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Glycine receptor antibodies in 2 cases of new, adult-onset epilepsy.

Zuliani L, Ferlazzo E, Andrigo C, Casano A, Cianci V, Zoccarato M, Leite MI, Waters P, Woodhall M, Della Mora E, Morra M, Giometto B, Aguglia U, Vincent A - Neurol Neuroimmunol Neuroinflamm (2014)

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology (L.Z., M.Z., B.G.), Ospedale Ca'Foncello, Treviso, Italy; Magna Græcia University of Catanzaro and Regional Epilepsy Centre, Bianchi-Melacrino-Morelli Hospital (E.F., V.C., U.A.), Reggio Calabria, Italy; Ospedale di Arzignano ULSS5 (C.A., A.C., E.D.M., M.M.) Ovest Vicentino, Italy; and Nuffield Department of Clinical Neurosciences (M.I.L., P.W., M.W., A.V.), University of Oxford, UK.

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Autoimmune encephalitides have been recognized in association with antibodies targeting neuronal surface antigen antibodies [NSAbs]... Here we report 2 patients with subacute onset of refractory temporal lobe seizures associated with behavioral changes and memory deficits whose sera were positive for GlyR-Abs... Over the following days, when sedation was withdrawn, seizures continued despite IV phenytoin and levetiracetam... An EEG performed on day 7 showed an ictal discharge over right temporal leads, clinically associated with a convulsive generalized seizure (figure, A)... EEG showed diffuse slow waves while brain MRI revealed a T2-weighted hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by status epilepticus (figure, B)... EEG revealed ictal discharges over right temporal leads (figure, C). 3T brain MRI showed a mild asymmetry of hippocampal volume (right 5.80 mL; left 5.24 mL; normal value 4.48–6.41 mL) with no different signal intensities on fluid-attenuated inversion recovery and T2-weighted images... She was given different AEDs (oxcarbazepine, lacosamide, valproate) without benefit... She was given IVIg 0.4 g/kg/d for 5 days with disappearance of the spasms and discharged home on oxcarbazepine and valproate... Over the following year, seizure frequency decreased but memory complaints persisted... Immunomodulation was effective in controlling symptoms in patient 1... In patient 2 IVIg provoked disappearance of spasms but only a mild reduction of seizure frequency and had no effect on attention and memory impairment; steroids were ineffective... Of note, GlyR expression is altered in patients with temporal lobe epilepsy and GlyR-Abs were identified in 3% of a large cohort of patients with epilepsy... We suggest that screening for NSAb could include GlyR-Ab in patients with autoimmune LE with seizures.

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Patient 1's ictal EEG and brain MRI and patient 2's ictal EEG and polygraphic recording(A) Patient 1's EEG showing an ictal discharge over right temporal leads followed by diffuse polyspikes. (B) Patient 1's fluid-attenuated inversion recovery brain MRI coronal section showing hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by the status epilepticus. (C) Patient 2's ictal EEG showing rhythmic sharp theta activity over the right temporal derivations lasting approximately 24 seconds, associated with above-mentioned symptoms. (D) Patient 2's polygraphic recording showing spasms involving upper limb muscles (arrows), more prominent proximally: Spontaneous (a), provoked by eye-opening (b), and provoked by intermittent photic stimulation (c). Note absence of EEG correlates. Ext R and L = extensor carpi muscle right and left; Int R and L = interosseous muscle right and left.
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Figure 1: Patient 1's ictal EEG and brain MRI and patient 2's ictal EEG and polygraphic recording(A) Patient 1's EEG showing an ictal discharge over right temporal leads followed by diffuse polyspikes. (B) Patient 1's fluid-attenuated inversion recovery brain MRI coronal section showing hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by the status epilepticus. (C) Patient 2's ictal EEG showing rhythmic sharp theta activity over the right temporal derivations lasting approximately 24 seconds, associated with above-mentioned symptoms. (D) Patient 2's polygraphic recording showing spasms involving upper limb muscles (arrows), more prominent proximally: Spontaneous (a), provoked by eye-opening (b), and provoked by intermittent photic stimulation (c). Note absence of EEG correlates. Ext R and L = extensor carpi muscle right and left; Int R and L = interosseous muscle right and left.

