Marchiafava Bignami disease is a rare neurologic condition that usually appears in chronic alcohol abusers, but has also been described in association with gynecologic malignancies as a paraneoplastic syndrome, consisting of corpus callosum demyelination and necrosis.
Marchiafava Bignami disease usually affects men who are chronic alcoholics and are middle- aged or elderly , prefer to consume hard liquor and are severely malnourished with vitamin B complex deficiency . Symptoms may commence in an abrupt or progressive manner. Some present with psychiatric disturbances like dementia, others with more or less severe neurologic impairment, like gait disturbances, seizures, hemiparesis or tetraparesis, limb spasticity, tremor or weakness. Aphasia and ideomotor apraxia, although less frequent, seem to be more specific . Diagnosis is complicated by the fact that the patients may also have alcoholic hepatitis or Wernicke-Korsakoff syndrome and can become even more difficult when the patient is experiencing delirium tremens, is in a coma or even lethargic, as some neurologic tests cannot be performed. In such cases, the interhemispheric disconnection, also called "split brain syndrome"  caused by corpus callosum demyelination is difficult to prove. The disconnection of the hemispheres may cause agraphia of the dominant hand  or hemianopia . However, any patient presenting with Wernicke-Korsakoff syndrome should be suspected to have Marchiafava Bignami disease.
The neurologic examination often reveals further abnormalities: dysarthria, mutism, nystagmus, gaze palsy, diplopia, peripheral sensory loss, deep tendon reflex loss or Babinski sign or even the presence of primitive reflexes. Mental state impairment may not be as severe and may consist of confusion, disorientation or memory loss and inability to memorize new information.
Jaw & Teeth
Neither tongue atrophy nor fasciculation were found. Bilateral upper and lower limb weakness with increased bilateral upper limb reflexes and Babinski reflexes were found. [ncbi.nlm.nih.gov]
Neither tongue atrophy nor fasciculation were found. The tongue could be protruded from the mouth and remained midline, and moved adequately from side to side. [bmcneurol.biomedcentral.com]
Muscle rigidity and facial trismus may be severe. Most patients presenting with the acute type of MB will go into coma and eventually die, although as illustrated in this report, some survive. [ajnr.org]
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 [ncbi.nlm.nih.gov]
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 [dx.doi.org]
Patients with Wernicke encephalopathy have ataxia, ophthalmoplegia, nystagmus, and confusion. Subacute form of MB is characterized by dementia, disarthria, and muscle hypertonia—may survive for years. [neuroradiologycases.com]
Gaze paretic nystagmus and finger-to-nose incoordination were also improved. [bmcneurol.biomedcentral.com]
Altered Mental Status
Marchiafava-Bignami disease, a rare alcohol-related disorder, characterized by altered mental status, seizure, and multifocal central nervous system signs, which results from progressive demyelination and necrosis of corpus callosum. [ncbi.nlm.nih.gov]
Case Report: A 36-year-old man was found with altered mental status on a street. The past medical history could not be obtained, but he appeared to be a chronic alcoholic with significant malnutrition. [e-jnc.org]
Case Description: Patient is a 30 year old left handed African-American with history of hypertension, diabetes type I, hypothyroidism, alcohol use disorder, who presented with c/o altered mental status, urinary incontinence, slurred speech and left-sided [omicsonline.org]
Case 2 A 65-year-old man known to be a chronic alcoholic and to have hepatic cirrhosis presented with an acute onset of altered mental status, seizures, and Parkinson-like movements. [ajnr.org]
"Crossed left hemispatial neglect" was not seen with the left hand, but neglect of the left hemifield was seen with the right hand. CT and MRI showed a lesion occupying almost the entire corpus callosum. [ncbi.nlm.nih.gov]
Clinical evidence of left hemispatial neglect remained 8 months later, with neuropsychological tests showing few changes. [elsevier.es]
Finally, no visual neglect was shown by the Albert line-crossing test performed with both hands. Figure 1. Top, Handwriting specimens (left hand, left; right hand, right). [jamanetwork.com]
Moriya, H. and Hirayama, K. (1993) “Crossed homonymous hemianopia” and “crossed left hemispatial neglect” in a case of MarchiafavaBignami disease. [scirp.org]
There were no lateralizing deficits and no signs of meningeal irritation. Laboratory tests showed a normal haemogram, liver and renal function tests and serum electrolytes. [indianjpsychiatry.org]
Normal serum electrolytes, absence of signs of meningeal irritation and normal CSF examination ruled out delirium due to electrolyte imbalance or meningo-encephalitis. [tropicalgastro.com]
Signs of meningeal irritation including neck rigidity and kernig sign were absent. Pupils were of normal size and normal reacting. Motor examination showed no neurological deficit. Laboratory results revealed normal haematological profile. [jcdr.net]
Coping Si Tratament.Alcohol depresses nerves that control involuntary actions such as breathing and the gag reflex, Abuse and Alcoholism (NIAAA A Word About Alcohol Poisoning.Alcohol poisoning can kill. irritate the stomach which causes vomiting and it [vygepulo.4fan.cz]
On admission, his Glasgow coma score was 7, and he showed no signs of meningeal irritation, focal deficits or cranial nerve abnormalities. IV thiamine (500 mg/day) and high doses of parenteral B vitamins were administered. [scielo.br]
Pyramidal Tract Signs
Type A: acute to subacute onset of consciousness impairment, pyramidal tract signs, limb hypertonia, seizures, hyperintense swelling of the corpus callosum on T2-weighted MR sequences and is associated with poor prognosis. [jcdr.net]
As soon as the patient is identified as a chronic alcohol abuser, the physician should order serum glucose, serum electrolytes, and toxicology screening. Blood workup should also include a complete cell blood count. If the individual presents with acute neurologic symptoms like seizures or coma, a cerebral mass or stroke must be urgently excluded using computer tomography or magnetic resonance imaging scans. The latter is considered the most useful diagnostic tool for this condition   and is able to describe myelin and neuron loss in the corpus callosum, as well as cyst formation. Corpus callosum initially becomes edematous, followed by the genu and the splenium . The edema is initially vasogenic and then converts to cytotoxic . Lesions may initially involve the central areas and spare the ventral and dorsal extremities. This finding is known as " the sandwich sign". When present, cysts are described as hyperintense, whereas myelin damage causes hypointensity. Magnetic resonance imaging may also reveal the presence of associated cortical lesions . The chronic phase involves corpus callosum atrophy.
