Research Article, J Addict Behav Ther Rehabil Vol: 8 Issue: 1
A Woman with Alcohol Use Disorder, Wernicke-Korsakoff Syndrome, and Central Pontine Myelinolysis: A Case Report and Review of the Literature
1Department of Internal Medicine, Greenwich Hospital, Yale New Haven Health, 5 Perry Ridge Road, Greenwich, CT, USA
2Department of Clinical Medicine, Yale University School of Medicine, Assistant Program Director, Internal Medicine Residency, Greenwich Hospital, 5 Perry Ridge Road Greenwich, CT, USA
*Corresponding Author : Annette Lukose
PGY-1 Resident, Internal Medicine, Greenwich Hospital, Yale New Haven Health,5 Perry Ridge Road, Greenwich, CT, USA
Fax: (203) 863-3924
Tel: (203) 863-3000
E-mail: annette.lukose@greenwichhospital.org
Received: February 11, 2019 Accepted: February 21, 2019 Published: February 22, 2019
Citation: Lukose A, Tyer NM (2019) A Woman with Alcohol Use Disorder, Wernicke-Korsakoff Syndrome, and Central Pontine Myelinolysis: A Case Report and Review of the Literature. J Addict Behav Ther Rehabil 8:1. doi: 10.4172/2324-9005.1000186
Abstract
We present a case report of a woman with a history of alcohol use disorder who was found to have both Wernicke-Korsakoff Syndrome (WKS) and Central Pontine Myelinolysis (CPM) on MRI. After a discussion of the case, we review the literature of the clinical manifestations, diagnostic criteria, and prognostic indicators of functional outcomes for both WKS and CPM. We compare and contrast the two diseases individually and simultaneously, as occurred in our patient
Keywords: Alcohol use disorder; Wernicke-Korsakoff Syndrome; Central Pontine Myelinolysis; Encephalopathy; Hypothyroidism
Introduction
Wernicke-Korsakoff syndrome (WKS) is a spectrum of neurologic disorders caused by thiamine deficiency, usually in the context of chronic alcohol use disorder and poor nutrition [1]. Wernicke’s encephalopathy (WE), the acute form the disease, is characterized by the classic triad of nystagmus, encephalopathy, and ataxia. Central pontine myelinolysis (CPM) is a demyelinating disorder of the brainstem that typically occurs with the rapid correction of serum sodium in hyponatremic patients. Diagnosis of both WKS and CPM requires a high index of suspicion. Though overlapping in some aspects of clinical manifestations, the prognosis and functional outcomes can differ between the two diseases so it is important to distinguish between the two processes.
Clinical Case
A 70-year-old female retired school teacher presented to our emergency room within 2 weeks of worsening confusion, blurred vision, difficulty walking, and short-term memory loss. Medical history was significant for hypothyroidism and alcohol use disorder with a previous ICU admission for alcohol withdrawal six months prior. Alcohol intake was estimated to be approximately 3-5 glasses of wine per day, though length of use was unclear; last drink was reportedly 3 days prior to presentation.
Neurologic exam revealed horizontal nystagmus, poor concentration and recall, tangential conversation with confabulation, and wide-based gait. Extensive work-up showed no abnormalities in the complete metabolic panel, CBC, TSH, Vitamin B12, HbA1c, ammonia, urinalysis, urine toxicology, and EEG. She tested negative for HIV, Lyme disease, and Syphilis. Lumbar puncture revealed normal protein, glucose, and negative gram stain and culture. CSF was negative for West Nile Virus, HSV, and VZV. The notable findings on work up were: a low thiamine level of 6 nmol/L (normal 8-30 nmol/L), and T2-FLAIR MRI of the brain with atypical white matter signal alteration of the central pons consistent with central pontine myelinolysis.
The constellation of symptoms (nystagmus, confusion, ataxia, and amnesia) in the setting of alcohol use disorder and low thiamine led to a working diagnosis of Wernicke-Korsakoff Syndrome. However, MRI findings in WKS typically reveal abnormalities in the mammary bodies and periventricular regions, not the pons.
