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Hypoglycemic encephalopathy

MedGen UID:
101772
Concept ID:
C0149877
Disease or Syndrome
Synonym: hypoglycemic encephalopathy
SNOMED CT: Hypoglycemic encephalopathy (64624009)
 
HPO: HP:0006929

Definition

Brain damage related to a lowering of blood glucose below a critical level (around 30 mg/dl), which may lead to confusion, lethargy and delirium followed by seizures and coma. Prolonged hypoglycemia may lead to irreversible brain damage. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Hypoglycemic encephalopathy

Conditions with this feature

Deficiency of 3-hydroxyacyl-CoA dehydrogenase
MedGen UID:
266222
Concept ID:
C1291230
Disease or Syndrome
3-hydroxyacyl-CoA dehydrogenase deficiency is an inherited condition that prevents the body from converting certain fats to energy, particularly during prolonged periods without food (fasting).\n\nInitial signs and symptoms of this disorder typically occur during infancy or early childhood and can include poor appetite, vomiting, diarrhea, and lack of energy (lethargy). Affected individuals can also have muscle weakness (hypotonia), liver problems, low blood glucose (hypoglycemia), and abnormally high levels of insulin (hyperinsulinism). Insulin controls the amount of glucose that moves from the blood into cells for conversion to energy. Individuals with 3-hydroxyacyl-CoA dehydrogenase deficiency are also at risk for complications such as seizures, life-threatening heart and breathing problems, coma, and sudden death. This condition may explain some cases of sudden infant death syndrome (SIDS), which is defined as unexplained death in babies younger than 1 year.\n\nProblems related to 3-hydroxyacyl-CoA dehydrogenase deficiency can be triggered by periods of fasting or by illnesses such as viral infections. This disorder is sometimes mistaken for Reye syndrome, a severe disorder that may develop in children while they appear to be recovering from viral infections such as chicken pox or flu. Most cases of Reye syndrome are associated with the use of aspirin during these viral infections.

Professional guidelines

PubMed

Cao DM, Liang QF, Zhang ZT, He WJ, Tang D
Chem Biodivers 2023 Sep;20(9):e202300434. Epub 2023 Aug 10 doi: 10.1002/cbdv.202300434. PMID: 37486314

Recent clinical studies

Etiology

Belenichev I, Aliyeva O, Popazova O, Bukhtiyarova N
Acta Biochim Pol 2023 Nov 22;70(4):751-760. doi: 10.18388/abp.2020_6953. PMID: 37991083
Simon Machado R, Mathias K, Joaquim L, Willig de Quadros R, Petronilho F, Tezza Rezin G
Brain Res 2023 Dec 15;1821:148611. Epub 2023 Oct 2 doi: 10.1016/j.brainres.2023.148611. PMID: 37793604
Diviccaro S, Cioffi L, Piazza R, Caruso D, Melcangi RC, Giatti S
Biomolecules 2023 Aug 29;13(9) doi: 10.3390/biom13091325. PMID: 37759725Free PMC Article
Sangare A, Marois C, Perlbarg V, Pyatigorskaya N, Valente M, Zyss J, Borden A, Lambrecq V, Le Guennec L, Sitt J, Weiss N, Rohaut B, Demeret S, Puybasset L, Demoule A, Naccache L
Neurocrit Care 2023 Apr;38(2):365-377. Epub 2022 Sep 15 doi: 10.1007/s12028-022-01594-0. PMID: 36109449
Young CW
Am J Med 1985 Sep 20;79(3B):8-11. doi: 10.1016/s0002-9343(85)80002-4. PMID: 3901746

Diagnosis

Mason Sharma A, Birnhak A, Sanborn E, Bhana N, Kazmi K, Thon JM, Thon OR, Siegler JE
J Neuroimaging 2023 Jul-Aug;33(4):467-476. Epub 2023 Apr 18 doi: 10.1111/jon.13106. PMID: 37070435
Álvarez Méndez A, Delgado Moya FP, Valiente Fernandez M
Med Intensiva (Engl Ed) 2023 Jun;47(6):361-362. Epub 2022 Nov 29 doi: 10.1016/j.medine.2022.10.014. PMID: 36460610
Sangare A, Marois C, Perlbarg V, Pyatigorskaya N, Valente M, Zyss J, Borden A, Lambrecq V, Le Guennec L, Sitt J, Weiss N, Rohaut B, Demeret S, Puybasset L, Demoule A, Naccache L
Neurocrit Care 2023 Apr;38(2):365-377. Epub 2022 Sep 15 doi: 10.1007/s12028-022-01594-0. PMID: 36109449
Kuriyama A, Sato K
CMAJ 2015 Oct 6;187(14):E437. Epub 2015 Jul 13 doi: 10.1503/cmaj.141040. PMID: 26170269Free PMC Article
Alkalay AL, Sarnat HB, Flores-Sarnat L, Simmons CF
Isr Med Assoc J 2005 Mar;7(3):188-92. PMID: 15792267

