Nystagmus in a child with nephrotic syndrome.

We report a child with steroid-dependent nephrotic syndrome presenting with excessive irritability, double vision and inability to walk for 5 days. On examination, the child was irritable with Glasgow coma sccale (GCS of 12/15, had bilateral convergent squint (R>L), vertical nystagmus, ataxia without any focal neurological deficits and normal fundus. MRI brain with venogram showed bilateral symmetric FLAIR hyperintensity in the medial thalamus and periaqueductal grey matter showing diffuse restriction with normal venogram. A possibility of Wernicke encephalopathy (WE) was considered and the child was started on thiamine supplementation, following which he had significant improvement in his symptoms. His irritability reduced with significant improvement in the range of eye movements and vertical nystagmus. At 3-month follow-up, the child is asymptomatic with normal gait. Although WE is uncommon in children with nephrotic syndrome, the possibility has to be kept in mind when a child presents with atypical neurological symptoms.

Atypical Wernicke's encephalopathy without mental status changes following bariatric surgery in an adolescent patient.

Morbid obesity is a systemic disease which can result in chronic complications, including hypertension, diabetes mellitus, depression, osteoarthritis and low self-esteem in the adolescent population.Bariatric surgery can be indicated to treat more severe forms of obesity, but these procedures are not without long-term risks. Therefore, adequate preoperative and postoperative care, which includes preoperative psychosocial evaluation for compliance, ongoing nutrition counselling and vitamin and micronutrient supplementation, is required for all patients, especially adolescent patients, who generally may not comply with medical therapies and/or be able to developmentally fully appreciate or comprehend the health consequences of their behaviours, prior to as well as after bariatric surgery to prevent complications.Thiamine pyrophosphate, an active form of thiamine (also known as vitamin B1, a water-soluble vitamin), which functions as a coenzyme in glucose and energy metabolism, is one such vitamin that requires supplementation postoperatively. It is mandatory for glucose to be administered concomitantly with thiamine, as glucose alone can precipitate Wernicke's encephalopathy (WE) in thiamine-deficient individuals. WE is a medical emergency, with a mortality rate of up to 20%. WE is best understood as a classic triad of mental confusion, gait ataxia and eye movement abnormalities, and atypical WE or Wernicke's syndrome (WS) is seen when the classic triad is not present. Cases that meet some, but do not necessarily meet all three criteria, are referred to as atypical WE or WS which can lead to delayed diagnosis. Atypical WE has an incidence of 19% which can lead to misdiagnosis of a preventable medical emergency with fatal complications.The following case reviews the consequences of post-bariatric thiamine supplementation therapy non-adherence and resulting in a deficiency in an adolescent patient.

Treatment variability and its relationships to outcomes among patients with Wernicke's encephalopathy: A multicenter retrospective study.

BACKGROUND: Despite guidelines and recommendations, Wernicke's encephalopathy (WE) treatment lacks evidence, leading to clinical practice variability. AIMS: Given the overall lack of information on thiamine use for WE treatment, we analyzed data from a large, well-characterized multicenter sample of patients with WE, examining thiamine dosages; factors associated with the use of different doses, frequencies, and routes; and the influence of differences in thiamine treatment on the outcome. METHODS: This retrospective study was conducted with data from 443 patients from 21 centers obtained from a nationwide registry of the Spanish Society of Internal Medicine (from 2000 to 2012). Discharge codes and Caine criteria were applied for WE diagnosis, and treatment-related (thiamine dosage, frequency, and route of administration) demographic, clinical, and outcome variables were analyzed. RESULTS: We found marked variability in WE treatment and a low rate of high-dose intravenous thiamine administration. Seventy-eight patients out of 373 (20.9%) received > 300mg/day of thiamine as initial dose. Patients fulfilling the Caine criteria or presenting with the classic WE triad more frequently received parenteral treatment. Delayed diagnosis (after 24h hospitalization), the fulfillment of more than two Caine criteria at diagnosis, mental status alterations, and folic acid deficiency were associated significantly with the lack of complete recovery. Malnutrition, reduced consciousness, folic acid deficiency, and the lack of timely thiamine treatment were risk factors for mortality. CONCLUSIONS: Our results clearly show extreme variability in thiamine dosages and routes used in the management of WE. Measures should be implemented to ensure adherence to current guidelines and to correct potential nutritional deficits in patients with alcohol use disorders or other risk factors for WE.

Diplopia after Sleeve Gastrectomy: The Canary in the Coal Mine.

