Metabolic Encephalopathy - Part II

2019 ◽  
Author(s):  
Rick Gill ◽  
Matthew McCoyd ◽  
Sean Ruland ◽  
José Biller
Keyword(s):  
Nutritional Intake ◽  
Osmotic Demyelination Syndrome ◽  
Wernicke Encephalopathy ◽  
Metabolic Encephalopathy ◽  
Improve Outcome ◽  
Myxedema Coma ◽  
Hashimoto Encephalopathy ◽  
Korsakoff Syndrome ◽  
Dialysis Disequilibrium Syndrome ◽  
Neurologic Function

Normal neurologic function requires a constantly balanced environment of electrolytes.  Normal hepatic and renal function is critical in maintaining this balance while removing toxins, maintaining a physiologic pH and regulating the excretion of electrolytes.  Nutritional intake provides essential nutrients but deficiencies can lead to characteristic syndromes such as Wernicke's encephalopathy and pellagra and exposure to neurotoxic substances such as heavy metals can lead to encephalopathy.  Thyroid and adrenal dysfunction are common endocrine causes of encephalopathy and symptoms can often improve rapidly with treatment.  A subset of idiopathic encephalopathy is increasingly being recognized as having an autoimmune basis, often presenting as a paraneoplastic process, and having a constellation of symptoms which can aide in the diagnosis.  Timely recognition and treatment of the autoantibodies which target neural structures, with immunosuppressive therapy, can improve outcome in these patients.   This review contains 4 figures, 3 tables, and 42 references. Key words: osmotic demyelination syndrome,hepatic encephalopathy, renal failure, triphasic waves, dialysis disequilibrium syndrome, Wernicke encephalopathy, Korsakoff syndrome, myxedema coma, Hashimoto encephalopathy

Metabolic Encephalopathy - Part II

2019 ◽  
Author(s):  
Rick Gill ◽  
Matthew McCoyd ◽  
Sean Ruland ◽  
José Biller
Keyword(s):  
Nutritional Intake ◽  
Osmotic Demyelination Syndrome ◽  
Wernicke Encephalopathy ◽  
Metabolic Encephalopathy ◽  
Improve Outcome ◽  
Myxedema Coma ◽  
Hashimoto Encephalopathy ◽  
Korsakoff Syndrome ◽  
Dialysis Disequilibrium Syndrome ◽  
Neurologic Function

Normal neurologic function requires a constantly balanced environment of electrolytes.  Normal hepatic and renal function is critical in maintaining this balance while removing toxins, maintaining a physiologic pH and regulating the excretion of electrolytes.  Nutritional intake provides essential nutrients but deficiencies can lead to characteristic syndromes such as Wernicke's encephalopathy and pellagra and exposure to neurotoxic substances such as heavy metals can lead to encephalopathy.  Thyroid and adrenal dysfunction are common endocrine causes of encephalopathy and symptoms can often improve rapidly with treatment.  A subset of idiopathic encephalopathy is increasingly being recognized as having an autoimmune basis, often presenting as a paraneoplastic process, and having a constellation of symptoms which can aide in the diagnosis.  Timely recognition and treatment of the autoantibodies which target neural structures, with immunosuppressive therapy, can improve outcome in these patients.   This review contains 4 figures, 3 tables, and 42 references. Key words: osmotic demyelination syndrome,hepatic encephalopathy, renal failure, triphasic waves, dialysis disequilibrium syndrome, Wernicke encephalopathy, Korsakoff syndrome, myxedema coma, Hashimoto encephalopathy

Metabolic Encephalopathy - Part II

2019 ◽  
Author(s):  
Rick Gill ◽  
Matthew McCoyd ◽  
Sean Ruland ◽  
José Biller
Keyword(s):  
Nutritional Intake ◽  
Osmotic Demyelination Syndrome ◽  
Wernicke Encephalopathy ◽  
Metabolic Encephalopathy ◽  
Improve Outcome ◽  
Myxedema Coma ◽  
Hashimoto Encephalopathy ◽  
Korsakoff Syndrome ◽  
Dialysis Disequilibrium Syndrome ◽  
Neurologic Function

