Anti-NMDA receptor encephalitis presenting with imaging findings and clinical features mimicking Rasmussen syndrome

Greiner, Hansel M.; Leach, James; Lee, Ki-Hyeong; Krueger, Darcy A.

doi:10.1016/j.seizure.2010.11.013

Cited by 46 publications

(31 citation statements)

References 15 publications

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“…In a severe case of NMDAR-ab encephalitis, symmetric hypometabolism of the occipital lobes and cerebellum and asymmetric hypermetabolism of the frontal, temporal and parietal lobes were detected 5. In less severe cases, only right frontolateral hypermetabolism,7 or only occipital hypometabolism, was described 8. These observations are in line with our findings.…”

Section: Discussionsupporting

confidence: 92%

Fluorodeoxyglucose positron emission tomography in anti-N-methyl-D-aspartate receptor encephalitis: distinct pattern of disease

Leypoldt

Buchert

Kleiter

et al. 2012

J Neurol Neurosurg Psychiatry

171

127

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Background Patients with encephalitis associated with antibodies against N-methyl-D-aspartate-receptor antibody (NMDAR-ab) encephalitis frequently show psychotic symptoms, amnesia, seizures and movement disorders. While brain MRI in NMDAR-ab encephalitis is often normal, abnormalities of cerebral glucose metabolism have been demonstrated by positron emission tomography (PET) with 18F-fluorodeoxyglucose (FDG) in a few usually isolated case reports. However, a common pattern of FDG-PET abnormalities has not been reported. Methods The authors retrospectively identified six patients with NMDAR-ab encephalitis in two large German centres who underwent at least one whole-body FDG-PET for tumour screening between January 2007 and July 2010. They analysed the pattern of cerebral uptake derived from whole-body PET data for characteristic changes of glucose metabolism compared with controls, and the changes of this pattern during the course of the disease. Results Groupwise analysis revealed that patients with NMDAR-ab encephalitis showed relative frontal and temporal glucose hypermetabolism associated with occipital hypometabolism. Cross-sectional analysis of the group demonstrated that the extent of these changes is positively associated with clinical disease severity. Longitudinal analysis of two cases showed normalisation of the pattern of cerebral glucose metabolism with recovery. Conclusions A characteristic change in cerebral glucose metabolism during NMDAR-ab encephalitis is an increased frontotemporal-to-occipital gradient. This pattern correlates with disease severity. Similar changes have been observed in psychosis induced by NMDAR antagonists. Thus, this pattern might be a consequence of impaired NMDAR function.

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Section: Discussionsupporting

confidence: 92%

Fluorodeoxyglucose positron emission tomography in anti-N-methyl-D-aspartate receptor encephalitis: distinct pattern of disease

Leypoldt

Buchert

Kleiter

et al. 2012

J Neurol Neurosurg Psychiatry

171

127

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“…Leypoldt et al (6) revealed a characteristic change in cerebral glucose metabolism during NMDAR-antibody encephalitis of an increased frontotemporal-to-occipital gradient; however, no specific pattern of FDG PET imaging could be identified in the majority of cases (7–10). The majority of cases demonstrated various degrees of hyper- or hypometabolism of a particular area of the brain, which was associated with different stages of the disease and the different structures involved at each stage.…”

Section: Discussionmentioning

confidence: 99%

“…Certain cases that underwent follow-up FDG PET imaging following treatment revealed an association between FDG PET imaging and clinical recovery of the patient. Greiner et al (7) reported the case of an 11-year-old female with a novel onset of explosive epilepsy. 18 F-FDG imaging at 24 days following the onset of seizure revealed asymmetric hypermetabolism in the superior right frontal lobe.…”

Section: Discussionmentioning

confidence: 99%

Marked improvement of anti-N-methyl-D-aspartate receptor encephalitis by large-dose methylprednisolone and plasmapheresis therapy combined with 18F-fluorodeoxyglucose positron emission tomography imaging: A case report

Chen

Wang

Yang

et al. 2014

Experimental and Therapeutic Medicine

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Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, a recently defined and frequently misdiagnosed disease characterized by psychiatric symptoms, seizures, movement disorders and autonomic dysfunction, has been observed predominantly in young females with ovarian teratoma. Conventional imaging techniques, including computed tomography (CT) and magnetic resonance imaging (MRI), are often ineffective for diagnosis of the disease. If diagnosed early, the initiation of immunotherapy and removal of the tumor (if present) may result in recovery. The current study presents the case of a 38-year-old female with classic clinical symptoms of anti-NMDAR encephalitis. The MRI brain scan results were unremarkable, cerebral spinal fluid (CSF) biochemistry indicated non-specific lymphocytic pleocytosis and the CSF microbiology studies were negative. 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) imaging revealed significant generalized asymmetric hypometabolism. The patient demonstrated marked recovery following treatment with a high dose of corticosteroids and plasmapheresis. Accordingly, the follow-up 18F-FDG PET imaging revealed significant improvement.

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“…Antibodies to neuronal antigens, such as leucin-rich glioma inactivated 1 (LGI1), α-amino-3-hydroxy-5-methyl-4-isoxazoleproprionic acid (AMPA), and gamma-aminobutyric acid (GABA) receptors, have been found in patients with limbic encephalitis, in which seizures may be a main component of the clinical profile [39]. The implications of these observations are particularly important for clinical diagnosis, as some of the neurological disorders mediated by these antibodies may mimic the initial stages of RE, as has been disclosed in a recent case of anti-NMDA encephalitis [40]. However, in the case of RE, the prevalence of anti-neuronal antibodies is low, and their presence likely represents an epiphenomenon secondary to the pathological process, rather than a causal factor.…”

Section: Autoantibodies In Pathogenesis Of Rementioning

confidence: 97%

Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES)

2014

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The mechanisms of epileptogenesis in pediatric epileptic syndromes are diverse, and may involve disturbances of neurodevelopmental trajectories, synaptic homeostasis, and cortical connectivity, which may occur during brain development, early infancy, or childhood. Although genetic or structural/metabolic factors are frequently associated with agespecific epileptic syndromes, such as infantile spasms and West syndrome, other syndromes may be determined by the effect of immunopathogenic mechanisms or energy-dependent processes in response to environmental challenges, such as infections or fever in normally-developed children during early or late childhood. Immune-mediated mechanisms have been suggested in selected pediatric epileptic syndromes in which acute and rapidly progressive encephalopathies preceded by fever and/or infections, such as febrile infection-related epilepsy syndrome, or in chronic progressive encephalopathies, such as Rasmussen encephalitis. A definite involvement of adaptive and innate immune mechanisms driven by cytotoxic CD8 +

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Anti-NMDA receptor encephalitis presenting with imaging findings and clinical features mimicking Rasmussen syndrome

Cited by 46 publications

References 15 publications

Fluorodeoxyglucose positron emission tomography in anti-N-methyl-D-aspartate receptor encephalitis: distinct pattern of disease

Fluorodeoxyglucose positron emission tomography in anti-N-methyl-D-aspartate receptor encephalitis: distinct pattern of disease

Marked improvement of anti-N-methyl-D-aspartate receptor encephalitis by large-dose methylprednisolone and plasmapheresis therapy combined with 18F-fluorodeoxyglucose positron emission tomography imaging: A case report

Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES)

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