Differentiating Oneiric Stupor in Agrypnia Excitata From Dreaming Disorders

Baldelli, Luca; Provini, Federica

doi:10.3389/fneur.2020.565694

Cited by 7 publications

(3 citation statements)

References 148 publications

(233 reference statements)

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“…The term agrypnia excitata (agrypnia meaning “chasing sleep away” referring to sleep loss of organic origin, and excitata referring to the motor and autonomic activation) defines a generalized overactivation syndrome characterized by severe and persistent insomnia and marked motor and autonomic sympathetic activation (including weight loss and hyperthermia) [ 14 ]. Oneiric stupor is described as a peculiar behavior of agrypnia excitata, with the recurrence of stereotyped gestures mimicking simple daily life activities associated with the reporting of a dream mentation consisting in a single oneiric scene [ 15 , 16 ]. Video PSG objective measures of agrypnia/sleep loss typically show reduction or disappearance of slow waves sleep, disappearance of spindles and the persistence of stage 1 non-rapid eye movement (NREM) sleep while rapid eye movement (REM) sleep, when identified, may persist but fails to stabilize, appearing in short recurrent episodes, either isolated, or mixed with stage 1 NREM sleep [ 14 , 17 ].…”

Section: Resultsmentioning

confidence: 99%

Proposal of new diagnostic criteria for fatal familial insomnia

et al. 2022

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Background The understanding of fatal familial insomnia (FFI), a rare neurodegenerative autosomal dominant prion disease, has improved in recent years as more cases were reported. This work aimed to propose new diagnostic criteria for FFI with optimal sensitivity, specificity, and likelihood ratio. Methods An international group of experts was established and 128 genetically confirmed FFI cases and 281 non-FFI prion disease controls are enrolled in the validation process. The new criteria were proposed based on the following steps with two-round expert consultation: (1) Validation of the 2018 FFI criteria. (2) Diagnostic item selection according to statistical analysis and expert consensus. (3) Validation of the new criteria. Results The 2018 criteria for possible FFI had a sensitivity of 90.6%, a specificity of 83.3%, with a positive likelihood ratio (PLR) of 5.43, and a negative likelihood ratio (NLR) of 0.11; and the probable FFI criteria had a sensitivity of 83.6%, specificity of 92.9%, with a PLR of 11.77, and a NLR of 0.18. The new criteria included more specific and/or common clinical features, two exclusion items, and summarized a precise and flexible diagnostic hierarchy. The new criteria for possible FFI had therefore reached a better sensitivity and specificity (92.2% and 96.1%, respectively), a PLR of 23.64 and a NLR of 0.08, whereas the probable FFI criteria showed a sensitivity of 90.6%, a specificity of 98.2%, with a PLR of 50.33 and a NLR of 0.095. Conclusions We propose new clinical diagnostic criteria for FFI, for a better refining of the clinical hallmarks of the disease that ultimately would help an early recognition of FFI and a better differentiation from other prion diseases.

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Section: Resultsmentioning

confidence: 99%

Proposal of new diagnostic criteria for fatal familial insomnia

et al. 2022

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“…Antibodies to CASPR2, and to a lesser extent, LGI1, are associated with peripheral nerve hyperexcitability (manifesting as neuropathic pain and neuromyotonia), as well as Morvan syndrome, which also features prominent neuropsychiatric symptoms, dysautonomia (especially hyperhidrosis and hemodynamic instability), and disordered sleep leading to a state once classically described as "agrypnia excitata" [89,90,[95][96][97]. Those with reactivity to CASPR2 frequently present with limbic symptoms at onset, more often seizures than cognitive dysfunction, and most have limbic involvement at some point during their clinical course.…”

