Diagnosis of pediatric anti-NMDAR encephalitis at the onset: A clinical challenge.

BACKGROUNDS: To investigate the clinical and instrumental features at the onset addressing to the diagnosis of anti-NMDAR encephalitis. METHODS: Twenty children (age: 15 months-17 years; 7 males, 13 females) with initial suspected diagnosis of autoimmune encephalitis, observed between January 2008 and March 2018, were included. The final diagnosis was anti-NMDAR encephalitis in 7 children, other/probable autoimmune encephalitis in 7 children, and primary psychosis in the remaining 6 children. RESULTS: At the clinical onset, anxiety disorder was the main symptom that helped in distinguishing the group of psychotic children from children with non-infectious encephalitis (P = 0.05 OR = 0.001), while epileptic seizures strongly predicted anti-NMDAR encephalitis (P = 0.04 OR = 28.6). At the onset, anti-NMDAR encephalitis could be distinguished from other/probable autoimmune encephalitis for the presence of sleep/wake rhythm alteration (P = 0.05 OR = 15). Among the symptoms occurring during the hospitalization, movement disorders (P = 0.031 OR = 12) were predictive of non-infectious encephalitis rather than primary psychosis. More specifically, the occurrence of language impairment (P = 0.03 OR = 33), epileptic seizures (P = 0.04 OR = 28.6) and catatonia (P = 0.03, OR = 33), were predictive of anti-NMDAR encephalitis. Also at this stage, anxiety disorder (P = 0.03 OR = 0.033) was predictive of primary psychosis. CONCLUSION: Our findings suggest that at the clinical onset epileptic seizures and sleep/wake rhythm alteration represent the main features addressing to the diagnosis of anti-NMDAR encephalitis rather than primary psychosis and other/probable autoimmune encephalitis, while anxiety disorder could be a solid predictor of primary psychosis.

From transformation to chronification of migraine: pathophysiological and clinical aspects.

Chronic migraine is a neurological disorder characterized by 15 or more headache days per month of which at least 8 days show typical migraine features. The process that describes the development from episodic migraine into chronic migraine is commonly referred to as migraine transformation or chronification. Ample studies have attempted to identify factors associated with migraine transformation from different perspectives. Understanding CM as a pathological brain state with trigeminovascular participation where biological changes occur, we have completed a comprehensive review on the clinical, epidemiological, genetic, molecular, structural, functional, physiological and preclinical evidence available.

Perceptions and experiences of environmental health risks among new mothers: a qualitative study in Ontario, Canada.

There is a growing awareness and concern in contemporary societies about potential health impacts of environmental contaminants on children. Mothers are traditionally more involved than other family members in managing family health and household decisions and thus targeted by public health campaigns to minimise risks. However little is known about how new mothers perceive and experience environmental health risks to their children. In 2010, we undertook a parallel case study using qualitative, in-depth interviews with new mothers and focus groups with public health key informants in two Public Health Units in Ontario Province, Canada. We found that the concern about environmental hazards among participants ranged from having no concerns to actively incorporating prevention into daily life. Overall, there was a common perception among participants that many risks, particularly in the indoor environment, were controllable and therefore of little concern. But environmental risks that originate outside the home were viewed as less controllable and more threatening. In response to such threats, mothers invoked coping strategies such as relying on the capacity of children's bodies to adapt. Regardless of the strategies adopted, actions (or inactions) were contingent upon active information seeking. We also found an optimistic bias in which new mothers reported that other children were at greater risk despite similar environmental circumstances. The findings suggest that risk communication experts must attend to the social and environmental contexts of risk and coping when designing strategies around risk reducing behaviours.

Early add-on immunoglobulin administration in Rasmussen encephalitis: the hypothesis of neuroimmunomodulation.

Rasmussen encephalitis (RE) is a chronic inflammatory disease leading to unilateral hemispheric atrophy, associated with progressive neurological dysfunction and intractable seizures. The best approach to RE is hemispherectomy. However long-term immunotherapy seems to prevent or slow down hemispheric tissue loss and the associated functional decline. We describe a girl with epilepsia partialis continua (EPC) and progressive neurological dysfunction compatible with RE. The brain MRI showed a lesion that was initially interpreted as focal cortical dysplasia. Combined antiepileptic and immunomodulation were administered for two years with initial beneficial effects. The follow-up MRI, 4 year later showed. atrophic change in right parietal region. The association of antiepileptic and immunomodulation therapies may inhibit pathogenetic mechanisms responsible for neuronal loss in RE, slowing down the progression of the disease.

Evaluation and management of nonsyndromic craniosynostosis.

