Decreased abundance of Akkermansia after adrenocorticotropic hormone therapy in patients with West syndrome.

BACKGROUND: Infants suffer from a severe epileptic encephalopathy known as West syndrome (WS). Treatment with adrenocorticotropic hormone (ACTH) indicates the involvement of the gut-brain axis in WS. Several pieces of evidence show the communication of the gut microbiota (GM) with the brain via the hypothalamic-pituitary-adrenal axis (HPA axis) and blood cytokines. This study aimed at (1) determining the GM diversity in infants having WS and (2) comparing the results of infants having WS with those of the healthy infants and also in the patients with WS before and after the ACTH therapy. RESULTS: In this study, 29 infants with WS and 29 healthy infants aged 3-13 months were recruited. Fecal samples were collected, and DNA was extracted and sequenced on the Illumina MiSeq platform. Kruskal-Wallis rank-sum test was used to analyze the between-group differences in the Chao1 index, Shannon index, and the abundances of GM at different taxonomy levels. R software was used to plot the graphs. The top five dominant GM genera between patients with WS and healthy infants showed no significant differences. However, the relative abundance of genus Akkermansia was observed to be significantly (P = 0.011) higher in the BT group than in the HC group and AT group. After 2 weeks of ACTH therapy, the relative abundance of Akkermansia significantly (P = 0.003) decreased. CONCLUSION: The relative abundance of Akkermansia was observed to be significantly higher in patients with WS than that in healthy infants. However, the relationship between Akkermansia and WS pathogenesis needs to be clarified in further studies.

Case report: Blue chromogenic dental staining in child with West syndrome.

BACKGROUND: Tooth discolouration can be caused by a variety of local and systemic factors. Extrinsic dental stains may be caused by predisposing factors, and other factors such as dental plaque, foods and beverages, chromogenic bacteria, metallic compounds and medications. Studies have reported a correlation between the colour of extrinsic staining and caries risk. CASE REPORT: A 4-year-old boy with West syndrome, characterised by epileptic seizures and severe muscle spasm, was referred to the paediatric dentistry clinic at School of Clinical Dentistry, Sheffield. He had a percutaneous endoscopic gastrostomy (PEG) tube and had no oral food or fluid intake. The presenting complaint was his parent's concern of trauma to the oral tissues from epileptic fits. An examination revealed an unusual navy-blue staining to his teeth that appeared extrinsic in nature. There was evidence of tooth-wear of his primary dentition, and marked calculus deposits. No caries was detected. TREATMENT: A further dental examination and treatment was carried out under general anaesthesia. The mandibular central incisors were extracted, due to imminent pulp exposure from bruxism, and were sent for histopathology to determine the nature of the staining. A moderate growth of Pseudomonas aeruginosa, a blue pigment-producing bacteria usually implicated in chronic pulmonary infections, was recovered from a swab sample. FOLLOW-UP: The patient was reviewed at 4 months at which time the staining had returned. CONCLUSION: The patient had no oral intake of food or drink, which placed him in a low caries risk category despite limited oral hygiene practice. His extensive lists of medications were not found to have extrinsic dental staining as a possible side effect. However, these may have altered the oral flora such that growth of pigmented bacteria, normally absent from the oral cavity, was favoured, causing generalised extrinsic staining.

[Clinical, neuro-radiological and prognostic aspects of post-encephalitic catastrophic epilepsies]. / Aspectos clínicos, neurorradiológicos y evolutivos de las epilepsias catastróficas postencefalíticas.

OBJECTIVE: To determine the prevalence of encephalitis and meningo encephalitis as the causative agents of catastrophic epilepsies (CE) and the incidence of post encephalitic CE, when catastrophic epilepsy is defined as often refractory to treatment and always associated with psychoneurological deterioration. PATIENTS AND METHODS: The prevalence of central nervous system (CNS) infections in determining West s syndrome (WS), Lennox Gastaut syndrome (LGS) and HHE syndrome (HHES) was detected in the large series published since 1980 in which the cause was stated. The incidence of CE in the course of meningoencephalitis was deduced from three studies done in the Virgen del Roc o Hospital: study 1 of 1,221 children admitted to hospital with the diagnosis of meningo encephalitis; study 2 of 55 cases of tuberculous meningitis; study 3 of 30 cases of encephalitis. RESULTS: CNS infections causing CE are responsible for from 3 to 11% of all WS, 3 to 8.2% of all LGS and 19% of the HHES with a catastrophic course. The commonest causes are infection due to cytomegalovirus and toxoplasmosis during the prenatal stage and the purulent meningitis, tuberculous meningitis and herpetic encephalitis during the neonatal and postnatal periods. The evidence of CE in meningo encephalitis varies according to the germ, age and severity of the aggression. CNS infections during the neonatal period in 3% of cases cause CE. In babies, newborn and subsequently, tuberculous meningitis (12.7%), measles meningo encephalitis (22%) and herpetic encephalitis (50%) lead to refractory epileptic seizures and very severe psychoneurological deterioration. CONCLUSIONS: 1. Encephalitis and meningo encephalitis are commoner than usually thought as a cause of CE. 2. They cause 3 11% of the WS, 3 8% of the LGS and 19% of the HHES. 3. The incidence of CE in the course of meningo encephalitis varies according to the germ involved and the severity of the aggression. 4. CE are very frequent during the course of herpetic encephalitis, measles meningo encephalitis and tuberculous meningo encephalitis. The latter two are becoming much less common. 5. The prognosis is extremely serious

[5th day convulsions of the newborn infant in rotavirus infections]. / 5-Tages-Krämpfe des Neugeborenen bei Rotavirusinfektionen.

AIM OF THE STUDY: The pathogenesis of fifth day fits in newborns has not yet been explained. From 1989-1991 we observed an epidemic occurrence of 21 cases in 1917 hospitalized newborn children. After finding rotavirus in the faeces of several cases we suspected a coincidence. METHODS: We tested the faeces of 19 fifth day fitters as well as 30 healthy newborns (4.-6. day of life) for rotavirus infection. The results of all clinically indicated rotavirus examinations in neonates of 4 to 6 days of age from 1989-1991 were analysed. In 6 rotavirus positive fifth day fitters we performed an immunologic and electron microscopic examination of cerebrospinal fluid. RESULTS: 18 of 19 examined children with fifth day fits (95%) had rotavirus in the faeces. Within the healthy control group we found 12 of 30 (40%, Chi-square 12.46, p < 0.001) and in the group of sick neonates without convulsions 98 of 202 (48.5%, Chi-square 12.44, p < 0.001). This shows a statistically significant coincidence of rotavirus positive faeces with fifth day fits. Rotavirus was not present in cerebrospinal fluid. CONCLUSIONS: We suggest a causal relation between fifth day fits and rotavirus infections. Pathogenetic details remain unclear.