Mentions: A 25-year-old man presented with a generalized convulsive status epilepticus (GCSE). Apathy and anxiety were noted over a few days. Status epilepticus was controlled with propofol. Vital signs, neurologic examination, routine blood tests, CSF analysis, and brain MRI were normal. Postictal EEG disclosed diffuse slow abnormalities. On day 2, GCSE recurred and he was transferred to the intensive care unit (ICU). Over the following days, when sedation was withdrawn, seizures continued despite IV phenytoin and levetiracetam. An EEG performed on day 7 showed an ictal discharge over right temporal leads, clinically associated with a convulsive generalized seizure (figure, A). Screening for pathogenic microorganisms, systemic autoimmunity, serum “onconeural,” GAD65, NMDAR, AMPAR, GABAbR, and voltage-gated potassium channel (VGKC)–complex autoantibodies was negative. Brain MRI was uninformative. On day 17, IV steroid (methylprednisolone 1 g/d for 7 days) was started, and 3 days later seizure frequency declined. Off sedation, the patient had only sporadic focal seizures. However, when steroids were tapered, status epilepticus reappeared. IV immunoglobulin (IVIg) (0.4 g/kg/d for 5 days) was started with marked improvement, and on day 40 he was discharged from the ICU. Seizures were controlled with a combination of phenobarbital, phenytoin, and zonisamide. EEG showed diffuse slow waves while brain MRI revealed a T2-weighted hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by status epilepticus (figure, B). A wider autoantibody screening with a live cell-based assay subsequently showed GlyR-Ab positivity in serum; CSF was not available for further testing. The patient was transferred to the rehabilitation department and discharged after 8 weeks. By telephone interview 5 months after discharge, he was seizure-free on the same antiepileptic drug (AED) combination.


Glycine receptor antibodies in 2 cases of new, adult-onset epilepsy.

Zuliani L, Ferlazzo E, Andrigo C, Casano A, Cianci V, Zoccarato M, Leite MI, Waters P, Woodhall M, Della Mora E, Morra M, Giometto B, Aguglia U, Vincent A - Neurol Neuroimmunol Neuroinflamm (2014)

Patient 1's ictal EEG and brain MRI and patient 2's ictal EEG and polygraphic recording(A) Patient 1's EEG showing an ictal discharge over right temporal leads followed by diffuse polyspikes. (B) Patient 1's fluid-attenuated inversion recovery brain MRI coronal section showing hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by the status epilepticus. (C) Patient 2's ictal EEG showing rhythmic sharp theta activity over the right temporal derivations lasting approximately 24 seconds, associated with above-mentioned symptoms. (D) Patient 2's polygraphic recording showing spasms involving upper limb muscles (arrows), more prominent proximally: Spontaneous (a), provoked by eye-opening (b), and provoked by intermittent photic stimulation (c). Note absence of EEG correlates. Ext R and L = extensor carpi muscle right and left; Int R and L = interosseous muscle right and left.
© Copyright Policy - open-access
Related In: Results  -  Collection

