Effects of Bacillus subtilis, butyrate, mannan-oligosaccharide, and naked oat (ß-glucans) on growth performance, serum parameters, and gut health of broiler chickens.

Four nonantibiotic alternative growth promoters for broiler chickens were evaluated. Ross 308 chicks were fed a control diet (mainly corn and soybean meal) or a diet supplemented with a probiotic (Bacillus subtilis Gallipro DSM 17299), encapsulated butyric acid (Novyrate C), mannan-oligosaccharide (Actigen MOS) or formulated with 20% naked oat (starter diet) and 30% naked oat (grower and finisher). The study was carried out as a complete random blocked design with 10 pens for each diet, 45 birds per pen. Compared to the control, the naked oat diet improved the average daily gain by 16% during the starter phase (up to d 10). The probiotic did so during the grower phase as did butyric acid in the finisher phase (up to d 34). For the experiment overall, the probiotic decreased average daily gain slightly. The best improvement in feed conversion ratio was obtained in the butyrate group (5%). No significant treatment effect on crop pH or on mortality was observed. The naked oat diet gave a slightly lower cecum pH on d 34. The MOS supplement decreased jejunal mass on d 34 and increased villus length (34%) and villus height/crypt depth ratio (32%) measured on d 10. Naked oat, butyric acid and MOS diets all reduced serum endotoxin levels. The probiotic increased serum C-reactive protein. All noncontrol diets reduced serum malondialdehyde. The naked oat diet reduced d 34 litter pH by about 0.3. Some effects of the proposed non-antibiotic growth promoters have been observed and could contribute to livestock performance. Their exact modes of action remained to be defined.

SQSTM1 mutation: Description of the first Tunisian case and literature review.

BACKGROUND: Mutations in SQSTM1 gene have been recently identified as a rare cause of progressive childhood neurodegenerative disorder. So far, only 25 patients from 10 unrelated families were reported. METHODS AND RESULTS: We report on the first Tunisian case of an 11-year-old girl with cerebellar ataxia, chorea and ophthalmoparesis. Brain MRI was normal. Whole-exome sequencing revealed a homozygous mutation c.823_824del(p.Ser275Phefs*17) in SQSTM1 gene (GenBank: NM_003900.4). CONCLUSION: By pooling our data to the data of literature, we delineated the phenotypic spectrum and stressed on genetic heterogeneity of this rare neurodegenerative disease.

Pyridoxine-dependent epilepsy: A novel mutation in a Tunisian child.

Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive metabolic disease characterized by seizures in neonates or infants, which is unresponsive to antiepileptic drugs but controlled by pyridoxine. Without prompt treatment, continued seizures and severe encephalopathy result. Mutations in the ALDH7A1 gene encoding α-amino-adipic semialdehyde (α-AASA) dehydrogenase (antiquitin) have been identified as the cause of PDE. We report on a novel ALDH7A1 mutation in a Tunisian child with PDE.

[Acute disseminated encephalomyelitis in Tunisia: Report of a pediatric cohort]. / Encéphalomyélite aiguë disséminée en Tunisie: étude d'une cohorte pédiatrique.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an inflammatory, demyelinating disorder of the central nervous system whose clinical features, management and outcome are incompletely understood in Tunisian population. OBJECTIVE: To describe clinical, neuroimaging and laboratory features; treatment and outcome in a cohort of Tunisian children with ADEM. METHODS: We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with ADEM between 2005 and 2015. Clinical, neuroimaging and laboratory features, therapeutic data and outcome were analyzed. RESULTS: There were 15 children (7 males and 8 females). The mean age at onset was 6.9 years. Thirteen (86.6%) patients had a prodromal event. The onset of neurological symptoms occurred within 17.6 days (4-30). Limb weakness was the most common presenting symptom (53.3%). Extrapyramidal syndrome was noticed in 6 patients (40%). Initial MRI showed a deep gray matter involvement in 7 cases (46.6%). Gadolinium enhancement at acute stage was observed in only 2 patients (13%). Cerebrospinal fluid findings did not show intrathecal oligoclonal bands. The use of high-dose IV methylprednisolone followed by oral steroid taper was associated with rapid recovery. Additional treatment with intravenous immunoglobulin was necessary in 2 patients. Complete recovery was obtained in 11 patients (73.3%). A monophasic course was noticed in 14 cases. Only one patient (5%) developed multiple sclerosis. CONCLUSION: The high frequency of prodromal events and extrapyramidal syndrome in addition to the low rate of gadolinium enhancement at acute stage seem to be the main features in our patients. Larger ADEM multicenter cohort studies in Tunisia and North Africa could provide more detailed information about this entity.

