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
Muscle rigidity and facial trismus may be severe. Most patients presenting with the acute type of Marchiafava-Bignami disease will go into coma and eventually die. [ijri.org]
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]
- Tongue Atrophy
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]
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]
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]
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. [symptoma.com]
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]
mental status and seizures. [1 ] The exact pathology leading to MBD has not yet been elucidated. [tropicalgastro.com]
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]
- Unstable Gait
A 62-year-old male Japanese, a heavy drinker for his last 40 years, was admitted to our hospital because of unstable gait of acute onset. [ncbi.nlm.nih.gov]
Case Reports Case 1 A 56-year-old man presented with vertigo and unstable gait of 2 days’ duration. The patient had been an alcoholic for 25 years and had been diagnosed with hepatic cirrhosis more than 10 years earlier. [ajnr.org]
- 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 .
- Plasmodium Falciparum
The triggering event was a Plasmodium falciparum infestation. Left tactile anomia was caused by callosal necrotic lesions as seen on brain MRI. [em-consulte.com]
Marchiafava Bignami disease has occasionally been described in non-alcoholic patients, such as individuals suffering from Plasmodium falciparum infection or gynecologic malignancies. [symptoma.com]
View Article : Google Scholar 3 Boutboul D, Lidove O, Aguilar C, Klein I and Papo T: Marchiafava-Bignami disease complicating SC hemoglobin disease and Plasmodium falciparum infection. Presse Med. 39:990–993. 2010. [dx.doi.org]
Marchiafava-Bignami disease complicating SC hemoglobin disease and Plasmodium falciparum infection. Presse Med. 2010; 39 :990–993. doi: 10.1016/j.lpm.2010.02.052. [ PubMed ] [ CrossRef ] [ Google Scholar ] 4. Tuntiyatorn L, Laothamatas J. [ncbi.nlm.nih.gov]
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]
Some treatments work extremely well with some patients and not at all with others. Some treatments include Therapy with thiamine and vitamin B complex. Alcohol consumption should be stopped. [en.wikipedia.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]
Comparison of Prognosis between the Two Groups Prognosis was not reported in 12 AMBD patients. The prognosis was significantly better in NAMBD patients than in the remaining AMBD patients (U = 1224, P = 0.012). [scirp.org]
The prognosis is especially grave with cortical involvement. However, the case presented above showed favorable prognosis. Key Words: Marchiafava-bignami disease · Cerebral cortex · Corpus callosum [e-jnc.org]
Early diagnosis and treatment can improve the prognosis of the patient. [dx.doi.org]
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]
MERS is not a so rare clinical entity even if epidemiological data are scanty; the clinical presentation is variable such as the etiologies of this syndrome. [neuro-la-cote.info]
^ 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]
MRI findings can reflect the underlying pathophysiology responsible for the morphological manifestations (see Table 2 ). Table 2: Correlation of imaging findings in MBS with pathophysiology [ 1, 6 ]. [hindawi.com]
Regarding pathophysiological changes, we believe that the lesions in these patients are demyelinating and ischaemic. [elsevier.es]
(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. [dx.doi.org]
Prevention Prevention depends on either maintaining a diet with a sufficient intake of thiamine, or supplementing an inadequate diet with vitamin preparations. [medical-dictionary.thefreedictionary.com]
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