Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

[Animal Models of Depression: Behavior as the Basis for Methodology, Assessment Criteria and Classifications].

Analysis of the current state modeling of depression in animals is presented. Criteria and classification systems of the existing models are considered as well as approaches to the assessment of model validity. Though numerous approaches to modeling of depressive states based on disturbances of both motivational and emotional brain mechanisms have been elaborated, no satisfactory model of stable depression state has been developed yet. However, the diversity of existing models is quite positive since it allows performing targeted studies of selected neurobiological mechanisms and laws of depressive state development, as well as to investigate mechanisms of action and predict pharmacological profiles of potential antidepressants.

Assessment of blood-brain barrier function and the neuroinflammatory response in the rat brain by using cerebral open flow microperfusion (cOFM).

Blood-brain barrier (BBB) impairment in systemic inflammation leads to neuroinflammation. Several factors including cytokines, chemokines and signal transduction molecules are implicated in BBB dysfunction in response to systemic inflammation. Here, we have adopted a novel in vivo technique; namely, cerebral open flow microperfusion (cOFM), to perform time-dependent cytokine analysis (TNF-alpha, IL-6 and IL-10) in the frontal cortex of the rat brain in response to a single peripheral administration of lipopolysaccharide (LPS). In parallel, we monitored BBB function using sodium fluorescein as low molecular weight reporter in the cOFM sample. In response to the systemic LPS administration, we observed a rapid increase of TNF-alpha in the serum and brain, which coincides with the BBB disruption. Brain IL-6 and IL-10 synthesis was delayed by approximately 1 h. Our data demonstrate that cOFM can be used to monitor changes in brain cytokine levels and BBB disruption in a rat sepsis model.

Assessment of cerebral tissue oxygen saturation in septic patients during acetazolamide provocation - a near infrared spectroscopy study.

UNLABELLED: Sepsis-associated encephalopathy is a multifactorially determined process of the brain parenchyma. Among other factors, vasogenic causes have been shown to play a role in its development. The aim of the present work was to assess whether cerebral tissue oxygen saturation is influenced by administration of acetazolamide in septic patients compared to controls. PATIENTS AND METHODS: 15 patients with severe sepsis and 10 healthy controls were studied. Cerebral oxygen saturation was assessed by INVOS 51 OOC Cerebral Oxymeter (NIRS) before and after administration of 15 mg/kg BW acetazolamide in both groups. RESULTS: The maximal rise that has been found in the partial pressure of CO(2) in the arterial blood of septic patients after administration of acetazolamide was from 35 ± 5 mmHg to 41.1 ± 6.3 mmHg. For the partial pressure of O(2) the observed increase was from 123.7 ± 47.1 mmHg to 139.9 ± 49 mmHg. Vasodilatory stimulus resulted in a similar maximal increase in cerebral oxygen saturation in septic patients and in controls (8.9 ± 6.5% for septic patients and 9.2 ± 4.6% for healthy persons, respectively). CONCLUSIONS: Cerebral vasoreactivity to acetazolamide is preserved in patients with severe sepsis.

Childhood convulsive status epilepticus: epidemiology, management and outcome.

Convulsive status epilepticus (CSE) in childhood is a medical emergency and its aetiology and outcome mean that it should be studied separately from adult CSE. The incidence in developed countries is between 17 and 23/100,000 with a higher incidence in younger children. Febrile CSE is the commonest single group with a good prognosis in sharp distinction to CSE related to central nervous system infections which have a high mortality. The aim of treatment is to intervene at 5 min and studies indicate that intravenous (i.v.) lorazepam may be a better first-line treatment than rectal diazepam and i.v. phenytoin a better second-line treatment than rectal paraldehyde. An epidemiological study strongly supports the development of prehospital treatment with buccal midazolam becoming a widely used but unlicensed option in the community. More than two doses of benzodiazepines increase the rate of respiratory depression without obvious benefit. The 1 year recurrence rate is 17% and the hospital mortality is about 3%.

Rasmussen's encephalitis: the relevance of neuropsychological assessment in patient's treatment and follow up.

Rasmussen's encephalitis is characterized by refractory epilepsy, neurological deterioration and progressive atrophy of one cerebral hemisphere. The objective of this study is to describe the importance of neuropsychological evaluation in the treatment decision and follow-up of patients with Rasmussens encephalitis. Neuropsychological assessment was performed in two steps. Firstly, the clinical history was obtained and the Vineland adaptative behavior scale (VABS) was applied. After this first step, the patients with social maturity level equal or higher than the inferior limit underwent a battery of neuropsychological assessment. We evaluated three patients before any specific treatment was started, and six months after the intervention (surgery or plasmapheresis). Patient 1 underwent left hemispherectomy and had global improvement on second neuropsychological assessment. This suggests that the decision of performing surgery was adequate. Patients 2 and 3 underwent plasmapheresis. They did not present cognitive decline between both evaluations which suggest that our decision of postponing surgery was adequate as well. We conclude that neuropsychological assessment is important when evaluating patients with Rasmussens encephalitis. That is especially true for patients in whom disease progression is slow, and surgery timing has to be carefully planned.

Cerebral monitoring by compressed spectral array.

Application of a frequency analysis technique, the compressed spectral array (CSA), permits the display of cerebral activity recorded by the electroencephalogram (EEG) over long periods of time in a succinct, graphic manner. This report is a description of a system which is being developed to investigate long-term monitoring of the EEGs of critically ill patients. The computer-based methodology which has been used to implement this system is described, with illustrations of CSA-transformed EEGs.