Figure 1
Axial Flair T2WI
Axial Flair T2 weighted cerebral MRI showing hyperintense periaqueductal signal.
Wernicke encephalopathy complicating sclerodermic dysphagia
SectionNeuroradiology
Case TypeClinical Cases
AuthorsF.Z Laamrani1, M.Lahkim1, B.Redouane, T.Amil
Connected authors
45 years, female
Clinical History
A 45-year-old woman followed for sclerodermia complicated by dysphagia treated by corticoids, consulted in our training for recent apyretic temporo-spatial disorientation associated to cerebellar ataxia and nystagmus and severe malnutrition. Parenteral nutrition was advocated, and an adjustment of the initial treatment without improvement.
Imaging Findings
Cerebral MRI was performed in T2 and FLAIR T2-weighted sequences, and objectified a T2 and Flair T2 periacqueductal (Fig. 1), bithalamic (Fig. 2) and mammillary hypersignal (Fig. 3).
Discussion
Although the prevalence of leukoaraiosis, depression and cognitive impairment is higher in patients suffering from sclerodermia than in the normal population, brain lesions associated to sclerodermia are exceptional. An acute encephalopathy possibly developed in sclerodermia was therefore dismissed.
The clinical triad of temporo-spatial disorientation, cerebellar ataxia and ocular disorders was suggestive of Wernicke encephalopathy, especially with subsequent severe dinutrition due to sclerodermic dysphagia.
The pathogenesis of this encephalopathy is explained by a deficiency in vitamin B1, also called thiamine, occurring most often in the context of chronic alcoholism, anorexia, severe malnutrition, or pregnancy vomiting [1].
Early thiamine supplementation provides a favourable evolution, which was the case of our patient. Otherwise, irreversible lesions lead to a Korsakoff syndrome characterised by memory disorders.
The Wernicke encephalopathy is a medical emergency, requiring the administration of thiamine. The clinical triad is often incomplete; the positive diagnosis is based primarily on morphological arguments [2, 3].
CT has a low sensitivity to lesions and may be suggestive in only 13%.
The magnetic resonance imaging shows T2 and Flair T2 hyper intense mammillary bodies, thalami and Sylvius, with the possibility of contrast enhancement after gadolinium T1 weighted. The existence of these imaging features in these specific locations seems to be related to their metabolism based primarily on vitamin B1.
Thus, the combination of a clinical triad, and one of the lesions early mentioned is sufficient to evocate Wernicke encephalopathy in order to establish an appropriate treatment with thiamine. The evolution is related to the earliness of treatment initiation [2, 3].
The clinical triad of temporo-spatial disorientation, cerebellar ataxia and ocular disorders was suggestive of Wernicke encephalopathy, especially with subsequent severe dinutrition due to sclerodermic dysphagia.
The pathogenesis of this encephalopathy is explained by a deficiency in vitamin B1, also called thiamine, occurring most often in the context of chronic alcoholism, anorexia, severe malnutrition, or pregnancy vomiting [1].
Early thiamine supplementation provides a favourable evolution, which was the case of our patient. Otherwise, irreversible lesions lead to a Korsakoff syndrome characterised by memory disorders.
The Wernicke encephalopathy is a medical emergency, requiring the administration of thiamine. The clinical triad is often incomplete; the positive diagnosis is based primarily on morphological arguments [2, 3].
CT has a low sensitivity to lesions and may be suggestive in only 13%.
The magnetic resonance imaging shows T2 and Flair T2 hyper intense mammillary bodies, thalami and Sylvius, with the possibility of contrast enhancement after gadolinium T1 weighted. The existence of these imaging features in these specific locations seems to be related to their metabolism based primarily on vitamin B1.
Thus, the combination of a clinical triad, and one of the lesions early mentioned is sufficient to evocate Wernicke encephalopathy in order to establish an appropriate treatment with thiamine. The evolution is related to the earliness of treatment initiation [2, 3].
Differential Diagnosis List
Wernicke encephalopathy
Ischaemic lesions
Venous infarctus
Final Diagnosis
Wernicke encephalopathy
References
[1] Zuccoli G, Pipitone N. (2009) Neuroimaging findings in acute Wernicke\'s encephalopathy: review of the literature. AJR Am J Roentgenol ;192(2):501-8. (PMID: 19155417)
[2] Zuccoli G, Gallucci M, Capellades J, Regnicolo L, Tumiati B, Giadás TC, Bottari W, Mandrioli J, Bertolini M. (2007) Wernicke encephalopathy: MR findings at clinical presentation in twenty-six alcoholic and nonalcoholic patients. AJNR Am J Neuroradiol 28(7):1328-31. (PMID: 19844698)
[3] Sechi G, Serra A (2007) Wernicke’s encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet neurol 6(5):442-455 (PMID: 17434099)
Case information
| URL: | https://www.eurorad.org/case/11165 |
| DOI: | 10.1594/EURORAD/CASE.11165 |
| ISSN: | 1563-4086 |
Figure 2
Axial Flair T2WI
Axial Flair T2 weighted cerebral MRI showing hyperintense mammillary bodies.
Figure 3
Axial Flair T2WI
Axial T2 weighted cerebral MRI shows bi-thalamic hypersignal.
Axial Flair T2WI
Axial Flair T2 weighted cerebral MRI showing hyperintense periaqueductal signal.
Service de Radioloie. Hopital militaire d\'instruction Mohamed V.
Service de Radioloie. Hopital militaire d\'instruction Mohamed V.
Axial Flair T2WI
Axial Flair T2 weighted cerebral MRI showing hyperintense mammillary bodies.
Service de Radiologie . Hopital militaire d\'instruction Mohamed V.
Service de Radiologie . Hopital militaire d\'instruction Mohamed V.
Axial Flair T2WI
Axial T2 weighted cerebral MRI shows bi-thalamic hypersignal.
service de Radiologie. Hopital militaire d\'instruction Mohamed V.
service de Radiologie. Hopital militaire d\'instruction Mohamed V.