UNLABELLED: Craniosynostosis (craniostenosis) is premature fusion of the sutures of the cranial vault. Several factors can affect the growth of the cranial vault during embryonic life and after birth, leading to different types of craniosynostosis; these can be classified on the basis of the specific sutures that are fused. Prognosis is improved by early diagnosis, and it is important to establish the correct approach to these patients on the basis of clinical and neuroradiological investigation. The first priority is to identify the type of craniosynostosis and to distinguish between the types that require surgical intervention and those that do not. We report on the different forms of nonsyndromic craniosynostosis, their clinical and neuroradiological diagnoses, and surgical strategies. CONCLUSION: The aim of this review is to provide to paediatricians a correct diagnostic approach and management of children affected from nonsyndromic craniosynostosis, for which a careful physical, ophthalmological and neurological examination is fundamental, whereas brain Computed tomography and magnetic resonance imaging are necessary for patients in which the diagnosis is uncertain or for cases of syndromic craniosynostosis.

Epileptic nystagmus: description of a pediatric case with EEG correlation and SPECT findings.

Epileptic nystagmus (EN) describes repetitive eye movements that result from seizure activity. We describe a patient with EN and vertigo first noted at the age of 4 yr and 10 mo. Brain MRI did not show anomalies. Ictal EEG recordings revealed epileptic activity during three episodes of horizontal, left-beating nystagmus not crossing the midline. Ictal 99mTc-ECD SPECT demonstrated the presence of active foci in multiple cerebral regions including bilateral prefrontal, bilateral parieto-temporo-occipital and the left parieto-insular-vestibular areas. A wide area of hypoperfusion was also evident in the right hemisphere, prevailing in the parieto-occipital regions and the medial prefrontal gyrus. Topiramate was started at a dose of 2 mg/kg/d with complete seizure control after 14 d. EEG and SPECT were repeated after a seizure-free period of 1 mo; disappearance of epileptic activity and modification of cerebral perfusion were evident. This case reaffirms the cortical origin and involvement of temporo-occipital and frontal cortex in the genesis of saccadic epileptic nystagmus. Rapid complete control of clinical events coincided with the normalization of EEG and improvement of the SPECT pattern.

Use of hair analysis for confirmation of self-reported cocaine use in users with negative urine tests.

INTRODUCTION: Identification of cocaine use based on a urine test may miss many cases because of the short elimination half-life of the drug. Our objective was to verify the sensitivity of the cocaine hair test in admitted users. PATIENTS AND METHODS: Admitted cocaine users (38), that were 18-70 years of age and reported to have refrained from using cocaine in the few days to months prior to the test, were compared to 10 controls who claimed never to have used cocaine. All had negative urine tests for cocaine and benzoylecgonine by thin-layer chromatography. Cocaine and benzoylecgonine were extracted from unwashed hair and tested by established immunoassays. RESULTS: The hair test was positive in 37/38 cases (97%) and in none of the controls. There was significantly more cocaine in black hair than in brown or blonde hair per mg of cocaine dose reported to have been consumed, highlighting a potential bias when interpreting test results in individuals with dark hair. There was a statistically significant correlation between reported dose used and hair concentrations of cocaine. DISCUSSION: The cocaine hair test appears to be highly sensitive and specific in identifying past cocaine use in the setting of a negative urine test.

Clinical utilization of the neonatal hair test for cocaine: a four-year experience in Toronto.

BACKGROUND: There has been a steady increase in the number of newborns affected by maternal drug use. Cocaine and its metabolites cross the placenta and have been routinely measured in neonatal urine; however, due to the short half-life of the drug many exposed fetuses have negative urine tests. We have developed a neonatal hair test for measuring cocaine and its metabolites by radioimmunoassay. Since the validation of this test we prospectively evaluated its clinical utility by physicians, hospital nurseries and social welfare agencies who requested neonatal hair analysis to verify clinical suspicion of maternal cocaine use during pregnancy. OBJECTIVE: The objective of the present research was to establish the sensitivity of the hair test in validating clinical suspicion of in utero exposure to cocaine in the presence of negative urine test. HYPOTHESIS: We hypothesized that the use of the hair test in cases of clinical suspicion but negative urine test will yield a substantially higher rate of positivity than expected in the general population. DESIGN: Between October 1991 and April 1995 we prospectively analyzed a total of 192 neonatal hair samples to confirm clinical suspicions of intrauterine exposure to cocaine. Of these, 10 did not have sufficient hair to analyze for cocaine metabolites. RESULTS AND DISCUSSION: Fifty-five (30%) of the remaining 182 were positive for cocaine metabolite. This rate was 5.5-fold higher than the 5.5% found by us in a population-based research study in three nurseries in Toronto (p < 0.001), thus documenting the efficiency of this test in confirming clinical suspicions of fetal exposure to cocaine. Benzoylecgonine concentrations in this cohort were 2-fold higher than among positive cases in a previous population-based screening study (p = 0.0001) indicating that when clinical suspicions prompted physicians to test neonatal hair, they identify a subgroup of heavy cocaine users, who are probably at higher perinatal risks.