[Epidemic of rubella encephalitis]. / Encéphalite rubéolique : observation récente d'une fréquence inhabituelle.

Rubella is a mild viral illness in children. Rubella encephalitis is an extremely uncommon complication of rubella affecting unvaccinated children, aged between 5 and 14 years. From May to June 2011, we observed 9 cases of rubella encephalitis diagnosed during an epidemic of rubella. All were previously healthy (8 boys and 1 girl). None of them had received rubella vaccine. The mean age was 11.6 years. The onset of neurological symptoms occurred within 1-5 days after the typical rush and was associated with seizures and altered consciousness in all cases. The presence of serum immunoglobulin M antibody against rubella virus was demonstrated in all patients. EEGs showed slow wave activity in all patients and brain MRI was normal in the 9 cases. Full recovery was obtained in all patients. However, 4 of them required intensive care unit referral. Acute encephalitis is an extremely rare complication of rubella. The main neurological findings are headache, ataxia, and hemiplegia. Epileptic seizure and altered consciousness are rarely observed. Rubella encephalitis is generally self-limiting with about 80% recovery rate with no sequelae. However, severe courses have been reported. These cases illustrated the potential severity of rubella and they should be prevented by encouraging widespread early childhood vaccination. In Tunisia, rubella encephalitis has been reported once previously and vaccination against rubella virus has only recently been included in the national vaccination program, prescribed only for adolescent females. Following this rubella epidemic, vaccination strategies in Tunisia have been revised.

[Congenital stenosis of interventricular foramina revealed by recurrent intracranial hypertension]. / Une sténose congénitale des foramens interventriculaires révélée par une hypertension intracrânienne à rechute.

Non-tumoral stenosis of interventricular foramen is a rare clinical condition. It can be either unilateral, causing monoventricular hydrocephalus, or bilateral leading to biventricular hydrocephalus. The pathophysiology of this misdiagnosed entity remains controversial. The non-tumoral stenosis of interventricular foramen can be either acquired or congenital. The latter usually manifesting with a neonatal hydrocephalus. We report a case of congenital bilateral stenosis of interventricular foramen, in an 8-year-old girl, revealed by recurrent intracranial hypertension. Diagnosis was relied on 3D-CISS sequences MRI. The child showed full recovery after neuroendoscopic septal fenestration and ventriculo-peritoneal shunt.

[Clinical and therapeutic aspects in Tunisian patients with dystonia: a 5-year prospective study]. / Aspects cliniques et thérapeutiques des dystonies en Tunisie : étude prospective sur cinq ans.

INTRODUCTION: Studies of dystonia are heterogeneous and there are no studies on this disease in Tunisia. The aim of our study was to determine the frequency of dystonia in the hospital population, to identify different forms of dystonia according to age of onset, distribution, to determine etiologies and to describe treatment. METHODS: We conducted a prospective study over a 5-year period (from January 2005 to November 2009) including all patients diagnosed with dystonia and followed at the Child and Adolescent Neurology Department and "Movement Disorders and Botulinum Toxin" consultation of the National Institute of Neurology of Tunis. RESULTS: Two hundred patients were included (2.2% of our patients). Mean age was 26.4±21.4 years and sex ratio H:F 1.3. Consanguinity rate was 29%. Main features of dystonia were action dystonia (78.5%), generalized forms (47%) and secondary forms (58%). A pyramidal syndrome and other movement disorders were the most common signs associated with dystonia (36.5% and 33.5% respectively). In the group of secondary dystonia, mains etiologies were dystonia due to exogenic agent (56%), neuro-metabolic diseases (26%), hereditary degenerative disease (13%) and psychogenic dystonia (5%). Dystonia was primary in 44% (84 patients). Different treatments were used and a dramatic improvement in some patients was noted with levodopa and botulinum toxin injections. A multidisciplinary approach associated with medical treatment led to recovery or improved prognosis. DISCUSSION AND CONCLUSION: Very few studies have been devoted to reporting a large series of dystonic patients. Our study is the first to describe both primary and secondary dystonia in 200 Tunisian patients. The presence of familial dystonia in our country suggests a genetic origin. Further work including genetic analysis with a screening of known mutations responsible for dystonia and the informative families with unknown mutations would be useful. Specific studies designed to identify new genes causal in dystonia are needed.

