CASE IN POINT An Atypical Long-Term Thiamine Treatment Regimen for Wernicke Encephalopathy Samra Shoaib, MD; Mehnaz Hyder, MD; and Margaret May, MD A patient with rapidly changing mental status responded to treatment with intramuscular or IV thiamine but not oral dosages, suggesting that presentation of thiamine deficiency can be highly variable, which can complicate the correct diagnosis and treatment. ernicke-Korsakoff syndrome is a exhibited none of the classical triad symp- Samra Shoaib is a Clinical cluster of symptoms attributed to a toms prior to death.4 Hence, these conditions Extern; Mehnaz Hyder is a Staff Psychiatrist, Inpatient Wdisorder of vitamin B1 (thiamine) are surmised to be significantly underdiag- Psychiatry; and Margaret deficiency, manifesting as a combined pre- nosed and misdiagnosed. May is a Staff Psychiatrist, sentation of alcohol-induced Wernicke Though consensus regarding the appro- Inpatient Psychiatry and Mental Health Evaluation encephalopathy (WE) and Korsakoff syn- priate treatment regimen is lacking for WE, Clinic; all at the Veterans 1 drome (KS). While there is consensus on a common protocol consists of high-dose Affairs Palo Alto the characteristic presentation and symp- parenteral thiamine for 4 to 7 days.5 This Health Care System in toms of WE, there is a lack of agreement is usually followed by daily oral thiamine California. Margaret May also is a Clinical Instructor on the exact definition of KS. The classic repletion until the patient either achieves (Affiliate), Department of triad describing WE consists of ataxia, complete abstinence from alcohol (ideal) or Psychiatry and Behavioral ophthalmoplegia, and confusion; however, decreases consumption. The goal is to allow Sciences, Stanford reports now suggest that a majority of pa- thiamine stores to replete and maintain at University School of Medicine in California. tients exhibit only 1 or 2 of the elements minimum required body levels moving for- Correspondence: of the triad. KS is often seen as a condition ward. In this case report, we highlight the Margaret May of chronic thiamine deficiency manifesting utilization of a long-term, unconventional ([email protected]) as memory impairment alongside a cogni- intramuscular (IM) thiamine repletion reg- Fed Pract. 2020;37(8):405-409. tive and behavioral decline, with no clear imen to ensure maintenance of a patient’s doi:10.12788/fp.0029 consensus on the sequence of appearance mental status, highlighting discrepancies of symptoms. The typical relationship is in our understanding of the mechanisms at thought to be a progression of WE to KS if play in WE and its treatment. untreated. From a mental health perspective, CASE PRESENTATION WE presents with delirium and confusion A 65-year-old male patient with a more whereas KS manifests with irreversible de- than 3-decade history of daily hard li- mentia and a cognitive deterioration. Though quor intake, multiple psychiatric hospi- it is commonly taught that KS-induced mem- talizations for WE, and a prior suicide ory loss is permanent due to neuronal dam- attempt, presented to the emergency de- age (classically identified as damage to the partment (ED) with increased frequency mammillary bodies - though other structures of falls, poor oral intake, confabulation, have been implicated as well), more recent and diminished verbal communication. A research suggest otherwise.2 A review pub- chart review revealed memory impairment lished in 2018, for example, gathered sev- alongside the diagnoses of schizoaffec- eral case reports and case series that suggest tive disorder and WE, and confusion that significant improvement in memory and was responsive to thiamine administration cognition attributed to behavioral and phar- as well as a history of hypertension, hy- macologic interventions, indicating this as an perlipidemia, osteoarthritis, and urinary area deserving of further study.3 About 20% retention secondary to benign prostatic of patients diagnosed with WE by autopsy hyperplasia (BPH). mdedge.com/fedprac SEPTEMBER 2020 • FEDERAL PRACTITIONER • 405 Wernicke Encephalopathy FIGURE Patient Treatment Timeline First Hospitalization Day 1 Day 4 Day 15 Day 22 Day 27 Day 42 Day 80 Day 83 Day 96 Day 126 Admitted with Actively Lithium Fall, confusion, IM thiamine Improved IM thiamine Lethargic, Slight Discharged poor oral intake, suicidal, started, confabulation, started appetite tapered in nonverbal, improvement; to SNF mutism, self-injurious disorganized and decreased at 200 mg and preparation unsteady thiamine confusion, and behaviors thinking oral intake twice daily interaction; for gait; IM increased to gait issues; continued continued; lithium no falls/ discharge thiamine 3 times daily paliperidone stopped due to confusion returned