Normal neurologic function requires a constantly balanced environment of electrolytes.  Normal hepatic and renal function is critical in maintaining this balance while removing toxins, maintaining a physiologic pH and regulating the excretion of electrolytes.  Nutritional intake provides essential nutrients but deficiencies can lead to characteristic syndromes such as Wernicke's encephalopathy and pellagra and exposure to neurotoxic substances such as heavy metals can lead to encephalopathy.  Thyroid and adrenal dysfunction are common endocrine causes of encephalopathy and symptoms can often improve rapidly with treatment.  A subset of idiopathic encephalopathy is increasingly being recognized as having an autoimmune basis, often presenting as a paraneoplastic process, and having a constellation of symptoms which can aide in the diagnosis.  Timely recognition and treatment of the autoantibodies which target neural structures, with immunosuppressive therapy, can improve outcome in these patients.   This review contains 4 figures, 3 tables, and 42 references. Key words: osmotic demyelination syndrome,hepatic encephalopathy, renal failure, triphasic waves, dialysis disequilibrium syndrome, Wernicke encephalopathy, Korsakoff syndrome, myxedema coma, Hashimoto encephalopathy

Gayet-Wernicke encephalopathy with rapid evolution toward dementia in alcoholic dependence – case report

Psihiatru ro ◽  
2019 ◽  
Vol 58 (3) (1) ◽  
pp. 18-20
Author(s):  
Cătălina Crişan ◽  
Laura Grosu ◽  
Oana Vanţa
Keyword(s):  
Case Report ◽  
Thiamine Deficiency ◽  
Withdrawal Syndrome ◽  
Rapid Evolution ◽  
Full Recovery ◽  
Wernicke Encephalopathy ◽  
Korsakoff Syndrome ◽  
Dementia Syndrome ◽  
Patients Experience

Gayet-Wernicke encephalopathy is an acute neuropsychiatric condition caused by thiamine deficiency. Only a small percentage of patients experience all three symptoms, with ophtalmoplegia, ataxia and confusion, and the full triad occurs more frequently among those who have overused alcohol. The evolution is toward full recovery, Korsakoff syndrome, dementia or death. We present the case of a 56-year-old patient, known with a diagnostic of alcoholism, who was admitted for a complicated withdrawal syndrome with delirium and who developed encephalopathy and dementia syndrome.

scholarly journals Unusual Presentation of Myxedema Coma in Type 1 Diabetes Mellitus With Hyperglycemia and Polysubstance Abuse

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A971-A972
Author(s):  
Yumna Hamid ◽  
Steven Douedi ◽  
Johnathan Nold ◽  
Raquel Kristin Ong ◽  
Jennifer Cheng ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Mortality Rate ◽  
Mental Status ◽  
White Male ◽  
Altered Mental Status ◽  
Close Monitoring ◽  
Urine Drug Screen ◽  
Metabolic Encephalopathy ◽  
Myxedema Coma

Abstract Background: Myxedema coma is a severe manifestation of hypothyroidism that typicallypresents with altered mental status and requires close monitoring in the intensive care unit dueto 30-60% mortality rate. Clinical Case: A 56 year old white male with type 1 diabetes with recurrent DKA, polysubstanceabuse, Bipolar disorder on lithium and post surgical hypothyroidism presented due to change inmental status after being brought in by sister. Patient was found to be lethargic with confusionthat worsened over the last week. The patient was admitted several times in the past monthsecondary to pneumonia, sepsis, and recurrent DKA. On physical examination, he found to have lethargy, macroglossia, hyporeflexia, and periorbitaledema. Patient had acute respiratory failure with metabolic encephalopathy, bradycardia,tachypnea, severe hyperglycemia, hypotension of 77/51, tachypnea of 31 breaths per minuteand hyponatremia. Laboratory findings showed T4 levels 2.87(n=5.28-9.87ug/dL) withundetectable FT4 and elevated TSH (>50, n=0.300-4.500uIU/mL). Electrolyte panel showedhyponatremia (133, n=136-145mmol/L), hyperglycemia up to 532mg/dL and lithium levels werewithin normal limits (n=0.5-1.5 mmol/L). Urine drug screen was positive for cocaine. A CT scanof the head was negative. His myxedema score was diagnostic (>60). The patient wasdiagnosed with myxedema coma and admitted to the ICU. Patient was treated with IV LT4 400mcg, LT3 10mcg and hydrocortisone 100mg and started onIV LT4 100mcg daily, LT3 2.5mcg daily and hydrocortisone 100mg Q8 hours. There was wideglycemic variation from 46-532 mg/dL on POCT. The patient improved clinically, with resolutionof lethargy, confusion, fatigue, improved appetite, and improved lab work of FT3 2.30 (n=2.28-3.96pg/mL), FT4 at 0.76 (n=0.50-1.26ng/dL) and was downgraded from the ICU. On hospitalday four, he was transitioned to oral levothyroxine and discharged home. Conclusion: It is important to diagnose early and promptly manage decompensatedhypothyroidism in the setting of other comorbidities such as hyperglycemia in diabetes andpolysubstance abuse. The cocaine in the system may cause tachypnea and tachycardia. Manyconditions may have altered mental status, but with a history of hypothyroidism, Myxedemacoma should be on the differential due to its high mortality rate.

