Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Benjumea-Cuartas, Vanessa; Eisermann, Monika; Simonnet, H; Hully, Marie; Nabbout, Rima; Desguerre, Isabelle; Kamińska, Anna

doi:10.1016/j.ebcr.2016.12.002

Cited by 6 publications

(5 citation statements)

References 41 publications

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“…Additionally, the unilateral nature of her manifestations raises concern regarding an underlying lesion that would necessitate, at the least, neuroimaging. This case also appears to confirm a propensity for unilateral symptoms in this condition as has been previously reported [ 5 ]. As noted, psychiatric conditions may be associated with comorbid movement disorders such as tic as in Tourette and stereotypy in autism.…”

Section: Discussionsupporting

confidence: 89%

“…A personal and family history of preceding neuropsychiatric symptoms produced diagnostic confusion and resulted in a significant diagnostic and therapeutic delay. Our case appears to confirm the unilateral movement manifestations that have been emphasized in recent reports [ 5 ]. Additionally, it emphasizes the need for involvement of neurologic as well as psychiatric services in the evaluation of such cases and demonstrates the importance of the neurologic examination in presentations with an initial psychiatric predominance.…”

Section: Introductionsupporting

confidence: 92%

“…Our case emphasizes both the expanded spectrum of movement phenomenon that might be seen in cases of anti-NMDA receptor antibody encephalitis and the variability of the encephalopathy at presentation and throughout the course. Additionally, recent reports indicate a propensity for unilateral presentations of the movement phenomenon as was clearly demonstrated in our patient with more than 8 months of hemiataxia without contralateral findings [ 5 ]. Testing for anti-NMDA antibody disorders has become readily available, with serum and spinal fluid analysis typically demonstrating positivity in affected individuals.…”

Section: Discussionsupporting

confidence: 71%

See 2 more Smart Citations

Hemiataxia: A Novel Presentation of Anti-NMDA Receptor Antibody Mediated Encephalitis in an Adolescent

Phillips

Jones

Callaghan

et al. 2017

Case Reports in Psychiatry

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Anti-NMDA receptor antibody associated encephalitis as a cause of new-onset neuropsychiatric manifestations in children and adults can represent a significant diagnostic challenge for clinicians. Clinical signs often include encephalopathy, new-onset psychosis, and movement phenomenon. Although orofacial dyskinesias were initially identified as a characteristic movement phenomenon in this type of encephalitis, an expanded range of abnormalities has recently been reported, including isolated ataxia. We report a case of isolated hemiataxia in a young adult with mild initial psychiatric manifestations. A personal and family history of preceding neuropsychiatric symptoms produced diagnostic confusion and resulted in a significant diagnostic and therapeutic delay. Our case confirms the unilateral movement manifestations that have been emphasized in recent reports. Additionally, it confirms the need for involvement of neurologic as well as psychiatric services in the evaluation of such cases and emphasizes the importance of the neurologic examination in presentations with an initial psychiatric predominance.

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Section: Discussionsupporting

confidence: 89%

Section: Introductionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 71%

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Hemiataxia: A Novel Presentation of Anti-NMDA Receptor Antibody Mediated Encephalitis in an Adolescent

Phillips

Jones

Callaghan

et al. 2017

Case Reports in Psychiatry

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“…As a matter of fact, the seizure types of our seropositive patients have been described in previous reports of autoimmune encephalitis with antibodies to voltage gated potassium channel complex, GAD, NMDAR, LGI1 and Hu (9)(10)(11)(12). Autoimmune encephalitis may also present with unilateral dominance of motor symptoms (similar to 5-year-old boy and 8-year-old girl in our cohort) and de novo status epilepticus (similar to 5-year-old girl in our cohort) (13,14). Focal motor seizures observed in two of our seropositive patients were indicative of peri-Rolandic cortex involvement and such extra-limbic motor symptoms have been reported in patients with Hu encephalitis (12).…”

Section: Figure 3 Immunolabeling (Brown Color) Of Frozen Rat Brain Se...supporting

confidence: 78%

Kelch-like Protein 11 (KLHL11) Antibodies in Children With Seizures of Undetermined Cause