to started; oral elevated creatinine twice daily thiamine initiated Second Hospitalization (within 24 h of discharge) Day 0 Day 1 Day 4 Day 12 Missed 2 doses of Admitted with AMS, ataxia, Patient activated, No AMS/mood symptoms IM thiamine disorganized speech, and fall; perseverating to go home; or gait disturbance; patient IV thiamine initiated thiamine therapy continued discharged Abbreviations: AMS, altered mental status; IM, intramuscular; SNF, skilled nursing facility. On examination the patient was found loss compared with the pattern typically to be disoriented with a clouded sensorium. seen in NPH. While the history of heavy daily alcohol In light of concern for WE and the pa- use was clear in the chart and confirmed by tient's history, treatment with IV thia- other sources, it appeared unlikely that the mine and IV fluids was initiated and the patient had been using alcohol in the pre- Liaison Psychiatry Service was consulted ceding month due to restricted access in his for cognitive disability and treatment of most recent living environment (a shared his mood. Administration of IV thiamine apartment with daily nursing assistance). rapidly restored his sensorium, but he He reported no lightheadedness, dizziness, became abruptly disorganized as the IV reg- palpitations, numbness, tingling, or any imen graduated to an oral thiamine dose of head trauma. He also negated the presence 200 mg 3 times daily. Simultaneously, as of active mood symptoms, auditory or vi- medical stabilization was achieved, the pa- sual hallucinations or suicidal ideation (SI). tient was transferred to the inpatient psychia- The patient was admitted to the Inter- try unit to address the nonresolving cognitive nal Medicine Service and received a workup impairment and behavioral disorganization. for the causes of delirium, including con- This specifically involved newly emerging, sideration of normal pressure hydrocepha- impulsive, self-harming behaviors like throw- lus (NPH) and other neurologic conditions. ing himself on the ground and banging his Laboratory tests including a comprehensive head on the floor. Such behaviors along with metabolic panel, thyroid stimulating hor- paucity of speech and decreased oral intake, mone, urinalysis, urine toxicology screen, ultimately warranted constant observation, and vitamin B12 and folate levels were in which led to a decrease in self-harming activ- normal ranges. Although brain imaging re- ity. All this behavior was noted even though vealed enlarged ventricles, NPH was con- the patient was adherent to oral adminis- sidered unlikely because of the absence of tration of thiamine. Throughout this time, ophthalmologic abnormalities, like gaze the patient underwent several transfers back nystagmus, and urinary incontinence; con- and forth between the Psychiatry and Inter- versely, there was some presence of urinary nal Medicine services due to ongoing con- retention attributed to BPH and required cern for the possibility of delirium or WE. an admission a few months prior. More- However, the Neurology and Internal Med- over, magnetic resonance images showed icine services did not feel that WE would that the ventricles were enlarged slightly explain the patient’s mental and behavioral out of proportion to the sulci, which can status, in part due to his ongoing adherence be seen with predominantly central volume with daily oral thiamine dosing that was 406 • FEDERAL PRACTITIONER • SEPTEMBER 2020 mdedge.com/fedprac Wernicke Encephalopathy not associated with improvement in mental versations. Some speech content remained status. disorganized particularly if engaged beyond Recollecting the patient’s improvement simple exchanges. with the parenteral thiamine regimen (IV The patient was discharged to a skilled and IM), the psychiatry unit tried a thia- nursing facility after a month of 3 times daily mine regimen of 200 mg IM and 100 mg oral IM administration of thiamine. Within the 2 times daily. After about 2 weeks on this reg- next 24 hours, the patient returned to the imen, the patient subsequently achieved re- ED with the originally reported symptoms markable improvement in his cognitive and of ataxia, agitation, and confusion. On in- behavioral status, with resolution of self- quiry, it was revealed that the ordered vials harming behaviors. The patient was noted of IM thiamine for injection had not arrived to be calmer, more linear, and more oriented, with him at the nursing facility and he had though he remained incompletely oriented missed 2 doses. The blood laboratory results, throughout his hospitalization. As improve- scans, and all other parameters were other- ment in sensorium was established and the wise found to be normal and the patient was patient’s hospital stay prolonged (Figure),