Wernicke's encephalopathy (WE) is a neurologic emergency requiring timely intravenous thiamine supplementation to prevent permanent neurologic deficits. Historically, the WE diagnosis was limited to individuals with alcohol use disorder. However, it is now widely recognized to occur in patients who are chronically malnourished, post-bariatric surgery, pregnant with hyperemesis gravidarum, and with severe anorexia nervosa. Here we present a young woman who developed WE after undergoing a recent sleeve gastrectomy followed by protracted emesis for several days. This case underscores the importance of performing a thorough neurological review of systems and physical exam in high-risk patients and having a low clinical threshold to initiate appropriate thiamine treatment.

A Case of Wernicke's Encephalopathy After Sleeve Gastrectomy.

BACKGROUND: Wernicke's encephalopathy, resulting from thiamine deficiency, is a rare but serious neurological complication of bariatric procedures. A clinical and radiologic diagnosis is often difficult, and thiamine blood tests are not broadly available. Only a few cases of Wernicke's encephalopathy after sleeve gastrectomy have been reported in the literature, nonetheless, subjects can be underdiagnosed, and their cases can be underreported. CASE PRESENTATION: We present the case of a 20-year-old female patient who developed Wernicke's encephalopathy after sleeve gastrectomy for grade II obesity with metabolic complications. She was presented to the Emergency Department showing confusion, gait ataxia and horizontal nystagmus two months after surgery. Persistent vomiting and lack of compliance with vitamin intake were reported. Cerebral MRI showed acute bilateral lesions in the periaqueductal and periventricular regions. Parenteral thiamine supplementation was administered, obtaining a progressive resolution of altered mental status, motor ataxia, and nystagmus. She was discharged on oral thiamine supplementation and underwent a multidisciplinary rehabilitation program, since anterograde, retrograde, and working memory impairment persisted. After a 2-year follow-up, she was compliant with a balanced fractionated diet and vitamin supplementation. A new cerebral MRI showed regression of the neuroradiological findings, but minimal memory impairment remained. CONCLUSION: Wernicke's encephalopathy is a concrete possibility after sleeve gastrectomy and should always be suspected in patients with recurrent vomiting, poor nutritional intake, and non-compliance to vitamin supplementation. Immediate and aggressive thiamine supplementation is mandatory to prevent patients from irreversible neurological impairment, even though full recovery is not always achieved.

Wernicke encephalopathy and beriberi disease presenting as STEMI-equivalent.

Thiamine deficiency is commonly associated with malnutrition, alcoholism and bariatric surgery. Thiamine deficiency can manifest in different ways, especially in developing countries: as peripheric neuropathy, as Wernicke encephalopathy or as beriberi disease. The authors present the case of a 72-year-old male, with a hiatal hernia that led to thiamine deficiency due to malnutrition. The initial clinical manifestation was an ST-elevation myocardial infarct equivalent, an ECG with a shark-fin pattern that evolved to a Wellens type B pattern. The patient evolved with severe altered mental status. A Wernicke encephalopathy diagnosis was confirmed by MRI; the patient was medicated with high-dose thiamine, with quick recovery, both neurologic and cardiac. The clinical history and response to treatment confirm the diagnosis of Wernicke encephalopathy and beriberi disease.

Clinical analysis of Wernicke encephalopathy after liver transplantation.

BACKGROUND: Wernicke encephalopathy (WE) is an acute neurological disease resulting from vitamin B1 deficiency, and there are only very few case reports of WE after liver transplantation. The present study aimed to investigate the clinical characteristics, etiology, magnetic resonance imaging (MRI) features, treatment and prognosis of patients with WE after liver transplantation. METHODS: Twenty-three patients with WE after liver transplantation from the First Affiliated Hospital, Zhejiang University School of Medicine and Jiangxi Provincial People's Hospital between January 2011 and December 2021 were retrospectively analyzed. RESULTS: Among the 23 patients diagnosed with WE after liver transplantation, 6 (26%) had a classic triad of impaired consciousness, oculomotor palsy and ataxia, and 17 (74%) had two features. The misdiagnosis rate was 65%. After treatment with high-dose vitamin B1, 19 (83%) patients showed improvement, whereas 4 (17%) showed no improvement, including 3 with residual short-term memory impairments and 1 with residual spatial and temporal disorientation and ataxia. CONCLUSIONS: The misdiagnosis rate is high in the early stage of WE, and the prognosis is closely associated with whether WE is diagnosed early and treated timely. High-dose glucose or glucocorticoids can trigger WE and cannot be administered before vitamin B1 treatment. Vitamin B1 is suggested to be used as a prophylactic treatment for patients with WE after liver transplantation.

