Marchiafava-Bignami disease is an uncommon condition that causes demyelination and necrosis of the corpus callosum. The nearby subcortical white matter may also be affected.
Marchiafava-Bignami disease should be considered in patients with persistent alcohol dependence or malnutrition who present with neurological symptoms, even though clinical characteristics might be highly diverse and vague.
Dementia altered mental state, dysarthria, spasticity, ataxia, seizures, and gait problems are the symptoms. Other clinical findings include bilateral symmetric movement desynchronization and unilateral symptoms of anomia, dyspraxia, apraxia, and agraphia.
Epidemiology
It is most typically found in malnourished individuals with chronic alcoholism. However, incidents have been reported in people with no alcohol use disorder, particularly those with poorly managed diabetes mellitus.
This disease has no ethnic, racial, or regional predisposition. However, men have a greater prevalence than women, most likely due to a stronger relationship with the consumption of alcohol. The average onset age is 45 years.
Anatomy
Pathophysiology
Depending on the quantity and frequency of consumption, ethanol is a significant risk factor for various brain problems.
Alcohol-related hypovitaminosis, primarily B1 and oxidative stress, can directly damage the corpus callosum, resulting in an initial phase of cytotoxic edema, blood-brain barrier breakdown, and a later stage of necrosis localized demyelination, which results in atrophy.
High myelin concentration indicates corpus callosum damage, the primary white matter commissure that links both hemispheres and allows the interchange of motor, cognitive, and sensory information.
Etiology
The disease’s etiology is unknown; however, it is believed to be caused by a combined effect of alcohol-induced neurotoxicity and a lack of B-complex vitamins. A manifestation of ketoacidosis caused by diabetes mellitus or alcohol is a sudden fluctuation in blood osmolality known as callosal myelinolysis.
Non-alcoholic malnutrition following gastric bypass surgery. Other non-alcoholic illnesses linked to it include sepsis, carbon monoxide overdose, sickle cell disease, cardiac cancer surgery, and cerebral malaria.
Genetics
Prognostic Factors
The prognosis of the disease varies. A patient may live for years with symptoms, recover completely, or deteriorate into a vegetative condition and die. It is theorized that partial lesions that spare the superior commissure fibers have a better prognosis than lesions that extend into the convolution white matter.
Cerebral lobe dysfunction, extracallosal lesions, significant disruption of consciousness, and high alcohol usage are all linked to a poor prognosis or severe dementia. Early diagnosis and effective management are thus critical to the patient’s recovery, and serial MRI has shown some examples of lesions disappearing with early identification and therapy.
Clinical History
Physical Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
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by Modality
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References
https://www.ncbi.nlm.nih.gov/books/NBK526007/
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Marchiafava-Bignami disease is an uncommon condition that causes demyelination and necrosis of the corpus callosum. The nearby subcortical white matter may also be affected.
Marchiafava-Bignami disease should be considered in patients with persistent alcohol dependence or malnutrition who present with neurological symptoms, even though clinical characteristics might be highly diverse and vague.
Dementia altered mental state, dysarthria, spasticity, ataxia, seizures, and gait problems are the symptoms. Other clinical findings include bilateral symmetric movement desynchronization and unilateral symptoms of anomia, dyspraxia, apraxia, and agraphia.
It is most typically found in malnourished individuals with chronic alcoholism. However, incidents have been reported in people with no alcohol use disorder, particularly those with poorly managed diabetes mellitus.
This disease has no ethnic, racial, or regional predisposition. However, men have a greater prevalence than women, most likely due to a stronger relationship with the consumption of alcohol. The average onset age is 45 years.
Depending on the quantity and frequency of consumption, ethanol is a significant risk factor for various brain problems.
Alcohol-related hypovitaminosis, primarily B1 and oxidative stress, can directly damage the corpus callosum, resulting in an initial phase of cytotoxic edema, blood-brain barrier breakdown, and a later stage of necrosis localized demyelination, which results in atrophy.
