scholarly journals 062 Alcohol associated toxic myelopathy with normal hepatic function

2019 ◽  
Vol 90 (e7) ◽  
pp. A20.2-A20
Author(s):  
Srimathy Vijayan ◽  
Scott Davies ◽  
Wai Leong

IntroductionAlcohol related neurological dysfunction affects the central and peripheral nervous system. We present a rare consequence of excessive alcohol consumption. A 42-year-old female consuming 2 bottles of wine daily for the preceding 5 years presented with 12 months of progressive gait disturbance. Examination revealed a spastic paraparesis with prominent dorsal column signs. Extensive work up found no alternate cause for her presentation. The final diagnosis was direct alcohol mediated toxic myelopathy. We reviewed the current literature on this rare condition.MethodsWe searched PubMED/OVID databases with the terms ‘alcohol AND myelopathy’.ResultsThere is a paucity of research on this clinical entity. Sage et al.1 described 5 well-nourished alcoholics presenting with a progressive myelopathy. As with our patient, routine laboratory tests, including nutritional screen, were normal. CT Myelogram revealed no structural cause. CSF was normal. In contrast, a myelopathic syndrome can occur in alcoholic hepatic failure, attributed to portosystemic shunting of blood, with resultant hyper-ammonaemia and demyelination of corticospinal tracts.2–4ConclusionAlcohol associated toxic myelopathy is a rare entity, distinct from hepatic myelopathy. It is thought to result from direct toxic effects of ethanol on corticospinal and large myelinated sensory tracts. The sensory involvement is unique for this entity, which distinguishes it from hepatic myelopathy, where corticospinal involvement predominates. It is prudent to look for other potentially treatable secondary causes of myelopathy. Abstinence from alcohol results in modest improvement in symptoms. Minimal literature exists on this clinical entity and further research is required.ReferencesSage JI, et al. Alcoholic myelopathy without substantial liver disease. A syndrome of progressive dorsal and lateral column dysfunction. Arch Neurol. 1984 Sep;41(9):999–1001.Premkumar M, et al. Hepatic Myelopathy in a Patient with Decompensated Alcoholic Cirrhosis and Portal Colopathy. Volume 2012, Article ID 735906, 4 pages.Koo JE, et al. Hepatic myelopathy as a presenting neurological complication in patients with cirrhosis and spontaneous splenorenal shunt. Korean J Hepatol. 2008 Mar;14(1):89–96.Kori P, et al. Hepatic myelopathy: an unusual neurological complication of chronic liver disease presenting as quadriparesis. BMJ Case Rep. 2013; 2013: bcr2013009078.

2020 ◽  
Vol 13 (6) ◽  
pp. e235090
Author(s):  
Jaspreet Kaur ◽  
Gautam Jesrani ◽  
Monica Gupta ◽  
Sarabmeet Singh Lehl

Hepatic myelopathy or spastic paraparesis of liver disease is an insidious onset condition with pure motor spastic paraparesis without sensory, bladder or bowel involvement in patients with chronic liver disease, in which the neurological dysfunction cannot be explained by other causes. It is a rare, relentlessly progressive and mostly irreversible neurological complication resulting from portosystemic shunts occurring spontaneously, created surgically or due to ‘functional shunting’. In some cases, no evidence of shunting is elicitable due to difficulty in locating the hidden collaterals. We report this rare case of a 33-year-old man with chronic liver disease presenting with spastic paraparesis after 11 months of resolution of an episode of hepatic encephalopathy.


2014 ◽  
Vol 8 (04) ◽  
pp. 551-553
Author(s):  
Jun Cheng ◽  
Shou-Wei Jiang ◽  
Zhong-Song Zhou ◽  
Qiu-Lin Sun

Progressive encephalomyelopathy is a rare neurological complication of chronic liver disease, even manifesting progressive spastic paraparesis. Few reports detailing the clinical and diagnostic aspects of this uncommon cause of neurological deterioration in patients with hepatic insufficiency have been published. Early recognition of this disorder will become more important in the future as patients with liver disease survive longer due to medical advances, including liver transplantation. The case of a patient with hepatic encephalomyelopathy associated with Budd-Chiari syndrome and HBV-related cirrhosis is presented.


2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Madhumita Premkumar ◽  
Avishek Bagchi ◽  
Neha Kapoor ◽  
Ankit Gupta ◽  
Gaurav Maurya ◽  
...  

