Central Neurogenic Hyperventilation In Anti-Nmda Receptor Encephalitis

Central neurogenic hyperventilation (CNH) is a rare condition that is generally associated with infiltrative tumors of the brainstem. Respiratory dysfunction, particularly central hypoventilation, is common in anti-N-methyl D-aspartate (NMDA) receptor encephalitis. CNH, to the best of our knowledge, has not been described previously in this disease. A 24-year-old woman was diagnosed with anti-NMDA receptor encephalitis secondary to ovarian teratoma. In addition to the typical symptoms of the disease, recurrent CNH episodes were observed during the course of the illness, which subsided with midazolam and propofol infusion. Supportive and disease-specific treatments, including oopherectomy, plasmapheresis and intravenous immunoglobulin, provided excellent recovery. These observations suggest that NMDA receptors may play a role in the pathophysiology of CNH.WoSScopu

Heart rate variability remains reduced and sympathetic tone elevated after temporal lobe epilepsy surgery

PURPOSE: There is evidence of autonomic dysregulation in temporal lobe epilepsy. The structures removed during temporal lobectomy are important centers of central cardiovascular control; therefore surgery may conceivably alter the cardiovascular autonomic function. The effects of temporal lobectomy on autonomic cardiac control are controversial. We investigated the effects of temporal lobectomy on heart rate variability (HRV) in the early and late postoperative periods. METHODS: We used 1-h ECG recordings to assess heart rate variability by spectral analysis in 24 consecutive patients who underwent temporal lobectomy due to intractable temporal lobe epilepsy. ECG recordings were performed before and twice (early and late) after surgery. The results were compared with age and sex matched controls. RESULTS: When compared with controls, all the time and frequency domain indices (SDRR, RMSSD, TP, LF and HF) were significantly lower in the patient group before surgery. Findings were similar in the early and late post-operative periods except that the LF/HF ratio increased in the patient group after the late post-operative period. Within the patient group, compared to pre-operative results, normalized HF was increased in the early post-operative period; however in the late post-operative period, LF/HF ratio was increased. CONCLUSIONS: These findings show that in patients with intractable temporal lobe epilepsy, HRV is decreased globally in both sympathetic and parasympathetic domains. While the total HRV remains reduced throughout the postoperative periods, the LF/HF ratio, i.e., sympathovagal balance is altered, in favor of parasympathetic side early after surgery, but towards the sympathetic side after the first postoperative month. rights reserved

A Novel De Novo Mutation Involving the Mll2 Gene in a Kabuki Syndrome Patient Presenting with Seizures

Kabuki syndrome is a rare multiple congenital anomaly disorder. Although mental retardation is one of the main features, various neurological symptoms such as hypotonia and seizures can occur. Here we report on a 18-year-old Turkish male patient who was diagnosed previously as Kabuki syndrome. Molecular genetic analysis showed a novel de novo heterozygous mutation (c. 12964C>T [p.Gln4322*]) in the MLL2 gene, that leads to the synthesis of a truncated protein. The aim of the present report is to increase the awareness of Kabuki Syndrome among adult neurologistsWoSScopu

Blockade of Gaba Synthesis Only Affects Neural Excitability Under Activated Conditions in Rat Hippocampal Slices

The primary goal of this study was to establish whether inhibition of GABA synthesis was sufficient to induce network hyperexcitability in a rat hippocampal slice model comparable to that seen with GABA receptor blockade. We used field and intracellular recordings from the CA1 region of rat hippocampal slices to determine the physiological effects of blocking GABA synthesis with the convulsant, 3-mercaptoproprionic acid (MPA). We measured the rate of synthesis of GABA and glutamate in slices using 2-13C-glucose as a label source and liquid chromatography-tandem mass spectrometry. There was little effect of 3.5 mM MPA on evoked events under control recording conditions. Tissue excitability was enhanced following a series of stimulus trains; this effect was enhanced when GABA transport was blocked. Evoked inhibitory potentials (IPSPs) failed following repetitive stimulation and MPA. Spontaneous epileptiform activity was seen reliably with elevated extracellular potassium (5 mM). GABA synthesis decreased by 49% with MPA alone and 45% with the combination of MPA and excess potassium; GABA content was not substantially altered. Our data indicate: (1) GABAergic inhibition cannot be significantly compromised by MPA without network activation; (2) GABAergic synaptic inhibition is mediated by newly synthesized GABA; (3) there is a depletable pool of GABA that can sustain GABAergic inhibition when synthesis is impaired under basal, but not activated conditions; (4) overt hyperexcitability is only seen when newly synthesized GABA levels are low. (c) 2008 Elsevier Ltd. All rights reserved.Wo

Predictive Factors for Postoperative Outcome in Temporal Lobe Epilepsy According to Two Different Classifications

Purpose: The determination of prognostic factors is important for predicting outcome after epilepsy surgery. We investigated the factors related to surgical outcome within a homogeneous group of patients suffering from pathologically proven mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS), and compared Engel's outcome classification system with the latest one proposed by the ILAE. Method: We included 109 patients with MTLE-HS who were followed-up for at least 1 year after epilepsy surgery. A retrospective chart review was performed to extract patients' demographic details, and potential pre- postoperative risk factors. Outcome of surgery was defined by the Engel's and ILAE classifications. In addition, the course of prognosis was determined according to the changes in ILAE classifications on an annual basis. Univariate and multivariate logistic regression analyses were used for the latest available outcomes and two different courses of prognosis. Results: The average duration of follow up was 4.78 +/- 2.55 years in the 109 patients with MTLE-HS. The univariate and multiple logistic regression analyses showed that the occurrence of seizures during the first month after surgery was a significant risk factor for a poor outcome. A history of trauma was also significant for patients with late recurrence of postsurgical seizures after at least 1-year seizure remission. Conclusion: The occurrence of seizures during the first month after surgery is a significant prognostic factor in patients with MTLE-HS. Ignoring early postoperative seizures in classification systems may result in difficulty in identifying the course of epilepsy after surgery. (C) 2008 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.WoSScopu

Brachial Diparesis Due To Motor Neuronopathy As One of the Predominant Presenting Signs of Occult Small Cell Lung Carcinoma

Sensory neuronopathy is a well-established presentation in paraneoplastic neurological syndromes that is mostly associated with small cell lung cancer and anti-Hu antibodies. Motor neuronopathy, on the other hand, is an extremely rare observation in this syndrome. A 56-year-old man presented with asymmetric brachial diparesis and sensory ataxia. Electrophysiological studies revealed sensory ganglionopathy and progressive anterior horn degeneration in cervical segments. Small cell lung carcinoma with associated anti-Hu antibodies was later diagnosed. The patient did not improve despite the administration of steroids and chemotherapy. Paraneoplastic syndromes may exceptionally present with a bilateral arm weakness. Cases accompanied by sensory ganglionopathy should therefore be promptly investigated for any underlying malignancy.WoSScopu