A Prospective Study of Arterial Spin Labelling in Paediatric Posterior Fossa Tumour Survivors: A Correlation with Neurocognitive Impairment.

AIMS: Posterior fossa tumours (PFTs), which account for two-thirds of paediatric brain tumours, are successfully treated in about 70% of patients, but most survivors experience long-term cognitive impairment. We evaluated arterial spin labelling (ASL), a common, non-invasive magnetic resonance imaging (MRI) technique, as a biomarker of cognitive impairment in a paediatric PFT survivor population. MATERIALS AND METHODS: Sixty participants were prospectively analysed. PFT survivors were at least 5 years post-treatment and had been treated as appropriate for their age and type of tumour. Group 1 had received radiotherapy and Group 2 had not. Group 3 were healthy controls matched to Group 1 for age, sex and handedness. All participants underwent cognitive assessment and multimodal MRI, including an ASL perfusion sequence. We used semi-quantitative ASL methods to assess differences in mean perfusion in the thalamus, caudate, putamen and hippocampus. RESULTS: Statistically, no significant associations between cognitive data and radiation doses were identified. Compared with healthy controls, Group 1 patients had significantly lower overall mean perfusion values (20-30% lower, depending on the cerebral structure) and Group 2 had slightly lower mean perfusion values (5-10% lower). Perfusion values did not correlate with total prescribed irradiation doses nor with doses received by different cerebral structures. Episodic and semantic memory test scores were significantly lower in Group 1 and correlated with lower mean absolute perfusion values in the hippocampus (P < 0.04). CONCLUSIONS: These preliminary results indicate that radiotherapy affects the perfusion of specific cerebral structures and identify perfusion as a potential biomarker of hippocampus-dependent memory deficit.

Lyme neuroborreliosis in pediatrics: A retrospective, descriptive study in southwest France.

BACKGROUND: The neurological effects of Lyme borreliosis in children are varied and their clinical progression is not widely reported in the French literature. We carried out a retrospective study to describe the clinical characteristics of Lyme neuroborreliosis in children in southwest France and their clinical progression at 6 months. METHODS: This study was carried out at Toulouse University Hospital during the period 2006-2017 using patient records. Case definition was based on the combined French clinical and laboratory diagnostic criteria. RESULTS: In total, 26 children were included. The median age was 8 years (4-14 years). The different neurological symptoms reported were: meningoradiculitis (62%), which was usually associated with facial palsy (54%); isolated facial palsy (15%); isolated meningitis (8%); polyradiculoneuritis (4%); benign intracranial hypertension (4%) and myelomeningoradiculitis (4%). The most common functional symptoms were headaches (54%), the perception of asthenia (42%), neck pain (27%), and a loss of appetite (19%). Patients with laboratory meningitis (84%) often had no signs of meningism or headaches (38%). CONCLUSION: The majority of the cases involved meningoradiculitis but other, less common, neurological conditions have been described. The clinical signs suggestive of meningitis are not very marked and might delay the diagnosis.

Specific Cues Can Improve Procedural Learning and Retention in Developmental Coordination Disorder and/or Developmental Dyslexia.

The present study investigates procedural learning of motor sequences in children with developmental coordination disorder (DCD) and/or developmental dyslexia (DD), typically-developing children (TD) and healthy adults with a special emphasis on (1) the role of the nature of stimuli and (2) the neuropsychological functions associated to final performance of the sequence. Seventy children and ten adults participated in this study and were separated in five experimental groups: TD, DCD, DD, and DCD + DD children and adults. Procedural learning was assessed with a serial reaction time task (SRTT) that required to tap on a specific key as accurately and quickly as possible when stimuli appeared on the screen. Three types of stimuli were proposed as cues: the classical version of the SRTT with 4 squares aligned horizontally on the screen, giving visuospatial cues (VS cues), and two modified versions, with 4 letters aligned horizontally on the screen (VS + L cues) and letters at the center of the screen (L cues). Reaction times (RT) during the repeated and random blocks allowed assessing three phases of learning: global learning, specific learning and retention of the sequence. Learning was considered as completed when RT evolved significantly in the three phases. Neuropsychological assessment involved, among other functions, memory and attentional functions. Our main result was that learning and retention were not influenced by the available cues in adults whereas learning improved with specific cues in children with or without neurodevelopmental disorders. More precisely, learning was not completed with L cues in children with neurodevelopmental disorders. For children with DD, learning was completed with the VS and VS + L cues whereas for children with DCD (with or without DD), learning was completed with combined VS + L cues. Comorbidity between DD and DCD had no more impact on procedural learning than DCD alone. These results suggest that learning depends on the nature of cues available during practice and that cues allowing learning and retention depend on the type of disorder. Moreover, selective attention was correlated with RT during retention, suggesting that this neuropsychological function is important for procedural learning whatever the available cues.