Cirrhotic or hepatic myelopathy is a rare neurological complication of chronic liver disease usually seen in adults and presents as a progressive pure motor spastic paraparesis which is usually associated with overt liver failure and a surgical or spontaneous systemic portocaval shunt. We describe the development of progressive spastic paraparesis, in a patient with alcoholic cirrhosis with portal hypertension and portal colopathy who presented with the first episode of hepatic encephalopathy. The patient had not undergone any shunt procedure.


2021 ◽  
Vol 12 (1) ◽  
pp. 1-14
Author(s):  
Lubomir Skladany ◽  
Tomas Koller ◽  
Svetlana Adamcova Selcanova ◽  
Janka Vnencakova ◽  
Daniela Jancekova ◽  
...  

AbstractChronic liver disease management is a comprehensive approach requiring multi-professional expertise and well-orchestrated healthcare measures thoroughly organized by responsible medical units. Contextually, the corresponding multi-faceted chain of healthcare events is likely to be severely disturbed or even temporarily broken under the force majeure conditions such as global pandemics. Consequently, the chronic liver disease is highly representative for the management of any severe chronic disorder under lasting pandemics with unprecedented numbers of acutely diseased persons who, together with the chronically sick patient cohorts, have to be treated using the given capacity of healthcare systems with their limited resources. Current study aimed at exploring potentially negative impacts of the SARS CoV-2 outbreak on the quality of the advanced chronic liver disease (ACLD) management considering two well-classified parameters, namely, (1) the continuity of the patient registrations and (2) the level of mortality rates, comparing pre-COVID-19 statistics with these under the current pandemic in Slovak Republic. Altogether 1091 registrations to cirrhosis registry (with 60.8% versus 39.2% males to females ratio) were included with a median age of 57 years for patients under consideration. Already within the very first 3 months of the pandemic outbreak in Slovakia (lockdown declared from March 16, 2020, until May 20, 2020), the continuity of the patient registrations has been broken followed by significantly increased ACLD-related death rates. During this period of time, the total number of new registrations decreased by about 60% (15 registrations in 2020 versus 38 in 2018 and 38 in 2019). Corresponding mortality increased by about 52% (23 deaths in 2020 versus 10 in 2018 and 12 in 2019). Based on these results and in line with the framework of 3PM guidelines, the pandemic priority pathways (PPP) are strongly recommended for maintaining tertiary care uninterrupted. For the evidence-based implementation of PPP, creation of predictive algorithms and individualized care strategy tailored to the patient is essential. Resulting classification of measures is summarized as follows: The Green PPP Line is reserved for prioritized (urgent and comprehensive) treatment of patients at highest risk to die from ACLD (tertiary care) as compared to the risk from possible COVID-19 infection. The Orange PPP Line considers patients at middle risk of adverse outcomes from ACLD with re-addressing them to the secondary care. As further deterioration of ACLD is still probable, pro-active management is ascertained with tertiary center serving as the 24/7 telemedicine consultation hub for a secondary facility (on a physician-physician level). The Red PPP Line is related to the patients at low risk to die from ACLD, re-addressing them to the primary care. Since patients with stable chronic liver diseases without advanced fibrosis are at trivial inherent risk, they should be kept out of the healthcare setting as far as possible by the telemedical (patient-nurse or patient- physician) measurements. The assigned priority has to be monitored and re-evaluated individually—in intervals based on the baseline prognostic score such as MELD. The approach is conform with principles of predictive, preventive and personalized medicine (PPPM / 3PM) and demonstrates a potential of great clinical utility for an optimal management of any severe chronic disorder (cardiovascular, neurological and cancer) under lasting pandemics.


2018 ◽  
Vol 19 (12) ◽  
pp. 3857 ◽  
Author(s):  
Marica Meroni ◽  
Miriam Longo ◽  
Raffaela Rametta ◽  
Paola Dongiovanni

Alcoholic liver disease (ALD), a disorder caused by excessive alcohol consumption is a global health issue. More than two billion people consume alcohol in the world and about 75 million are classified as having alcohol disorders. ALD embraces a wide spectrum of hepatic lesions including steatosis, alcoholic steatohepatitis (ASH), fibrosis, cirrhosis, and hepatocellular carcinoma (HCC). ALD is a complex disease where environmental, genetic, and epigenetic factors contribute to its pathogenesis and progression. The severity of alcohol-induced liver disease depends on the amount, method of usage and duration of alcohol consumption as well as on age, gender, presence of obesity, and genetic susceptibility. Genome-wide association studies and candidate gene studies have identified genetic modifiers of ALD that can be exploited as non-invasive biomarkers, but which do not completely explain the phenotypic variability. Indeed, ALD development and progression is also modulated by epigenetic factors. The premise of this review is to discuss the role of genetic variants and epigenetic modifications, with particular attention being paid to microRNAs, as pathogenic markers, risk predictors, and therapeutic targets in ALD.


