[Listeria monocytogenes sepsis. Fatal course in an immunocompetent child]. / Sepsis à Listeria monocytogenes Issue fatale chez un enfant immunocompétent.

The occurrence of non-viral meningitis remains a heavy diagnosis carrying undeniable pejorative aspects that may vary according to several factors such as the age and the presence or absence of an immunodeficiency. In some cases, uncommon germs can also lead to a bad prognosis such as Listeria monocytogenes that can be responsible of an extremely severe septic state. We report the case of a girl with Listeria meningitis, whose evolution was unfortunately quickly negative.

La survenue d'une méningite reste, à l'exception de la plupart des méningites virales, un diagnostic lourd, comportant un aspect péjoratif incontestable et source d'inquiétude pour les familles. Ce caractère péjoratif peut, toutefois, varier en fonction de certains éléments cliniques, tels que l'âge de l'enfant, la présence ou non d'une immunodéficience ou d'autres facteurs favorisants. Le type de germe incriminé ainsi que sa virulence jouent aussi un rôle incontestable. Nous rapportons le cas d'une petite fille, sans antécédents médicaux, atteinte d'une méningite à Listeria monocytogenes dont l'évolution fut malheureusement rapidement fatale.

[Dystonia 12: A rare and difficult diagnosis]. / Dystonie de type 12 : un diagnostic rare et difficile.

We report a case of dystonia 12, also called rapid-onset dystonia-parkinsonism, which occurred in a young 12-year-old boy. Type 12 dystonia is a genetic syndrome characterized by a pathogenic mutation on ATP1A3 gene encoding the subunit alpha 3 of Na-K-ATPase protein, resulting in neuronal dysfunctions. It remains a rare syndrome with less than 100 cases described in the literature. Its atypical presentation and its rarity may lead to a wandering diagnosis, even in some cases to a conversion hysteria diagnosis. Today, unfortunately, there is no effective treatment.

[A rare case of lacunar skull deformity associated with craniosynostosis]. / LE CAS CLINIQUE DU MOIS. Une présentation rare de craniosténose associée à un crâne lacunaire.

The lacunar skull is a radiologic description characterised by the presence of lacunae in the cranial vault. Its physiopathology remains up to now poorly understood; it is mostly associated with neural tube defects. The association of a lacunar skull with a craniosynostosis has rarely been described in the literature. The case of a 9-month-old patient presenting a multisutural craniosynostosis with a lacunar skull is reported in this article. The surgical treatment allowed to remodel the skull and to hope for a spontaneous regression of the lacunae.

[Two case reports of meningococcemia. Review of the literature on chronic meningococcemia]. / À propos de deux cas de méningococcémie. Revue de la littérature sur la méningococcémie chronique.

INTRODUCTION: Neisseria meningitidis is a Gram-negative bacteria that can be highly pathogenic in humans and responsible for life-threatening and acute diseases. In a few cases, it can lead to an atypical form of sepsis-acute, subacute, or chronic-which, even if progression is slower, may carry the same risks for patients as conventional acute forms. CASES: Case 1 presented with recurrent fever, polyarthralgia, and generalized macular rash. Case 2 had fever for the previous 10days with neck pain and macular rash. Blood cultures were positive for group B meningococcus in both cases. Polymerase chain reaction (PCR) analysis for the same pathogen was also positive in the cerebrospinal fluid (CSF) of Case 2. Following intravenous antibiotic treatment, the progression was favorable in both cases. DISCUSSION: Chronic meningococcemia is characterized by a triad of clinical features, including a fever lasting for at least 1week, arthralgia, and a cutaneous rash. This condition evolves positively without any sequelae if treated properly. PCR and blood culture are two complimentary tools that support the diagnosis. CONCLUSION: Meningococcal infection can manifest in a variety of ways. Chronic meningococcemia diagnosis should be considered in a triad of prolonged fever, rash, and arthralgia. Better access to state-of-the-art techniques such as PCR is recommended in the future so as to provide optimal care of patients suffering from this condition.

[Prolonged seizures in children in sub-Saharan Africa Clinical aspects and management]. / Convulsions prolongées chez l'enfant en Afrique subsaharienne Aspects cliniques et prise en charge.

