ABSTRACT
Agrypnia excitata represents a triad of three subgroups of disorders having similar clinical characteristics, namely, delirium tremens caused by alcohol withdrawal, fatal familial insomnia (an autosomal dominant prion disease) and Morvan syndrome (autoimmune encephalitis). All three disorders are characterised by motor, autonomic and hormonal hyperactivity. Clinically, the involvement of the peripheral, central and autonomic nervous systems combined with insomnia symptomatology should be considered indicative of agrypnia excitata because early diagnosis and treatment is essential for preventing disease-related mortality. Herein, we present the case of a patient who was admitted with complaints of sudden-onset diplopia, loss of balance, hallucinations and insomnia and diagnosed with delirium tremens. A literature review, including data obtained most recently on agrypnia excitata, delirium tremens, fatal familial insomnia and Morvan syndrome, was conducted with this context.
Keywords: Agrypnia excitata, oneiric stupor, delirium tremens
References
1
Lugaresi E, Provini F, Cortelli P. Agrypnia excitata. Sleep Med 2011;12(Suppl 2):S3-10.
2
Hazin R, Abuzetun JY, Giglio P, Khan F. Agrypnia excitata: current concepts and future prospects in management. J Neuropsychiatry Clin Neurosci 2009;21:126-31.
3
Provini F, Cortelli P, Montagna P, Gambetti P, Lugaresi E. Fatal insomnia and agrypnia excitata: Sleep and the limbic system. Revue Neurologique 2008;164:692-700.
4
Lugaresi E, Provini F. Agrypnia excitata: Clinical features and pathophysiological implications. Sleep Med Rev 2001;5:313-22.
5
Montagna P, Lugaresi E. Agrypnia excitata: a generalized overactivity syndrome and a useful concept in the neurophysiopathology of sleep. Clin Neurophysiol 2002;113:552-60.
6
Provini F. Agrypnia excitata. Curr Neurol Neurosci Rep 2013;13:1-6.
7
Rahman A, Paul M. Delirium Tremens. 2020 Aug 29. In: StatPearls [Internet]. StatPearls Publishing, Treasure Island (FL), 2020.
8
Porcel FJ, Schutta HS. From antiquity to the n-methyl-d-aspartate receptor: a history of delirium tremens. J Hist Neurosci 2015;24:378-95.
9
Hughes JR. Alcohol withdrawal seizures. Epilepsy Behav 2009; 2:92-7.
10
Sarff M, Gold JA. Alcohol withdrawal syndromes in the intensive care unit. Crit Care Med 2010;38:494-501.
11
Baldelli L, Provini F. Differentiating oneiric stupor in agrypnia excitata from dreaming disorders. Front Neurol 2020;11:565694.
12
Wright T, Myrick H, Henderson S, Peters H, Malcolm R. Risk factors for delirium tremens: a retrospective chart review. Am J Addict 2006;15:213-9.
13
Berggren U, Fahlke C, Berglund KJ, Blennow K, Zetterberg H, Balldin J. Thrombocytopenia in early alcohol withdrawal is associated with development of delirium tremens or seizures. Alcohol Alcohol 2009;44:382-6.
14
Eyer F, Schuster T, Felgenhauer N, Pfab R, Strubel T, Saugel B, Zilker T. Risk assessment of moderateto severe alcohol withdrawal predictors for seizures and delirium tremens in the course of withdrawal. Alcohol Alcohol 2011;46:427-33.
15
Mainerova B, Prasko J, Latalova K, Axmann K, Cerna M, Horacek R, Bradacova R. Alcohol withdrawal delirium diagnosis, course and treatment. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2015;159:44-52.
16
Lee JA, Duby JJ, Cocanour CS. Effect of early and focused benzodiazepine therapy on length of stay in severe alcohol withdrawal syndrome. Clin Toxicol (Phila) 2019;57:624-7.
17
Grover S, Ghosh A. Delirium tremens: assessment and management. J Clin Exp Hepatol 2018;8:460-70.
18
Levine AR, Thanikonda V, Mueller J, Naut ER. Front-loaded diazepam versus lorazepam for treatment of alcohol withdrawal agitated delirium. Am J Emerg Med 2021;44:415-8.
19
Kosten TR, O’Connor PG. Management of drug and alcohol withdrawal. N Engl J Med 2003;348:1786-95.
20
Schuckit MA. Recognition and management of withdrawal delirium (delirium tremens). N Engl J Med 2014;371:2109-13.
21
Malcolm R, Myrick H, Roberts J, Wang W, Anton RF, Ballenger JC. The effects of carbamazepine and lorazepam on single versus multiple previous alcohol withdrawals in an outpatient randomized trial. J Gen Intern Med 2002;17:349-55.
22
Farrokh S, Roels C, Owusu KA, Nelson SE, Cook AM. Alcohol withdrawal syndrome in neurocritical care unit: assessment and treatment challenges. Neurocrit Care 2021;34:593-607.
23
Montagna P. Fatal familial insomnia and the role of the thalamus in sleep regulation. Handb Clin Neurol 2011;99:981-96.
24
Baldelli L, Provini F, Cortelli P. Fatal familial ınsomnia: a rare disease with unique clinico-neurophysiological features. Mov Disord Clin Pract 2020;8:162-3.
