Wernicke-Korsakoff Syndrome Clinical Presentation

Ocular/visual disturbances include the following:

• Painless vision abnormalities
• Diplopia (double vision)
• Strabismus

Gait abnormalities include the following:

• New, wide-based, short-stepped gait
• Inability to stand or walk without assistance

Mental status changes include the following:

• Apathy, indifference, paucity of speech
• Hallucination, agitation
• Confabulation - Patient fills in gaps of memory with data that can be recalled at that moment

The classical triad of confusion, ataxia, and nystagmus is only present in about 16-38% of patients.^[11]

Ocular abnormalities

The diagnosis of Wernicke encephalopathy is made most reliably on the basis of the following ocular abnormalities, which can occur singly or in combination:

• Nystagmus, vertical and horizontal
• Weakness or paralysis of lateral rectus muscles - Occurs bilaterally but can be asymmetrical and is accompanied by diplopia and internal strabismus
• Weakness or paralysis of conjugate gaze
• Nonreacting miotic pupils and complete loss of ocular movements (in advanced cases)
• Ptosis, small retinal hemorrhages, involvement of near-far focusing mechanism, and optic neuropathy (occasionally)
• Papilledema (very rare)

Ataxia

Ataxia is manifested as an abnormality of stance and gait. Vestibular paresis also plays a role in ataxia in the early stages of disease. The following characteristics are demonstrated by the patient:

• Mildest form evident on tandem walking only
• Wide-based stance
• Slow and uncertain, short-stepped gait
• In the most severe form, inability to walk without support
• Abnormal results on caloric testing (indicates vestibular paresis)

Mental status

Alterations in consciousness can present simultaneously with ophthalmoplegia and ataxia but more commonly follow these signs and symptoms by days to weeks. These changes develop in 90% of patients and present in various forms.

Patients with long-term alcoholism are likely to present disheveled and unkempt, but appearance on presentation can range to a well-kept individual. No characteristic perceptual disturbances exist, but those of delirium tremens are present if it coexists.

The patient’s mood can range from calm and blunted or apathetic affect to stupor; agitation can occur in acute delirium and tremens can be found in a patient with alcohol withdrawal. The rare patient presenting in the Korsakoff amnestic state is alert and oriented but lacks the ability to provide adequate history.

No characteristic speech pattern exists. Vocal tremor may be present in a patient undergoing alcohol withdrawal. Reduced verbal content may occur in those with apathy.

Form and content of thought vary depending on patient presentation. Themes may include a lack of concern about the patient’s own current health status or state of affairs.

Sensorium and cognition vary with the level of consciousness. The patient may be in a state of altered sensorium, with decreased attention and concentration (inability to perform "serial 7's" or spell "WORLD" backwards); disorientation is present in the acute state, consistent with other delirium (or encephalopathy). For a patient who is not in delirium, impaired recall or orientation to date or location may occur. Knowledge of historical facts (eg, naming of presidents) is often impaired in persons with Korsakoff syndrome. A patient may cover up the memory deficit by confabulating information.

Suicidal or homicidal ideation is generally not associated with this disorder, although any person in the midst of delirium can become self-injurious or violent.

A global confusional state is the most common early manifestation and is characterized by apathy, inattentiveness, and indifference to surroundings. Spontaneous speech is minimal, and provoked speech indicates general disorientation with regard to time, place, and purpose. Prompt administration of thiamine often results in increased attentiveness and orientation.

Stupor or coma can be observed in more severe cases but is rare as an initial presentation. If patients remain untreated, the condition will progress to death, as in the initial cases described by Wernicke.

Patients may present with varying degrees of alcohol withdrawal. Alcohol use is the most common etiology leading to a poor nutritional state that results in Wernicke-Korsakoff syndrome.

Korsakoff amnestic state

The Korsakoff amnestic state is observed in a small number of patients. Individuals present as alert and responsive. On examination, they demonstrate the amnestic features of Korsakoff psychosis as the only manifestation of mental confusion. This state appears after the initial confusional state begins to resolve with thiamine administration and persists to some degree in the most severely affected individuals.

The Korsakoff state is characterized by anterograde (ie, learning) and retrograde (ie, memory of past events) amnesia. Anterograde amnesia is severe but incomplete. It is demonstrated by the ability of the patient to repeat a series of numbers or objects as they are stated, along with an inability to recall the registered information after 3-5 minutes.

Retrograde amnesia is demonstrated by gaps in patients' memories of the recent and remote past that antedate the onset of illness. These gaps in memory are what lead to the characteristic feature of confabulation.

Confabulation represents filling in of memory gaps with data the patient can readily recall. Debate continues as to whether this action represents a deliberate attempt by patients to hide their memory deficits. In either case, confabulation is a fascinating defense mechanism. Confabulation is classically described in Korsakoff dementia, although it may be present in other dementias and does not necessarily need to be present for a diagnosis to be made.

In actual patient scenarios, the patient often greets the examiner cordially as if he or she knows the individual, despite having never met him or her. When asked about prior encounters, the patient may tell the examiner, for example, that he or she met the examiner 2 weeks ago in the hospital but does not precisely recall the topic of the conversation. The patient may then proceed to tell the examiner that currently he or she is doing well and provide a basic history about current symptoms, as well as express uncertainty about where he or she will live. However, when asked about the year and the president, the patient might reply (in many cases, without hesitation) that the year is 1955 and that the president is Dwight Eisenhower, even though the actual year is 2011 and the president is Barack Obama.

In such cases, the examiner may not have detected any deficits until specific orientation questions were asked. Other aspects of conversation generally lack specificity and/or depth.

Other manifestations

Hypothermia presents secondary to damage in temperature regulating centers. Associated peripheral neuropathy is found in 80% of patients.

Cardiovascular dysfunction may be observed. Overt signs of beriberi heart disease are rare in patients with Wernicke-Korsakoff syndrome. The following symptoms may be observed and generally improve with administration of thiamine:

• Postural hypotension
• Tachycardia
• Syncope