eventually diagnosed with MBD. RESULTS: Magnetic Resonance showed lesions in the splenium and the body of corpus callosum and bilateral lesions of the frontal cortex. The patient showed late-onset atypical psychiatric symptoms which were drug resistant. DISCUSSION: The case we describe seems to support the existing few ones describing cortical involvement in MBD, which suggest that this is associated with a poorer prognosis. Psychiatric symptoms may be challenging to treat because of drug resistance. CONCLUSIONS: The involvement of psychiatrists together with neurologists and radiologists, with a consultation-liaison approach proved important for the achievement of diagnosis and of the most appropriate management and treatment for this patient.

[Diagnostic imaging of marchiafava-bignami disease].

Marchiafava-Bignami disease (MBD) is a rare alchol related disorder characterized by demyelination of the corpus callosum. Clinical features include impaired consciousness, seizure, dysarthria, limb hypertonus, frontal lobe symptoms in the acute stage and interhemispheric disconnection syndromes in the chronic stage. While autopsy was historically necessary for a definitive diagnosis of MBD, imaging systems such as X-ray computed tomography and magnetic resonance imaging have enabled in vivo diagnosis. We reviewed 91 reported MBD cases additional to our 9 own cases focusing on their imaging. The corpus callosum in MBD has irreversible necrosis secondary to edema and demyelination, often complicated by bleeding in the subacute stage and it becomes atrophic in the chronic stage. The observed patterns of corpus callosal lesions were highly variable, sometimes complicated by extra-callosal lesions in the frontal cortex, white matter, and putamen. Moreover, these lesions are sometimes detected in non-alcoholic patients. Although the underlying causes of MBD are still unknown, investigating its imaging characteristics together with clinical and pathological features will contribute to elucidating its etiology. Furthermore, MBD as an alcohol related disease is linked with neurological emergency presentation. Thus, early diagnosis and treatment are essential.

[Marchiafava-Bignami disease]. / Enfermedad de Marchiafava-Bignami.

TITLE: Enfermedad de Marchiafava-Bignami.

Diagnosis and management of Marchiafava-Bignami disease: a review of CT/MRI confirmed cases.

OBJECTIVE: Marchiafava-Bignami disease (MBD) is a rare condition mainly associated with alcoholism, although it may be mimicked by several other disorders that cause corpus callosum lesions. Our objective was to identify helpful features for differential diagnosis and assess whether any treatment can be recommended. METHODS: We reviewed 122 reports containing data on 153 subjects with confirmed MBD that was associated with either alcoholism or malnutrition, and 20 reports with data on 53 subjects with conditions mimicking MBD. All the cases had been verified antemortem by brain imaging. Unconditional logistic regression was used to demonstrate factors that were associated with the outcome of MBD. RESULTS: The mimicking conditions were differentiated from MBD by the occurrence of solitary and rapidly disappearing splenial lesions; fewer signs and symptoms with exception of seizures, hemiparesis and tetraparesis; nystagmus; and rapid and complete recovery. MBD occurred most frequently among alcoholics, but it was also reported in 11 non-alcoholics (7.2% of all the MBD cases). A better outcome was observed among those who were treated within 2 weeks after onset of symptoms with parenteral thiamine (p=0.033). CONCLUSIONS: As thiamine deficiency is frequently associated with alcoholism, malnutrition and prolonged vomiting; we recommend prompt treatment of MBD with parenteral thiamine in such subjects. Recovery should be followed by repeated neuropsychological and MRI examinations, preferably using diffusion tensor imaging.

Acute Marchiafava-Bignami disease with extensive diffusion restriction and early recovery: case report and review of the literature.

Marchiafava-Bignami disease (MBD) is a neurological disorder that has been found to be associated with chronic alcoholism and malnutrition. MBD classically results in acute edema and demyelination of the corpus callosum. Edema of the complete corpus callosum has been described to be an unfavorable prognostic factor. We present an acute onset of MBD with diffusion restriction of the complete corpus callosum and symmetric bilateral extension into the semioval center, that almost completely resolved clinically as well as in MRI only 3 days later. With early detection and treatment, the prognosis of MBD may be good even in cases with severe diffusion restriction of the complete corpus callosum.

"Ears of the lynx" sign in a marchiafava-bignami patient: structural basis and fiber-tracking DTI contribution to the understanding of this imaging abnormality.

The "ears of the lynx" sign was previously reported as a neuroimaging finding observed in patients with autosomal recessive hereditary spastic paraplegia in association with a thin corpus callosum (ARHSP-TCC). We report a patient with a chronic form of Marchiafava-Bignami disease (MBD) that presented with this imaging feature. Diffusion tensor imaging (DTI) and fiber-tracking data support that this finding is a consequence of the structural derangement, which enlarges a preexisting border zone of the bundles of fibers from the corpus callosum (CC) genu to the forceps minor and anterior corona radiata. Therefore, we assume that despite their pathological differences, damage to the anterior portion of the CC is responsible for the imaging similarities between MBD and ARHSP-TCC.

[Relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease].

Marchiafava-Bignami disease is a rare alcohol-associated disorder. Clinical features include not only disturbed consciousness, dysarthria, tetraparesis, and astasia-abasia as initial symptom but also cognitive deficits and symptoms of interhemispheric disconnection as clinical outcomes. The clinical significance of cerebral microhemorrhage has been recognized in patients with cognitive deficits. We have recently examined the clinical significance of cerebral microhemorrhage in Marchiafava-Bignami disease and demonstrated that demented patients showed higher severity of cerebral microhemorrhage than patients with normal cognitive function. However, the relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease has not been fully examined. The aim of the present study was to clarify the relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease. For this purpose, we report four patients with Marchiafava-Bignami disease. All cases had a history of chronic alcohol abuse and symmetrical lesions in the corpus callosum. Clinical symptoms include not only coma, dysarthria, and astasia-abasia as initial symptom but also dementia as clinical outcomes. Susceptibility-weighted imaging showed asymmetrical hypointense areas in the multiple cortico-subcortical regions, indicating the presence of cerebral microhemorrhage. There were no apparent relationships between the extension of callosal lesion and the severity of cognitive deficits or cerebral microhemorrhage. Our present report indicates that cerebral microhemorrhage, an important. factor for the severity of dementia in Marchiafava-Bignami disease as clinical outcomes, is independent of the callosal lesion.