Cystic fibrosis associated with Wernicke's encephalopathy in an older adult.

Here we report the first case of an association between cystic fibrosis and Wernicke's encephalopathy. The patient had a history of cystic fibrosis diagnosed in her early 60s associated with pancreatitis and chronic lung disease. She presented with a traumatic hip fracture requiring operative repair. On examination, she was found to have bilateral nystagmus. MRI revealed enhancement of the mammillary bodies. Laboratory results were notable for thiamine deficiency, which in context of the radiographic and physical examination findings, confirmed a diagnosis of Wernicke's encephalopathy. The cause of her low thiamine was thought to be poor dietary intake, weight loss and malabsorption associated with exocrine pancreatic insufficiency in the setting of a history of recurrent pancreatitis. The patient had complete resolution of her symptoms with the initiation of thiamine supplementation and pancreatic enzymes. Although classically associated with fat soluble vitamin deficiencies, there are increasing reports of water-soluble vitamin deficiencies associated with cystic fibrosis.

Wernicke's encephalopathy in women with hyperemesis gravidarum - Case series and literature review.

Whilst nausea affects around 80% of pregnant women, hyperemesis gravidarum, an extreme form of the same, affects only 0.5% to 3%, but may lead to severe nutritional deficiency. Wernicke's encephalopathy is an acute neuropsychiatric disorder which occurs due to thiamine deficiency and needs emergency treatment to prevent neurological morbidity and mortality. Wernicke's encephalopathy is characterised by a clinical triad of oculomotor abnormalities, cerebellar dysfunction and altered mental state. Korsakoff's psychosis is a chronic condition and consequence of Wernicke's encephalopathy, resulting from its delayed treatment. Wernicke's encephalopathy is a well-known complication of chronic alcohol abuse. Not many are aware of its association with hyperemesis gravidarum. Although it is a rare complication, if not diagnosed and treated promptly, it may result in permanent and irreversible neurological sequelae. The objective of our retrospective observational study was to analyse the clinical profile and outcome (short and long term) in a rare yet preventable complication of pregnancy.

Atypical neuroleptic malignant syndrome and non-alcoholic Wernicke's encephalopathy.

We report the case of a middle-aged woman with a history of bipolar disorder, in the absence of alcohol or substance misuse. The patient had been maintained on fluphenazine decanoate depot and now presented acutely with cognitive dysfunction and rigidity. Laboratory tests revealed elevated creatine kinase, acute kidney injury with metabolic acidosis and transaminitis, leading to a provisional diagnosis of neuroleptic malignant syndrome (NMS). Neuroleptics were withheld; dialysis was commenced; and blood biochemistry parameters improved in tandem. However, mental status changes persisted, and re-evaluation revealed multidirectional nystagmus with bilateral past-pointing. MRI confirmed the diagnosis of Wernicke's encephalopathy (WE). Prompt recovery followed treatment with high-dose intravenous thiamine. We discuss the co-occurrence of NMS and non-alcoholic WE-highlighting the need for a high index of suspicion for these relatively rare neuropsychiatric diagnoses which are often missed in those with atypical presentations.

[Orientation disorder, ophthalmoplegia and ataxia in a 48-year-old male]. / Orientierungsstörung, Ophthalmoplegie und Ataxie bei einem 48-Jährigen.

This case report concerns a 48-year-old male patient presenting the classic clinical picture of Wernicke encephalopathy with orientation disorder, ophthalmoplegia and ataxia. Wernicke encephalopathy is a neurological emergency that should be treated immediately with high-dose intravenous thiamine for a week, if suspected. Recognition of the disease in the emergency room is essential. Due to the limited compliance in patients with alcohol-use disorder, early diagnosis can be challenging.

Case of hypoactive delirium precipitated by thiamine deficiency.

Thiamine is an essential cofactor in the process of nucleic acid synthesis. Neuronal tissues are especially sensitive to thiamine deficiency, manifesting as Wernicke's encephalopathy (WE). The typical triad of WE, encephalopathy, oculomotor dysfunction and gait ataxia, is only present in less than one-third of the cases. We present the case of a middle-aged man with hypoactive delirium due to presumed thiamine deficiency, who had a prolonged hospital course and a delayed diagnosis of the cause of altered mental status. The presentation of this disorder solely as a decreased level of consciousness is uncommon but has been reported in the literature. It is essential to recognise WE as a treatable condition that may manifest only as a hypoactive delirium. The delay in the diagnosis and treatment may lead to coma and death.

