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Marchiafava Bignami Disease

Marchiafava-Bignami Disease

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.


Presentation

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.

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]
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[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]
  • 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]
  • At the same time, our patient did not present ophthalmoplegia, nystagmus, or ataxia and did not show MR findings typical of Wernicke's encephalopathy, such as symmetrical hyperintense lesions surrounding the third ventricle and aqueduct.[ncbi.nlm.nih.gov]
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]
Neglect
  • "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]
  • This is the first known case of Marchiafava-Bignami disease in a non-alcoholic woman and the first case in the forensic setting of neglect.[ncbi.nlm.nih.gov]
  • ., Moriya, H. and Hirayama, K. (1993) “Crossed homonymous hemianopia” and “crossed left hemispatial neglect” in a case of MarchiafavaBignami disease.[scirp.org]
  • Neglect-associated fatal Marchiafava-Bignami disease in a non-alcoholic woman . Int J Legal Med. 2001 ; 115 ( 2 ): 90 – 3 . 5. Celik , Y , Temizoz , O , Genchellac , H , Cakir , B , Asil , T.[cambridge.org]
  • Kamaki M, Kawamura M, Moriya H, Hirayama K (1993) “Crossed homonymous hemianopia” and “crossed left hemispatial neglect” in a case of Marchiafava-Bignami disease. J Neurol Neurosurg Psychiatry 56:1027–1032 PubMed Google Scholar 31.[dx.doi.org]
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]
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]

Workup

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].

Plasmodium Falciparum
  • Marchiafava Bignami disease has occasionally been described in non-alcoholic patients, such as individuals suffering from Plasmodium falciparum infection or gynecologic malignancies.[symptoma.com]
  • 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]
  • 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]
  • Marchiafava-Bignami disease complicating SC hemoglobin disease and Plasmodium falciparum infection. Presse Med 2010; 39 : 990–993. 21. Cui Y, Zheng L, Wang X, Zhang W, Yuan D, Wei Y. Marchiafava-Bignami disease with rare etiology: a case report.[dx.doi.org]

Treatment

  • 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]
  • Our objective was to identify helpful features for differential diagnosis and assess whether any treatment can be recommended.[ncbi.nlm.nih.gov]
  • Because both MBD and CPM can have fatal outcomes, early recognition and treatment can result in a better prognosis.[ncbi.nlm.nih.gov]
  • In any case, the effectiveness of steroids and other treatments has remained hypothetical.[ncbi.nlm.nih.gov]
  • Early diagnosis and treatment can improve the prognosis of the patient.[dx.doi.org]

Prognosis

  • 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]
  • Early diagnosis with imaging and prompt treatment may improve the prognosis of MBD.[ncbi.nlm.nih.gov]
  • This case raises questions about some previous ideas on this disease, such as the prognosis of its acute forms and the significance of the extracallosal lesions seen on neuroimaging.[ncbi.nlm.nih.gov]
  • This case further supports the notion that cortical involvement in patients with MBD is a predictor of a poor prognosis.[ncbi.nlm.nih.gov]
  • With early detection and treatment, the prognosis of MBD may be good even in cases with severe diffusion restriction of the complete corpus callosum.[ncbi.nlm.nih.gov]

Etiology

  • 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]
  • It is associated with chronic alcohol consumption caused by either a toxic or nutritional etiology.[ncbi.nlm.nih.gov]
  • To date, the etiology of MBD is incompletely understood.[dx.doi.org]
  • Etiology and Pathophysiology It has long been accepted that the etiology of Marchiafava-Bignami disease (MBD) is likely either toxic or nutritional, as it is seen predominantly in malnourished alcoholics.[emedicine.com]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Functional neuroimages would be useful for understanding the pathophysiologic processes of MBD.[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]

Prevention

  • 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]
  • 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]
  • 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]

References

Article

  1. 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.
  2. Geibprasert S, Gallucci M, Krings T. Alcohol-induced changes in the brain as assessed by MRI and CT. Eur Radiol. 2010;20:1492-1501.
  3. 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.
  4. Berlucchi G. Frontal callosal disconnection syndromes. Cortex. 2012;48:36–45.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami Disease: MR findings in two patients. Am J Neuroradiol. 2003;24:1955–1957.
  11. 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.
  12. 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.
  13. Fang SC. EEG coherence for a patient with Marchiafava-Bignami disease. Clin EEG Neurosci. 2007;38(4):207.

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Last updated: 2019-06-28 09:57