Encephalopathy due to carnitine deficiency in an adult patient with gluten enteropathy

Karakoç, Ebru; Erdem, Sevim; Sökmensüer, Cenk; Kansu, Tülay

doi:10.1016/j.clineuro.2005.10.012

Cited by 10 publications

(5 citation statements)

References 17 publications

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“…Headache, usually migraine, has been reported as the first manifestation of CD in several case reports [ 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ].…”

Section: Resultsmentioning

confidence: 99%

“…Sixteen papers provided information regarding imaging findings [ 7 , 14 , 22 , 26 , 28 , 29 , 30 , 31 , 33 , 34 , 35 , 36 , 37 , 41 , 42 , 43 ].…”

Section: Resultsmentioning

confidence: 99%

“…Crosato et al were the first to describe a case of a nine-year-old boy with a history of seizures, headaches, episodes of drowsiness and cerebral calcification on CT who, because of his very low folate levels, was eventually diagnosed with CD [ 29 ]. Kakoraç et al reported a case of a 48-year old man who presented with two episodes of headache, confusion, and seizures and normal MRI, and because of carnitine deficiency, was eventually diagnosed with CD [ 34 ].…”

Section: Resultsmentioning

confidence: 99%

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Headache Associated with Coeliac Disease: A Systematic Review and Meta-Analysis

2018

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Objective: The aim of this systematic review was to explore the relationship between coeliac disease (CD) and headache. The objectives were to establish the prevalence of each entity amongst the other, to explore the role of gluten free diet (GFD), and to describe the imaging findings in those affected by headaches associated with CD. Methodology: A systematic computer-based literature search was conducted on the PubMed database. Information regarding study type, population size, the age group included, prevalence of CD amongst those with headache and vice versa, imaging results, the nature of headache, and response to GFD. Results: In total, 40 articles published between 1987 and 2017 qualified for inclusion in this review. The mean pooled prevalence of headache amongst those with CD was 26% (95% CI 19.5–33.9%) in adult populations and 18.3% (95% CI 10.4–30.2%) in paediatric populations. The headaches are most often migraine-like. In children with idiopathic headache, the prevalence of CD is 2.4% (95% CI 1.5–3.7%), whereas data for adult populations is presently unavailable. Brain imaging can be normal, although, cerebral calcifications on CT, white matter abnormalities on MRI and deranged regional cerebral blood flow on SPECT can be present. GFD appears to be an effective management for headache in the context of CD, leading to total resolution of headaches in up to 75% of patients. Conclusions: There is an increased prevalence of CD amongst idiopathic headache and vice versa. Therefore, patients with headache of unknown origin should be screened for CD, as such patients may symptomatically benefit from a GFD.

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“…Headache, usually migraine, has been reported as the first manifestation of CD in several case reports [ 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ].…”

Section: Resultsmentioning

confidence: 99%

“…Sixteen papers provided information regarding imaging findings [ 7 , 14 , 22 , 26 , 28 , 29 , 30 , 31 , 33 , 34 , 35 , 36 , 37 , 41 , 42 , 43 ].…”

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Headache Associated with Coeliac Disease: A Systematic Review and Meta-Analysis

2018

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“…Treatment with L-carnitine ameliorated symptoms of encephalopathy subsequent to long term use of valproate [79], and the antibiotic cefditoren pivoxil [80]. Case reports indicate that improvement with L-carnitine treatment was also seen in hyperammonemic encephalopathy caused by carnitine deficiency that manifested several years after gastrointestinal bypass surgery [83] and in encephalopathy secondary to gluten enteropathy [84]. Additionally, a randomized clinical trial reported some improvement in Friedreich’s ataxia patients treated with L-carnitine [85].…”

Section: Neuroprotection Afforded By L-carnitine Supplementation In Pmentioning

confidence: 99%

l-Carnitine and Acetyl-l-carnitine Roles and Neuroprotection in Developing Brain

2017

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L-Carnitine functions to transport long chain fatty acyl CoAs into the mitochondria for degradation by β-oxidation. Treatment with L-carnitine can ameliorate metabolic imbalance in many inborn errors of metabolism. In recent years there has been considerable interest in the therapeutic potential of L-carnitine and its acetylated derivative acetyl-L-carnitine (ALCAR) for neuroprotection in a number of disorders including hypoxia-ischemia, traumatic brain injury, Alzheimer’s disease and in conditions leading to central or peripheral nervous system injury. There is compelling evidence from preclinical studies that L-carnitine and ALCAR can improve energy status, decrease oxidative stress and prevent subsequent cell death in models of adult, neonatal and pediatric brain injury. ALCAR can provide an acetyl moiety that can be oxidized for energy, used as a precursor for acetylcholine, or incorporated into glutamate, glutamine and GABA, or into lipids for myelination and cell growth. Administration of ALCAR after brain injury in rat pups improved long-term functional outcomes, including memory. Additional studies are needed to better explore the potential of L-carnitine and ALCAR for protection of developing brain as there is an urgent need for therapies that can improve outcome after neonatal and pediatric brain injury.

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“…Carnitine insufficiency leading to inhibition of oxidative metabolism of fatty acids could be responsible for several rare extraintestinal manifestations which can be observed among CD patients, such as cardiomyopathy, encephalopathy, myopathy with hypotonia and hyporeflexia, or disturbed liver function. Until recently, two CD patients with dilated cardiomyopathy [61,62] and one with encephalopathy [63] were published in the literature. All of them presented disturbed carnitine homeostasis presumably due to their malabsorption disorder.…”

Section: Celiac Diseasementioning

confidence: 99%

Mass Spectrometric Analysis of L-carnitine and its Esters: Potential Biomarkers of Disturbances in Carnitine Homeostasis

Bene

Szabó

Komlósi

et al. 2020

CMM

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Purpose: After a golden age of classic carnitine research three decades ago, the spread of mass spectrometry opened new perspectives and a much better understanding of the carnitine system is available nowadays. In the classic period, several human and animal studies were focused on various distinct physiological functions of this molecule and these revealed different aspects of carnitine homeostasis in normal and pathological conditions. Initially, the laboratory analyses were based on the classic or radioenzymatic assays, enabling only the determination of free and total carnitine levels and calculation of total carnitine esters’ amount without any information on the composition of the acyl groups. The introduction of mass spectrometry allowed the measurement of free carnitine along with the specific and sensitive determination of different carnitine esters. Beyond basic research, mass spectrometry study of carnitine esters was introduced into the newborn screening program because of being capable to detect more than 30 metabolic disorders simultaneously. Furthermore, mass spectrometry measurements were performed to investigate different disease states affecting carnitine homeostasis, such as diabetes, chronic renal failure, celiac disease, cardiovascular diseases, autism spectrum disorder or inflammatory bowel diseases. Results: This article will review the recent advances in the field of carnitine research with respect to mass spectrometric analyses of acyl-carnitines in normal and various pathological states. Conclusion: The growing number of publications using mass spectrometry as a tool to investigate normal physiological conditions or reveal potential biomarkers of primary and secondary carnitine deficiencies shows that this tool brought a new perspective to carnitine research.

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Encephalopathy due to carnitine deficiency in an adult patient with gluten enteropathy

Cited by 10 publications

References 17 publications

Headache Associated with Coeliac Disease: A Systematic Review and Meta-Analysis

Headache Associated with Coeliac Disease: A Systematic Review and Meta-Analysis

l-Carnitine and Acetyl-l-carnitine Roles and Neuroprotection in Developing Brain

Mass Spectrometric Analysis of L-carnitine and its Esters: Potential Biomarkers of Disturbances in Carnitine Homeostasis

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