Acute demyelination in Marchiafavabignami syndrome: Axial DW images (a and b) show symmetric hyperintensities in the splenium of corpus callosum, which show reduced diffusivity on the ADC maps
Marchiafava–Bignami disease (MBD)
Marchiafava-Bignami Disease: Longitudinal MR Imaging and MR Spectroscopy Study
AJNR 2003 24: 249-253
Anna Gambinia, Andrea Falinia, Lucia Moiolab, Giancarlo Comia,b and Giuseppe Scotti
Summary: A case of Marchiafava-Bignami disease was serially evaluated with MR
imaging and MR spectroscopy at 1, 2, 4, and 11 months after the onset of symptoms.
The first MR imaging study showed extensive abnormal signal intensity of the corpus
callosum without macroscopic changes; a diagnosis of Marchiafava-Bignami disease
was made, and vitamin therapy was initiated.
Follow-up studies showed progressive reduction of signal intensity abnormalities and
residual callosal atrophy. MR spectroscopy revealed progressive reduction of the Nacetylaspartate:creatine ratio, with partial recovery in the last study, and a
normalization of the choline:creatine ratio, which was initially slightly increased.
Lactate was detectable during the subacute phase and was replaced by lipids after 4
months. This study confirmed the role of MR imaging in diagnosing MarchiafavaBignami disease and particularly the value of MR spectroscopy in focusing the
pathogenesis of the disease, monitoring its evolution and changes related to therapy.
MR Imaging Findings in 56 Patients with Wernicke Encephalopathy: Nonalcoholics
May Differ from Alcoholics
AJNR January 2009 30: 171-176
G. Zuccolia, D. Santa Cruzd, M. Bertolinib, A. Rovirae, M. Galluccif, C. Carollogand N. Pipitonec
BACKGROUND AND PURPOSE: Wernicke encephalopathy (WE) is a severe neurologic disorder
resulting from dietary vitamin B1 deficiency. This study was undertaken to analyze and compare MR imaging
findings and neurologic manifestations at clinical presentations of patients with WE with and without a
history of alcohol abuse.
MATERIALS AND METHODS: WE patients were identified using diagnostic neurologic data bases. 56
patients (29 females, 27 males) diagnosed between 1999 and 2008 with WE who improved within 1 month
from the onset of thiamine administration were included in the analysis. Patients’ records were reviewed for
clinical manifestations and imaging studies’ findings. MR imaging was performed in the acute phase of the
disease at a field strength of 1T (16 patients) and 1.5T (40 patients). All MR images were of acceptable to
good quality and were retrospectively reviewed. We compared imaging findings and clinical presentation in
the alcoholic (AL) group versus the non-alcoholic (NA) group using the 2-tailed Fisher exact test and the Phi
coefficient as appropriate.
RESULTS: Forty-three percent of the patients were in the AL group, whereas 57% were in the NA group.
Eighty-nine percent showed changes in consciousness, 75% had ocular manifestations, and 54% had ataxia.
On MR imaging, 80% of the patients had evidence of symmetric lesions in the medial thalami and in the
periventricular region of the third ventricle; 59%, in the periaqueductal area; 45%, in the mamillary bodies;
36%, in the tectal plate; and 7%, in the periventricular gray matter located anteriorly to the fourth ventricle.
Signal-intensity alterations in areas considered atypical for the disease were noted only in the NA group and
always in association with the typical findings. Contrast enhancement of the thalamus and mamillary bodies
was significantly associated with alcohol abuse.
CONCLUSIONS: Contrast enhancement in the mamillary bodies and thalamus is a typical finding of the
disease in AL patients. Atypical MR imaging findings characterize NA patients.
Topographic distribution of the lesions in AL and NA patients with WE†
Neurologic symptoms at clinical onset in the AL and NA groups
CC indicates changes in consciousness; OA, ocular abnormalities; AT,
ataxia; T, classic triad of the disease.
American Journal of Roentgenology 2009 192:2, 501-508
Neuroimaging Findings in Acute Wernicke's Encephalopathy: Review
of the Literature
Giulio Zuccoli1 and Nicolò Pipitone2
OBJECTIVE. Wernicke's encephalopathy is an acute neurologicalsyndrome resulting
from thiamine (vitamin B1) deficiency. Early recognition is important because timely
thiamine supplementation can reverse the clinical features of the disease. The aim of this
article is to provide an update on the typical and atypical neuroimaging findings of the
acute phase of the disease.
