Immunotherapy in a case of low titre GAD65 antibody-associated spectrum neurological disorders.

We present a rare case of low titre GAD65 antibody-associated autoimmune encephalitis and status epilepticus in a young woman. She initially presented with left arm dystonic movements, contractures and status epilepticus. Due to the concern of autoimmune encephalitis and seizures, the patient received intravenous immunoglobulin empirically. After the detection of low serum GAD65 antibodies, the patient underwent immunomodulation therapy with significant improvement. This case demonstrated that in autoimmune encephalitis, it is important to monitor serum GAD65 antibodies levels and consider immunotherapy, despite mildly elevated serum levels. The patient's history of left arm dystonic movements without impaired awareness may have been due to limb dystonia, a presenting symptom of stiff person syndrome (SPS), despite SPS more commonly affecting axial muscles. This case further demonstrates that GAD65 antibody-related syndromes can manifest with different neurological phenotypes including co-occurrence of epilepsy with possible focal SPS despite low GAD65 antibodies titres.

Meningioma Related Epilepsy- Pathophysiology, Pre/postoperative Seizures Predicators and Treatment.

Meningiomas are the most common primary brain tumors accounting for about 30% of all brain tumors. The vast majority of meningiomas are slow-growing and of benign histopathology rendering them curable by surgery alone. Symptomatic lesions depend on the location with signs of mass effect or neurological deficits. Seizures are the presenting symptoms in approximately 30% of cases, which negatively affect quality of life, limit independence, impair cognitive functioning, as well as increase the risk for psychiatric comorbidities including depression. Although surgical resection may offer seizure freedom in 60-90% of meningiomas, seizures persist after surgical resection in approximately 12-19% of patients. Anti-seizure medications (ASMs) are employed in management, however, are limited by adverse neurocognitive side-effects and inefficacy in some patients. The potential predictors of pre- and post-operative seizures in meningioma patients have been identified in the literature. Understanding various factors associated with seizure likelihood in meningioma patients can help guide more effective seizure control and allow for better determination of risk before and after surgery.

Striking MRI Changes of Focal Cortical Dysplasia Over Time: A Case Series and Literature Review.

PURPOSE OF REVIEW: Brain MRI findings of focal cortical dysplasia (FCD) can undergo dramatic changes over time, which may be related to long-term epilepsy or a combination of histopathologic changes that necessitate further investigation. RECENT FINDINGS: We describe 2 cases of FCD type IIb that initially displayed inconspicuous findings on MRI, however progressed to obvious signal changes on subsequent MRI 10-17 years later. Pathologic analysis indicates that the interval changes are likely attributed to reactive astrogliosis and diffuse parenchymal rarefaction. A few case reports and case series showing similar MRI changes have been described in the literature, the majority in pediatric patients. The adult cases we present add to the scientific evidence of these changes occurring in the adult population. SUMMARY: Our observations lead to several clinical suggestions, including closer interval follow-up imaging for nonlesional cases, the addition of postprocessing imaging methods, earlier surgical intervention, and meticulous surgical planning.

Epilepsy Surgery for Skull-Base Temporal Lobe Encephaloceles: Should We Spare the Hippocampus from Resection?

The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE) with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL) and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG) monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI) and confirmed by maxillofacial head computed tomography (CT) scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG) with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy) is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone.

Cranial nerve palsies: sarcoidosis to systemic lupus erythematosus.

Cranial palsies are a very rare feature of SLE. Similarly, peripheral sensory-motor axonal neuropathy is very uncommon in SLE. The combination of the two as the presenting symptoms of SLE is a diagnostic challenge particularly in an elderly male patient with a known diagnosis of sarcoidosis. This case serves to highlight the diagnostic considerations in such a patient. The lack of response to standard therapy and the presence of subtle clues like anemia, proteinuria, and mild serositis should prompt the physician to look for alternate diagnoses. The potential association of SLE and sarcoidosis is also discussed. SLE can be present in elderly male patients with cranial and peripheral neuropathy.

Bilateral and independent Broca areas confirmed by Wada test and functional magnetic resonance imaging.

Accurate preoperative evaluation of language dominance is critical when evaluating potential patients for temporal lobe epilepsy surgery. Although most people have left-sided language dominance, a minority of patients have been described with either bilateral or right hemispheric dominance. We present a patient with right temporal lobe epilepsy who presented with bilateral and functionally independent Broca areas, as confirmed by Wada testing and functional magnetic resonance imaging.