[An overview of neurometabolic diseases in Tunisia. a 3-year prospective study]. / Diagnostic et prise en charge des maladies neurométaboliques en Tunisie. Etude prospective sur trois ans.

INTRODUCTION: Neurometabolic diseases are a large group of genetic diseases. In our country, the diagnostic and therapeutic approach to theses diseases is rather difficult. The aim of our study was to determine the frequency of neurometabolic diseases in the hospital population, to describe the problems in diagnosing these conditions and difficulties encountered during patient care. Our goal was to propose guidelines for a practical diagnostic and therapeutic approach to neurometabolic disorders in our country. METHODS: We have conducted a prospective study over a 3-year period including all patients diagnosed with "metabolic disease" and followed at the Child and Adolescent Neurology Department of the National Institute of Neurology of Tunis. RESULTS: One hundred and thirty-six patients were included (2.4% of our patients). Mean age was 7.3 +/- 5.1 years. Mean age at onset was 4.3 years. There was a high consanguinity rate. Respiratory chain defects were the most frequently suspected diseases (16.9%), followed by lysosomal diseases (8.8%). Chromatography, initially systematically prescribed, became targeted with a higher diagnostic efficacy. Metabolic diseases diagnosed as certain, represented 22% of the studied cases. This can be explained by the insufficiency of available laboratory tests of confirmation. The prescription of specific treatment was insufficient, even for confirmed pathologies (14.7%) because of the high cost of these therapies. CONCLUSION: The diagnostic approach has to be rational, targeted, multidisciplinar and conducted within a care network. Diagnostic priority should focus on treatable neurometabolic diseases. The establishment of a systematized registry and neonatal screening for the main treatable neurometabolic diseases constitute the final objective of our work to prepare for biochemical and genetic studies.

[Cerebromediastinal tuberculosis in a child with a probable Say-Barber-Miller syndrome: a causative link?]. / Tuberculose cérébromédiastinale chez un enfant atteint de syndrome de Say-Barber-Miller probable : un lien de causalité ?

INTRODUCTION: Tuberculosis continues to be a public health problem in emerging countries with a recent evidence of increased incidence of extrapulmonary localization in developed countries probably linked to HIV. To our knowledge the occurrence of cerebro-mediastinal tuberculosis in an immuno-competent child has not been previously described; moreover the child we describe has a probable Say-Barber-Miller syndrome. We discuss a putative causative link between this syndrome and the occurrence of tuberculosis. CASE REPORT: A seven-year-old girl presented to our department with a history of infantile encephalopathy since birth characterized by a facial dysmorphy (evocative of a bird face), microcephaly, and mental retardation, and with recurrent infections. The child had complained of back pain for several months; the parents reported anorexia, loss of weight. Spinal and cerebral MRI showed a mediastinal mass involving the spine and cerebral lesions evocative of tuberculomas. The tuberculin interdermal reaction was positive. Culture of a vertebral biopsy was positive for Koch bacillus. Anti-tuberculosis treatment improved general and local status. An extensive immunological work-up was normal. CONCLUSION: [corrected] This observation is exceptional in many aspects: very early age of onset of extrapulmonary tuberculosis, no immune deficit, association with a rare congenital neurological syndrome. We discuss the possible link between this entity and the occurrence of tuberculosis.