Nutritional Deficiencies and Toxicities

2017 ◽  
Author(s):  
Karl E. Misulis ◽  
E. Lee Murray
Keyword(s):  
Vitamin D ◽  
Vitamin D Deficiency ◽  
Copper Deficiency ◽  
Protein Energy Malnutrition ◽  
Nutritional Deficiencies ◽  
Wernicke Encephalopathy ◽  
Diagnosis And Management ◽  
Protein Energy ◽  
Korsakoff Syndrome ◽  
B12 Deficiency

Nutritional disorders are often encountered in hospital neurology practice, especially deficiencies of vitamins B1 and B12. Medical conditions can predispose to nutritional disorders. This chapter discusses the presentation, diagnosis, and management of B12 deficiency, B1 deficiency, protein-energy malnutrition, folate deficiency especially in the context of pregnancy, B6 deficiency, B6 toxicity, copper deficiency, and vitamin D deficiency. Wernicke encephalopathy and Korsakoff syndrome are also discussed.

scholarly journals Wernicke–Korsakoff syndrome associated with mtDNA disease

2020 ◽  
Vol 13 ◽  
pp. 175628642093897
Author(s):  
Idris Janos Jimoh ◽  
Barbara Sebe ◽  
Peter Balicza ◽  
Mariann Fedor ◽  
Ilona Pataky ◽  
...  
Keyword(s):  
Mitochondrial Dysfunction ◽  
Negative Symptoms ◽  
Brain Mri ◽  
Mitochondrial Disorder ◽  
Vitamin B1 ◽  
Antipsychotic Treatment ◽  
High Dose ◽  
Wernicke Encephalopathy ◽  
Mtdna Mutation ◽  
Korsakoff Syndrome

Introduction: Wernicke encephalopathy (WE) and Wernicke–Korsakoff syndrome (WKS) are well-known disorders caused by thiamine deficiency. In addition to the classical concept of these diseases, some literature data suggest a connection between mitochondrial dysfunction and WE/WKS. Psychotic disorders and WKS seem to run in families, as the deficiency of the oxidative phosphorylation can be a trigger factor in psychotic events and WE/WKS as well. We present a patient harbouring the m.A3243G mtDNA mutation with the clinical and magnetic resonance imaging (MRI) findings of WKS who developed schizophrenia with predominantly negative symptoms some years later. Case presentation: A 27-year-old woman was referred to our clinic with severe weight loss after severe vomiting episodes, memory dysfunction and gait ataxia. Family history, as well as clinical, imaging and laboratory findings suggested a mitochondrial aetiology of her symptoms. Brain MRI detected bilateral mild thalamic lesions and loss of corpus mammillae, indicating Wernicke encephalopathy. Genetic testing detected an m.A3243G mtDNA mutation, which has been frequently associated with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes. High-dose vitamin B1 supplementation with supportive antioxidant therapy improved the patient’s memory and learning disturbance; however, some months later she developed psychosis with predominantly negative symptoms and her cognitive functions deteriorated again. Both cognitive and negative symptoms responded well to cariprazine monotherapy. Discussion: Mitochondrial disease due to mtDNA alteration can be a rare cause of WE. In addition to vitamin B1 supplementation, cariprazine with significant dopamine D3 receptor binding can be useful to treat the predominantly negative symptoms and cognitive dysfunction in patients with mitochondrial dysfunction. Conclusion: We assume that patients with a mitochondrial disorder might be prone to develop WE/WKS and therefore need tailored supportive therapy during metabolic crisis as well as symptom-based personalized antipsychotic treatment.

scholarly journals CONFABULATIONS IN A CHRONIC ALCOHOLIC- A SUBTLE PRESENTATION OF KORSAKOFF SYNDROME

2019 ◽  
Vol 7 (7) ◽  
pp. 58-60
Author(s):  
Yan Leyfman
Keyword(s):  
Case Report ◽  
Alcohol Abuse ◽  
Retrograde Amnesia ◽  
Vitamin B1 ◽  
Wernicke Encephalopathy ◽  
Chronic Alcoholic ◽  
Present Case Report ◽  
Personality Changes ◽  
Korsakoff Syndrome ◽  
Vitamin B1 Deficiency

Wernicke Encephalopathy is an acute neuropsychiatric condition, characterized by confusion, nystagmus, ataxia, and ophthalmoplegia, resulting from thiamine (Vitamin B1) deficiency, typically secondary to alcohol abuse. Failure to properly diagnose, which occurs in 80% of the time, can result in gradual progression to irreversible Korsakoff Syndrome, characterized by irreversible personality changes, and anterograde and retrograde amnesia. The present case report seeks to highlight this patient’s chronology to Korsakoff Syndrome and our attempted interventions.