Therapy

Hasebe M, Shibue K, Honjo S, Hamasaki A
QJM 2022 Jul 9;115(7):478-479. doi: 10.1093/qjmed/hcac125. PMID: 35583317
Shimizu S, Sakamoto S, Horikawa R, Fukuda A, Uchida H, Takeda M, Yanagi Y, Irie R, Yoshioka T, Kasahara M
Liver Transpl 2020 Jan;26(1):57-67. Epub 2019 Nov 26 doi: 10.1002/lt.25649. PMID: 31587472
Chuang KI, Hsieh KL, Chen CY
BMC Med Imaging 2019 Jan 24;19(1):11. doi: 10.1186/s12880-019-0310-z. PMID: 30678650Free PMC Article
Miura K, Kameda T, Suzukawa M
Intern Med 2015;54(8):989. Epub 2015 Apr 15 doi: 10.2169/internalmedicine.54.3683. PMID: 25876589
Young CW
Am J Med 1985 Sep 20;79(3B):8-11. doi: 10.1016/s0002-9343(85)80002-4. PMID: 3901746

Prognosis

Sangare A, Marois C, Perlbarg V, Pyatigorskaya N, Valente M, Zyss J, Borden A, Lambrecq V, Le Guennec L, Sitt J, Weiss N, Rohaut B, Demeret S, Puybasset L, Demoule A, Naccache L
Neurocrit Care 2023 Apr;38(2):365-377. Epub 2022 Sep 15 doi: 10.1007/s12028-022-01594-0. PMID: 36109449
Lee YE, Lee EJ, Lee SE, Park J
Front Endocrinol (Lausanne) 2022;13:956367. Epub 2022 Aug 16 doi: 10.3389/fendo.2022.956367. PMID: 36051391Free PMC Article
Ma JH, Kim YJ, Yoo WJ, Ihn YK, Kim JY, Song HH, Kim BS
Neuroradiology 2009 Oct;51(10):641-9. Epub 2009 Jun 17 doi: 10.1007/s00234-009-0544-5. PMID: 19533113
Gallucci M, Limbucci N, Paonessa A, Caranci F
Neuroradiology 2007 Jul;49(7):541-4. Epub 2007 May 24 doi: 10.1007/s00234-007-0235-z. PMID: 17522852
Young CW
Am J Med 1985 Sep 20;79(3B):8-11. doi: 10.1016/s0002-9343(85)80002-4. PMID: 3901746

Clinical prediction guides

Sangare A, Marois C, Perlbarg V, Pyatigorskaya N, Valente M, Zyss J, Borden A, Lambrecq V, Le Guennec L, Sitt J, Weiss N, Rohaut B, Demeret S, Puybasset L, Demoule A, Naccache L
Neurocrit Care 2023 Apr;38(2):365-377. Epub 2022 Sep 15 doi: 10.1007/s12028-022-01594-0. PMID: 36109449
Lee YE, Lee EJ, Lee SE, Park J
Front Endocrinol (Lausanne) 2022;13:956367. Epub 2022 Aug 16 doi: 10.3389/fendo.2022.956367. PMID: 36051391Free PMC Article
Ikeda T, Takahashi T, Sato A, Tanaka H, Igarashi S, Fujita N, Kuwabara T, Kanazawa M, Nishizawa M, Shimohata T
Diabetes Res Clin Pract 2013 Aug;101(2):159-63. Epub 2013 Jun 29 doi: 10.1016/j.diabres.2013.05.007. PMID: 23820485
Witsch J, Neugebauer H, Flechsenhar J, Jüttler E
J Neurol 2012 Oct;259(10):2172-81. Epub 2012 Apr 11 doi: 10.1007/s00415-012-6480-z. PMID: 22491856
Ma JH, Kim YJ, Yoo WJ, Ihn YK, Kim JY, Song HH, Kim BS
Neuroradiology 2009 Oct;51(10):641-9. Epub 2009 Jun 17 doi: 10.1007/s00234-009-0544-5. PMID: 19533113

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