BACKGROUND: There is a paucity of epidemiologic studies of prolonged seizures (persisting for more than 5 minutes) in the Democratic Republic of Congo (DRC) and in Rwanda. OBJECTIVE: We sought to analyze the clinical presentation, causes, pharmacologic management, and shortterm course of these seizures. METHODS: We enrolled 436 children, aged five months to ten years, who presented with prolonged seizures at the pediatric emergency departments of nine hospitals. Findings: Overall, 57.8% of the children were younger than three years; 7% had pre-existing psychomotor delay. Although 21% had had previous seizures, only 13% were receiving antiepileptic therapy. On presentation, 63.5% of the patients had fever and 26% were in status epilepticus. The seizures were focal in 21% of the cases. Malaria was the most common cause, involving 63% of the cases. The recurrence rate was 38%, and the mortality rate 4%. CONCLUSION: Prolonged seizures in DRC and Rwanda are frequently associated with fever, most commonly caused by malaria. The immediate use of long-acting antiepileptic drug could improve their outcomes.

[Epidemiology and characteristics of febrile seizures in children]. / Epidémiologie et caractéristiques des convulsions fébriles de l'enfant.

Febrile Seizures (FS), despite their usual benign clinical course, are still subject of controversies regarding the need for further investigation and treatment with anti-epileptic drugs (AEDs). Our study aimed to inventory the clinical findings, laboratory and imaging data associated with FS and eventually influencing their management. 275 episodes admitted with FS at the emergency ward of the Liege CHR over a 5 year period were retrospectively analyzed regarding precipitating factors; clinical features; laboratory, electroencephalographic, and imaging studies; as well as treatment response. FS represented 1.4% of admissions to the pediatric service. 31.3% of patients had a family history of seizure disorder. 9% percent of seizures were focal, 11.7% recurrent, and 12.3% prolonged (greater than 10 minutes). Upper respiratory tract and otorhinolaryngologic viral infections were the most often implicated provoking factors, occurring in 69.5% of patients. Laboratory, electroencephalographic and radiographic studies were normal in more than 90% of cases. 73.8% of seizures resolved without intervention. An AED was required to manage the remaining 26.2%. This study confirms the favorable outcomes of FS as demonstrated in previous studies. This happens without requiring AEDs for resolution, and without recurrence. Laboratory, electroencephalographic and imaging studies, as well as initiation of AEDs should be based primarily on clinical severity.

Diastematomyelia: pre- and postnatal multimodal diagnostic approach.

Diastematomyelia is a relatively rare congenital abnormality presenting as a sagittal separation of the spinal cord. Although cases of diastematomyelia have been previously reported, fully documented approaches by both prenatal and postnatal diagnostic workup are rare in the literature. We present a fully studied case of diastematomyelia type I investigated by prenatal US and MRI and postnatal US, MRI and radiography.

[Update in drug therapy in neuropediatry]. / Actualités thérapeutiques en neuropédiatrie.

The most recent antiepileptic drugs used in children are lamotrigine, topiramate, oxcarbamaz6pine and levetiracetam. Their efficacy is proven, depending on the type of crisis, but in Belgium they are reimbursed only in certain conditions. The treatment of children with attention deficit hyperactivity disorder (ADHD), which was only constituted of methylphenidate, can now benefit from atomoxetine whose mechanism of action is different.

[Management of medulloblastoma in children: the experience of a single institution in Liege from 1991 to 2005]. / Prise en charge du médulloblastome de l'enfant.

We present the experience of the Citadelle Hospital (Liege, B) in the diagnosis, treatment and follow-up of medulloblastoma in children. A retrospective study of 10 cases of medulloblastoma was performed. Five years after diagnosis, the event-free survival was 77%.

[Prader-Willi syndrome: specific management by a multidisciplinary team]. / Le syndrome de Prader Willi: intérêt d'une prise en charge pluridisciplinaire.

Prader Willi syndrome can be viewed as a physiopathological model of obesity. Such patients deserve specific management, preferably in a multidisciplinary setting. The paper reports on 6 patients followed in the paediatric endocrine service at the University of Liege.

Hyperekplexia in Kurdish families: a possible GLRA1 founder mutation.

Autosomal recessive hyperekplexia is due to loss-of-function mutations in the GLRA1 gene. The authors describe six patients from two consanguineous families with a homozygous deletion of the first seven GLRA1 exons and provide evidence of a founder effect in Kurds from Turkey. Hyperekplexia may be misdiagnosed as epilepsy.