25
Watts JC, Balachandran A, Westaway D. The expanding universe of prion diseases. PLoS Pathog 2006;2:e26. doi: 10.1371/journal.ppat.0020026
26
Baldelli L, Provini F. Fatal familial insomnia and Agrypnia Excitata: autonomic dysfunctions and pathophysiological implications. Auton Neurosci 2019;218:68-86.
27
Cracco L, Appleby BS, Gambetti P. Fatal familial insomnia and sporadic fatal insomnia. Handb Clin Neurol 2018;153:271-99.
28
Xiao K, Shi Q, Zhou W, Dong XP. Different post-mortem brain regions from three Chinese FFI patients induce different reactive profiles both in thefirst and second generation RT-QuIC assays. Prion 2020;14:163-9.
29
Guaraldi P, Calandra-Buonaura G, Terlizzi R, Montagna P, Lugaresi E, Tinuper P, Cortelli P, Provini F. Oneiric stupor: the peculiar behaviour of agrypnia excitata. Sleep Med 2011;12(Suppl 2):S64-7.
30
Roguski A, Gill AC. The role of the mammalian prion protein in the control of sleep. Pathogens 2017;6:58.
31
Dauvilliers Y, Cervena K, Carlander B, Espa F, Bassetti C, Claustrat B, Laplanche JL, Billiard M, Touchon J. Dissociation in circadian rhythms in a pseudohypersomnia form of fatal familial insomnia. Neurology 2004;63:2416-8.
32
Montagna P, Gambetti P, Cortelli P, Lugaresi E. Familial and sporadic fatal insomnia. Lancet Neurol 2003;2:167-76.
33
Mastrangelo V, Merli E, Rucker JC, Eggenberger ER, Zee DS, Cortelli P. Neuro-Ophthalmological Findings in Early Fatal Familial Insomnia. Ann Neurol 2021;89:823-7.
34
Froböse T, Slawik H, Schreiner R, Veselý Z, Wiegand M, Bäuml J, Förstl H. Agomelatine improves sleep in a patient with fatal familial insomnia. Pharmacopsychiatry 2012;45:34-6.
35
Schenkein J, Montagna P. Self-management of fatal familial insomnia, part 2: case report. Med Gen Med 2006;8:66.
36
Forloni G, Tettamanti M, Lucca U, Albanese Y, Quaglio E, Chiesa R, Erbetta A, Villani F, Redaelli V, Tagliavini F, Artuso V, Roiter I. Preventive study in subjects at risk of fatal familial insomnia: innovative approach to rare diseases. Prion 2015;9:75-9.
37
Toribio-Díaz E, Quintas S, Peláez-Hidalgo A, Villacieros-Álvarez J, GarcíaCobos E, GarcíaDi-Ruggiero E. Fatal familial insomnia: a new case description with early response to immunotherapy. J Neuroimmunol 2020;346:577321.
38
Liguori R, Vincent A, Clover L, Avoni P, Plazzi G, Cortelli P, Baruzzi A, Carey T, Gambetti P, Lugaresi E, Montagna P. Morvan’ssyndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain 2001;124:2417-26.
39
Vincent A, Buckley C, Schott JM, Baker I, Dewar BK, Detert N, Clover L, Parkinson A, Bien CG, Omer S, Lang B, Rossor MN, Palace J. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 2004;127:701-12.
40
Cornelius JR, Pittock SJ, McKeon A, Lennon VA, Aston PA, Josephs KA, Tippmann-Peikert M, Silber MH. Sleep manifestations of voltage-gated potassium channel complex autoimmunity. Arch Neurol 2011;68:733-8.
41
Masood W, Sitammagari KK. Morvan Syndrome. 2020 Aug 16. In: StatPearls [Internet]. StatPearls Publishing, Treasure Island (FL), 2020.
42
Masrori P, Vaesen Bentein H, Raskin J, Montagna M, De Picker L, De Volder I, Van Schil PE, Janssens A, Mercelis R. Caspr2 autoantibody-associated Morvan syndrome predating thymoma relapse by 30 months. Lung Cancer 2021;153:117-9.
43
Katirji B. Peripheral nerve hyperexcitability. Handb Clin Neurol 2019;161:281-90.
44
Provini F, Marconi S, Amadori M, Guaraldi P, Pierangeli G, Cortelli P, Lugaresi E, Montagna P, Tinuper P. Morvan chorea and agrypnia excitata: when video- polysomnographic recording guides the diagnosis. Sleep Med 2011;12:1041-3.
45
Lanuzza B, Aricò D, Cosentino F, Provini F, Ferri R. Video-polysomnographicstudy of a patient with Morvan’s Fibrillary Chorea. Sleep Med 2012;13:550-3.
46
Morvan A. De la chorée fibrillaire. Gaz Hebd Med Chir 1890;27:173-200.
47
Hudson LA, Rollins YD, Anderson CA, Johnston-Brooks C, Tyler KL, Filley CM. Reduplicative paramnesia in Morvan’s syndrome. J Neurol Sci 2008;267:154-7.
48
Aytaç E, Acar T. A case report of Morvan syndrome. Ideggyogy Sz 2019;72:285-8.