Central pontine myelinolysis is usually iatrogenic from rapid correction of serum sodium. On further questioning, it was discovered that the patient was hospitalized at an OSH (Outside Hospital) for Legionnaire’s Disease six months prior to presentation. It was hypothesized that she suffered a rapid correction of hyponatremia at that time which resulted in mild, unrecognized central pontine myelinolysis. Outside medical records were reviewed, but there was no evidence for hyponatremia to support this hypothesis. On review of the literature, central pontine dysfunction on MRI has been associated with WKS.
Intravenous thiamine was given, and the patient was discharged home with oral thiamine, outpatient cognitive therapy, and alcohol cessation management. On her post-discharge follow-up, she showed improvement in her short-term memory and returned to baseline shortly after discharge, which is seen in only a minority of patients with WKS.
Interestingly, the patient re-presented to the ED six months after hospitalization with a similar presentation of acute onset confusion, this time with a normal thiamine level. Her work-up was unrevealing and MRI was unchanged, revealing stable white matter signal alteration of central pons. She was again admitted to the hospital and treated with IV thiamine for WE with improvement in her mental status during her hospital stay.
Discussion
Wernicke-Korsakoff syndrome is a spectrum of neurologic disorders caused by thiamine deficiency, usually in the context of chronic alcohol use disorder and poor nutrition [1]. Wernicke’s encephalopathy, the acute form the disease, is characterized by the classic triad of nystagmus, encephalopathy, and ataxia. The prognosis of the disease is dependent on prompt treatment with IV thiamine as well as alcohol cessation [2]. Additionally, a retrospective study of 25 patients with WKS also noted that prognosis in WKS is favorable when there are fewer radiologic abnormalities detected at onset. The MRI of patients with WKS may reveal white signal abnormalities in the mammary bodies and periventricular regions, and therefore, the presence of radiological signs may also be helpful in diagnosis and prognosis.
| Wernicke’s Encephalopathy | Korsakoff Syndrome | Central Pontine Myelinolysis | |
|---|---|---|---|
| Clinical Presentation: | Wernicke’s Encephalopathy is an acute condition, characterized by triad of confusion, oculomotor dysfunction, and gait ataxia. Symptomatic improvement with thiamine administration and cessation of alcohol. | Korsakoff syndrome typically occurs in patients who have history of Wernicke’s Encephalopathy and will present as chronic selective short-term and long-term memory difficulties, confabulation, and hallucinations. The memory impairment is often irreversible. No or minimal improvement with thiamine administration and cessation of alcohol. | Presentation is highly variable, ranging from asymptomatic to ataxia to coma. Neurologic and neuropsychiatric manifestations include confusion, personality changes, acute psychosis, hallucinations, and catatonia. Motor manifestations include rapidly evolving paraparesis, quadriparesis, pseudo bulbar symptoms (dysphagia, dysarthria), locked-in syndrome. |
| Diagnostic Criteria: | High index of suspicion in alcoholic patient with triad of acute delirium, ataxia, and nystagmus. Diagnosis is clinical and requires demonstration of low thiamine levels. | High index of suspicion in alcoholic patient with WE presenting with new amnesia (anterograde and/or retrograde), confabulation, and lack of insight. Diagnosis is clinical and requires demonstration of low thiamine levels. | High index of suspicion in hyponatremic patient with rapid overcorrection of serum sodium. Diagnosis is confirmed by imaging revealing symmetrical hypo intense lesions of the pons on T1-weighted or T2-weighted MRI. |
| Predictors of Favorable Outcome: | Early treatment with IV thiamine. Abstinence from alcohol. Lack of radiologic abnormalities on MRI. | Lack of radiologic abnormalities on MRI. | Higher GCS scores. Better scores in functional scales in hospital. Less severe hypernatremia. Chronic hypernatremia. Absence of hypokalemia. |
Table 1: Clinical Presentation, Diagnostic Criteria, and Predictors of Favorable Outcome in Wernicke’s encephalopathy, Korsakoff Syndrome, and Central Pontine Myelinolysis.
