Serial MRI and 1H-MRS of Wernicke's encephalopathy: report of a case with remarkable cerebellar lesions on MRI

https://doi.org/10.1016/S0925-4927(01)00304-3Get rights and content

Abstract

Before and after the administration of thiamine (vitamin B1), MRI and proton magnetic resonance spectroscopy (1H-MRS) were serially performed in a patient with Wernicke's encephalopathy demonstrating remarkable cerebellar lesions on MRI. Before thiamine administration, high signal intensities were observed in the thalamus around the third ventricle and in the superior portion of the cerebellar vermis and hemisphere on fluid-attenuated inversion recovery (FLAIR) and T2-weighted MR images. After thiamine administration, the high signal intensity in the former region disappeared immediately, while that in the latter regions persisted. The low level of N-acetylaspartate (NAA)/creatine (Cr) in the thalamus before thiamine administration improved to some degree on the 1[H]-MRS images taken after thiamine administration. In the cerebellum, a lactate peak was observed before thiamine administration, and the NAA/Cr level did not improve after thiamine administration, suggesting that irreversible necrosis occurred. It is suggested that serial MRI/1H-MRS observation may be helpful in determining the neuronal viability of Wernicke's encephalopathy and the prognostic implications of sequelae such as Korsakoff's syndrome and cerebellar ataxia.

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.

Section snippets

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

References (28)

  • M. Cole et al.

    Extra-ocular palsy and thiamine therapy in Wernicke's encephalopathy

    American Journal of Clinical Nutrition

    (1969)
  • R. Kreis et al.

    Absolute quantitation of water and metabolites in the human brain. II. Metabolite concentrations

    Journal of Magnetic Resonance Series B

    (1993)
  • P.B. Barker et al.

    Quantitative proton spectroscopy of canine brain: in vivo and in vitro correlations

    Magnetic Resonance in Medicine

    (1994)
  • J.P. Bonjour

    Vitamins and alcoholism. IV. Thiamine

    International Journal for Vitamin and Nutrition Research

    (1980)
  • R.E. Brenner et al.

    The proton NMR spectrum in acute EAE: the significance of the change in the Cho:Cr ratio

    Magnetic Resonance in Medicine

    (1993)
  • R.F. Butterworth et al.

    Thiamine-dependent enzyme changes in the brains of alcoholics: relationship to the Wernicke–Korsakoff syndrome

    Alcoholism: Clinical and Experimental Research

    (1993)
  • J.F. Donnal et al.

    MR of reversible thalamic lesions in Wernicke syndrome AJNR

    American Journal of Neuroradiology

    (1990)
  • J.F. Feinberg

    The Wernicke–Korsakoff syndrome

    American Family Physician

    (1980)
  • J. Frahm et al.

    Localized proton NMR spectroscopy in different regions of the human brain in vivo. Relaxation times and concentrations of cerebral metabolites

    Magnetic Resonance in Medicine

    (1989)
  • M. Gallucci et al.

    Wernicke encephalopathy: MR findings in five patients. AJNR

    American Journal of Neuroradiology

    (1990)
  • J.V. Hajnal et al.

    Use of fluid attenuated inversion recovery (FLAIR) pulse sequences in MRI of the brain

    Journal of Computer Assisted Tomography

    (1992)
  • A.M. Hakim

    The induction and reversibility of cerebral acidosis in thiamine deficiency

    Annals of Neurology

    (1984)
  • C.G. Harper

    The incidence of Wernicke's encephalopathy in Australia: a neuropathological study of 131 cases

    Journal of Neurology, Neurosurgery and Psychiatry

    (1983)
  • H. Lee et al.

    In vivo localized proton NMR spectroscopy of thiamine-deficient rat brain

    Magnetic Resonance in Medicine

    (1995)
  • Cited by (0)

    View full text