A non-alcoholic Japanese patient with Wernicke's encephalopathy and Marchiafava-Bignami disease

In Japan Wernicke's encephalopathy is not rare, but only one case of Marchiafava-Bignami disease has been reported. In our case, Wernicke lesions were found in the mamillary bodies, septal nuclei, and periventricular regions. Involvement of the septal nuclei is rare in this encephalopathy. A characteristic Marchiafava-Bignami lesion was observed only in the anterior part of the corpus callosum. From the clinicopathologic viewpoint, Wernicke's encephalopathy might have preceded Marchiafava-Bignami disease in our case. To our knowledge, only two cases without a history of alcoholism and both suffering from Wernicke's encephalopathy and Marchiafava-Bignami disease have been documented. However, in both cases Wernicke lesions were atypical, since characteristic lesions were absent in the mamillary bodies and in the regions surrounding the third ventricle and the aqueduct. Ours appears to be the first report on a non-alcoholic patient with typical lesions of both diseases.     

Marchiafava-Bignami disease with favorable development

A 35 year-old right-handed woman with a history of alcoholism presented signs of interhemispheric disconnection. CT of the brain revealed a low-density areas in the corpus callosum. Two months later, clinical examination was normal, but the CT showed cystic area in the genu and splenium. MRI was also consistent with a lesion of the corpus callosum. CT and MRI did not show lesions in the hemispheric white matter.     

MRI in non-alcoholic Wernicke's encephalopathy

Wernicke's encephalopathy (WE) is a potentially reversible disorder. It is often not considered in non-alcoholic patients unless MRI demonstrates lesions in the appropriate sites. However, specific MRI sequences only highlight some areas of abnormality and hence WE may not be considered unless a more complete study is performed and this is highlighted in the case described herein. The neuropathological basis for the imaging findings is also discussed.     

X-ray scanning in Marchiafava-Bignami disease

CT scan in 4 patients with Marchiafava-Bignami's disease in its acute alcoholic form, who died after 12, 18, 30 and 50 days respectively, demonstrated large symmetrical bilateral hypodense areas in the hemispheric white substance. These areas were either widespread or more marked posteriorly, and were independent of the corpus callosum. Pathologic examination of the brains showed necrotic regions, often with cavities, or areas of demyelinization of the centrum ovale correlating with the hypodense areas as seen on CT scans. In two cases CT scan further showed regions of linear hypodensity in front of and medial to the frontal ventricular horns which could have corresponded to the corpus callosum necrosis found on the brain sections. CT scan can thus contribute to the diagnosis of Marchiafava-Bignami's disease.     

Marchiafava-Bignami病1例报告

Marchiafava-Bignami病(MBD)是一种罕见的大脑白质脱髓鞘性疾病,以胼胝体受累为突出表现,是导致胼胝体变性、渐进性脱髓鞘和坏死的中毒性疾病,大多见于慢性酒精中毒的患者.     

Marchiafava-Bignami病的临床及影像学研究

目的 探讨Marchiafava-Bignami病(MBD)的临床及影像学改变.方法 回顾性分析了7例MBD患者的临床和CT、MRI资料,包括病灶形态、分布、信号或密度改变等影像学特征:4例同时行CT和MRI检查,2例仅行CT检查,1例仅行MRJ检查.结果 本组患者急性型5例,均表现为胼胝体肿胀及长T1、长T2信号改变,均有双侧脑室周围白质、额叶皮层下白质对称性累及:慢性型2例,胼胝体明显萎缩变薄,并呈长T1、长T2信号及FLAIR像点片状或线样低信号灶.5例患者DWI显示病灶区信号明显增高并有2例出现弥散受限改变.结论 MBD具有特征性MRJ表现,其影像学改变可能反映其临床及预后.     

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.     

Transient encephalopathy with reversible white matter lesions in children

We report on two children with acute encephalopathy showing mild clinical manifestations and reversible white matter lesions. In both patients, MRI revealed high intensities on T (2)-weighted imaging and marked reductions of water diffusion in the white matter of the bilateral centrum semiovale and the corpus callosum. These abnormalities disappeared along with the neurological symptoms within a week in both patients. These children represent a characteristic group of patients among childhood acute encephalopathy.     

Transient encephalopathy with reversible white matter lesions: a case report

We report on a boy with bilateral optic nerve hypoplasia and mild psychomotor retardation. At 1 year and 9 months of age, he was admitted to hospital with a cluster of febrile convulsions and unconsciousness. Magnetic resonance imaging (MRI) revealed widespread areas of high signal intensity on diffusion-weighted imaging of the deep cerebral white matter and corpus callosum. This imaging disappeared at five days of illness. No atrophy or abnormalities were noted on the 6-month follow-up MRI. Despite full recovery after the acute episode, the patient showed retarded developmental progress. We discuss the differential diagnosis for this case.     

Hemispheric disconnection syndrome with a "crossed avoiding" reaction in a case of Marchiafava-Bignami disease.

A clinicopathological study is presented of a case of Marchiafava-Bignami disease with a hemispheric disconnection syndrome, an association that does not appear to have been reported previously. Gross and microscopic examination of the brain revealed necrosis of the corpus callosum (sparing a small area in front of the splenium) and of the anterior commissure, cortical and subcortical infarction of the right lingual gyrus, diffuse cortical lesions of the laminar sclerosis type, and lacunae in the basal ganglia and the pons. The patient was unable to grasp objects presented to the right visual half-field with the left hand, or to respond to contralateral somaesthetic stimuli with either of the upper limbs. This motor inhibition, with the associated extended posture, is described as a "crossed avoiding reaction", and attributed to the inability of one hemisphere to respond to visual or somaesthetic stimuli projected to the other hemisphere. Clinicopathological correlations and visuomotor coordination mechanisms are discussed in the light of previous clinical and experimental studies. Anomia to pictures projected tachistoscopically to the left visual field, disturbances in the transfer of somaesthetic information, left sided ideomotor apraxia with agraphia, right sided dyscopia, and ideational apraxia especially marked in the right visual field were observed.     

A patient with Marchiafava-Bignami disease as a complication of diabetes mellitus treated effectively with cortico steroid

We report a 37-year-old woman with no history of alcohol consumption or malnutrition who had Marchiafava-Bignami disease (MBD) as a complication of diabetes mellitus. The patient suddenly developed dizziness and could not speak words fluently. Neurological examination revealed acalculia, agraphia, left blepharoptosis, and mild left facial palsy. Her blood glucose was 391 mg/dL, and her glycated hemoglobin (HbA1c) was 16.0%. Her brain MRI revealed hyperintense changes in the corpus callosum on T2-weighted, fluid-attenuated inversion recovery and diffusion-weighted images. In addition to therapy for diabetes mellitus, prednisolone was commenced. All of the symptoms gradually improved, and after three months, only slight dysarthria, acalculia, and agraphia were observed. Brain MRI also revealed a reduction in lesion size. In conclusion, MBD may occur even with metabolic disorders. It is important to diagnose MBD in the early stages with MRI and to treat the symptoms with cortico steroids.     

Rheumatoid disease with encephalopathy

A 63-year-old woman developed progressive rheumatoid disease complicated by a confusional state as well as persistent cerebrospinal fluid pleocytosis and hypoglycorrhachia. Neuropathological examination revealed extensive rheumatoid lesions in the cranial dura, falx, and choroid plexus. The choroid plexus has not been affected in any of the 16 previously reported cases of pathologically verified rheumatoid disease of the central nervous system. The findings in this patient support the observations of others regarding the role of the choroid plexus in development of CNS manifestations in systemic immune complex disease.