Journal of Emergencies, Trauma, and Shock
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 Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 8  |  Issue : 1  |  Page : 52-54
Marchiafava-Bignami disease in a trauma patient


1 Department of Surgery, Lincoln Medical and Mental Health Center, Bronx, New York, USA
2 Department of Radiology, Lincoln Medical and Mental Health Center, Bronx, New York, USA
3 Department of Medicine, Lincoln Medical and Mental Health Center, Bronx, New York, USA

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Date of Submission16-Apr-2014
Date of Acceptance14-May-2014
Date of Web Publication30-Jan-2015
 

   Abstract 

Marchiafava-Bignami disease (MBD) is a rare pathological condition affecting the corpus callosum (CC), characterized by progressive demyelination and necrosis. While usually found in patients with chronic alcoholism, it has rarely been characterized in non-alcoholics. We describe a trauma patient with an unknown mechanism of injury, who was found to have MBD after remaining comatose for a prolonged period of time. Magnetic resonance imaging (MRI) demonstrated restricted diffusion involving the genu, body, and splenium of the CC. The patient eventually awoke but was non-communicative and uncomprehending prior to discharge to a nursing facility. We reviewed the literature and report here the first case of MBD encountered in a trauma patient. In conclusion, MBD is an extremely rare condition in non-alcoholic patients, and the use of MRI is crucial for its identification.

Keywords: Marchiafava-Bignami disease, trauma, corpus callosum

How to cite this article:
Jorge JM, Gold M, Sternman D, Prabhakaran K, Yelon J. Marchiafava-Bignami disease in a trauma patient. J Emerg Trauma Shock 2015;8:52-4

How to cite this URL:
Jorge JM, Gold M, Sternman D, Prabhakaran K, Yelon J. Marchiafava-Bignami disease in a trauma patient. J Emerg Trauma Shock [serial online] 2015 [cited 2019 Nov 13];8:52-4. Available from: http://www.onlinejets.org/text.asp?2015/8/1/52/150399



   Introduction Top


Marchiafava-Bignami disease (MBD) was first described in Italian heavy red wine drinkers in 1903. [1] A relatively rare condition classically found in chronic alcoholics, a small number of cases of MBD has been reported in non-alcoholics. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12] Clinical features classically include reduced consciousness or coma, neuropsychiatric manifestations, seizures, hemiparesis, dysarthria, ataxia and apraxia, and symptoms of hemispheric disconnection. It was historically confirmed by pathological diagnosis; however, magnetic resonance imaging (MRI) has become the diagnostic method of choice by easily revealing the pathognomonic features.

We describe a young non-alcoholic male who presented after being found unresponsive at the bottom of a flight of stairs, who failed to make a full neurological recovery despite having no acute traumatic intracerebral findings on brain imaging.


   Case report Top


A 28-year-old male was found with altered mental status and unknown mechanism of injury at the bottom of a flight of stairs. He reportedly had gone to a party the evening before, which was the last time he was seen by his wife. He regularly smoked an average of "one nickel bag" (0.5 gm/10-20 cigarettes) of marijuana daily. His Glasgow Coma Scale (GCS) on presentation was seven, and he was intubated for airway protection. A trauma workup including physical examination revealed a left scalp hematoma and some minor facial fractures. Non-contrast computed tomography (CT) imaging of the brain demonstrated a left temporal and parietal scalp hematoma but no intracranial injury. CT angiography of the neck demonstrated no vascular injury. Ethyl alcohol was undetectable in his serum; his toxicology panel revealed cannabinoids in his urine but was negative for barbiturates, benzodiazepines, and cocaine, methamphetamines, opiates, or phencyclidine. Liver enzymes were within normal limits (aspartate transaminase, AST 50; alanine transaminase, ALT 20; alkaline phosphatase 78; total bilirubin 0.9).

Over the next several days, the patient's neurological status remained poor, with an average GCS of eight, and intermittent agitation requiring sedation. Repeat CT imaging of the brain demonstrated no new findings. His electroencephalography (EEG) was abnormal with delta slowing and left frontal spikes indicative of encephalopathy. Unenhanced MRI of the brain demonstrated restricted diffusion and hyperintense signal on fluid attenuated inversion recovery (FLAIR) imaging throughout the corpus callosum (CC) [Figure 1]. Additionally, two small areas of microhemorrhage were identified in the right hemisphere on the gradient echo sequence. The CC did not enhance on a follow-up MRI exam. MRI of the cervical spine demonstrated a small syrinx at the C6 level.

Clinical and neurological findings were consistent with MBD. High dose of thiamine was started empirically based on anecdotal support in the literature. The patient's mental status subsequently improved, with spontaneous eye opening, incoherent vocalization, and random limb movement but no ability to follow commands. Five weeks after the patient's admission, the patient was discharged to a nursing facility.
Figure 1: Diffusion-weighted imaging demonstrates restricted diffusion in the body (a) and splenium (b) of the corpus callosum. Hyperintense signal is demonstrated on FLAIR in the splenium (c)

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   Discussion Top


MBD has been reported worldwide in less than 300 patients. It is a primary degenerative condition of the CC characterized by symmetrical demyelination and necrosis. While other pathologies such as ischemic stroke, diffuse axonal injury, inflammatory demyelination, brain tumors, Wernicke encephalopathy, and  Wallerian degeneration More Details can also involve the CC, they can be differentiated based on history, physical exam, and MRI. Two subtypes of MBD have been described, differentiating between either acute or chronic onset. [13] Type A MBD is characterized by acute to subacute onset of impaired consciousness, pyramidal tract signs, limb hypertonia, seizures, hyperintense swelling on T2-weighted MR sequences of the CC, and poor prognosis. Type B MBD has a less precipitous onset and carries a more favorable prognosis. Sequential MR imaging may demonstrate reversal of lesions, suggesting underlying edema rather than demyelination. It appears that our patient's presentation was more consistent with the acute form of MBD.