When WE is not treated, around 80% of patients will advance to the chronic form of the disease, Korsakoff Syndrome (KS). Clinical manifestations of KS include the classic WE triad as well as amnesia and confabulation [3]. KS generally carries a poorer prognosis compared to WE, as KS is typically irreversible despite treatment with thiamine and alcohol cessation. In contrast to patients with WE, patients with KS rarely recover and will require some form of social support.
Central pontine myelinolysis is a demyelinating disorder of the brainstem and typically occurs with the rapid correction of serum sodium in hyponatremic patients [4]. As with WKS, CPM is also seen in the setting of nutritional and electrolyte stress, including chronic alcohol use disorder and malnourishment . The diagnosis of CPM is based on correlation of clinical findings with radiologic studies, in which an MRI reveals a symmetric area of central pontine demyelination .The treatment of CPM is usually supportive. Interestingly, the clinical presentation of CPM can vary from asymptomatic, to neuropsychiatric, to paralysis, coma, or death [5]. The neurological findings that were seen in our patient include disinhibiting, confusion, impaired cognition, and gait instability. It is important to note that it is difficult to distinguish the mild forms of CPM from WKS as both diseases present with similar neurological findings.
While CPM can vary from incidental finding to a fatal diagnosis, there are only a few studies that examine the prognostic indicators and functional outcomes. A retrospective cohort study of 25 patients with CPM demonstrated that higher GCS (Glasgow Coma Scale) scores (≥ 11), better scores in functional scales, less severe hyponatremia ([Na+] > 115 mEq/L), and absence of superadded hypokalemia predict favorable outcome [6]. Another article reviews the homeostatic mechanism by which severe acute hyponatremia (serum [Na+] dropping to < 120 mEq/L in ≤ 48-hours) causes a more serious presentation compared to a slowly progressive chronic hyponatremia [7]. If untreated, severe acute hyponatremia may lead to brain edema, irreversible neurologic damage, respiratory arrest, brainstem herniation, and even death. In contrast, in chronic or less severe hyponatremia, symptoms usually do not arise until the serum [Na+] falls below 120 mEq/L and they are initially nonspecific (e.g., headache, lethargy, nausea) [8].Ultimately, however, the rate of sodium correction is more important than the nadir sodium concentration in the development of CPM.
Unlike WKS, where clinical outcome was favorable when fewer radiological signs were detected at onset, the extent of pontine signal abnormality in CPM does not predict clinical outcome. A retrospective cohort study of 24 patients with CPM concluded that the volume of demyelination on MRI was not associated with disease severity. This information is helpful and can prevent both premature and pessimistic prognostication based on radiologic abnormalities [9].
In patients with WKS complicated by CPM, as in our patient, it is especially important to consider the prognostic factors in the context of both WKS and CPM, and to assess the patient’s functional status prior to discharge. Using the prognostic factors for WKS we describe in this paper, our patient had only one favorable predictor in the context of WKS, which was immediate treatment with IV thiamine. It is also important to note that she had improvement in her short-term memory, a finding that is rare in patients with WKS. Additionally, the presence of MRI abnormalities is an unfavorable prognostic indicator for our patient in the context of WKS.
Using the prognostic factors of CPM we describe in this paper, our patient had several findings that would predict favorable outcome: no evidence of hyponatremia or hypokalemia, functional independence (ambulation without assistance), and GCS score of 13-14 throughout hospital stay.
Conclusion
In addition to treating the patient for WKS with thiamine, CPM in the setting of WKS also necessitates assessment of the patient’s functional status. Only a few small studies exist that examine the predictors of disease severity and functional outcomes in WKS and CPM. There is very little literature about patients with both concurrent WKS and CPM as in our patient. This case is important because it highlights the subtle and overlapping differences between WKS and CPM from a clinical, diagnostic, and outcomes perspective.
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