A novel technique, the fluid-attenuated inversion recovery is also contributory, offering important information when available, such as the extent of myelin damage.
Electroencephalography is uninformative in Marchiafava Bignami disease .
All had a favorable outcome after treatment with thiamine. Only one case had extensive extracallosal lesions and this case also had a good recovery after treatment. Now, MBD is not a fatal disease and early diagnosis and treatment are crucial. [ncbi.nlm.nih.gov]
Early diagnosis and treatment can improve the prognosis of the patient. [dx.doi.org]
Early diagnosis by relatively specific performance of bisymmetric lesions in corpus callosum of diffusion-weighted imaging (DWI) may affect the prognosis. [ncbi.nlm.nih.gov]
Etiology is the deficiency of vitamin B complex. Magnetic resonance imaging (MRI) in MBD typically reveals focal lesions of high T2 and FLAIR signal intensity in the corpus callosum. [ncbi.nlm.nih.gov]
To date, the etiology of MBD is incompletely understood. [dx.doi.org]
So we postulate MBD as the possible etiology of acquired athetosis. The term athetosis, comes from a Greek word meaning “without attachment” or “changing.” [karger.com]
MBD is in most instances seen in chronic alcoholics, and as such the epidemiology reflects this demographic, with middle-aged to elderly male patients (40-60 years of age) being most commonly affected 2. [radiopaedia.org]
PMID 14625216. a b c "Marchiafava-Bignami Disease: Background, Etiology and Pathophysiology, Epidemiology". 2017-07-11. a b Parmanand H. T. Marchiafava–Bignami disease in chronic alcoholic patient. [en.wikipedia.org]
Various magnetic resonance imaging (MRI) modalities were performed sequentially to elucidate the pathophysiology. A 52-year-old man with chronic alcoholism developed acute confusion and dysarthria. [ncbi.nlm.nih.gov]
(See Etiology and Pathophysiology.) In 1903, Italian pathologists Marchiafava and Bignami described 3 alcoholic men who died after having seizures and coma. [emedicine.com]
The appropriate administration of thiamine may prevent poor outcomes. [ncbi.nlm.nih.gov]
Abstract Background: Although Wernicke encephalopathy (WE) is a preventable and treatable disease it still often remains undiagnosed during life. Objectives: To create practical guidelines for diagnosis, management and prevention of the disease. [oadoi.org]
Clinical screening for interhemispheric disconnection syndrome may lead to identify sickle cell/HbSC disease patients who require more aggressive treatment, such as preventive red blood cell transfusions in order to avoid cognitive function decline [ [em-consulte.com]
- Hlaihel C, Gonnaud P, Champin S, et al. Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: follow-up studies. Neuroradiology. 2005;47:520-524.
- Geibprasert S, Gallucci M, Krings T. Alcohol-induced changes in the brain as assessed by MRI and CT. Eur Radiol. 2010;20:1492-1501.
- Hirayama K, Tachibana K, Abe N, et al. Simultaneously cooperative, but serially antagonistic: a neuropsychological study of diagonistic dyspraxia in a case of Marchiafava-Bignami disease. Behav Neurol. 2008;19(3):137-144.
- Berlucchi G. Frontal callosal disconnection syndromes. Cortex. 2012;48:36–45.
- Rosa A, Demiati M, Cartz L, et al. Marchiafava-Bignami disease, syndrome of interhemispheric disconnection, and right-handed agraphia in a left-hander. Arch Neurol. 1991;48:985–988.
- Kamaki M, Kawamura M, Moriya H, et al. Crossed homonymous hemianopia and crossed left hemispatial neglect in a case of Marchiafava-Bignami disease. J Neurol Neurosurg and Psych. 1993;56:1027–1032.
- Boutboul D, Lidove O, Aquilar C, et al. Marchiafava-Bignami disease complicating SC hemoglobin disease and Plasmodium Falciparum infection. Presse Med. 2010;39:990–993.
- Lee S, Kim S, Kim S, et al. Acute Marchiafava-Bignami disease with selective involvement of the precentral cortex and splenium: a serial magnetic resonance imaging study. Neurologist. 2011;17(4):213-217.
- Yoshizaki T, Hashimoto T, Fujimoto K, et al. Evolution of Callosal and Cortical Lesions on MRI in Marchiafava-Bignami Disease. Case Rep Neurol. 2010;2(1):19-23.
- Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami Disease: MR findings in two patients. Am J Neuroradiol. 2003;24:1955–1957.
- Doelken M, Lanz S, Alibek S, et al. Differentiation of cytotoxic and vasogenic edema in a patient with reversible posterior leukoencephalopathy syndrome using diffusion-weighted MRI. Diagn Interv Radiol. 2007;13:125–128.
- Ménégon P, Sibon I, Pachai C, et al. Marchiafava-Bignami disease: Diffusion-weighted MRI in corpus callosum and cortical lesions. Neurology. 2005;65:475–477.
- Fang SC. EEG coherence for a patient with Marchiafava-Bignami disease. Clin EEG Neurosci. 2007;38(4):207.