Hyperammonemic Encephalopathy Caused by Carnitine Deficiency

Limketkai, Berkeley N.; Zucker, Stephen D.

doi:10.1007/s11606-007-0473-0

Cited by 59 publications

(41 citation statements)

References 33 publications

(45 reference statements)

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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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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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“…In primary systemic carnitine deficiency, an autosomal recessive condition, the organic cation transporter gene OCTN2 is mutated, resulting in hypocarnitinemia secondary to reduced renal tubular reabsorption of carnitine. Patients with this often present with hyperammonemia, encephalopathy, hypoglycemia, and myopathies [72].…”

Section: Drug Related Causesmentioning

confidence: 99%

Hyperammonemia in review: pathophysiology, diagnosis, and treatment

2011

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Ammonia is an important source of nitrogen and is required for amino acid synthesis. It is also necessary for normal acid-base balance. When present in high concentrations, ammonia is toxic. Endogenous ammonia intoxication can occur when there is impaired capacity of the body to excrete nitrogenous waste, as seen with congenital enzymatic deficiencies. A variety of environmental causes and medications may also lead to ammonia toxicity. Hyperammonemia refers to a clinical condition associated with elevated ammonia levels manifested by a variety of symptoms and signs, including significant central nervous system (CNS) abnormalities. Appropriate and timely management requires a solid understanding of the fundamental pathophysiology, differential diagnosis, and treatment approaches available. The following review discusses the etiology, pathogenesis, differential diagnosis, and treatment of hyperammonemia.

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“…[7][8][9] Carnitine deficiency alone has been documented to cause encephalopathy, 10 and, therefore, administration of valproate in these patients could further reduce levels of carnitine, predisposing them to encephalopathy. [7][8][9] Carnitine deficiency alone has been documented to cause encephalopathy, 10 and, therefore, administration of valproate in these patients could further reduce levels of carnitine, predisposing them to encephalopathy.…”

Section: Discussionmentioning

confidence: 99%