[Epilepsy in children with cerebral palsy]. / Epilepsie chez des enfants infirmes moteurs-cérébraux.

The incidence of epilepsy in 110 patients with cerebral palsy (the majority with spastic tetraplegia) was 46,4%. Almost half of the patients with spastic tetraplegia (45%) and hemiplegia (52%) had epilepsy. The incidence was lower in patients with spastic diplegia (32%). Half of epilepsy in spastic hemiplegia were partial seizures and the other half consisted of generalized seizures, while generalized tonic-clonic episodes predominated in all other forms of C.P. A high incidence of West syndrome was observed in patients with spastic tetraplegia. Etiological factors of C.P. were perinatal in 75%. 16,65% had neonatal antecedents of convulsions; most of them had spastic tetraplegia (75%) and a significant mental retardation. A low intelligence quotient (I.Q.) was seen in most of the children with epilepsy, and patients with tetraplegia had significantly lower intelligence quotient than other groups.

Bony syngnathia, vertebral segmentation defect, coloboma, microcephaly and mental retardation: confirmation of Dobrow syndrome and review of syndromal syngnathias.

Congenital bony fusion of the maxilla and mandible is a rare condition. Two classifications were previously proposed dealing exclusively with craniofacial malformations. Most of the reported cases to date represent either aglossia-adactylia or hemifacial microsomia syndromes. We report a young girl with bony syngnathia associated with multiple defects (severe microcephaly, coloboma, vertebral segmentation defects), growth and mental delay. This patient is very similar to the patient described by Dobrow in 1983 and confirms the existence of this extremely rare disorder.

[Epileptic and non-epileptic paroxysmal phenomenons in the child]. / Principaux phénomènes paroxystiques epileptiques et non epileptiques propres a l'enfant.

In neuropediatry, paroxysmal phenomenons characteristic of the child can be of an epileptic or non epileptic origin. A correct clinical diagnosis has to be done straightaway in order to best select the complementary exams, establish the prognosis and decide the treatment. We will describe the clinical presentation of the main paroxysmal phenomenons found in children.

[Catecholaminergic polymorphic ventricular tachycardia in a child: an often unrecognized diagnosis]. / La tachycardie ventriculaire catécholergique du jeune enfant: un diagnostic souvent méconnu.

UNLABELLED: Catecholaminergic polymorphic ventricular tachycardia is important to be diagnosed as an underlying disease in children with syncope and normal heart, because of its poor prognosis. CASE REPORT: A 3-year-old boy was referred for stress and emotion induced syncope. Primary ventricular arrhythmia, consisting of salvos of bidirectional ventricular tachycardia, was reproducibly induced by physical exertion. The syncopal events and severe arrhythmia disappeared with beta-blocking therapy. CONCLUSION: Despite its rare occurrence, catecholaminergic polymorphic ventricular tachycardia is an important cause of stress and emotion induced syncope and sudden death in children.

[How I investigate...difficult cases of tuberculous meningitis]. / Comment j'explore ... certains cas difficiles de méningite tuberculeuse.

To formally document the presence of the bacillus of Koch in meninges still remains difficult and depends on many variables. We report two cases where the diagnosis proved difficult. The presentation of tuberculous meningitis can take several aspects. Mantoux reaction is frequently negative. The BK cultures, which demonstrate the diagnosis, depend on the volume of CSF and on the importance of BK dissemination. It, unfortunately, remains difficult to make the diagnosis of tuberculous meningitis even though this pathology can have extremely deleterious consequences.

Infantile and juvenile presentations of Alexander's disease: a report of two cases.

We describe 2 new cases of Alexander's disease, the first to be reported in Belgium. The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third of heterozygotic twins, the 2 others having developed normally and being alive at age 5 years. The second patient developed at age 10 years and over a decade spastic paraparesis, palatal myoclonus, nystagmus, thoracic hyperkyphosis and thoraco-lumbar scoliosis with radiological findings of bilateral anterior leukoencephalopathy. Brain stereotactic biopsy at age 16 years demonstrated numerous RFs. With these 2 cases, we review the literature on the various clinico-pathological conditions reported as Alexander's disease. We discuss the nosology of this entity and the pathogeny of RFs formation and dysmyelination. Clues to the diagnosis of this encephalopathy in the living patient are briefly described.