A different problem in management may arise once the patient has recovered from Wernicke disease and the amnesic syndrome becomes prominent. Only a minority of such patients (fewer than 20 percent in Victor’s series) recover entirely; moreover, the time of recovery may be delayed for several weeks or even months, and then it proceeds very slowly over a period of many months. The extent to which the amnesic symptoms will recover cannot be predicted during the acute stages of the illness. Interestingly, some patients with alcoholism who develop Korsakoff syndrome, once more or less recovered, seldom demand alcohol but will drink it if it is offered.
This designates an acute and frequently fatal disease of infants, which until recently was common in rice-eating communities of the Far East. It affects only breast-fed infants, usually in the second to the fifth months of life. Acute cardiac symptoms dominate the clinical picture, but neurologic symptoms (aphonia, strabismus, nystagmus, spasmodic contraction of facial muscles, and convulsions) have been described in many of the cases. This syndrome can be reversed dramatically by the administration of thiamine, so that some authors prefer to call it acute thiamine deficiency in infants. In the few neuropathologic studies that are available, changes like those of Wernicke disease in the adult have been described. Occasionally, there are outbreaks of this condition due to inadequately formulated baby foods that lack thiamine.
Infantile beriberi bears no consistent relationship to beriberi in the mother. Infants of mothers with overt signs of beriberi may be quite normal. The absence of beriberi in the mothers of affected infants suggests that infantile beriberi might be due to the result of a toxic factor in breast milk, but such a factor, if it exists, has never been isolated. Rarely, the clinical manifestations of beriberi in infancy represent an inherited (autosomal recessive) thiamine-dependent state, responding to the continued administration of massive doses of thiamine (Mandel et al; see also Table 40-3, further on).
NUTRITIONAL POLYNEUROPATHY (NEUROPATHIC BERIBERI) (SEE ALSO CHAP. 43)
Beriberi is a distinct clinical entity characterized by a predominantly cardiac disorder occurring among people whose diet is dominated by polished rice. The milling process, or “polishing,” removes the husk that contains most of the vitamin nutrients. In fact, beriberi is not confined to any particular part of the world. In addition to affecting the heart, it also affects the peripheral nerves (which may be affected separately), with or without edema, the latter feature providing the basis for the old division into “wet” and “dry” forms. The cardiac manifestations range from tachycardia and exertional dyspnea to acute and rapidly fatal heart failure, the latter being the most dramatic but uncommon manifestation of beriberi. Here we emphasize the peripheral neuropathy associated with beriberi.
The notion that the neurologic manifestation of beriberi is essentially a peripheral neuropathy was established in the late nineteenth century by the studies of the Dutch investigators Eijkman, Pekelharing and Winkler, and