Section: Paraneoplastic Encephalitidesmentioning

confidence: 99%

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

et al. 2023

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Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associated with antibodies against neural surface antigens, known as “intermediate- or low-risk” antibodies, are less frequently associated with cancer. In this narrative review, we will focus on PNS of the central nervous system (CNS). Clinicians should have a high index of suspicion with acute/subacute encephalopathies to achieve a prompt diagnosis and treatment. PNS of the CNS exhibit a range of overlapping “high-risk” clinical syndromes, including but not limited to latent and overt rapidly progressive cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome, paraneoplastic (and limbic) encephalitis/encephalomyelitis, and stiff-person spectrum disorders. Some of these phenotypes may also arise from recent anti-cancer treatments, namely immune-checkpoint inhibitors and CAR T-cell therapies, as a consequence of boosting of the immune system against cancer cells. Here, we highlight the clinical features of PNS of the CNS, their associated tumors and antibodies, and the diagnostic and therapeutic strategies. The potential and the advance of this review consists on a broad description on how the field of PNS of the CNS is constantly expanding with newly discovered antibodies and syndromes. Standardized diagnostic criteria and disease biomarkers are fundamental to quickly recognize PNS to allow prompt treatment initiation, thus improving the long-term outcome of these conditions.

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“…O fenótipo clínico da IFF coincide com as áreas cerebrais afetadas e sua marca clínica é a Agrypnia Excitata (AE), embora tal síndrome não seja uma manifestação exclusiva da IFF, estando presente em outras patologias como a Síndrome de Morvan, uma forma de encefalite autoimune, e o Delirium Tremens, uma síndrome de abstinência álcoólica (Baldelli & Provini, 2019). Além de insônia grave persistente e um estado de superativação simpática motora e autonômica, a AE também é marcada Research, Society and Development, v. 10, n. 14, e168101421719, 2021 (CC BY 4.0) | ISSN 2525-3409 | DOI: http://dx.doi.org/10.33448/rsd-v10i14.21719 3 por um quadro peculiar de estupor onírico advindo da desestruturação da arquitetura cíclica fisiológica do sono (Baldelli & Provini, 2020).…”

Section: Introductionunclassified

Índice de prevalência e principais manifestações clínicas iniciais da insônia familiar fatal

Barbosa

Santos

Sousa

et al. 2021

RSD

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Introdução: A insônia familiar fatal (IFF) é uma doença priônica autossômica dominante e neurodegenerativa causada por uma mutação no gene da proteína príon celular (PrPC). O objeivo dessa revisão é identificar as manifestações clínicas iniciais da IFF e sua distribuição geográfica durante a última década. Metodologia: Trata-se de uma revisão sistemática da literatura incluindo relatos de casos sobre a IFF, publicados no período de 2011 a 2021, indexados na base de dados do PubMed/MEDLINE. Resultados: Foram detectados 49 indivíduos portadores de IFF. Os sintomas iniciais mais comuns foram insônia progressiva (55%), déficits cognitivos (25%), redução ponderal (25%), alterações da marcha (25%) e desorientação (25%). Alguns casos apresentaram sintomas sugestivos de outras hipóteses diagnósticas como tremores (15%) e alterações do humor (10%). Os EUA apresentaram frequência absoluta de 17 pessoas distribuídas em três famílias. A China teve um total de dez afetados por IFF distribuídos em cinco famílias. Brasil, Alemanha e Coreia do Sul apresentaram duas famílias afetadas cada. Porém, quanto ao total de portadores de IFF, Brasil apresentou sete e Coreia do Sul, França e Espanha, três portadores cada. Conclusão: a China e o EUA apresentam as maiores frequências absolutas de IFF, contudo, alguns países europeus, como Espanha e França, possuem uma frequência relativa maior. Apesar de a insônia ser o sintoma inicial mais comum, existem casos nos quais a IFF pode se manifestar inicialmente com sintomas que mimetizam outras patologias neurodegenerativas ou patologias de ordem psicológica, dificultando o diagnóstico.

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Differentiating Oneiric Stupor in Agrypnia Excitata From Dreaming Disorders

Cited by 7 publications

References 148 publications

Proposal of new diagnostic criteria for fatal familial insomnia

Proposal of new diagnostic criteria for fatal familial insomnia

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

Índice de prevalência e principais manifestações clínicas iniciais da insônia familiar fatal

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