Anti-Ma2 encephalitis in a phenotypic female with XY gonadal dysgenesis: A case report.

Anti-Ma2 encephalitis is an autoimmune disorder that typically involves the brainstem, limbic system, and diencephalon. It can be paraneoplastic and is more common in males. We describe an unusual presentation of anti-Ma2 encephalitis in a patient with an XY chromosome and a female phenotype. She experienced various neurological symptoms, including olfactory hallucinations, episodic nausea, per-ictal water drinking, and hypersomnolence, that were poorly controlled by antiseizure medications (ASMs) and immunotherapy. Brain MRI showed abnormalities in right medial temporal and frontal regions, and blood tests detected anti-Ma2 antibodies. Screening for malignancies yielded no tumors. Pelvic CT showed bilateral inguinal masses and the absence of a uterus, while genetic studies revealed an XY karyotype. Surgical removal of the masses, shown to be primitive gonads, offered temporary relief, necessitating ongoing ASMs and immunotherapy.

Frontal disconnection surgery for drug-resistant epilepsy: Outcome in a series of 16 patients.

OBJECTIVE: To evaluate the effectiveness of frontal disconnection surgery in seizure control and related consequences in a consecutive patient series. METHODS: We conducted a retrospective analysis of patients who underwent frontal disconnection surgery for drug-resistant epilepsy (DRE). Baseline epilepsy characteristics, detailed presurgical evaluation including epileptogenic zone (EZ) localization, magnetic resonance imaging (MRI) detection of epileptogenic lesion, and pathological findings were reviewed. Patients were followed postoperatively for seizure outcome at 1 year. RESULTS: A total of 16 patients were identified (six children and 10 adults). Most patients had a childhood onset of DRE with a median duration of epilepsy of 6.5 years (interquartile range 3.5-17.5 years) before surgery. In 10 (62.5%) patients, the EZ was localized to the frontal lobe, while in six patients, the EZ involved also adjacent lobes or consisted of multiple foci. In 10 (62.5%) patients, an epileptogenic lesion was detected on presurgical MRI, four of which (40%) had all MRI abnormalities confined to the frontal lobe. Two-thirds of the patients (11/16; 68.8%) underwent isolated frontal disconnection procedure, while remaining patients had frontal disconnection combined with resection of an adjacent lobe. Of the 12 patients in whom biopsy was taken from the disconnected frontal lobe, six (50%) had pathology-proven focal cortical dysplasia. We observed surgical-related complications in three (18.8%) cases, neurological deficits in other three (18.8%) patients, and worsening cognitive abilities in one (6.3%) patient. Overall, eight (50%) patients became completely seizure-free (ILAE 1) at one-year follow-up. SIGNIFICANCE: Frontal disconnection surgery for DRE can result in seizure freedom in certain patients, especially when the EZ is strictly limited to the ipsilateral frontal region, and the MRI shows an epileptogenic lesion that is purely frontal in location. Frontal lobe disconnection procedure is safe and has a limited complication rate. However, further studies with larger patient population will yield more significance.

Inhibition of cell survival, invasion, tumor growth and histone deacetylase activity by the dietary flavonoid luteolin in human epithelioid cancer cells.

Phytochemical compounds and histone deacetylase (HDAC) inhibitors are emerging as a new generation of anticancer agents with limited toxicity in cancer patients. We investigated the impact of luteolin, a dietary flavonoid, on survival, migration, invasion of cancer cells in vitro, and tumor growth in vivo. Luteolin (25-200µM) decreased the viability of human cancer cell lines originating from the lung (LNM35), colon (HT29), liver (HepG2) and breast (MCF7/6 and MDA-MB231-1833). Luteolin effectively increased the sub-G1 (apoptotic) fraction of cells through caspase-3 and -7 dependent pathways. We provide evidence that luteolin at sub-lethal/non-toxic concentrations inhibited the invasive potential of LNM35, MCF-7/6 and MDA-MB231-1833 cancer cells using Matrigel as well as the chick heart and Oris invasion assays. Moreover, we demonstrate for the first time that luteolin is a potent HDAC inhibitor that potentiates the cytotoxicity of cisplatin in LNM35 cells and decreases the growth of LNM35 tumor xenografts in athymic mice after intraperitoneal injection (20mg/kg/day for 18days) Thus, luteolin, in combination with standard anticancer drugs such as cisplatin, may be a promising HDAC inhibitor for the treatment of lung cancer.