scholarly journals Hyperemesis Gravidarum, Wernicke Encephalopathy and Korsakoff Syndrome: A Critical Appraisal of the Relevant Literature

2019 ◽  
pp. 1-18
Author(s):  
Nasreen Khawer Salahuddin ◽  
Arindam Dutta ◽  
Naseem Akhtar Qureshi
Keyword(s):  
Pregnant Women ◽  
Hyperemesis Gravidarum ◽  
Early Stage ◽  
Relevant Literature ◽  
Emergency Admission ◽  
Successful Outcome ◽  
Electrolyte Balance ◽  
Wernicke Encephalopathy ◽  
Metabolic Disturbances ◽  
Korsakoff Syndrome

Background: Hyperemesis gravidarum tends to rapidly progress into Wernicke encephalopathy and Korsakoff syndrome and, therefore, needs to be recognized early and managed promptly with targeted multimodal therapies. Objective: This study critically reviewed the relevant literature on clinical perspectives of hyperemesis gravidarum, Wernicke encephalopathy and Korsakoff syndrome. The secondary objective of this study was to improve the awareness, emphasis on early diagnosis and immediate intervention concerning these sequential syndromes in pregnant women across the board. Methods: Electronic searches (since inception-2019) of PubMed/MEDLINE, Google Scholar, OvidSP, Dove Medical Press, ScienceDomain International (SDI) and Hindawi.com were conducted using keywords and Boolean Operators. Hundreds of thousands articles were retrieved which were reviewed independently by two authors and finally 144 articles retained that addressed clinical components of these sequential syndromes along with relevance of thiamine deficiency. Results: Evidently, vulnerable women in early stage of gestation tend to develop hyperemesis gravidarum characterized by persistent severe pernicious nausea and vomiting that causes Wernicke encephalopathy defined by variable oculomotor disturbances, ataxia, confusion, metabolic disturbances and Korsakoff syndrome linked with gross memory impairment, confabulation and constipation. The women with these conditions need diagnostic evaluation by means of clinical history, relevant laboratory tests, abdominal ultrasound and brain computerized tomography and magnetic resonance imaging. Most patients need emergency admission, prompt treatment with optimal doses of antiemetics, vitamin B1, and followed by fluid replenishment and electrolyte balance with follow up till the end of pregnancy. Successful maternal and fetal outcome of pregnancy depends on multiple determinants including associated systemic diseases. Conclusion: Evidently, a variety of etiological and risk factors in pregnant women determine the initiation of hyperemesis gravidarum that subsequently causes Wernicke encephalopathy and Korsakoff syndrome, and each of which needs prompt multimodal treatment in order to reduce maternal morbidity and increase successful outcome of pregnancy. Although clinical literature concerning these sequential syndromes is huge, further studies are needed to understand their underlying pathophysiological pathways across the world.

Review of thiamine deficiency disorders: Wernicke encephalopathy and Korsakoff psychosis

2018 ◽  
Vol 30 (2) ◽  
pp. 153-162 ◽  
Author(s):  
Abin Chandrakumar ◽  
Aseem Bhardwaj ◽  
Geert W. ‘t Jong
Keyword(s):  
Thiamine Deficiency ◽  
Clinical Presentation ◽  
Active Form ◽  
Biologically Active ◽  
Therapeutic Interventions ◽  
Thiamine Pyrophosphate ◽  
Wernicke Encephalopathy ◽  
Carbohydrate Utilization ◽  
Korsakoff Syndrome ◽  
Neurological Conditions

Abstract Wernicke encephalopathy (WE) and Korsakoff psychosis (KP), together termed Wernicke–Korsakoff syndrome (WKS), are distinct yet overlapping neuropsychiatric disorders associated with thiamine deficiency. Thiamine pyrophosphate, the biologically active form of thiamine, is essential for multiple biochemical pathways involved in carbohydrate utilization. Both genetic susceptibilities and acquired deficiencies as a result of alcoholic and non-alcoholic factors are associated with thiamine deficiency or its impaired utilization. WKS is underdiagnosed because of the inconsistent clinical presentation and overlapping of symptoms with other neurological conditions. The identification and individualized treatment of WE based on the etiology is vital to prevent the development of the amnestic state associated with KP in genetically predisposed individuals. Through this review, we bring together the existing data from animal and human models to expound the etiopathogenesis, diagnosis, and therapeutic interventions for WE and KP.

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