2022 ◽  
Vol 23 (2) ◽  
pp. 774
Author(s):  
Yoon Mee Yang ◽  
Ye Eun Cho ◽  
Seonghwan Hwang

Alcoholic liver disease (ALD) is characterized by the injury, inflammation, and scarring in the liver owing to excessive alcohol consumption. Currently, ALD is a leading cause for liver transplantation. Therefore, extensive studies (in vitro, in experimental ALD models and in humans) are needed to elucidate pathological features and pathogenic mechanisms underlying ALD. Notably, oxidative changes in the liver have been recognized as a signature trait of ALD. Progression of ALD is linked to the generation of highly reactive free radicals by reactions involving ethanol and its metabolites. Furthermore, hepatic oxidative stress promotes tissue injury and, in turn, stimulates inflammatory responses in the liver, forming a pathological loop that promotes the progression of ALD. Accordingly, accumulating further knowledge on the relationship between oxidative stress and inflammation may help establish a viable therapeutic approach for treating ALD.


Author(s):  
Mohd Riyazuddin ◽  
Arisha Shahid

Abstract Non-alcoholic Fatty Liver Disease (NAFLD) is one of the diseases that have evolved lately into a major challenge for gastroenterologists. Although, the term NAFLD has not been familiar to the medical world since long, other conditions resembling the presentation of NAFLD have been there since primitive times. It is a reversible condition of the liver, wherein large vacuoles of triglyceride fat accumulates in liver cells via the process of steatosis, despite any evidence of excessive alcohol consumption. In the developed countries NAFLD is reported to be the most common liver disorder, with a worldwide prevalence of 6–35%, in India its prevalence has been increasing gradually. Unani physicians have described liver as one of the principal organs of the body. It is the primary source of natural faculties, where the functions of digestion, concoction, absorption and excretion are performed, normally temperament of liver is hot and moist which can get converted to cold due to mutable dietary habits, consumption of fatty and cold food in abundance etc. In Unani System of Medicine, NAFLD has not been described as such, but it can be studied under Su’-i-Mizāj Kabid Bārid due to correlation of most of the symptoms. Its management mainly consists of elimination of morbid matter which is accumulated in the liver and correction of Su’-i-Mizāj Kabid Bārid by using drugs having opposite temperament (Ilaj bil zid).


2019 ◽  
Vol 90 (e7) ◽  
pp. A20.3-A21
Author(s):  
Srimathy Vijayan ◽  
Carolyn Orr ◽  
Catherine Franconi

ObjectivesPrimary headache disorders are common with migraine and tension headache accounting for the vast majority of cases. A smaller proportion suffer from trigeminal autonomic cephalgia (TAC). We present a 23-year-old Caucasian female who described characteristic, episodic headaches starting with a dull retro-orbital/bi-frontal pressure evolving, over the course of 1 minute, to experience florid periorbital ecchymosis. While this phenomenon has been described in the literature, the characteristics of our case are unique and noteworthy of reporting.MethodsWe reviewed the literature surrounding this rare entity by using PubMed/OVID databases and the search terms ‘Headache AND ecchymosis’.ResultsCase reports exist in older patients1–3, where the headache is side locked and associated with other autonomic characteristics such as periorbital oedema, conjunctival injection and tearing. Our case is a young female with only ecchymosis in a unilateral and/or bilateral manner and no other autonomic or indeed migraine features. The patient underwent vascular/cranial imaging and blood tests to exclude haematological, autoimmune, vasculitic causes for this presentation which were unrewarding.ConclusionVariations on this clinical entity are described;1–4 we hope this report may bring attention to this fascinating phenomenon. The pathophysiological process is likely to be similar to those implicated in TACs, namely activation of the trigemino-neurovascular system and facial autonomic pathways. The release of neuromediators such as CGRP, VIP and Substance P cause blood vessel fragility resulting in diapedesis. Optimal treatment regimens are unknown but various agents have been trialled. Our patient declined treatment and continues to be followed.ReferencesDeBroff B, et al. Migraine Associated with Periorbital Ecchymosis. Headache 1990;30:260–263.Dafer R, et al. Atypical Chronic Headache and Recurrent Facial Ecchymosis: A Case Report. Neuro-Ophthalmology 2011;35:76–77.Nozzolillo D, et al. Migraine associated with facial ecchymoses ipsilateral to the symptomatic side. J Headache Pain ( 2004) 5:256–259.Sethi PK, et al. Teaching neuroimages: Red forehead dot syndrome and migraine revisited. Neurology 2015;85;e28.


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