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NEURORADIOLOGY Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 779-781
MRI findings in marchiafava - bignami disease with central pontine myelinolysis : A case report


Primus, G. S Road, Bhangagarh, Guwahati - 781 005, Assam, India

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Date of Submission28-Nov-2006
Date of Acceptance02-Dec-2006
 

Keywords: Marchiafava Bignami Disease, Corpus Callosum, Demyelination

How to cite this article:
Goswami P, Medhi N, Sarma P K, Sarmah B J. MRI findings in marchiafava - bignami disease with central pontine myelinolysis : A case report. Indian J Radiol Imaging 2006;16:779-81

How to cite this URL:
Goswami P, Medhi N, Sarma P K, Sarmah B J. MRI findings in marchiafava - bignami disease with central pontine myelinolysis : A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:779-81. Available from: http://www.ijri.org/text.asp?2006/16/4/779/32346

   Introduction Top


Marchiafava-Bignami Disease is rare toxic disease of an unknown etiology but strongly associated with alcoholism. Marchiafava-Bignami disease is a disorder characterized by progressive demyelination and necrosis of the corpus callosum. The process may extend laterally into the neighboring white matter and occasionally as far as the subcortical regions. In this article, we present the classical MR findings of Marchiafava-Bignami disease with associated central pontine myelinolysis in a female patient.


   Case report Top


A forty years old female presented with depressed consciousness and a variety of other symptoms of two days duration. The patient had been an alcoholic for over fifteen years. History of short - and long- term memory deficits were also present. Physical examination showed a thin built female with overall diminished reflexes and rigidity. Relevant laboratory values were low serum level of albumin and mild hyponatremia. MR imaging demonstrated areas of T1 hypointense and T2 hyperintense signal involving the corpus callosum on the T1 weighted and T2 weighted axial images respectively (Fig.1A and 1B). T2 weighted sagittal MR image showed mild atrophy and hyperintense signal involving almost the entire central layers of corpus callosum, with sparing of dorsal and ventral layers producing the sandwich sign (Fig. 2). The sagittal T2 weighted image also showed high signal intensity areas in the pons. The findings were consistent with degenerative necrosis of corpus callosum. Few high T2 signal intensity areas were seen in the periventricular and subcortical white matter (Fig. 3). High signal intensity areas within the midpons could be better appreciated on the T2 - weighted axial image, with surrounding rim of normal appearing pontine parenchyma and sparing of the corticospinal tracts (Fig. 4). On the basis of clinical history, findings on physical examination and imaging features, the diagnosis of chronic form of Marchiafava-Bignami disease with central pontine myelinolysis was made. The patient was treated with vitamin B complex and gradual correction of electrolyte imbalance and showed relative improvement of symptoms within next few days and finally made a reasonably good recovery.


   Discussion Top


Marchiafava-Bignami disease is a rare neurologic disorder, primarily affecting the corpus callosum and has strong association with chronic alcohol consumption [1],[2],[3]. At first, Marchiafava-Bignami disease was thought to be particular in individuals living in the central region of Italy and consuming large amounts of inexpensively manufactured Chanti red wine. It is now known that Marchiafava-Bignami disease occurs worldwide and has been described in poorly nourished nondrinkers [1],[5]. Most patients are male, between forty and sixty years of age and have a history of chronic alcoholism and malnutrition [1]. Non-specific general symptoms and encephalopathy in an alcoholic may harbour undiagnosed Marchiafava-Bignami disease. The incidence of Marchiafava-Bignami disease may be higher and its prognosis may be milder than generally believed [3].

Marchiafava-Bignami disease is a fatal disorder characterized by demyelination of the corpus callosum. It is diagnosed by the imaging of demyelination at the corpus callosum with MRI [4]. The central layers of the corpus callosum are affected, with sparing of the dorsal and ventral layers producing sandwich sign [2]. The disease typically affects the body of the corpus callosum, followed by genu, and finally the splenium. The entire corpus callosum may also be involved. Other white matter tracts such as the anterior and posterior commissures and the cortico-spinal tracts may be involved. Lesions may also be found in the hemispheric white matter and in the middle cerebellar peduncles. The subcortical U-fibers tend to be spared. The corpus callosum degenerate and splits into three layers ("layered necrosis). The necrosis leads to cystic cavities. Occasionally, the lateral putamina and the cortex are involved [1].

