Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies
<i>Background:</i> Atypical Parkinsonian syndromes with prominent frontal lobe involvement can occur in the 4R-taupathies progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Secondary forms of movement disorders may occur in the context of autoimmune encephalitis wi...
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MDPI AG
2020-06-01
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| Series: | Brain Sciences |
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| Online Access: | https://www.mdpi.com/2076-3425/10/6/399 |
| _version_ | 1797564391748534272 |
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| author | Dominique Endres Harald Prüss Michel Rijntjes Tina Schweizer Rita Werden Kathrin Nickel Sophie Meixensberger Kimon Runge Horst Urbach Katharina Domschke Philipp T. Meyer Ludger Tebartz van Elst |
| author_facet | Dominique Endres Harald Prüss Michel Rijntjes Tina Schweizer Rita Werden Kathrin Nickel Sophie Meixensberger Kimon Runge Horst Urbach Katharina Domschke Philipp T. Meyer Ludger Tebartz van Elst |
| author_sort | Dominique Endres |
| collection | DOAJ |
| description | <i>Background:</i> Atypical Parkinsonian syndromes with prominent frontal lobe involvement can occur in the 4R-taupathies progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Secondary forms of movement disorders may occur in the context of autoimmune encephalitis with antineuronal antibodies, such as anti-glycine receptor (anti-GlyR) antibodies, which are typically associated with Stiff-Person spectrum syndrome, or progressive encephalomyelitis with rigidity and myoclonus. Overlaps between neurodegenerative and immunological mechanisms have been recently suggested in anti-IgLON5 disease. In this case study, the authors describe a patient with a Parkinsonian syndrome with frontal lobe involvement and anti-GlyR antibodies. <i>Case presentation</i>: The patient presented was a 63-year-old female. Her symptoms had begun with insomnia at the age of 60, after which, since the age of 61, increasing personality changes developed, leading to a diagnosis of depression with delusional symptoms. Severe cognitive deficits emerged, along with a left-side accentuated Parkinsonian syndrome with postural instability. The personality changes involved frontal systems. Magnetic resonance imaging (MRI) showed low-grade mesencephalon atrophy. [<sup>18</sup>F]fluorodeoxyglucose positron emission tomography (FDG PET) depicted a moderate hypometabolism bilateral frontal and of the midbrain, while [<sup>123</sup>I]FPCIT single-photon emission computed tomography (SPECT) revealed severely reduced dopamine transporter availability in both striata, indicating pronounced nigrostriatal degeneration. In addition, anti-GlyR antibodies were repeatedly found in the serum of the patient (max. titer of 1:640, reference: <1:20). Therefore, an anti-inflammatory treatment with steroids and azathioprine was administered; this resulted in a decrease of antibody titers (to 1:80) but no detectable clinical improvement. The cerebrospinal fluid (CSF) and electroencephalography diagnostics showed inconspicuous findings, and negative CSF anti-GlyR antibody results. <i>Conclusion</i>: The patient presented here was suffering from a complex Parkinsonian syndrome with frontal lobe involvement. Because of the high anti-GlyR antibody titers, the presence of an autoimmune cause of the disorder was discussed. However, since no typical signs of autoimmune anti-GlyR antibody syndrome (e.g., hyperexcitability, anti-GlyR antibodies in CSF, or other inflammatory CSF changes) were detected, the possibility that the anti-GlyR antibodies might have been an unrelated bystander should be considered. Alternatively, the anti-GlyR antibodies might have developed secondarily to neurodegeneration (most likely a 4-repeat tauopathy, PSP or CBD) without exerting overt clinical effects, as in cases of anti-IgLON5 encephalopathy. In this case, such antibodies might also potentially modify the clinical course of classical movement disorders. Further research on the role of antineuronal antibodies in Parkinsonian syndromes is needed. |
