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The Wernicke-Korsakoff Syndrome

Wernicke disease and the Korsakoff amnesic state are common neurologic disorders that have been
recognized since the 1880s. Wernicke disease (originally called polioencephalitis hemorrhagica
superioris) is characterized by nystagmus, abducens and conjugate gaze palsies, ataxia of gait, and
mental confusion. These symptoms develop acutely or subacutely and may occur singly or, more
often, in various combinations. Wernicke disease is due specifically to a deficiency of thiamine and is
observed mainly though far from exclusively in alcoholics.
The Korsakoff amnesic state (Korsakoff psychosis) is a unique mental disorder in which retentive
memory is impaired out of proportion to all other cognitive functions in an otherwise alert and
responsive patient. This amnesic disorder, like Wernicke disease, is most often associated with
alcoholism and malnutrition, but it may be a symptom of various other diseases that have their basis
in lesions of the medial thalami or the inferomedial portions of the temporal lobes, such as infarction in
the territory of the temporal lobe branches of the posterior cerebral arteries, hippocampal damage
after cardiac arrest, third ventricular tumors, or herpes simplex encephalitis. A Korsakoff type of
memory disturbance may also follow lesions that involve the basal septal nuclei of the frontal lobe.
Transient impairments of retentive memory of the Korsakoff type may be the salient manifestations of
temporal lobe epilepsy, concussive head injury, and a unique disorder known as transient global
amnesia. The anatomic basis of the Korsakoff amnesic syndrome is discussed further in Chap. 21. In
the alcoholic, nutritionally deficient patient, Korsakoff amnesia is usually associated with and follows
the occurrence of Wernicke disease. Stated another way, Korsakoff psychosis is the psychic
manifestation of Wernicke disease. For this reason and others elaborated below, the term Wernicke
disease or Wernicke encephalopathy should be applied to a symptom complex of ophthalmoparesis,
nystagmus, ataxia, and an acute apathetic-confusional state. If an enduring defect in learning and
memory is
added, the symptom complex is appropriately designated as the Wernicke-Korsakoff syndrome.
Historical Note In 1881, Carl Wernicke first described an illness of sudden onset characterized by
paralysis of eye movements,
ataxia of gait, and mental confusion. Swelling of the optic discs and retinal hemorrhages were also said
to be present. His observations were made in three patients, of whom two were alcoholics and one was
a young woman with persistent vomiting following the ingestion of sulfuric acid. In each of these
patients there was progressive stupor and coma, culminating in death. The pathologic changes
described by Wernicke consisted of punctate hemorrhages,
primarily affecting the gray matter around the third and fourth ventricles and aqueduct of Sylvius; he
considered these changes to be inflammatory in nature and confined to the gray matter, hence his
designation “polioencephalitis hemorrhagica superioris.” In the belief that Gaˆyet had described an
identical disorder in 1875, the term
Gaˆyet-Wernicke is used frequently by French authors. Such a designation is hardly justified insofar as
the clinical signs and pathologic changes in Gaˆyet’s patient differed from those of Wernicke’s patients
in all essential details.
In a similar vein, a number of early writers, beginning with Magnus Huss in 1852, made casual
reference to a disturbance of memory in the course of chronic alcoholism. However, the first
comprehensive account of this disorder was given by the Russian psychiatrist S. S. Korsakoff in a series
of articles published between
1887 and 1891 (for English translation and commentary, see reference by Victor and Yakovlev).
Korsakoff stressed the relationship between “neuritis” (a term used at that time for all types of
peripheral nerve disease) and the characteristic disorder of memory, which he believed to be “two
facets of the same disease” and which he called “psychosis polyneuritica.” But he also made the
points, generally disregarded by subsequent authors, that neuritis need not accompany the
characteristic amnesic syndrome and that both disorders could affect nonalcoholic as well as alcoholic
patients. His clinical descriptions were remarkably complete and have hardly been surpassed to the
present day. It is of interest that the relationship between Wernicke disease and Korsakoff polyneuritic
psychosis
was appreciated neither by Wernicke nor by Korsakoff. Murawieff, in 1897, first postulated that a single
cause was responsible for both. The intimate clinical relationship was established by Bonhoeffer in
1904, who stated that in all cases of Wernicke disease he found neuritis and an amnesic psychosis.
Confirmation of this relationship on pathologic grounds came much later (for further details, see the
monograph by Victor et al). Clinical Features The incidence of the Wernicke-Korsakoff syndrome
cannot be stated with precision, but it has, until relatively
recent years, been a common disorder, judging from our experience. At the Cleveland Metropolitan
General Hospital, for example, in a consecutive series of 3548 autopsies in adults (for the period 1963
to 1976), our colleague M. Victor found the pathognomonic lesions in 77 cases (2.2 percent). The
disease affects males only slightly more often than females, and the age of onset is fairly evenly
distributed between 30 and 70 years. In the past two decades,
the incidence of the Wernicke-Korsakoff syndrome has fallen significantly in the alcoholic population.
However, it is being recognized with increasing frequency among nonalcoholic patients in a variety of
clinical settings, mainly iatrogenic ones. The triad of clinical features described by Wernicke—
ophthalmoplegia, ataxia, and disturbances of mentation and consciousness—is still clinically useful
provided that the diagnosis is suspected and the signs are carefully sought. Often the disease begins
with ataxia, followed in a few days or weeks by mental confusion;
or there may be the more or less simultaneous onset of ataxia, nystagmus, and ophthalmoparesis with
or without confusion. Less often, one component of this triad may be the sole manifestation of the
disease. Timely treatment with thiamine prevents the permanent amnesic component of the disease. A
description of each
of the major manifestations follows. Oculomotor Abnormalities The diagnosis of Wernicke disease is
made most readily on the basis of the ocular signs. These consist of (1) nystagmus that is both
horizontal and vertical, (2) weakness or paralysis of the lateral rectus muscles, and (3) weakness or pa-