A Case of Wernicke Encephalopathy Secondary to Anorexia Nervosa Complicated by Refeeding Syndrome and Takotsubo Cardiomyopathy.

BACKGROUND Wernicke encephalopathy (WE) is a neurological condition commonly associated with sustained alcohol abuse. However, it should be noted that disorders resulting in severe malnutrition, such as anorexia nervosa (AN), can precipitate nonalcoholic WE. AN is a life threatening psychological and eating disorder defined by inappropriate weight loss from food restriction due to the fear of gaining weight and immoderate desire to be thin. Treatment of those suffering with AN can often be complicated by severe electrolyte derangements after caloric intake termed refeeding syndrome. Although extremely rare, severe cardiomyopathy and ultimately death may occur in patients from AN. CASE REPORT Herein describes the case of a 20-year-old female with AN induced WE complicated by refeeding syndrome and hemodynamic compromise in the setting of findings consistent with takotsubo cardiomyopathy. She required ventilatory and hemodynamic support with aggressive intravenous thiamine and phosphorus repletion. Nutritional supplementation was imperative and carefully administered throughout her hospitalization. Her symptoms improved over the course of a few weeks with an ultimate reversal of her cardiomyopathy. CONCLUSIONS Given the morbidity surrounding AN, practitioners should exhibit caution when caring for those with severe nutritional deficiencies. Clinicians must monitor for severe electrolyte abnormalities and offer aggressive repletion. In addition to electrolyte derangements, severe cardiomyopathy may result as a rare sequela of the aforementioned complications associated with AN. Moreover, it is imperative to understand that patients with AN have the highest mortality of any psychiatric disorder and early intervention is necessary for survival in this vulnerable patient population.

Wernicke's encephalopathy post hyperemesis gravidarum misdiagnosed as Guillain-Barre syndrome: lessons for the frontline.

We report a case of a 26-year-old pregnant woman, who presented with subacute limb weakness. This was initially suspected to be Guillain-Barre syndrome but subsequently found to be the motor neuropathy of dry beriberi (vitamin B1, thiamine deficiency) along with associated Wernicke's encephalopathy (WE). The underlying cause was revealed as hyperemesis gravidarum (HG). HG complicates up to 3% of pregnancies and if severe, without nutritional supplements, may lead to electrolyte disturbances, calorie loss and vitamin deficiency. Although the association of HG and WE was first reported in 1939, it remains an under diagnosed condition with potential for serious and permanent neurological deficits, and some mortality, in both mother and baby. Early recognition of the problem, with timely and careful fluid, electrolyte, glucose and vitamin replacement is needed to avoid complications. We highlight current best practice in the treatment of WE. An open mind to the possibility of HG complications in any pregnant woman presenting with neurological symptoms is probably the most important lesson to learn from the front line.

Reversible Wernicke encephalopathy caused by hyperemesis gravidarum in the second trimester of pregnancy: a case report.

Wernicke encephalopathy is a potentially life-threatening neurologic syndrome caused by acute thiamine (vitamin B1) deficiency. It is usually associated with excessive alcohol consumption. Less frequently, this syndrome can be caused by persistent vomiting. This is a case report of a 33-year-old woman diagnosed with Wernicke encephalopathy (WE) during the second trimester of pregnancy. The presence of neurological and ophthalmological symptoms in the context of hyperemesis gravidarum led us to evoke the diagnosis of WE, and it was confirmed when specific lesions were found in the brain magnetic resonance imaging (MRI). Luckily for our patient, WE was diagnosed promptly and the signs were reversible after thiamine supplementation. In conclusion, any first line care taker or midwife must know the symptoms of Wernicke encephalopathy because prompt diagnosis and treatment can lead to recovery.

Wernicke's encephalopathy - An oddball complication of nephrotic syndrome.

Metabolic encephalopathies are a common cause of altered mental status in various states of malnutrition. However, a high index of suspicion is required to recognize them and differentiate Metabolic Disorders from other causes of altered mental status such as infections. A 6-year old with steroid-resistant nephrotic syndrome (NS), peritonitis, and prolonged diarrhea and vomiting, developed a brief episode of altered mental status six days after starting tacrolimus. On imaging, there were features suggestive of Wernicke's encephalopathy and it ruled out other causes of seizure in the given scenario. The child was treated with thiamine supplementation and the changes reversed four weeks after treatment. This is to emphasize that although an uncommonly reported complication of nephrotic state, one should have a high index of suspicion for these metabolic encephalopathies, especially in the setting of malnutrition, where these children are highly predisposed to multivitamin deficiency.