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Figure 1: Patient 1's ictal EEG and brain MRI and patient 2's ictal EEG and polygraphic recording(A) Patient 1's EEG showing an ictal discharge over right temporal leads followed by diffuse polyspikes. (B) Patient 1's fluid-attenuated inversion recovery brain MRI coronal section showing hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by the status epilepticus. (C) Patient 2's ictal EEG showing rhythmic sharp theta activity over the right temporal derivations lasting approximately 24 seconds, associated with above-mentioned symptoms. (D) Patient 2's polygraphic recording showing spasms involving upper limb muscles (arrows), more prominent proximally: Spontaneous (a), provoked by eye-opening (b), and provoked by intermittent photic stimulation (c). Note absence of EEG correlates. Ext R and L = extensor carpi muscle right and left; Int R and L = interosseous muscle right and left.
Mentions: A 25-year-old man presented with a generalized convulsive status epilepticus (GCSE). Apathy and anxiety were noted over a few days. Status epilepticus was controlled with propofol. Vital signs, neurologic examination, routine blood tests, CSF analysis, and brain MRI were normal. Postictal EEG disclosed diffuse slow abnormalities. On day 2, GCSE recurred and he was transferred to the intensive care unit (ICU). Over the following days, when sedation was withdrawn, seizures continued despite IV phenytoin and levetiracetam. An EEG performed on day 7 showed an ictal discharge over right temporal leads, clinically associated with a convulsive generalized seizure (figure, A). Screening for pathogenic microorganisms, systemic autoimmunity, serum “onconeural,” GAD65, NMDAR, AMPAR, GABAbR, and voltage-gated potassium channel (VGKC)–complex autoantibodies was negative. Brain MRI was uninformative. On day 17, IV steroid (methylprednisolone 1 g/d for 7 days) was started, and 3 days later seizure frequency declined. Off sedation, the patient had only sporadic focal seizures. However, when steroids were tapered, status epilepticus reappeared. IV immunoglobulin (IVIg) (0.4 g/kg/d for 5 days) was started with marked improvement, and on day 40 he was discharged from the ICU. Seizures were controlled with a combination of phenobarbital, phenytoin, and zonisamide. EEG showed diffuse slow waves while brain MRI revealed a T2-weighted hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by status epilepticus (figure, B). A wider autoantibody screening with a live cell-based assay subsequently showed GlyR-Ab positivity in serum; CSF was not available for further testing. The patient was transferred to the rehabilitation department and discharged after 8 weeks. By telephone interview 5 months after discharge, he was seizure-free on the same antiepileptic drug (AED) combination.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology (L.Z., M.Z., B.G.), Ospedale Ca'Foncello, Treviso, Italy; Magna Græcia University of Catanzaro and Regional Epilepsy Centre, Bianchi-Melacrino-Morelli Hospital (E.F., V.C., U.A.), Reggio Calabria, Italy; Ospedale di Arzignano ULSS5 (C.A., A.C., E.D.M., M.M.) Ovest Vicentino, Italy; and Nuffield Department of Clinical Neurosciences (M.I.L., P.W., M.W., A.V.), University of Oxford, UK.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Autoimmune encephalitides have been recognized in association with antibodies targeting neuronal surface antigen antibodies [NSAbs]... Here we report 2 patients with subacute onset of refractory temporal lobe seizures associated with behavioral changes and memory deficits whose sera were positive for GlyR-Abs... Over the following days, when sedation was withdrawn, seizures continued despite IV phenytoin and levetiracetam... An EEG performed on day 7 showed an ictal discharge over right temporal leads, clinically associated with a convulsive generalized seizure (figure, A)... EEG showed diffuse slow waves while brain MRI revealed a T2-weighted hyperintensity with mild swelling of right hippocampus, likely due to local edema induced by status epilepticus (figure, B)... EEG revealed ictal discharges over right temporal leads (figure, C). 3T brain MRI showed a mild asymmetry of hippocampal volume (right 5.80 mL; left 5.24 mL; normal value 4.48–6.41 mL) with no different signal intensities on fluid-attenuated inversion recovery and T2-weighted images... She was given different AEDs (oxcarbazepine, lacosamide, valproate) without benefit... She was given IVIg 0.4 g/kg/d for 5 days with disappearance of the spasms and discharged home on oxcarbazepine and valproate... Over the following year, seizure frequency decreased but memory complaints persisted... Immunomodulation was effective in controlling symptoms in patient 1... In patient 2 IVIg provoked disappearance of spasms but only a mild reduction of seizure frequency and had no effect on attention and memory impairment; steroids were ineffective... Of note, GlyR expression is altered in patients with temporal lobe epilepsy and GlyR-Abs were identified in 3% of a large cohort of patients with epilepsy... We suggest that screening for NSAb could include GlyR-Ab in patients with autoimmune LE with seizures.

No MeSH data available.


Related in: MedlinePlus