The disease can follow one of three clinical courses, a fulminate acute form, or subacute and chronic forms [1],[2],[5]. Patients present acutely with mental confusion, disorientation, neurocognitive deficits and seizures. 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. Patients with the subacute type of Marchiafava-Bignami disease - which is characterized by dementia, dysarthria and muscle hypertonia - may survive for years. The chronic form of Marchiafava-Bignami disease is characterized by a chronic dementia. Patients with Marchiafava-Bignami disease may also present with hyponatremia. [1]. There have been reports of survival after acute onset with a fairly good recovery after vitamin B complex substitution [1],[5],[6].

The acute form affects the genu and splenium, whereas the chronic form most commonly affects the body. On MR images, in the acute form, the central corpus callosum enlarges, presumably because of edema. The corpus callosum is of low signal on T1-weighted images and high signal on T2-weighted images and often enhances. In the subacute and chronic forms, the lesions involve the central part of the body most commonly and are hypointense on T1-weighted images and hyperintense on T2-weighted images. Focal hypointensity on T2-weighted images within the corpus callosum has been described, which has been speculated to represent haemosiderin deposits [2],[5]. Associated findings of Wernicke encephalopathy or central pontine myelinolysis may also been seen in these patient [5]. MR spectroscopy has been used to evaluate patient with Marchiafava-Bignami disease. The initial MR spectra show a mildly increased choline level (probably secondary to acute demyelination), low N-acetyl aspartate (probably secondary to neuronal damage), and the presence of lactate. On long term follow up after treatment, the spectra show nearly complete normalization of the peak heights [7].

Diagnosis is made on the basis of clinical findings in combination with imaging features. Increased T2 signal intensity involving the corpus callosum, when identified in the correct clinical setting, the diagnosis of Marchiafava-Bignami disease can be made. Prompt therapy may result in survival of these patients [1].

In our patient classical MRI findings of chronic form of Marchiafava-Bignami disease were present along with very infrequently associated central pontine myelinolysis, which was probably responsible for the acute clinical presentation of the patient.

 
   References Top

1.Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami Disease : MR Findings in Two Patients. AJNR Am J Neuroradiol 2003; 24 (10): 1955-1957  Back to cited text no. 1    
2.Bourekas EC, Varakis K, Bruns D, et al. Lesions of the Corpus Callosum : MR Imaging and Differential Considerations in Adults and Children. AJR Am J Roentgenol 2002; 179:251-257  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Helenius J, Tatlisumak T, Soinne L, Valanne L, Kaste M : Marchiafava-Bignami disease : two cases with favourable outcome. Eur J Neurol 2001; 8 (3) : 269-272  Back to cited text no. 3    
4.Celik Y, Kaya M, Sengun S, Utku U. : Marchiafava-Bignami disease : cranial MRI and SPECT findings. Clin Neurol Neurosurg 2002 ; 104 (4) : 339-341  Back to cited text no. 4    
5.Nusbaum AO, Fung KM, Atlas SW. White Matter Diseases and Inherited Metabolic Disorders. In :Atlas SW, ed. Magnetic Resonance Imaging of the Brain and Spine, 3rd ed. Philadelphia : Lippincott Williams & Wilkins, 2002 : 504-505  Back to cited text no. 5    
6.Rosa A, Demiati M, Cartz L, Mizon JP : Marchiafava-Bignami disease, syndrome of interhemispheric disconnection, and right-handed agraphia in a left-hander. Arch Neurol 1991;48 (9):986-988   Back to cited text no. 6    
7.Gambini A, Falini A, Moiola L, et al. Marchiafava-Bignami disease : longitudinal MR imaging and MR spectroscopy study. AJNR Am J Neuroradiol 2003; 24: 249-253  Back to cited text no. 7  [PUBMED]  [FULLTEXT]

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Correspondence Address:
P Goswami
MD Consultant Radiologist, Primus G. S Road, Bhangagarh Guwahati - 781 005, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32346

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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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