Cite this article as: Patterson JF Asymptomatic Marchiafava-Bignami Disease. Int J Psychopath Psychopharmacol Psychother 1996, 1 (1). URL http://www.psycom.net/ijppp.v1n1.html
A 35 year old right handed Caucasian male was admitted to the hospital for alcohol rehabilitation. Following completion of high school, he had served in the Marine Corps. He was discharged honorably and had worked at odd jobs since his discharge. He had been a chronic alcoholic for 16 years. His father and brother were chronic alcoholics but not his mother or sisters. He had been through several alcohol rehabilitation programs in the past without obtaining sobriety. He had no neurological symptoms or signs on admission. His only psychiatric complaint was one of fluctuating irritability.
Physical examination on admission was unremarkable. Neurological examination revealed no neurological deficit. The only mental status abnormality was in the area of remote memory which was sketchy. Laboratory data performed during hospitalization consisted of a wide battery of studies which were normal including: a complete blood count, serological test for syphilis, HIV testing, thyroid studies, B-12 and folate levels, liver function studies, as well as a hepatitis screen. Due to the patient's mental status of sketchy remote memory with no other abnormality, magnetic resonance imaging of the head was obtained and revealed marked atrophy of the corpus collosum as well as a hypodense area in the splenum of the corpus collosum consistent with cystic necrosis. A SPECT scan revealed normal blood flow and distribution with no abnormalities. Psychometric testing including a Wechler Adult Intelligence Scale-Revised, Wide Range Achievement Tests-Revision Three, Rey Auditory Verbal Learning Test, Halstead-Reitan Category subtest, Wechler Memory Scale-Revised, Stroop, and a Boston Naming Test (COWAT) revealed a full scale IQ of 106 with only minimal deficit in the area of logical reasoning. No other areas of neuropsychometric deficit were demonstrated. A neurological consultation was in agreed with the diagnosis of Marchiafava-Bignami disease and recommended treatment with B vitamins for the long term.
The patient successfully completed the alcohol rehabilitation program. Throughout his hospitalization and upon discharge, he was treated with high doses of B vitamins. At no time during his rehabilitation did he reveal any evidence of mental status abnormality or neurological deficiency. Follow up evaluation at six months and one year revealed no progressive psychiatric or neurological symptoms. An MRI of the head at six months failed to reveal any change in the degree of atrophy or cavitation of the corpus callosum; however, at one year, the MRI revealed small multi-focal hypodense areas surrounding the original cystic cavitation. Repeat neuropsychometric testing on both occasions were not signifcantly altered from the original testing. Further investigation suggested strongly that the patient had begun to consume alcohol in signicant amounts beginning approximately four months after completion of the alcohol rehabilitation program.
Merritt reported that the characteristic neuropathology of Marchiafava- Bignami disease is necrosis of the medial zone of the corpus collosum. The necrosis varies from softening and discoloration to cavitation and cyst formation. Usually all stages of the degeneration are found in any given case. In the majority of cases, the rostral portion of the corpus collosum is effected first. The lesions arise as small symmetrical foci which extend and become confluent. All of the lesions have a constant bilateral symmetry (Merritt HH, 1979). Few neurological diseases have such a well demarcated neuropathological picture.
Marchiafava-Bignami disease is a rare complication of alcoholism. Diagnosis has been based on autopsy findings or magnetic resonance imaging in patients with alcoholism and definitive neurological deficits. The clinical course of MBD is highly variable. Valk and van der Knapp distinguished an acute, subacute, and chronic form of the disorder. The acute form is characterized by sudden onset, impaired vigilance, epileptic seizures, and rapid death; the subacute by limb hypertonia, dysarthria, and rapidly progressive dementia which evolves over several years into a chronic form(Valk J, van der Knaap MS, 1989). Considered to be progressively lethal, several cases of remission have been described (Izquierdo G, Quesada MA, Chacon J, et al., 1992). Marjama (Marjama J, Yoshino MT, and Reese C, 1994) described an additional type of case as relapsing. With this case, these classifications must be questioned. It would appear that a separate disease state which is asymptomatic possiblly exists for MBD.
The neuropsychometric testing is of particular importance in this patient. No evidence could be gained from the testing which would point in the direction of MBD or any other severe neurological disease.
Magnetic resonance imaging is the only diagnostic modality that allows the diagnosis of MBD to be made in this patient. The question must arise as to when magnetic resonance imaging should be performed in chronic alcoholics. Given the cost factor and the relatively high incidence of head trauma, guidelines need to be established. It is clear that if the chronic alcoholic patient is demonstrating neurological deficiency, particularly symptoms of inner hemispheric disconnection, magnetic resonance imaging of the brain needs to be performed. Likewise, any chronic alcoholic patient demonstrating corpus collosal symptoms or signs, bilateral frontal lobe signs, or mental status changes, however diffuse, which remit without treatment, should have magnetic resonance imaging of the brain. However, for the vast majority of chronic alcoholics who present with neither of the two above situations, the differential diagnosis is much more problematical.
In this particular patient, the diagnosis is highly probable from the magnetic resonance imaging studies. This is especially true of the progression of the disease state upon resumption of alcohol consumption as evidenced by follow up MRI scans. However, repetitive closed head trauma or congential abnormality of the corpos callosum can not be completely eliminated from the differential diagnoses. Only by postmortem examination can a truly definitive diagnosis be made. The question is raised by this case as to the prodromal time frame for Marchiafava-Bignami disease.
Treatment in this case must be guided at further alcohol abstinence, minimal exposure to further head trauma, vitamin supplementation, and close follow up examination.
Berek K, Wagner M, Chemelli AP, et al. (1994) Hemespheric disconnection in Marchiafava-Bignami disease: clinical,neuropsychological and MRI findings. Journal of the Neurological Sciences. 123;2-5.
Izquierdo G, Quesada MA, Chacon J, et al. (1992) Neuroradiological abnormalities in Marchifava-Bignami disease of benign evolution. Eur Journal of Radiology. 15;71-4.
Humbert T, De Guilhermier P, Maktouf C, et al. (1992) Marchiafava-Bignami Disease. A Case Studied by Structural and Functional Brain Imaging. Eur Arch Psychiatry Clin Neurosci. 242;69-71
Marchiafava E and Bignami A. (1903) Sopra un alterazione del corpo calloso observata in saggertti alcoolisti. RIV PATOL NERV. 8;544-549.
Marjama J, Yoshino MT, and Reese C. (1994) Marchiafava-Bignami Disease: Premortem Diagnosis of an Acute Case Utilizing Magnetic Resonance Imaging. Journal of Neuroimaging. 2;106-108.
Merritt HH. (1979) A Textbook of Neurology. Philadelphia: Lea & Febiger.
Rosa E, Demiati M, Cartz L, et al. (1992) Marchiafava-Bignami Disease, syndrome of interhemispheric disconnection and right handed agraphia in a left hander. Arch Neurol. 48;986-988.
Valk J, van der Knaap MS. (1989) Magnetic Resonance of Myelin, Myelination and Myelin Disorders. New York: Springer-Verlag.