Global cerebral involvement and l-arginine use in a patient with MELAS syndrome

Torre, Paula Pérez; Acebrón-Herrera, F.; Barragán, N García; Corral, Íñigo

doi:10.1016/j.nrleng.2018.03.021

Cited by 2 publications

(5 citation statements)

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“…Three patients discontinued oral treatment in a 2-year open-label trial due to increased frequency of seizures (n = 1), concurrent pneumonia (n = 1), and unverified efficacy (n = 1). Of the 5 patients treated acutely with oral l -arginine, 21-25 emesis occurred in a single patient. 24…”

Section: Resultsmentioning

confidence: 99%

“…frequency of seizures (n = 1), concurrent pneumonia (n = 1), and unverified efficacy (n = 1). Of the 5 patients treated acutely with oral L-arginine, [21][22][23][24][25] emesis occurred in a single patient. 24 Mortality (IV and Oral L-Arginine) Eleven (12%) of all 91 patients treated with L-arginine (both oral and IV) died during follow-up or while on prophylactic (oral) treatment (Table 5).…”

Section: Clinical Responsementioning

confidence: 99%

“…Positive: N = 15 (54%); n = 18 (55%) Within 24 h (N = 5; n = 6) 20,24,41,43 ; few days (N = 1; n = 1) 44 ; 1 wk (N = 1; n = 1) 39 ; 3-4 wk (N = 2; n = 3), 37,40 ; 6 wk (N = 2; n = 2) 22,25 Improved; timing N/R (N = 3; n = 4) 26,38,42 Partial improvement (N = 1; n = 3 over 27 mo) 23 Positive: Within 24 h of treatment, improvement (episode or patient): headache 31/39 (79%); disability 25/31 (81%); nausea/vomiting 32/36 (89%); positive visual symptoms 20/31 (65%) 10,11 Positive: N = 1; n = 1, improved consciousness within 4 wk of treatment 21 Positive: N = 5 (56%); n = 8 (47%) within 24 h of end of treatment. All treatment immediately on hospital presentation 9…”

Section: Clinical Responsementioning

confidence: 99%

“…Negative: N = 4; n = 4 3 mo after discharge, symptom decline preceding death (N = 1; n = 1) 13 1 d after treatment, symptoms deteriorated (N = 1; n = 1) 17 AEDs administered to control NCSE (N = 2; n = 2) 14,15 NA Negative: N = 1; n = 1, developed refractory seizures and PIS 16 No response: N = 6 (67%); n = 7 (41%) within 24 h of end of treatment Unclear: N = 6/10 patient had 0 ictuses assessed by MRI 11 NA N/R: (N = 10; n = 14) 14,18,25,35,39,40,42,45 NA N/R: N = 4; n = 4 16,21,23,34 Abbreviations: AED = antiepileptic drug; DWI = diffusion-weighted imaging; FLAIR = fluid-attenuated inversion recovery; MRS = magnetic resonance spectroscopy; n = number of stroke-like episodes; N = number of patients; N/R = not reported; NA = not applicable; NAA/Cho = N-acetylaspartate/choline; NSCE = nonconvulsive status epilepticus; PIS = propofol infusion syndrome; rCBF = regional cerebral blood flow.…”

Section: Clinical Responsementioning

confidence: 99%

“…Positive: N = 7, N = 6 on AEDs Remained free of attacks except when treatment was stopped on 2 occasions during which she had recurrent cerebral metabolic attacks (N = 1) 23 Prevented further stroke-like episodes (N = 5) 12,22,25,45,46 No stroke-like episodes and his visual field defect improved. Occasional seizures but less than before (N = 1) 48 Positive: N = 6 Frequency and severity of stroke-like episodes (score based on symptoms) decreased after 18 mo 4…”

Section: Clinical Responsementioning

confidence: 99%

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l -Arginine in Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes

Stefanetti

Errington

et al. 2022

Neurology

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Objective:Stroke management in the context of primary mitochondrial disease is clinically challenging and the best treatment options for patients with stroke-like episodes remain uncertain. We sought to perform a systematic review on the safety and efficacy of L-arginine use in the acute and prophylactic management of stroke-like episodes in patients with mitochondrial disease.Methods:The systematic review was registered in PROSPERO (CRD42020181230). We searched six databases from inception − 15/01/2021: MEDLINE, Embase, Scopus, Web of Science, CINAHL and ClinicalTrials.gov. Original articles and registered trials available, in English, reporting L-arginine use in the acute or prophylactic management of stroke-like episodes in patients with genetically confirmed mitochondrial disease were eligible for inclusion.Data on safety and treatment response were extracted and summarized by multiple observers. Risk of bias was assessed by the methodological quality of case reports, case series and a risk-of-bias checklist for non-randomized studies. Quality of evidence was synthesized using the Oxford Centre for Evidence-Based Medicine Levels of Evidence and Grade of Recommendations. The predetermined main outcome measures were clinical response to L-arginine treatment, adverse events, withdrawals, and deaths (on treatment and/or during follow up), as defined by the author.Results:Thirty-seven articles met inclusion [0=randomised controlled trials (RCTs); 3 open-label; 1 retrospective cohort; 33 case reports/case series] (N = 91 patients; 86% m.3243A>G). In the case reports, 54% of patients reported a positive clinical response to acute L-arginine, of which 40% were concomitantly treated with AEDs. Improved headache at 24-hours was the greatest reported benefit in response to IV L-arginine in the open-label trials (31/39, 79%). Of 15/48 patients (31%) who positively responded to prophylactic L-arginine, AEDs were either used (7/15) or unreported (8/15). Moderate adverse events were reported in the follow-up of both IV and oral L-arginine treatment and 11 patients (12%) died during follow up or while on prophylactic treatment.Conclusion:The available evidence is of poor methodological quality and classified as Level 5. IV and/or oral L-arginine confers no demonstrable clinical benefit in either the acute or prophylactic treatment of MELAS, with more robust controlled trials required to assess its efficacy and safety profile.

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