Assessing the management of pediatric bone and joint infections according to French guidelines.

BACKGROUND: Short treatments for acute bone and joint infections (BJI) are recommended. We implemented a protocol in 2009 to improve diagnosis and bacteriological documentation, and to shorten antibiotic therapies as per French guidelines (French Pediatric Infectious Disease Group, GPIP). METHODS: To assess the impact of the new clinical protocol for BJI, we conducted a retrospective study from January 1st, 2006 to August 31st, 2012. Two successive cohorts were compared, before and after protocol implementation. All children suspected of community-acquired BJI were included. Confirmed osteomyelitis and septic arthritis required a positive bacterial isolate; otherwise, cases were considered probable. We compared clinical, biological, and radiological data; duration of antibiotic therapy and hospital length of stay; and complications and sequelae. RESULTS: A total of 377 children with suspected BJI were included. The bacteriological identification improved from 32% to 44% when patients were completely evaluated. Isolated bacteria were Staphylococcus aureus (53%), Kingella kingae (17%), Streptococcus pyogenes (15%), and Streptococcus pneumoniae (8%). Before protocol implementation, 70% of patients had a central venous line versus 9% after implementation. Mean duration of IV antibiotics (11 days versus 6 days), mean duration of total antibiotic therapy (45 days versus 32 days) and mean hospital length of stay (13 days versus 7 days) had significantly improved. CONCLUSION: Improvement in bacteriological diagnosis and shorter antibiotic regimens lead to shorter hospital length of stays with no additional morbidity. Simplifying the protocol and better diffusion among health professionals should contribute to shortening BJI treatment duration.

[Severe nutritional rickets in young children: Resurgence of an old disease]. / Rachitisme carentiel sévère du nourrisson : de nouveau d'actualité.

Nutritional rickets remains a significant public health issue for children worldwide. Although it has almost disappeared in industrialized countries following routine vitamin D supplementation, recent evidence suggests an increasing incidence, especially in young children. In addition to the classical clinical consequences on bone and the growth plate, rickets may also be associated with life-threatening neurological and cardiac complications in the most severe forms. Consequently, early screening and treatment are required. Here, we report the case of a 2-year-old child who presented with severe nutritional rickets associated with seizure and cardiomyopathy. Family screening revealed rickets in all the siblings. This case report emphasizes the importance of being aware of this disease, notably in population with sociocultural risk factors.

[Hallucinations: Etiological analysis of children admitted to a pediatric emergency department]. / Hallucinations : analyse étiologique chez des enfants admis en service d'accueil des urgences.

Children's hallucinations pose the dual challenge of diagnosing a possible medical emergency and a possible psychiatric disorder. PURPOSE: The main objective was to analyze the causes of such hallucinations in children presenting to a pediatric emergency department. MATERIAL AND METHODS: We conducted a single-center, observational, retrospective study including all children aged less than 15 years experiencing hallucinations and admitted to our tertiary level pediatric emergency department between 1 January 2007 and 31 December 2015. The data collected were demographic; medical: previous medical or psychiatric history, current medications, associated clinical or psychiatric symptoms, type and character of hallucinations, length and recurrence of hallucinatory phenomena; and other biological, radiological and neurological explorations. RESULTS: Sixty-eight patients were included (29 boys). The mean age was 9.1±3 years (range, 2-14 years and 10 months; median, 9.2 years). Admissions were seasonal with a bimodal distribution (a peak during springtime and another one during fall). Hallucinations were mainly visual (90%), acute (77%) and complex (63%). Visual hallucinations were associated with other types of hallucinations: auditory (n=17), somatosensory (n=7). Fifteen children had a psychiatric history and had already experienced hallucinatory phenomena (93%). Among 47 patients (69%), these hallucinations were associated with other symptoms: agitation (41%), headaches (28%), hyperthermia (21%) and negative symptoms of the schizophrenia spectrum (15%). On admission, 20 patients (29%) had one or more treatments under way (34 drugs, 41% known for hallucinogenic adverse effects). Neurological explorations were undertaken in half of the cases. Toxicological analysis prescribed in 19 children was positive in five cases (26%). Fifty-three percent of patients were hospitalized and 51 children received a specialized follow-up (by a neurologist and/or a psychiatrist). A nonpsychiatric origin of these hallucinations was diagnosed in 29 patients (43%): neurological causes (n=10), infectious diseases (n=10), intoxications (n=5) and a medication side effect (n=4). CONCLUSION: Hallucinations with a suspected underlying psychiatric cause differed on several factors: chronic duration (p=0.02), an onset after 10 years of age (p=0.004), previous identical episodes (p=0.014) and a parental psychiatric history (p=0.036), auditory hallucinations (p=0.0009), absence of fever (p=0.005), headaches (p=0.036) and the presence of negative symptoms of the schizophrenic spectrum (p=0.02).

