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N-type calcium channel antibody-mediated autoimmune encephalitis: An unlikely cause of a common presentation.

Finkel L, Koh S - Epilepsy Behav Case Rep (2013)

Bottom Line: He was also treated with anticonvulsants for one month.No tumor has been found to date.Complete resolution of brain lesion, seizure freedom, and full recovery of function following early and aggressive immunotherapy demonstrate that a high index of suspicion is crucial for early recognition and treatment of autoimmune encephalitis.

View Article: PubMed Central - PubMed

Affiliation: Neurology and Epilepsy, Ann and Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Avenue, Chicago, IL 60611, USA.

ABSTRACT
We report, to our knowledge, the only known pediatric case with encephalopathy and significantly elevated titers of N-type voltage-gated calcium channel antibody (N-type VGCC). The patient, an 8th grader, was previously healthy and presented with a one-week history of confusion, aphasia, transient fever, headaches, and dizziness. An underlying autoimmune process was suspected because of inflammatory changes in the brain MRI and multiple focal electrographic seizures captured in the EEG in the absence of CSF pleocytosis. Within 24 h of presentation, the patient was empirically started on immune-modulatory therapy, and a full recovery was achieved within 3 months of the initial presentation. Immune therapy included high-dose intravenous (IV) methylprednisolone followed by a 2-week course of dexamethasone and 2 monthly courses of IV immunoglobulin (IVIG). He was also treated with anticonvulsants for one month. No tumor has been found to date. There is a paucity of reports on autoimmune epilepsy or encephalopathy associated with N-type VGCC. Complete resolution of brain lesion, seizure freedom, and full recovery of function following early and aggressive immunotherapy demonstrate that a high index of suspicion is crucial for early recognition and treatment of autoimmune encephalitis.

No MeSH data available.


Related in: MedlinePlus

Interictal and ictal activities during overnight vEEG monitoring demonstrated marked left posterior dysfunction and epileptogenicity.A.Intermittent, prominent slowing noted in the left hemisphere maximum in the temporal and occipital regions. A moderate fast rhythm, 18–20 Hz, was often noted in the left occipital region (arrow). Note a lack of posterior dominant rhythm (PDR) on the left and preserved 9-Hz PDR on the right hemisphere.B.Multiple brief electrographic seizures were captured from the awake and sleep states. The seizure onset was subtle and stuttering. Ictal EEG correlate suggests posterior (left temporal/occipital) seizure origin. Posterior maximum left hemisphere fast activity spread throughout the left hemisphere and evolved in amplitude and frequency.C.Evolution of rhythmic delta activity spreading throughout the left hemisphere.
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f0005: Interictal and ictal activities during overnight vEEG monitoring demonstrated marked left posterior dysfunction and epileptogenicity.A.Intermittent, prominent slowing noted in the left hemisphere maximum in the temporal and occipital regions. A moderate fast rhythm, 18–20 Hz, was often noted in the left occipital region (arrow). Note a lack of posterior dominant rhythm (PDR) on the left and preserved 9-Hz PDR on the right hemisphere.B.Multiple brief electrographic seizures were captured from the awake and sleep states. The seizure onset was subtle and stuttering. Ictal EEG correlate suggests posterior (left temporal/occipital) seizure origin. Posterior maximum left hemisphere fast activity spread throughout the left hemisphere and evolved in amplitude and frequency.C.Evolution of rhythmic delta activity spreading throughout the left hemisphere.

Mentions: Head computed tomography (CT) was unremarkable, and a lumbar puncture (LP) was performed to rule out meningoencephalitis, which showed only 5 white blood cells (WBCs) with normal glucose and protein (Table 1). Overnight video electroencephalogram (vEEG) showed marked left hemisphere dysfunction, a lack of a posterior dominant rhythm on the left and epileptogenicity with temporal and occipital maximum. Electroencephalogram captured multiple, 20- to 30-second-long, subtle/stuttering electrographic seizures emanating from the left hemisphere (Fig. 1). Magnetic resonance imaging (MRI) revealed cortical thickening involving the left hippocampal and parahippocampal gyri and temporal lobes with associated abnormal FLAIR signal and restriction. The imaging pattern was most suggestive of an underlying inflammatory process such as encephalitis or prolonged seizure activity (Fig. 2).


