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Ann Indian Acad Neurol
Ann Indian Acad Neurol
AIAN
Ann Indian Acad Neurol
Annals of Indian Academy of Neurology
0972-2327
1998-3549
Wolters Kluwer - Medknow India

AIAN-27-451
10.4103/aian.aian_50_24
Letters to the Editor
Stroke-Like Lesions or Epiphenomena of Seizures in COQ8A-Related Coenzyme-Q10 Deficiency
Finsterer Josef
Department of Neurology, Neurology and Neurophysiology Center, Vienna, Austria
Address for correspondence: Prof. Josef Finsterer, Postfach 20, 1180 Vienna, Austria. E-mail: fifigs1@yahoo.de
Jul-Aug 2024
15 5 2024
27 4 451452
19 1 2024
09 3 2024
Copyright: © 2024 Annals of Indian Academy of Neurology
2024
https://creativecommons.org/licenses/by-nc-sa/4.0/ This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
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pmcDear Editor,

We read with interest Ramesh et al.’s[1] article about a 62-year-old man with intractable myoclonus epilepsy as a manifestation of coenzyme-Q deficiency due to the variant p.Arg299Trp in COQ8A. In addition, the patient presented with recurrent stroke-like episodes (SLEs) at the age of 57 years (left parieto-occipital region), 61 years (left temporoparietal region and thalamus), 61 years (right frontal and parietal regions), and 62 years (right frontal region).[1] He also developed Parkinson syndrome and global and cerebellar atrophy.[1] Despite polypragmatic antiepileptic treatment, steroids, and supplements, the patient died of sepsis in super-refractory status epilepticus.[1] The study is impressive, but several points require discussion.

The major limitation of the study is that only diffusion-weighted imaging (DWI) results were reported and presented in Figure 1.[1] To assess whether the four DWI hyperintensities in 2018, 2022, and 2023 were indeed stroke-like lesions (SLLs) or epiphenomena of recurrent seizure activity, it is imperative to present the results of T2/fluid-attenuated inversion recovery (FLAIR), perfusion-weighted imaging (PWI), and oxygen extraction fraction (OEF) imaging of all four described SLEs. Classic SLLs, the morphologic equivalent of SLEs on imaging, appear as hyperintensity on T2/FLAIR, DWI, and PWI and as hypointensity on OEF.[2] Another characteristic of SLLs is that they do not conform to a vascular territory. The third feature of SLLs is that they progress by increasing in size to regress after reaching a nadir, with no or residual structural brain damage. The latter includes cysts, white matter lesions, focal atrophy, laminar cortical necrosis, and the toenail sign.[2] The fourth feature of SLLs is that magnetic resonance spectroscopy (MRS) typically shows a lactate peak and a reduced N-acetyl aspartate (NAA) peak.[2] On fluorodeoxyglucose-positron emission tomography (FDG-PET), SLLs typically show hypometabolism.[3] We should know whether these criteria of an SLL have been met.

Surprisingly, cerebrospinal fluid (CSF) examinations were reported as normal. We should know if CSF lactate was measured and if it was elevated. We should also know whether MRS showed a lactate peak and a reduced NAA peak or not. Elevated CSF lactate is a typical feature of SLLs in all syndromic and nonsyndromic mitochondrial disorders.

Another limitation is that the reasons for administering steroids were not explained.[1] Was there evidence of immune encephalitis or acute disseminated encephalomyelitis? Steroids are known to have detrimental effects in at least certain mitochondrial phenotypes.[4]

Since the patient received maximum doses of propofol, we should know whether the worsening of super-refractory status epilepticus was due to the occurrence of propofol infusion syndrome.[5] Propofol is known to be a mitochondrion toxin and should therefore be avoided in patients with a mitochondrial disorder.

To sum up, the excellent study has limitations that should be addressed before final conclusions are drawn. Clarifying the weaknesses would strengthen the conclusions and improve the study. SLLs should be confirmed by multimodal magnetic resonance imaging, MRS, and FDG-PET. Propofol should be administered with caution to patients with mitochondrial disorder and myoclonus epilepsy, so that it does not cause intractable super-refractory status epilepticus.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.
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REFERENCES

1. Ramesh R Hariharan S Sundar L Stroke-like episodes and epilepsy in a patient with COQ8A-related coenzyme Q10 deficiency Ann Indian Acad Neurol 2023 26 980 2 38229639
2. Finsterer J Characteristics of stroke-like lesions on cerebral imaging Ideggyogy Sz 2023 76 5 10 36892301
3. Kim JH Lim MK Jeon TY Rha JH Eo H Yoo SY Diffusion and perfusion characteristics of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode) in thirteen patients Korean J Radiol 2011 12 15 24 21228936
4. Finsterer J Frank M Glucocorticoids for mitochondrial disorders Singapore Med J 2015 56 122 3 25715858
5. Finsterer J Mitochondrial disorders are prone to propofol infusion syndrome Acute Med Surg 2020 7 e495 33408871