[Anthropological approach to current parental perceptions of children's seizures]. / Approche anthropologique des représentations parentales actuelles des convulsions chez l'enfant.

UNLABELLED: Pediatric seizures are a common symptom, especially when associated with fever. This phenomenon is still shocking and traumatic for parents. The study analyzed current parental perceptions of seizures in order to improve the quality of management, care, and explanations provided to families at our emergency unit. METHODS: Using an anthropological approach, we analyzed 28 interviews of 37 parents whose child was admitted to our pediatric emergency unit between November 2007 and August 2008 due to a first seizure. RESULTS: The parental experience of the crisis was marked by upsetting memories of a "scary"-looking body and the perception of imminent death. Parental interpretations of the pathophysiology of the event were often wrong; very few mentioned the possibility of its cerebral origin, leading to inappropriate rescue attempts (e.g., giving CPR). The meaning attributed by parents to the word "seizure" and "epilepsy" usually referred to an exact clinical description of the phenomenon, but many admitted being unfamiliar with the term or at least its origin. Many studies have found the expectation of imminent death as well as inappropriate behaviors. This is the first study to consider interpretations expressed by parents around the convulsive phenomenon and to confirm a low level of knowledge of the symptom. Some historical interpretations persisted (e.g., the influence of excessive mood, anger, menstruation, demonic possession). CONCLUSION: Understanding and integrating these parental interpretations seems essential to improving care for families who first experience this symptom. This study motivated the implementation of a special educational workshop on seizures in 2010.

[Value of lumbar puncture after a first febrile seizure in children aged less than 18 months. A retrospective study of 157 cases]. / Utilité de la ponction lombaire lors de la première convulsion fébrile chez l'enfant de moins de 18 mois. Étude rétrospective de 157 cas.

AIM: Because meningitis symptoms are not very specific under the age of 18 months, lumbar puncture (LP) was widely recommended in children presenting a febrile seizure (FS). Recent retrospective studies have challenged this age criterion. In 2011, the American Academy of Pediatrics updated its guidelines for the first episode of simple FS: LP is indicated if signs suggestive of meningitis are present and remains "an option" in case of prior antibiotic treatment or between the age of 6 and 12 months if the child is not properly vaccinated against Haemophilus and Streptococcus pneumoniae. Because the meningitis epidemiology and the vaccination coverage are different, the objective of this study was to evaluate whether these new guidelines were applicable in France. PATIENTS AND METHODS: Between 2009 and 2010, we conducted a retrospective single-center study including 157 children aged less than 18 months admitted to the pediatric emergency department (Children's Hospital, Toulouse, France) for their first febrile seizure. The data collected were: type of seizure, knowledge of prior antibiotic treatment, neurological status, signs of central nervous system infection, and biological results (LP, blood cultures). RESULTS: Lumbar puncture was performed in 40% of cases (n=63). The diagnosis of meningitis/encephalitis was selected in eight cases: three cases of viral meningitis, three bacterial meningitis (Streptococcus pneumoniae), and two non-herpetic viral encephalitis. The incidence of bacterial meningitis in our study was 1.9%. The risk of serious infection, bacterial meningitis or encephalitis, was increased when there was a complex FS (14% versus 0% with a simple FS, P=0.06). The presence of other suggestive clinical symptoms was strongly associated with a risk of bacterial meningitis/encephalitis (36% in case of clinical orientation versus 0% in the absence of such signs, P<0.001). DISCUSSION: All severe clinical presentations were associated with complex FS (prolonged, focal, and/or repeated seizures) and the presence of other suggestive clinical signs (impaired consciousness lasting longer than 1h after the seizure, septic aspect, behavior disorders, hypotonia, bulging fontanel, neck stiffness, petechial purpura). The risk of bacterial meningitis or encephalitis associated with a simple FS and followed by a strictly normal clinical examination is extremely low. CONCLUSION: After a simple febrile seizure without any other suggestive signs of meningitis, systematic lumbar puncture is not necessary even in children younger than 18 months. LP remains absolutely indicated if clinical symptoms concentrate on central nervous system infection and should be discussed in case of complex seizures, prior antibiotic treatment, or incomplete vaccination.