CONCLUSION. Wernicke's encephalopathy is characterized by a quite distinct pattern of
MR alterations, which include symmetrical alterations in the thalami, mamillary bodies,
tectal plate, and periaqueductal area, but atypical alterations may also been seen. A
thorough knowledge of the neuroimaging findings of Wernicke's encephalopathy will
assist in arriving at an early diagnosis, thus reducing the morbidity and mortality
associated with this disease.
61-year-old alcoholic man with Wernicke encephalopathy during acute phase of
disease. A, Axial T2-weighted image shows asymmetric edema of mamillary bodies
(arrows). B, Multiplanar gradient-recalled image shows blooming consistent with
hemorrhage (arrow) in left mamillary body. C, Symmetric involvement of medial
thalami (arrows) is seen on T2-weighted image. D, Contrast enhancement of mamillary
bodies (arrows) is seen on T1-weighted image.
53-year-old alcoholic man affected by Marchiafava-Bignami disease. A, Multiple
cavitations and atrophy of corpus callosum are noted (arrows) on sagittal T1weighted images. B, Axial FLAIR image shows cavitations of splenium of corpus
53-year-old alcoholic woman with Marchiafava-Bignami disease during subacute
phase. A, Sagittal FLAIR image shows signal intensity alteration involving inferior
aspect of corpus callosum (arrows). B, Axial FLAIR image depicts two curvilinear
hyperintensities (arrows) in splenium of corpus callosum.
Acute demyelination in Marchiafava bignami syndrome: Axial DW images (a and b) show
symmetric hyperintensities in the splenium of corpus callosum, which show reduced diffusivity
Marchiafava bignami syndrome: Axial DW image (a) shows symmetric hyperintensities in the splen
T2-w axial (b), coronal (c) and sagittal (f), and, FLAIR axial (d) and sagittal (e) images show
(A), diffusion-weighted image (DWI) (B), and the apparent diffusion coefficient (ADC)
map (C), showing hyperintense lesions in the precentral gyrus bilaterally and the
splenium of the corpus callosum.
(A), DWI(B), and ADC map (C) 17 days after admission, showing disappearance of the
(Left) Sagittal T2WI MR shows a swollen corpus callosum splenium with high signal in the
middle white matter layers (arrow) and peripheral sparing, classic for acute MarchiafavaBignami disease.
(Right) Axial FLAIR MR in the same case shows high signal in the corpus callosum splenium
(arrows) without other identifiable abnormalities. Male patient with a history of alcohol
abuse and seizures.
(Left) Axial T2WI MR shows symmetric hyperintensities in the medial thalami (arrows). Patient
also had hyperintensities in the periaqueductal gray matter (not shown), classic for WE.
(Right) Coronal T1 C+ MR in the same case shows enhancement in the tectal plate bilaterally
(arrows). Nonalcoholic WE seen in a patient who had had a bone marrow transplant and
This MRI shows typical high signal intensities (SIs) in the medial thalamus (A), periaqueductal gray
(B), mamillary bodies (C), cerebellar vermis (B, C, D), and paravermian superior cerebellum (D). All
the lesions represent high SIs on the DWI (E–H). The ADC images of the cerebellar vermis (K, L) and
paravermian superior cerebellum (L) show low SIs (arrowheads), whereas other described areas (I
J) show iso-SIs (arrows). Neurology. Apr 8 2008;70(15):e48.
Marchiafawa bignami disease possibly related to consumption of a
locally brewed alcoholic beverage: Report of two cases
Jagdeo P. Rawat, Charles Pinto1, Kapil S. Kulkarni, M. Ananthi K. Muthusamy, Malay
Marchiafava Bignami disease is a rare toxic disease seen mostly in chronic alcoholics,
resulting in progressive demyelination and necrosis of the corpus callosum. Initially it
was thought to be specific to individuals in central Italy, consuming large amounts of
Chianti red wine; however, alcoholic beverages worldwide are presently implicated. In
our case series of two cases, locally made “illicit” liquor (Mahuwa Alcohol) could be
the causative factor. In radiological point of view typically the corpus callosum is
affected, with involvement of the body, genu, and splenium in order of occurrence.
Occasionally the entire callosum may be also involved. Clinical presentation varies
from case to case.