Marchiafava-Bignami disease (MBD) is a rare toxic-based demyelinating disease that affects the central nervous system. It is often associated with malnutrition and alcoholism. Clinically, it can manifest with pleomorphic pictures in which; however, neurological symptoms such as altered consciousness, dementia, dysarthria, pyramidal tract signs, seizures, ataxia, and signs of interhemispheric disconnection predominate. This pathology must be differentiated from Wernicke’s encephalopathy, multiple sclerosis, encephalitis, infectious leukoencephalopathy, paraneoplastic syndromes, cerebral stroke and dementias, both degenerative (Alzheimer’s disease, Pick’s fronto-temporal dementia), and secondary multiinfarct type. The diagnosis of MBD is performed with radiological criteria and depends on the MRI results of the hyperintensity of the corpus callosum in T2 sequences and T2 with fluid-attenuated inversion recovery, with or without extracallous lesions. Two clinical-radiological subtypes of MBD have been described, distinguishing between acute and chronic onset: type A and type B, with significant differences regarding the clinical course and prognosis. Type A is characterized by hyperintense T2 swelling of the entire corpus callosum on initial MRI; type B shows partial calloused lesions on MRI. The use of magnetic resonance imaging in diagnosis allowed early administration of parenteral treatment with thiamine and improved the prognosis of MBD from often fatal to less than 8% mortality. Administration of thiamine, within 14 days of symptom onset, has been shown to have statistically better results than delayed treatment. We present a clinical case of MBD diagnosed in a 74-year-old woman, who presented in a state of stupor-coma, following a hospitalization in Emergency Medicine for urinary tract infection. In this particular case, the use of MRI was able to discriminate MBD from hypokinetic delirium, which is very common in the population of elderly hospitalized for infectious status. Early diagnosis also allowed a rapid improvement in the patient’s condition which, if left undiagnosed, would have led inexorably to exitus.
Marchiafava-Bignami or delirium ipoactive: a case report presentation
Fraccalini T.;Minniti D.;Maina G.;
2025-01-01
Abstract
Marchiafava-Bignami disease (MBD) is a rare toxic-based demyelinating disease that affects the central nervous system. It is often associated with malnutrition and alcoholism. Clinically, it can manifest with pleomorphic pictures in which; however, neurological symptoms such as altered consciousness, dementia, dysarthria, pyramidal tract signs, seizures, ataxia, and signs of interhemispheric disconnection predominate. This pathology must be differentiated from Wernicke’s encephalopathy, multiple sclerosis, encephalitis, infectious leukoencephalopathy, paraneoplastic syndromes, cerebral stroke and dementias, both degenerative (Alzheimer’s disease, Pick’s fronto-temporal dementia), and secondary multiinfarct type. The diagnosis of MBD is performed with radiological criteria and depends on the MRI results of the hyperintensity of the corpus callosum in T2 sequences and T2 with fluid-attenuated inversion recovery, with or without extracallous lesions. Two clinical-radiological subtypes of MBD have been described, distinguishing between acute and chronic onset: type A and type B, with significant differences regarding the clinical course and prognosis. Type A is characterized by hyperintense T2 swelling of the entire corpus callosum on initial MRI; type B shows partial calloused lesions on MRI. The use of magnetic resonance imaging in diagnosis allowed early administration of parenteral treatment with thiamine and improved the prognosis of MBD from often fatal to less than 8% mortality. Administration of thiamine, within 14 days of symptom onset, has been shown to have statistically better results than delayed treatment. We present a clinical case of MBD diagnosed in a 74-year-old woman, who presented in a state of stupor-coma, following a hospitalization in Emergency Medicine for urinary tract infection. In this particular case, the use of MRI was able to discriminate MBD from hypokinetic delirium, which is very common in the population of elderly hospitalized for infectious status. Early diagnosis also allowed a rapid improvement in the patient’s condition which, if left undiagnosed, would have led inexorably to exitus.
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