Serial MRI and 1H-MRS of Wernicke's encephalopathy: report of a case with remarkable cerebellar lesions on MRI
Introduction
Wernicke's encephalopathy, which results from a deficiency of thiamine (vitamin B1), shows the three classical signs of progressive unconsciousness, ataxia and ophthalmoplegia (Victor et al., 1971), and is characterized by specific lesions localized to parts of the brain such as the thalamus, periventricular region and mammillary body (Harper, 1983, Yokote et al., 1991).
It was also reported that lesions in the cerebellum, which have been detected in more than half of patients with Wernicke's encephalopathy on neuropathological autopsy, are closely related to ataxia (Victor et al., 1971, Victor et al., 1989). Activities of the thiamine-dependent enzyme alpha-ketoglutarate dehydrogenase (αKGDH), a rate-limiting tricarboxylic acid cycle enzyme, are significantly reduced in autopsied cerebellar vermis samples from patients with Wernicke's encephalopathy (Butterworth et al., 1993). Concerning in vivo cerebellar neuroimaging associated with alcohol-related disorders, Sullivan et al. (2000) published a large controlled quantitative MRI study on alcoholism and Korsakoff's syndrome, and reported significant vermian volume deficits and a relationship between a quantitative ataxia measure and the volume deficits. Proton magnetic resonance spectroscopy (1H-MRS) studies have shown metabolite abnormalities in the cerebellar vermis in chronic uncomplicated alcoholic patients and their normalization after abstinence (Martin et al., 1995, Seitz et al., 1999). However, there have been few studies that longitudinally evaluated MRI/1H-MRS abnormalities in the cerebellum from the acute stage to the transitional stage to Korsakoff's syndrome in patients with Wernicke's encephalopathy.
When thiamine is replenished in the early period, good recovery can be anticipated (Cole et al., 1969, Bonjour, 1980); otherwise severe and irreversible sequelae such as cerebellar symptoms and Korsakoff's syndrome may persist (Victor et al., 1971, Feinberg, 1980). To search for indices that can provide clues to the threshold of reversibility with treatment and to the pathophysiology of Wernicke's encephalopathy, further studies are needed. In the present study, we serially performed MRI and 1H-MRS immediately after onset in a patient with acute Wernicke's encephalopathy demonstrating remarkable cerebellar lesions on MRI, and examined reactivity to thiamine and the usefulness of MRI and 1H-MRS to determine the prognostic implications of sequelae such as Korsakoff's syndrome and cerebellar ataxia.
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Case report
The patient was a 79-year-old male paperhanger who developed a cerebral infarction at the age of 70 years. The patient, who had drunk approximately 550 ml sake (rice wine) at supper every night for 40 years, had an independent daily life. After eating poorly for several days due to a cold, the patient demonstrated gait disturbance and inconsistent speech and behavior around 20 January, 2000, and was admitted to our hospital on 25 January.
On admission, the level of consciousness was 13/15 (E
Discussion
The patient was considered to suffer from chronic thiamine deficiency due to chronic alcoholism, and demonstrated acute Wernicke's encephalopathy induced by acute pharyngolaryngitis. Among the main clinical symptoms of Wernicke's encephalopathy, ophthalmoplegia and polyneuropathy are rapidly improved by thiamine supplementation (Cole et al., 1969, Bonjour, 1980), while cerebellar symptoms and Korsakoff's syndrome often persist for a prolonged period and are considered irreversible (Victor et
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