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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

Perfusion SPECT imaging of the brain performed with Tc 99m EDC (Neurolite). 2D grayscale and color maps were produced using iterative reconstruction with and without attenuation correction. There is asymmetrically increased radiotracer uptake throughout the left cerebral hemisphere in comparison to the right, which is most pronounced in the left occipital and posterior parietal lobes. These findings correspond to the areas of signal abnormality and gyral swelling in the left cerebral hemisphere on MRI and suggest that images were acquired during seizure activity, likely an ictal SPECT.
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f0015: Perfusion SPECT imaging of the brain performed with Tc 99m EDC (Neurolite). 2D grayscale and color maps were produced using iterative reconstruction with and without attenuation correction. There is asymmetrically increased radiotracer uptake throughout the left cerebral hemisphere in comparison to the right, which is most pronounced in the left occipital and posterior parietal lobes. These findings correspond to the areas of signal abnormality and gyral swelling in the left cerebral hemisphere on MRI and suggest that images were acquired during seizure activity, likely an ictal SPECT.

Mentions: One gram of intravenous (IV) methylprednisolone (Solumedrol) was started soon after MRI and EEG and within 24 h of admission. The patient was also loaded with fos-phenytoin and started on maintenance fos-phenytoin and topiramate. Intravenous Solumedrol was continued for a total of 3 days followed by dexamethasone (6 mg TID × 5 days, 6 mg BID × 5 days, 6 mg daily × 5 days, 3 mg daily × 5 days). He continued to demonstrate waxing and waning mental status after a high-dose Solumedrol burst. Repeat video-EEG monitoring on day 9 no longer detected seizures but continued to show absence of a posterior dominant rhythm on the left. Single photon emission computed tomography scan was performed on day 9 to address continued concern for ongoing subclinical seizure activity that was not being captured on EEG. It demonstrated an asymmetric hyperperfusion throughout the left cerebral hemisphere with posterior predominance, corresponding to the findings seen on MRI, which may have reflected ongoing seizure activity (Fig. 3). Repeated doses of lorazepam were given at times of aphasia, confusion, and sleepiness and produced a transient clinical improvement. The patient was, therefore, started on a daily dose of clobazam. Repeat MRI of the brain (with and without contrast) on day 10 of the hospital stay displayed marked improvement; only subtle T2 hyperintensity, with minimal cortical thickening in the left occipital and inferior temporal lobes, was noted. The repeat lumbar puncture on day 10 was again unremarkable (Table 1). The patient was given 2 doses of 1-g/kg intravenous immunoglobulin (IVIG) on days 11 and 12 of hospitalization. On day 18, he was transferred to inpatient rehabilitation with a plan for clobazam monotherapy (10 mg BID).


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

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

Perfusion SPECT imaging of the brain performed with Tc 99m EDC (Neurolite). 2D grayscale and color maps were produced using iterative reconstruction with and without attenuation correction. There is asymmetrically increased radiotracer uptake throughout the left cerebral hemisphere in comparison to the right, which is most pronounced in the left occipital and posterior parietal lobes. These findings correspond to the areas of signal abnormality and gyral swelling in the left cerebral hemisphere on MRI and suggest that images were acquired during seizure activity, likely an ictal SPECT.
© Copyright Policy
Related In: Results  -  Collection

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

f0015: Perfusion SPECT imaging of the brain performed with Tc 99m EDC (Neurolite). 2D grayscale and color maps were produced using iterative reconstruction with and without attenuation correction. There is asymmetrically increased radiotracer uptake throughout the left cerebral hemisphere in comparison to the right, which is most pronounced in the left occipital and posterior parietal lobes. These findings correspond to the areas of signal abnormality and gyral swelling in the left cerebral hemisphere on MRI and suggest that images were acquired during seizure activity, likely an ictal SPECT.
Mentions: One gram of intravenous (IV) methylprednisolone (Solumedrol) was started soon after MRI and EEG and within 24 h of admission. The patient was also loaded with fos-phenytoin and started on maintenance fos-phenytoin and topiramate. Intravenous Solumedrol was continued for a total of 3 days followed by dexamethasone (6 mg TID × 5 days, 6 mg BID × 5 days, 6 mg daily × 5 days, 3 mg daily × 5 days). He continued to demonstrate waxing and waning mental status after a high-dose Solumedrol burst. Repeat video-EEG monitoring on day 9 no longer detected seizures but continued to show absence of a posterior dominant rhythm on the left. Single photon emission computed tomography scan was performed on day 9 to address continued concern for ongoing subclinical seizure activity that was not being captured on EEG. It demonstrated an asymmetric hyperperfusion throughout the left cerebral hemisphere with posterior predominance, corresponding to the findings seen on MRI, which may have reflected ongoing seizure activity (Fig. 3). Repeated doses of lorazepam were given at times of aphasia, confusion, and sleepiness and produced a transient clinical improvement. The patient was, therefore, started on a daily dose of clobazam. Repeat MRI of the brain (with and without contrast) on day 10 of the hospital stay displayed marked improvement; only subtle T2 hyperintensity, with minimal cortical thickening in the left occipital and inferior temporal lobes, was noted. The repeat lumbar puncture on day 10 was again unremarkable (Table 1). The patient was given 2 doses of 1-g/kg intravenous immunoglobulin (IVIG) on days 11 and 12 of hospitalization. On day 18, he was transferred to inpatient rehabilitation with a plan for clobazam monotherapy (10 mg BID).

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