Marchiafava Bignami disease usually affects men who are chronic alcoholics and are middle- aged or elderly [1], prefer to consume hard liquor and are severely malnourished with vitamin B complex deficiency [2]. 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 [3]. 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" [4] caused by corpus callosum demyelination is difficult to prove. The disconnection of the hemispheres may cause agraphia of the dominant hand [5] or hemianopia [6]. 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.

Marchiafava Bignami disease has occasionally been described in non-alcoholic patients, such as individuals suffering from Plasmodium falciparum infection [7] or gynecologic malignancies.

• Hypoxemia

  Hypercarbia and hypoxemia. Glycemia changes. Osmolarity changes. Inborn metabolism disorders (eg. porphyria, Wilson's). Central Nervous System disorders: Epileptic seizures. Contusion. Hypertensive encephalopathy. Intracranial hypertension. [ncbi.nlm.nih.gov]

• Diffuse Muscle Wasting

  On neurological examination, he showed an ataxic gait, and a diffuse muscle wasting and weakness (more proximal than distal). His face was hypo-mimic, and his speech was dysarthric and slow. [itmedicalteam.pl]

• Nystagmus

  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]

  The patient was found to have gross difficulty in walking, normal higher functions with MMSE of 28/30, absence of apraxia, dysarthria, and nystagmus; she had normal power in the limbs, normal deep jerks, and negative Babinski response. [neurologyindia.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]

• Irritability

  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 [8] [9] 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 [10]. The edema is initially vasogenic and then converts to cytotoxic [11]. 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 [12]. 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.

A central nervous system or meningeal infection sometimes must also be ruled out, and that is accomplished using a lumbar puncture.

Electroencephalography is uninformative in Marchiafava Bignami disease [13].

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]

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]

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]

Prognosis Prevention Introduction[1] The syndrome was first described by Marchiafava and Bignami (two Italian pathologists) in 1903. [patient.info]

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]

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]

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]

In this article Introduction Epidemiology Presentation Differential diagnosis Investigations Management Prognosis Prevention Marchiafava-Bignami Syndrome In this article Introduction Epidemiology Presentation Differential diagnosis Investigations Management [patient.info]

Marchiafava-Bignami disease 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]

^ 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]

Published online 2015 October 16 DOI: http://dx.doi.org/10.3346/jkms.2015.30.11.1675 5 The Epidemiology of Alcohol Use Disorders Hae Kook Lee, Bo Hye Lee J Korean Diabetes.2012;13(2):69-75. [synapse.koreamed.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]

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]

^ 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]

Optic neuropathy and a reversible splenial lesion after gastric bypass: Shared pathophysiology? J Neurol Sci 2010;291:92-4. [cyberleninka.org]

Regarding pathophysiological changes, we believe that the lesions in these patients are demyelinating and ischaemic. [elsevier.es]

These findings can conclude in the possibility of aggressive nutritional supplementation with a reduction in drinking to prevent the development of MBD in alcoholic patients. [pubmed.ncbi.nlm.nih.gov]

Prevention Prevention of alcohol-related problems is through education and mental health support. Yoshizaki T, Hashimoto T, Fujimoto K, et al; Evolution of Callosal and Cortical Lesions on MRI in Marchiafava-Bignami Disease. [patient.info]

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]

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