N-type calcium channel antibody-mediated autoimmune encephalitis: An unlikely cause of a common presentation.

Finkel L, Koh S - Epilepsy Behav Case Rep (2013)

Interictal and ictal activities during overnight vEEG monitoring demonstrated marked left posterior dysfunction and epileptogenicity.A.Intermittent, prominent slowing noted in the left hemisphere maximum in the temporal and occipital regions. A moderate fast rhythm, 18–20 Hz, was often noted in the left occipital region (arrow). Note a lack of posterior dominant rhythm (PDR) on the left and preserved 9-Hz PDR on the right hemisphere.B.Multiple brief electrographic seizures were captured from the awake and sleep states. The seizure onset was subtle and stuttering. Ictal EEG correlate suggests posterior (left temporal/occipital) seizure origin. Posterior maximum left hemisphere fast activity spread throughout the left hemisphere and evolved in amplitude and frequency.C.Evolution of rhythmic delta activity spreading throughout the left hemisphere.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4150614&req=5

f0005: Interictal and ictal activities during overnight vEEG monitoring demonstrated marked left posterior dysfunction and epileptogenicity.A.Intermittent, prominent slowing noted in the left hemisphere maximum in the temporal and occipital regions. A moderate fast rhythm, 18–20 Hz, was often noted in the left occipital region (arrow). Note a lack of posterior dominant rhythm (PDR) on the left and preserved 9-Hz PDR on the right hemisphere.B.Multiple brief electrographic seizures were captured from the awake and sleep states. The seizure onset was subtle and stuttering. Ictal EEG correlate suggests posterior (left temporal/occipital) seizure origin. Posterior maximum left hemisphere fast activity spread throughout the left hemisphere and evolved in amplitude and frequency.C.Evolution of rhythmic delta activity spreading throughout the left hemisphere.
Mentions: Head computed tomography (CT) was unremarkable, and a lumbar puncture (LP) was performed to rule out meningoencephalitis, which showed only 5 white blood cells (WBCs) with normal glucose and protein (Table 1). Overnight video electroencephalogram (vEEG) showed marked left hemisphere dysfunction, a lack of a posterior dominant rhythm on the left and epileptogenicity with temporal and occipital maximum. Electroencephalogram captured multiple, 20- to 30-second-long, subtle/stuttering electrographic seizures emanating from the left hemisphere (Fig. 1). Magnetic resonance imaging (MRI) revealed cortical thickening involving the left hippocampal and parahippocampal gyri and temporal lobes with associated abnormal FLAIR signal and restriction. The imaging pattern was most suggestive of an underlying inflammatory process such as encephalitis or prolonged seizure activity (Fig. 2).

Bottom Line: He was also treated with anticonvulsants for one month.No tumor has been found to date.Complete resolution of brain lesion, seizure freedom, and full recovery of function following early and aggressive immunotherapy demonstrate that a high index of suspicion is crucial for early recognition and treatment of autoimmune encephalitis.

View Article: PubMed Central - PubMed

Affiliation: Neurology and Epilepsy, Ann and Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Avenue, Chicago, IL 60611, USA.

ABSTRACT
We report, to our knowledge, the only known pediatric case with encephalopathy and significantly elevated titers of N-type voltage-gated calcium channel antibody (N-type VGCC). The patient, an 8th grader, was previously healthy and presented with a one-week history of confusion, aphasia, transient fever, headaches, and dizziness. An underlying autoimmune process was suspected because of inflammatory changes in the brain MRI and multiple focal electrographic seizures captured in the EEG in the absence of CSF pleocytosis. Within 24 h of presentation, the patient was empirically started on immune-modulatory therapy, and a full recovery was achieved within 3 months of the initial presentation. Immune therapy included high-dose intravenous (IV) methylprednisolone followed by a 2-week course of dexamethasone and 2 monthly courses of IV immunoglobulin (IVIG). He was also treated with anticonvulsants for one month. No tumor has been found to date. There is a paucity of reports on autoimmune epilepsy or encephalopathy associated with N-type VGCC. Complete resolution of brain lesion, seizure freedom, and full recovery of function following early and aggressive immunotherapy demonstrate that a high index of suspicion is crucial for early recognition and treatment of autoimmune encephalitis.

No MeSH data available.


Related in: MedlinePlus