Heavy alcohol consumption has been the classic associative factor with MBD. While the exact role of alcohol in MBD is unknown, it has been hypothesized that a toxin present in the alcohol may be responsible for demyelination in the CC. Alternatively in non-drinkers, there may be exposure from a different source. [14] It has also been suggested that severe liver dysfunction in chronic alcoholics may lead to elevated serum ammonia levels and encephalopathy, callosal edema, and demyelination. [11] Various vitamin deficiencies and malnutrition in general have also been linked with MBD.

Our patient was unique in that he had neither a history of alcohol abuse nor malnutrition. His family reported him to drink alcohol only occasionally. He did have a strong pattern of cannabis use, averaging up to 20 marijuana cigarettes per day. The presentation of our patient, being "found down" with a scalp hematoma, suggested traumatic brain injury. Routine urine toxicology revealed only cannabinoids but this does not exclude the possibility of other illicit drug use just before admission, not included within the screen. Further, the marijuana our patient was exposed to could have been laced with unknown potentially neurotoxic adulterants. Clinical, laboratory, and diagnostic imaging features of other substance abuse or any other cause of sudden coma were absent.

Our patient was described as being asymptomatic just hours prior to admission implying a diagnosis of type A or acute onset MBD. Additionally, it is unclear whether the patient's improvement in neurological status two weeks into his hospital admission was due to supplemental thiamine or the passage of time and supportive medical care. Scant recovery over five weeks left the patient with severe neurologic impairment at the time of discharge.


   Conclusion Top


MBD is an extremely rare neurological disorder that carries an unfavorable prognosis. To the best of our knowledge, this is the first reported case of MBD following injury in the setting of habitual marijuana abuse. Other ambiguities include the possibility of other undetected illicit drug intoxication and the possibility of an unknown injury mechanism.

 
   References Top

1.
Marchiafava E, Bignami A. Upon an alteration of the corpus callosum observed in a subject with alcoholism. 1903;8:544-9.  Back to cited text no. 1
    
2.
Caulo M, Briganti C, Notturno F, Committeri G, Mattei PA, Tartaro A, et al. Non-alcoholic partially reversible Marchiafava-Bignami disease: Review and relation with reversible splenial lesions. Neuroradiol J 2009;22:35-40.  Back to cited text no. 2
    
3.
Leong AS. Marchiafava-Bignami disease in a non-alcoholic Indian male. Pathology 1979;11:241-9.  Back to cited text no. 3
[PUBMED]    
4.
Ma KC, Chen KJ. Primary necrosis of corpus callosum with dystrophic astrogliosis and Rosenthal-like fiber formation. The first Chinese case of Marchiafava-Bignami′s disease (MBD). Acta Neuropathol 1983;60:283-90.  Back to cited text no. 4
[PUBMED]    
5.
Kosaka K, Aoki M, Kawasaki N, Adachi Y, Konuma I, Iizuka R. A non-alcoholic Japanese patient with Wernicke′s encephalopathy and Marchiafava-Bignami disease. Clin Neuropathol 1984;3:231-6.  Back to cited text no. 5
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Rickert CH, Karger B, Varchmin-Schultheiss K, Brinkmann B, Paulus W. Neglect-associated fatal Marchiafava-Bignami disease in a non-alcoholic woman. Int J Legal Med 2001;115:90-3.  Back to cited text no. 6
    
7.
Hlaihel C, Gonnaud PM, Champin S, Rousset H, Tran-Minh VA, Cotton F. Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: Follow up studies. Neuroradiology 2005;47:520-4.  Back to cited text no. 7
    
8.
Mas G, Gonzalez G, Martinez-Ortiz MJ, Sáez-Castán J. Marchiafava-Bignami disease in a non-alcoholic patient. Rev Neurol 2006;42:637-8.  Back to cited text no. 8
    
9.
Rusche-Skolarus LE, Lucey BP, Vo KD, Snider BJ. Transient encephalopathy in a postoperative non-alcoholic female with Marchiafava-Bignami disease. Clin Neurol Neurosurg 2007;109:713-5.  Back to cited text no. 9
    
10.
Celik Y, Temizoz O, Genchellac H, Cakir B, Asil T. A non-alcoholic patient with acute Marchiafava-Bignami disease associated with gynecologic malignancy: Paraneoplastic Marchiafava-Bignami disease? Clin Neurol Neurosurg 2007;109:505-8.  Back to cited text no. 10
    
11.
Tao H, Kitagawa N, Kako Y, Yamanaka H, Ito K, Denda K, et al. A case of anorexia nervosa with Marchiafava-Bignami disease that responded to high-dose corticosteroid administration. Psychiatry Res 2007;156:181-4.  Back to cited text no. 11
    
12.
Yadala S, Luo JJ. Marchiafava-Bignami disease in a non-alcoholic diabetic patient. Case Rep Neurol Med 2013;2013:979383.  Back to cited text no. 12
    
13.
Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol 2004;251:1050-9.  Back to cited text no. 13
    
14.
Murthy SB, Jawaid A, Bock JE, Qureshi SU, Schulz PE. Marchiafava-Bignami disease (MBD) in a non-alcoholic patient: A case report. Can J Neurol Sci 2010;37:138-40.  Back to cited text no. 14
    

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Correspondence Address:
Juaquito M Jorge
Department of Surgery, Lincoln Medical and Mental Health Center, Bronx, New York
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2700.150399

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