Electroclinical insights into autoimmune epilepsy

Purpose: Chronic autoimmune epilepsy is an increasingly recognised entity however its clinical and electrographic features remain poorly understood. We present a case undergoing diagnostic Stereo-electro-encephalography implantation that was found to have a multifocal perisylvian epilepsy with unique electrographic features and is now seizure free with immunotherapy.Methods: The patient had antibody negative refractory perisylvian epilepsy and underwent implantation of the perisylvian-temporal networks. Immunomodulatory treatment was administered during SEEG.Results: SEEG demonstrated a multifocal perisylvian epilepsy with strong involvement of the posterior insula. There was almost continuous spiking seen interictally from multiple foci within the right hemisphere and independent seizures were generated from 5 locations. After treatment with intravenous methylprednisone and immunoglobulin during SEEG, spiking and seizures terminated while still off anti-seizure medications. The patient remains seizure free on immunotherapy.Conclusion: This case highlights the importance of considering autoimmunity in the differential diagnosis of refractory epilepsy, especially perisylvian epilepsy. It also highlights the need to define a clinical phenotype associated with autoantibodies in epilepsy, as there are likely many cases who are not positive for one of the commercially available tests. This case also provides insights into the possible features of an electroclinical syndrome associated with autoimmunity

Refractory epilepsy secondary to anti-GAD encephalitis treated with DBS post SEEG evaluation: a novel case report based on stimulation findings

We report a case of medically refractory anti-GAD encephalitis which was treated with deep brain stimulation (DBS) after seizure termination was achieved using cortical stimulation during stereo-electroencephalography (SEEG) evaluation. The patient underwent bilateral SEEG implantation and cortical stimulation. Upon stimulation, mimicking the intrinsic seizures (at 1Hz), it was possible to induce seizures with typical semiology, on multiple attempts. Stimulation during these seizures with high frequency (50Hz) resulted in complete termination of the seizure. DBS was inserted after the SEEG evaluation, targeting the bilateral anterior nucleus of the thalamus. There was a sustained reduction in seizure frequency and severity 12 months post insertion. There were also improvements in quality of life. To the best of our knowledge, this is the only case reported in which DBS was successfully used to treat refractory epilepsy in a patient with seizures that were proven to be responsive to electrical stimulation during SEEG recording

A woman with visual loss, amenorrhoea and polyuria:The first reported case of nodular lymphocyte-predominant hodgkin lymphoma presenting with hypopituitarism

SUMMARY: A 34-year-old woman presented 18 months post-partum with blurred vision, polyuria, amenorrhoea, headache and general malaise. Comprehensive clinical examination showed left superior temporal visual loss only. Initial investigations revealed panhypopituitarism and MRI demonstrated a sellar mass involving the infundibulum and hypothalamus. Lymphocytic hypophysitis was suspected and high dose glucocorticoids were commenced along with desmopressin and thyroxine. However, her vision rapidly deteriorated. At surgical biopsy, an irresectable grey amorphous mass involving the optic chiasm was identified. Histopathology was initially reported as granulomatous hypophysitis. Despite the ongoing treatment with glucocorticoids, her vision worsened to light detection only. Histopathological review revised the diagnosis to partially treated lymphoma. A PET scan demonstrated avid uptake in the pituitary gland in addition to splenic involvement, lymphadenopathy above and below the diaphragm, and a bone lesion. Excisional node biopsy of an impalpable infraclavicular lymph node confirmed nodular lymphocyte-predominant Hodgkin lymphoma. Hyper-CVAD chemotherapy was commenced, along with rituximab; fluid-balance management during chemotherapy (with its requisite large fluid volumes) was extremely complex given her diabetes insipidus. The patient is now in clinical remission. Panhypopituitarism persists; however, her vision has recovered sufficiently for reading large print and driving. To the best of our knowledge, this is the first reported case of Hodgkin lymphoma presenting initially as hypopituitarism. LEARNING POINTS: Lymphoma involving the pituitary is exceedingly rare and, to the best of our knowledge, this is the first reported case of nodular lymphocyte-predominant Hodgkin lymphoma presenting as hypopituitarism. There are myriad causes of a sellar mass and this case highlights the importance of reconsidering the diagnosis when patients fail to respond as expected to appropriate therapeutic intervention. This case highlights the difficulties associated with managing panhypopituitary patients receiving chemotherapy, particularly when this involves large volumes of i.v. hydration fluid