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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 3 | Page : 642-643
A rare case of Marchiafava–Bignami disease: Extracallosal lesions involving bilateral medipeduncle
LI Xuehan, LV Zhiyu, Ping Wang, Xiu Chen
Department of Neurology, The Affiliated Hospital of Southwest Medical University, Luzhou, China
|Date of Web Publication||9-May-2017|
Department of Neurology, The Affiliated Hospital of Southwest Medical University, Taiping Street 25, Luzhou, Sichuan - 646000
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Xuehan L I, Zhiyu L V, Wang P, Chen X. A rare case of Marchiafava–Bignami disease: Extracallosal lesions involving bilateral medipeduncle. Neurol India 2017;65:642-3
|How to cite this URL:|
Xuehan L I, Zhiyu L V, Wang P, Chen X. A rare case of Marchiafava–Bignami disease: Extracallosal lesions involving bilateral medipeduncle. Neurol India [serial online] 2017 [cited 2020 Jan 28];65:642-3. Available from: http://www.neurologyindia.com/text.asp?2017/65/3/642/205928
Marchifava–Bignami disease (MBD) is a rare condition that mostly affects middle-aged males, often causing necrosis or demyelination in the corpus callosum symmetrically. This disease was first described by two Italian pathologists, Ettore Machiafava and Amico Bignami. It has been accepted that the classic MBD injures the center part of the corpus callosum, sparing the dorsal and ventral layers. In addition, it is widely believed that extensive lesions indicate a worse prognosis. Articles commonly report that MBD may also cause lesions at sites other than the corpus callosum. To the best of our knowledge, a case with extracallosal lesions involving bilateral medipeduncle is rare.
A 48-year-old man was admitted to our department with repeated generalized seizures for more than 6 months. According to his family, these episodes lasted for approximately 2–3 minutes. The frequency of the seizures and their precipitating factors were unknown. After waking up, he became tired and started the clonic movements heralding the seizure but did not vomit or have bladder incontinence. The patient had a 20-year history of alcoholism, averaging more than 250 ml per day. On admission, the neurological examination revealed an intact cognition with a slurred speech. His pupils were equal in size and reactive to light and accommodation. His eye movements were normal without nystagmus. He had no VIIth or XIIth nerve palsy. The muscle strength of the patient's extremities was grade 5, with a normal muscle tone. The patient had no sensory disturbance, and his physiological reflexes were intact and normal.
Cerebral magnetic resonance imaging indicated abnormal signals in bilateral medipeduncle and the corpus callosum [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f. Laboratory tests showed a glutamic–pyruvic transaminase value of 95.2 U/L and a glutamic–oxalacetic transaminase value of 96.0 U/L. The patient did not undergo a liver ultrasound. Routine blood, kidney function, blood lipid, and blood coagulation tests indicated no overt abnormality. He did not undergo a lumbar puncture.
|Figure 1: (a) T1 weighted imaging (T1WI): Hypointensity of the splenium of corpus callosum; (b) T2-weighted imaging (T2WI): Hyperintensity of the genu of the corpus callosum; (c) Fluid attenuated inversion recovery (FLAIR): Hypointensity of splenium of the corpus callosum; (d) Diffusion weighted imaging (DWI): Hyperintensity of splenium of the corpus callosum; (e) FLAIR: Hyperintensity of bilateral medipeduncle; (f) DWI: Hyperintensity of bilateral medipeduncle|
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His diagnosis was MBD. He received a large dose of B-complex vitamins, piracetam, and sodium valproate. He was discharged 8 days later with normal cognition and vital signs. At follow-up 3 months later, he reported that he had changed his job because of his clumsiness.
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| » References|| |
Navarro JF, Noriega S. Marchiafava-Bignami disease. Rev Neurol 1999;28:519-23.
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava-Bignami disease: A review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry 2014;85:168-73.
Namekawa M, Nakamura Y, Nakano I. Cortical involvement in Marchiafava-Bignami disease can be a predictor of a poor prognosis: A case report and review of the literature. Intern Med 2013;52:811-3.
Kawarabuki K, Sakakibara T, Hirai M, Yoshioka Y, Yamamoto Y, Yamaki T. Marchiafava-Bignami disease: Magnetic resonance imaging findings in corpus callosum and subcortical white matter. Eur J Radiol 2003;48:175-7.