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| spelling | doaj.art-c3b1ff79d32c434cbad5422fa6e47bb82023-11-20T04:43:36ZengMDPI AGBrain Sciences2076-34252020-06-0110639910.3390/brainsci10060399Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor AntibodiesDominique Endres0Harald Prüss1Michel Rijntjes2Tina Schweizer3Rita Werden4Kathrin Nickel5Sophie Meixensberger6Kimon Runge7Horst Urbach8Katharina Domschke9Philipp T. Meyer10Ludger Tebartz van Elst11Section for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanyDepartment of Neurology and Experimental Neurology, Charité-Universitätsmedizin Berlin, 10117 Berlin, GermanyDepartment of Neurology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, GermanySection for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanySection for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanySection for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanySection for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanySection for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanyDepartment of Neuroradiology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, GermanyDepartment of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, GermanyDepartment of Nuclear Medicine, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, GermanySection for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79104 Freiburg, Germany<i>Background:</i> Atypical Parkinsonian syndromes with prominent frontal lobe involvement can occur in the 4R-taupathies progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Secondary forms of movement disorders may occur in the context of autoimmune encephalitis with antineuronal antibodies, such as anti-glycine receptor (anti-GlyR) antibodies, which are typically associated with Stiff-Person spectrum syndrome, or progressive encephalomyelitis with rigidity and myoclonus. Overlaps between neurodegenerative and immunological mechanisms have been recently suggested in anti-IgLON5 disease. In this case study, the authors describe a patient with a Parkinsonian syndrome with frontal lobe involvement and anti-GlyR antibodies. <i>Case presentation</i>: The patient presented was a 63-year-old female. Her symptoms had begun with insomnia at the age of 60, after which, since the age of 61, increasing personality changes developed, leading to a diagnosis of depression with delusional symptoms. Severe cognitive deficits emerged, along with a left-side accentuated Parkinsonian syndrome with postural instability. The personality changes involved frontal systems. Magnetic resonance imaging (MRI) showed low-grade mesencephalon atrophy. [<sup>18</sup>F]fluorodeoxyglucose positron emission tomography (FDG PET) depicted a moderate hypometabolism bilateral frontal and of the midbrain, while [<sup>123</sup>I]FPCIT single-photon emission computed tomography (SPECT) revealed severely reduced dopamine transporter availability in both striata, indicating pronounced nigrostriatal degeneration. In addition, anti-GlyR antibodies were repeatedly found in the serum of the patient (max. titer of 1:640, reference: <1:20). Therefore, an anti-inflammatory treatment with steroids and azathioprine was administered; this resulted in a decrease of antibody titers (to 1:80) but no detectable clinical improvement. The cerebrospinal fluid (CSF) and electroencephalography diagnostics showed inconspicuous findings, and negative CSF anti-GlyR antibody results. <i>Conclusion</i>: The patient presented here was suffering from a complex Parkinsonian syndrome with frontal lobe involvement. Because of the high anti-GlyR antibody titers, the presence of an autoimmune cause of the disorder was discussed. However, since no typical signs of autoimmune anti-GlyR antibody syndrome (e.g., hyperexcitability, anti-GlyR antibodies in CSF, or other inflammatory CSF changes) were detected, the possibility that the anti-GlyR antibodies might have been an unrelated bystander should be considered. Alternatively, the anti-GlyR antibodies might have developed secondarily to neurodegeneration (most likely a 4-repeat tauopathy, PSP or CBD) without exerting overt clinical effects, as in cases of anti-IgLON5 encephalopathy. In this case, such antibodies might also potentially modify the clinical course of classical movement disorders. Further research on the role of antineuronal antibodies in Parkinsonian syndromes is needed.https://www.mdpi.com/2076-3425/10/6/399parkinsonian syndromesfrontal dementiaglycine receptorantibodyPERMstiff-person syndrome |
| spellingShingle | Dominique Endres Harald Prüss Michel Rijntjes Tina Schweizer Rita Werden Kathrin Nickel Sophie Meixensberger Kimon Runge Horst Urbach Katharina Domschke Philipp T. Meyer Ludger Tebartz van Elst Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies Brain Sciences parkinsonian syndromes frontal dementia glycine receptor antibody PERM stiff-person syndrome |
| title | Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies |
| title_full | Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies |
| title_fullStr | Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies |
| title_full_unstemmed | Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies |
| title_short | Parkinsonian Syndrome with Frontal Lobe Involvement and Anti-Glycine Receptor Antibodies |
| title_sort | parkinsonian syndrome with frontal lobe involvement and anti glycine receptor antibodies |
| topic | parkinsonian syndromes frontal dementia glycine receptor antibody PERM stiff-person syndrome |
| url | https://www.mdpi.com/2076-3425/10/6/399 |
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