TZARTOS,

PECHLIVANIDOU,

BOSVELI

et al. 2023

In Vivo

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Background/Aim: Kelch-like protein 11 (KLHL11)antibody may be found in paraneoplastic neurological disorders presenting with epileptic seizures. The aim of this study was to investigate the prevalence and clinical significance of KLHL11-antibody in epilepsy. Patients and Methods: Sera of 42 pediatric and 59 adult patients with seizures of undetermined cause were screened using a cellbased assay. Results: KLHL11-antibody was found in three of 168 control patients with paraneoplastic neurological disorders and four pediatric patients (4-8-year-old, 2 boys/2 girls) with seizures of unknown cause presenting with myoclonic-atonic epilepsy, generalized epilepsy or childhood epilepsy with centrotemporal spikes. In these four cases, seizures continued for 2-7 months, responded promptly and favorably to conventional anti-seizure drugs and did not recur in follow-up durations ranging between 2-5 years. Patients had normal brain MRI findings and motor-mental development before and after seizures. KLHL11-antibody was not detected in adult epilepsy patients with undetermined cause, MOG antibody-positive patients and healthy controls. Conclusion: KLHL11-antibody may be detected in pediatric epilepsy patients with a relatively benign disease course.Antibodies targeting Kelch-like protein 11 (KLHL11) were initially identified in young men presenting with rhombencephalitis and testicular seminoma (1). However, recent studies have shown that KLHL11 antibody can be detected in patients without cancer (2-4). Moreover, the associated clinical spectrum is more heterogeneous than previously recognized and includes limbic encephalitis, psychosis, memory deficits and opsoclonus-myoclonus syndrome (2-4). Seizures are occasionally encountered in KLHL11 encephalitis at frequencies ranging from 18% to 23% (3, 4).Antibodies directed against neuronal intracellular or cellsurface antigens are detected in a sizeable proportion of epilepsy patients of undetermined cause (5). Thus, to identify whether KLHL11 antibody belongs to the anti-neuronal antibodies associated with epilepsy, we screened adult and pediatric patients with epilepsy of undetermined cause together with disease controls using a cell-based assay. Patients and MethodsPatient cohorts. We consecutively recruited 42 pediatric patients (female/male 22/20; mean age±standard deviation 7.2±2.5; range for age 1-9 years; mean duration of epilepsy 3.6±1.9 years) admitted to our outpatient clinic with seizures of undetermined cause. Informed consent was obtained from all participants included in the study.All procedures performed in the study involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (Istanbul University 351

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“…Electro-encephalographic abnormalities have also been investigated in anti-NMDAR encephalitis patients, i.e. human models of NMDAR deficiency (Gitiaux et al, 2013; Nosadini et al, 2015; Benjumea-Cuartas et al, 2017). However, to the best of our knowledge, this is the first study comparing slow wave characteristics during NREM sleep between patients with anti-NMDAR encephalitis and healthy individuals.…”

Section: Discussionmentioning

confidence: 99%

Altered EEG markers of synaptic plasticity in a human model of NMDA receptor deficiency: anti-NMDA receptor encephalitis

Gefferie

Maric

Critelli

et al. 2020

Preprint

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Plasticity of synaptic strength and density is a vital mechanism enabling memory consolidation, learning, and neurodevelopment. It is strongly dependent on the intact function of N-methyl-D-aspartate receptors (NMDAR). The importance of NMDAR is further evident as their dysfunction is involved in many diseases such as schizophrenia, Alzheimer's disease, neurodevelopmental disorders, and epilepsies. Synaptic plasticity is thought to be reflected by changes of sleep slow wave slopes across the night, namely higher slopes after wakefulness at the beginning of sleep than after a night of sleep. Hence, a functional NMDAR deficiency should theoretically lead to altered overnight changes of slow wave slopes. Here we investigated whether pediatric patients with anti-NMDAR encephalitis, being a very rare but unique human model of NMDAR deficiency due to autoantibodies against receptor subunits, indeed show alterations in this sleep EEG marker for synaptic plasticity. We retrospectively analyzed 12 whole-night EEGs of 9 patients (age 4.3-20.8 years, 7 females) and compared them to a control group of 45 healthy individuals with the same age distribution. Slow wave slopes were calculated for the first and last hour of non-rapid eye movement (NREM) sleep (factor 'hour') for patients and controls (factor 'group'). There was a significant interaction between 'hour' and 'group' (p = 0.013), with patients showing a smaller overnight decrease of slow wave slopes than controls. Moreover, we found smaller slopes during the first hour in patients (p = 0.022), whereas there was no group difference during the last hour of NREM sleep (p = 0.980). Importantly, the distribution of sleep stages was not different between the groups, and in our main analyses of patients without severe disturbance of sleep architecture, neither was the incidence of slow waves. These possible confounders could therefore not account for the differences in the slow wave slope values, which we also saw in the analysis of the whole sample of EEGs. These results suggest that quantitative EEG analysis of slow wave characteristics may reveal impaired synaptic plasticity in patients with anti-NMDAR encephalitis, a human model of functional NMDAR deficiency. Thus, in the future, the changes of sleep slow wave slopes may contribute to the development of electrophysiological biomarkers of functional NMDAR deficiency and synaptic plasticity in general.

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Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Cited by 6 publications

References 41 publications

Hemiataxia: A Novel Presentation of Anti-NMDA Receptor Antibody Mediated Encephalitis in an Adolescent

Hemiataxia: A Novel Presentation of Anti-NMDA Receptor Antibody Mediated Encephalitis in an Adolescent

Kelch-like Protein 11 (KLHL11) Antibodies in Children With Seizures of Undetermined Cause

Altered EEG markers of synaptic plasticity in a human model of NMDA receptor deficiency: anti-NMDA receptor encephalitis

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