Non-alcoholic beriberi, Wernicke encephalopathy and long-term eating disorder: case report and a mini-review.

INTRODUCTION: Nowadays, reports of beriberi are rare in developed countries. Wernicke encephalopathy may be present in about 25% of patients with beriberi. CASE REPORT: We report the case of a woman with history of depression and chronic eating disorder, who complained Wernicke encephalopathy and beriberi. Sural nerve and muscular biopsy were performed, showing severe axonal neuropathy. Thiamine supplementation was started with rapid improvement of the pulmonary and cardiac affections; improvement of peripheral neuropathy was incomplete. CONCLUSIONS: Thiamine deficiency can be misdiagnosed. Beriberi is an important cause of acute flaccid paralysis; hence, clinicians should consider this diagnosis and prompt start thiamine treatment to avoid permanent neurological sequelae.

Resolved External Ophthalmoplegia and Hearing Loss in Wernicke's Encephalopathy With Thiamine Replacement.

BACKGROUND: Wernicke encephalopathy (WE) is classically described by a clinical triad consisting of confusion, ataxia, and ophthalmoplegia, but recent reports emphasize a history of malnutrition along with 2 elements of the WE triad (Caine's criteria) to enhance diagnostic sensitivity. The ophthalmoplegia, vestibular, and auditory expeditious improvement with intravenous thiamine usually confirms the diagnosis; serum levels generally provide additional diagnostic certainty. METHODS: Here, we discuss the case of a woman with a distant history of gastric sleeve, poor nutrition and protracted vomiting, who developed acute confusion, imbalance, near-total external ophthalmoplegia (EO), and hearing loss. The baseline thiamine level was 28 πmol/L (Normal: 70-180 πmol/L). We performed serial neurological, vestibular, and audiological examination to document over 5 days, the effect of intravenous (IV) thiamine, and again at 3 months with continued oral supplementation. We provide serial documentation with photographs and video recording of oculomotor abnormalities, audiometric testing, and a video of horizontal head impulse testing, and imaging findings. RESULTS: Over the course of 5 days of IV thiamine supplementation, we demonstrate our patient's resolution of near complete EO. We assessed vestibular paresis with horizontal head impulse testing, after complete resolution of the EO. The initially positive bilateral h-HIT showed decreased gain and overt corrective saccades, it clinically resolved by day 5, but video h-HIT testing demonstrated persistent decreased horizontal vestibulo-ocular reflex (VOR) gain and covert horizontal saccades, which persisted at the 3-month examination. By contrast, the vertical VOR gain was normal without corrective saccades. Bedside audiometry completed during the acute phase demonstrated severely restricted auditory speech comprehension, which normalized 3 months later. Severe truncal ataxia improved as well. CONCLUSIONS: This case is an example of how awareness of the variations in the clinical presentation of WE can be crucial in achieving an early diagnosis and obtaining better outcomes. A history of the poor nutritional status can be an important clue to aid in this early diagnosis.

Wernicke's encephalopathy-induced hearing loss complicating sleeve gastrectomy.

A 25-year-old woman brought to the hospital with symptoms of acute confusion, disorientation, diplopia, hearing loss and unsteady gait which started 4 days prior to her presentation with rapid worsening in its course until the day of admission. She had a surgical history of laparoscopic sleeve gastrectomy 2 months earlier which was complicated by persistent vomiting around one to three times per day. She lost 30 kg of her weight over 2 months and was not compliant to vitamin supplementation. CT of the brain was unremarkable. Brain MRI was done which showed high signal intensity lesions involving the bilateral thalamic regions symmetrically with restricted diffusion on fluid-attenuated inversion recovery imaging. Other radiological investigations, such as magnetic resonance venography and magnetic resonance angiography of the brain were unremarkable. An official audiogram confirmed the sensorineural hearing loss. A diagnosis of Wernicke's encephalopathy due to thiamin deficiency post-sleeve gastrectomy was made based on the constellation of her medical background, clinical presentation and further supported by the distinct MRI findings. Consequently, serum thiamin level was requested and intravenous thiamin 500 mg three times per day for six doses was started empirically, then thiamin 250 mg intravenously once daily given for 5 more days. Marked improvement in cognition, eye movements, strength and ambulation were noticed soon after therapy. She was maintained on a high caloric diet with calcium, magnesium oxide, vitamin D supplements and oral thiamin with successful recovery of the majority of her neurological function with normal cognition, strength, reflexes, ocular movements, but had minimal resolution of her hearing deficit. Serum thiamin level later was 36 nmol/L (67-200).