[Wernicke-Korsakow syndrome: clinical aspects, pathophysiology and therapeutic approaches]. / Das Wernicke-Korsakow-Syndrom: Klinik, Pathophysiologie und therapeutische Ansätze.

In ICD-10 or DSM IV, the Wernicke-Korsakow Syndrome (WKS) is mentioned among the criteria for the alcohol amnestic disorder. Rarely seen in clinical practice, the Wernicke-Korsakow Syndrome is important in differential diagnosis of amnestic syndromes, because life-threatening conditions can occur. A specific treatment is available for the acute form of Wernicke encephalopathy, in many cases a Wernicke encephalopathy shows a progression to the Korsakow-Syndrome, which is the chronic form of this disease. The aim of this article is to review recent developments in clinical, etiological and pathophysiological research of WKS. The role of thiamin-dependent enzymes like transketolase and the importance of the NMDA (N-Methyl-D-Aspartate) System is discussed. New trends in diagnostics are shown, as are differential diagnosis and epidemiology. Finally, the rarely investigated drug therapy of the Korsakow syndrome with clonidine and fluvoxamine is reviewed.

Thiamine pyrophosphate effect and normalized erythrocyte transketolase activity ratio in Wernicke-Korsakoff patients and acute alcoholics undergoing detoxification.

Thiamine deficiency may be assessed clinically by an abnormally low specific erythrocyte transketolase activity and/or by abnormally large activation by thiamine diphosphate in vitro (or 'TPP effect'). In the present investigation, we report erythrocyte transketolase activation by TPP in acute alcoholics and Wernicke-Korsakoff patients undergoing detoxification. A new age-dependent parameter was used to improve the reliability of transketolase activity as an indicator of marginal thiamine deficiency. Thus normalized transketolase activity ratio (NTKZ), primary activation ratio (PAR) and further activation ratio (FAR) were measured in 29 acute alcoholics and 12 Wernicke-Korsakoff patients upon admission, and also on 47 control subjects. It was possible to follow up 14 of the 29 acute alcoholics after 7 days of treatment. Twenty-one per cent of the acute alcoholics and 33% of the Wernicke-Korsakoff patients, on admission to the detoxification Unit, had NTKZ values beyond the defined critical conditions for thiamine deficiency, whereas 7% of the former and 25% of the latter had PAR values beyond these critical conditions. Furthermore, all three parameters were significantly different in the Wernicke-Korsakoff patients compared to the other groups. The pattern of improvement of the different parameters on follow-up varied considerably and is difficult to explain, as only the NTKZ was statistically significant. Hence, only eight out of 14 acute alcoholics showed improvement in NTKZ, seven showed improvement of PAR and six showed improvement of FAR after treatment. Five patients showed improvement of both NTKZ and PAR and none of the patients showed improvement of all three parameters. We conclude that our findings confirm previous reports and that this modified transketolase activation test improves its reliability as an indicator of marginal thiamine deficiency.

Korsakoff minus Wernicke syndrome.

It is commonly believed that Korsakoff's amnesic state in alcoholics is characteristically the sequel to Wernicke's disease. However, in a study of 44 Korsakoff patients it was found that 33 had developed serious memory impairment without having had alarming neurological symptoms. This insidious nature of the onset of the disease indicates that all alcohol-dependent patients should be treated with vitamin B.

Blood and serum thiamin and thiamin phosphate esters concentrations in patients with alcohol dependence syndrome before and after thiamin treatment.

The blood and serum concentrations of free thiamin and its three phosphate esters were determined concomitantly by a new high-performance liquid chromatography (HPLC) method in 30 patients with alcohol dependence syndrome on admission to hospital and 24 hr after thiamin injection. We studied 24 men and 6 women; mean age, 50 years (range 21 to 69); mean ethanol consumption during the last 30 days, 164 +/- 119 g/day. A control group included 40 healthy volunteers (25 men, 15 women), of whom 10 were given the same thiamin injection as were the patients. Thiamin monophosphate was significantly reduced in the patients compared with controls before treatment (men 2.9 +/- 2.3 and 5.9 +/- 3.1 nmol/liter) and after (8.1 +/- 5.1 and 19.5 +/- 8.1 nmol/liter). On admission, free thiamin and thiamin diphosphate were similar in controls and in patients in whole blood (B) and serum (S) and increased similarly after treatment (mean B-thiamin diphosphate in male patients: 149 +/- 64 to 238 +/- 88 nmol/liter, in controls: 179 +/- 40 to 289 +/- 18 nmol/liter). However, seven patients had extremely high free thiamin values. The phosphorylation ratio was lower in patients than in controls (p less than 0.05), before and after treatment. Finally, the mean B-diphosphate was lower in patients not taking vitamins (116 +/- 48 nmol/liter and 172 +/- 57 nmol/liter) and in patients with polyneuropathy (118 +/- 54 nmol/liter and 173 +/- 52 nmol/liter), compared with the other patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Mental stimulation of a patient with Wernicke-Korsakoff's syndrome]. / Mental stimulering af en patient med Wernicke-Korsakoff's syndrom.

A mental stimulation programme employed in a woman aged 37 years suffering from Wernicke-Korsakoff's syndrome is described. This resulted in such improvement of her condition that full-time institutionalization could be avoided.

Treatment of chronic organic mental disorders associated with alcoholism.

Considering the magnitude of the problem, the treatment of alcoholism-associated chronic organic mental disorders has not been extensively studied. Alcoholic organic brain disease is heuristically viewed as the admixture of clinical syndromes of impairment [alcoholic amnestic disorder or Korsakoff's psychosis (KP) and dementia associated with alcoholism (DAA)], each with its distinguishing cognitive, neuropathological, and neurochemical characteristics. Differences between KP and DAA are highlighted by studies that compare KP patients with those having Alzheimer's disease or depression. Furthermore, treatment of cognitive deficits in KP and DAA may be modeled after strategies that have proved effective for these other neuropsychiatric disorders. Although abstinence and proper nutrition remain the cornerstones of treatment, pharmacological modification of neurotransmitter function and/or enhancement of cerebral metabolism combined with behavioral methods may also be beneficial. Serotonergic approaches to improve memory in detoxified alcoholics may also reduce alcohol intake, and this has implication for treatment of less impaired alcoholics.