Marchiafava-Bignami disease (MBD) is a rare CNS disorder usually seen in the context of alcoholism and malnutrition. The condition classically involves the. Marchiafava-Bignami disease is a rare disorder characterized by demyelination and necrosis of the corpus callosum, commonly seen in. Images in Clinical Medicine from The New England Journal of Medicine — Marchiafava–Bignami Disease.
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Alcohol consumption should be stopped. Some patients survive, but with residual brain damage and dementia. Others remain in comas that eventually lead to death. Nutritional counseling is also recommended.
It is believed that he had protein, folic acid, and thiamine deficiencies, which are what caused the demyelination of the marchiafava bignami callosum.
The patient was diagnosed through MRI, but countless other neurological diseases needed to be ruled out initially. He was diagnosed with liver cirrhosis. He was confused and had a lack of motor coordination.
He also had altered sensorium and seizures.
Marchiafava-Bignami Disease: Uncertain MRI Predictors of Outcome
A Diffusion-weighted images show hyperintensity of the corpus callosum; B Apparent diffusion coefficient images show a corresponding less prominent marchiafava bignami in the marchiafava bignami. FLAIR coronal and axial images show frontal lesion as hyper-intense areas. A more detailed neuropsychological evaluation was impossible because of the patient inability to understand the tasks and to maintain an adequate attention.
On the basis of the above-mentioned features, a diagnosis of Marchiafava-Bignami disease was made.
Because of the history of alcohol abuse, a diagnosis of Wernicke Encephalopathy was initially considered but excluded on the basis of clinical and MRI findings. The same applied to the hypothesis of an alcohol withdrawal syndrome. Similarly, other diseases marchiafava bignami the corpus marchiafava bignami, such as multiple sclerosisencephalitislymphoma, infarction or solid tumors were not substantiated by the history, clinical features and clinical findings.
Marchiafava–Bignami disease - Wikipedia
We did not marchiafava bignami steroid therapy. Three days after hospitalization the patient was started on a physical therapy program. The program started with Early Active Mobilization Protocol; gradually the rehabilitation program included also the Frenkel and Rhythmic Stabilization Exercises.
Seven to ten days after admission, we noticed a progressive improvement of the ideomotor performance. The patient was able to walk slowly without support. He was more ready to interact with the medical and nursing staff. He continued being disoriented in space and time, but there were no clear signs of hemispheric disconnection.
Two weeks later admission, the marchiafava bignami underwent to brain TC as a control, without significant changes. After 25 days in the neurological ward, his conditions were such to allow him to be discharged, in the context of a rehabilitative program.
At the release, the neurocognitive performance were slight better, although a complete evaluation was not possible MMSE: Six month later his clinical conditions were stable: He lived in a nursing home, depending on the activities of daily living; his relatives rejected additional follow up investigations.
Discussion and Conclusion MBD is characterized by a degenerative lesion of the corpus callosum, probably related to deficiency of nutritional factors [ 3 ].
It is considered a form of toxic demyelination seen in alcoholic patients, especially males between 40 and 60 years of age. MBD was observed in persons of all ethnic groups and drinking any type of alcoholic beverage, and even in rare nonalcoholic patients [ 45 ].
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 [ 67 ].
Neuroimaging can help in diagnosis and staging of MBD. In the acute phase, related to cellular damage and cytotoxic edema, MRI lesions are marchiafava bignami across the corpus callosum body and genu, then spleniumhypointense in T1 and hyperintense in T2 sequences, with restricted diffusion in DWI without a mass effect and rarely they may have contrast enhancement.
Occasionally, similar lesions can involve other areas, such as anterior and posterior commissures, brachium pontis [ marchiafava bignami ], optic chiasm, putamen [ 9 ], and frontal cortex [ 110 ].