High myelin concentration indicates corpus callosum damage, the primary white matter commissure that links both hemispheres and allows the interchange of motor, cognitive, and sensory information.
The disease’s etiology is unknown; however, it is believed to be caused by a combined effect of alcohol-induced neurotoxicity and a lack of B-complex vitamins. A manifestation of ketoacidosis caused by diabetes mellitus or alcohol is a sudden fluctuation in blood osmolality known as callosal myelinolysis.
Non-alcoholic malnutrition following gastric bypass surgery. Other non-alcoholic illnesses linked to it include sepsis, carbon monoxide overdose, sickle cell disease, cardiac cancer surgery, and cerebral malaria.
The prognosis of the disease varies. A patient may live for years with symptoms, recover completely, or deteriorate into a vegetative condition and die. It is theorized that partial lesions that spare the superior commissure fibers have a better prognosis than lesions that extend into the convolution white matter.
Cerebral lobe dysfunction, extracallosal lesions, significant disruption of consciousness, and high alcohol usage are all linked to a poor prognosis or severe dementia. Early diagnosis and effective management are thus critical to the patient’s recovery, and serial MRI has shown some examples of lesions disappearing with early identification and therapy.
https://www.ncbi.nlm.nih.gov/books/NBK526007/
medtigo
Marchiafava-Bignami Disease
Updated :
October 25, 2022
Marchiafava-Bignami disease is an uncommon condition that causes demyelination and necrosis of the corpus callosum. The nearby subcortical white matter may also be affected.
Marchiafava-Bignami disease should be considered in patients with persistent alcohol dependence or malnutrition who present with neurological symptoms, even though clinical characteristics might be highly diverse and vague.
Dementia altered mental state, dysarthria, spasticity, ataxia, seizures, and gait problems are the symptoms. Other clinical findings include bilateral symmetric movement desynchronization and unilateral symptoms of anomia, dyspraxia, apraxia, and agraphia.
It is most typically found in malnourished individuals with chronic alcoholism. However, incidents have been reported in people with no alcohol use disorder, particularly those with poorly managed diabetes mellitus.
This disease has no ethnic, racial, or regional predisposition. However, men have a greater prevalence than women, most likely due to a stronger relationship with the consumption of alcohol. The average onset age is 45 years.
Depending on the quantity and frequency of consumption, ethanol is a significant risk factor for various brain problems.
Alcohol-related hypovitaminosis, primarily B1 and oxidative stress, can directly damage the corpus callosum, resulting in an initial phase of cytotoxic edema, blood-brain barrier breakdown, and a later stage of necrosis localized demyelination, which results in atrophy.
High myelin concentration indicates corpus callosum damage, the primary white matter commissure that links both hemispheres and allows the interchange of motor, cognitive, and sensory information.
The disease’s etiology is unknown; however, it is believed to be caused by a combined effect of alcohol-induced neurotoxicity and a lack of B-complex vitamins. A manifestation of ketoacidosis caused by diabetes mellitus or alcohol is a sudden fluctuation in blood osmolality known as callosal myelinolysis.
Non-alcoholic malnutrition following gastric bypass surgery. Other non-alcoholic illnesses linked to it include sepsis, carbon monoxide overdose, sickle cell disease, cardiac cancer surgery, and cerebral malaria.
The prognosis of the disease varies. A patient may live for years with symptoms, recover completely, or deteriorate into a vegetative condition and die. It is theorized that partial lesions that spare the superior commissure fibers have a better prognosis than lesions that extend into the convolution white matter.
Cerebral lobe dysfunction, extracallosal lesions, significant disruption of consciousness, and high alcohol usage are all linked to a poor prognosis or severe dementia. Early diagnosis and effective management are thus critical to the patient’s recovery, and serial MRI has shown some examples of lesions disappearing with early identification and therapy.
https://www.ncbi.nlm.nih.gov/books/NBK526007/
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