Opisthotonus (arc de cercle) in anti-NMDAR encephalitis

Delgado-García, Guillermo; Cano-Nigenda, Vanessa; Abundes-Corona, Arturo; Carrillo-Loza, Karina; Calleja-Castillo, Juan Manuel; Flores‐Rivera, José

doi:10.1590/0004-282x20180047

Cited by 3 publications

(3 citation statements)

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“…The similarities between opisthotonus movements in anti-NMDAR encephalitis and the arc de cercle in hystero-epilepsy ( Fig. 1) have been indicated previously [28]. However, repetitive opisthotonus movements are not uniquely seen in anti-NMDAR encephalitis.…”

Section: Discussionsupporting

confidence: 78%

Reanalysis of the patients who were diagnosed with hystero-epilepsy by Charcot

Komagamine¹,

Kokubun²,

Hirata³

2020

Preprint

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Background: The term "hysteria" is now recognized to refer to non-organic and functional disorders. Jean-Martin Charcot once attempted to identify the lesions responsible for hysteria as an organic disorder in his later years, but his effort failed. He classi ed hysteria as usual and grand. Grand hysteria was also called hystero-epilepsy, which became obsolete after Charcot's death and has been an enigma in modern neurology. After anti-NMDA receptor (NMDAR) encephalitis was established in the twenty-rst century, a paradigm shift emerged in the interpretation of acute onset of neuropsychiatric symptoms. Patients with anti-NMDAR encephalitis demonstrate a characteristic multistage progression and stereotypical movements, including opisthotonic posture, which are analogous to the staging progressions and ' arc de cercle' observed in Charcot's hystero-epilepsy.Methods: To elucidate the full picture of Charcot's hystero-epilepsy, we reviewed the patients presented in "the Tuesday Lessons".Results: Eleven patients were shown to have hystero-epilepsy by Charcot himself. Of the 11 patients, 4 patients had acute-onset neuropsychiatric symptoms, and 2 patients had a condition that resembled anti-NMDAR encephalitis. Although the clinical courses were similar to those of anti-NMDAR encephalitis, we could not make conclusive diagnoses of anti-NMDAR encephalitis without examinations due to the lack of evidence of teratomas. One patient with alcohol withdrawal syndrome and one patient with chronic pain and morphine withdrawal syndrome were also classi ed as having hystero-epilepsy by Charcot. The other patients, who manifested chronic courses or varying hypnosis-induced neurological symptoms, could be diagnosed with conversion and dissociative disorders today.Conclusions: Charcot's hystero-epilepsy has been suspected of being a syndrome encompassing conversion and dissociative disorders, drug withdrawal syndrome, and anti-NMDAR encephalitis-like manifestations. Charcot's observations combined with the current progress in molecular biology suggest that a common dysfunctional basis exists, such as glutamate/NMDAR system dysfunction, among these conditions.

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Section: Discussionsupporting

confidence: 78%

Reanalysis of the patients who were diagnosed with hystero-epilepsy by Charcot

Komagamine¹,

Kokubun²,

Hirata³

2020

Preprint

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“…Maple syrup urine disease [17] Meningitis [18,19] Monoamine neurotransmitter defect [20,21] Encephalitis [22,23] Wilson disease [24] Tetanus [1,[25][26][27][28] Neurodegeneration with brain iron accumulation (NBIA), e.g., pantothenate kinase-associated neurodegeneration (PKAN) [15] Rabies [29] Other genetic disorders 1 [21,30] Neurosyphilis [31] Cerebral malaria [12] Toxins/elevated toxic metabolites Kernicterus [12] Strychnine [32] Phencyclidine [32] Phenothiazines [32] Propofol [33,34] Methoxphenidine [35] Neonatal caffeine overdose [32] Increased Intracranial Pressure Subarachnoid hemorrhage [5] Intracerebral hemorrhage [5] Hydrocephalus [36] Mass/Tumor [36] Autoimmune…”

Section: Infectious/post-infectiousmentioning

confidence: 99%

“…Stiff-person syndrome [37] Anti-NMDA receptor encephalitis [22] Other Functional (psychogenic) dystonia [3,4,38] Tardive dystonia [6,30,[39][40][41][42][43] Malignant catatonia [29] Brainstem/cerebellar malformation [44] Epilepsy [12] 1 Any genetic syndromes with prominent dystonia have the potential to manifest opisthotonus.…”

Section: Infectious/post-infectiousmentioning

confidence: 99%

Botulinum Neurotoxin Injections in Childhood Opisthotonus

Hull

Parnes

Jankovic

2021

Toxins

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Opisthotonus refers to abnormal axial extension and arching of the trunk produced by excessive contractions of the paraspinal muscles. In childhood, the abnormal posture is most often related to dystonia in the setting of hypoxic injury or a number of other acquired and genetic etiologies. The condition is often painful, interferes with ambulation and quality of life, and is challenging to treat. Therapeutic options include oral benzodiazepines, oral and intrathecal baclofen, botulinum neurotoxin injections, and deep brain stimulation. Management of opisthotonus within the pediatric population has not been systematically reviewed. Here, we describe a series of seven children who presented to our institution with opisthotonus in whom symptom relief was achieved following administration of botulinum neurotoxin injections.

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Autoimmune Encephalitis in Latin America: A Critical Review

et al. 2021

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Autoimmune encephalitis is an increasingly recognized cause of encephalitis. The majority of case series report patients residing in developed countries in the northern hemisphere. The epidemiologic features of autoimmune encephalitis in Latin America are still unclear. The aim of the study was to perform a review of the clinical presentation of autoimmune encephalitis in Latin America and compare to world literature. References were identified by an in-depth literature search and selected on the basis of relevance to the topic and authors' judgment. We selected clinical studies and case reports published from 2007 to July, 2020 including patients from Latin American countries. Of the 379 patients included, the majority were cases of anti-NMDA receptor encephalitis (93.14%), followed by anti-VGKC-complex encephalitis (N = 17; 4.48%), anti-GAD encephalitis (N = 9; 2.37%), anti-AMPA receptor encephalitis (N = 1; 0.26%), anti-GABA receptor encephalitis (N = 1; 0. 26%), anti-mGluR5 encephalitis (N = 1; 0. 26%), and anti-mGluR1 encephalitis (N = 1; 0. 26%). Reported cases of Anti-NMDA encephalitis in Latin-America had a very slight female predominance, lower prevalence of associated tumors and a lower incidence of extreme delta brush on electroencephalogram. Autoimmune encephalitis is possibly underdiagnosed in underdeveloped countries. Its outcome after treatment, however, appears to be similarly favorable in Latin American patients as has been reported in developed countries based on available case reports and case series. Regional specificities in the manifestation of autoimmune encephalitis could be related to epidemiologic factors, such as the presence of different triggers and different genetic and immunologic background, that need to be studied by future research.

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Opisthotonus (arc de cercle) in anti-NMDAR encephalitis

Cited by 3 publications

References 1 publication

Reanalysis of the patients who were diagnosed with hystero-epilepsy by Charcot

Reanalysis of the patients who were diagnosed with hystero-epilepsy by Charcot

Botulinum Neurotoxin Injections in Childhood Opisthotonus

Autoimmune Encephalitis in Latin America: A Critical Review

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