[Respiratory syncytial virus brainstem encephalitis in a 7-year-old boy]. / Rhombencéphalite liée au virus respiratoire syncytial chez un garçon de 7 ans.

The literature reports that neurological complications of childhood respiratory diseases due to respiratory syncytial virus (RSV) fluctuate between 1 and 40% of cases. They mostly involve central apnea - often the first symptom of infection - anoxia, and ischemic brain damage due to severe sudden weakness in infants, and seizures and consciousness disorders more or less associated with focalized neurological deficiency proving an encephalitis lesion. We report the case of brainstem encephalitis in a 7-year-old boy with RSV A nasopharyngitis, with meningitis, positive polymerase chain reaction in cerebrospinal fluid and magnetic resonance imaging (MRI) abnormalities, which was explained by viral replication encephalitis. Based on a literature review, we discuss the main aspects of epidemiology and physiopathology of the main neurological complication of RSV. Most of them have not been fully investigated and only a few articles report encephalitis. As far as central apnea is concerned, an animal experimental hypothesis surprisingly suggests a peripheral mechanism.

[Guillain-Barré syndrome in a child with normal tendon reflexes]. / Un cas pédiatrique de syndrome de Guillain-Barré à réflexes conservés.

We describe the case of a 10-year-old child with the acute motor axonal neuropathy (AMAN) form of Guillain-Barré syndrome (GBS) with preserved tendon reflexes, 6 days after a bout of gastroenteritis. The child quickly showed weakness of the distal muscles of his four limbs, with preserved tendon reflexes and a raised CSF protein concentration with no cells. Nerve conduction studies showing motor axonal degeneration confirmed the diagnosis of GBS in spite of preserved tendon reflexes. The serum was positive for IgG antibodies to gangliosides GM1 and GD1b. The child received intravenous immunoglobulins, which resulted in a favorable progression. This case proves that GBS with normal tendon reflexes exists. The other cases of SGB with preserved tendon reflexes already described in the literature were the AMANs form with antibodies to gangliosides in the serum and only adults were affected.

[Neurobiological bases and neurophysiological correlates of developmental coordination disorders]. / Trouble de l'acquisition de la coordination : bases neurobiologiques et aspects neurophysiologiques.

Among psychomotor disorders in children, developmental coordination disorder (DCD) is characterized by a motor skill impairment that interferes with psychomotor development, academic performance and activities of daily living, despite normal intelligence. The main behavioural phenomena (lack of postural control, coordination and motor learning) suggest involvement of cerebellum, basal ganglia and frontal and parietal lobes. Our studies on a synchronisation/syncopation task, with EEG recording (coherence analysis and evoked potential), show that DCD children (8 to 12 years old) exhibit major interindividual variability and do not improve performance with repetition. In younger DCD children, an increase of coherence between fronto-central regions was reported, and, for evoked potential, an increase of motor preparation component and a N100 latency longer than control children. These findings support the idea of a general synchronization disorder in DCD children and furnish elements allowing a better understanding of intra- and interindividual variability.

[Treatment and prognosis of idiopathic intracranial hypertension in children. Retrospective study (1995-2009) and literature review]. / Traitement et pronostic de l'hypertension intracrânienne idiopathique de l'enfant. Étude rétrospective (1995-2009) et revue de la littérature.

AIM: Idiopathic intracranial hypertension (IIH) may cause severe visual loss due to the optic nerve damage. Routine management involves mainly medical treatment. The aim of this study was to improve diagnosis and management of IIH in children. METHODS: The medical records of all patients with definite IIH seen at the children's hospital of Toulouse between 1995 and 2009 were reviewed. Cases of secondary intracranial hypertension were included because they did not present any cerebral lesions and underwent a similar therapeutic approach. The clinical and ophthalmological data at the beginning and at the end of their treatment was collected. RESULTS: Eighteen children were included in this study. The average age was 10 years and the sex-ratio was equal to 1. There were 3 cases of secondary idiopathic intracranial hypertension in this pediatric group. The main features encountered were headache (15 children) and diplopia (8 children). Abnormal neurological examination was found for 11 patients with abducens nerve paresis in 8 cases, rachialgia in 6 cases, and neurogenic pains (neuralgia, dysesthesia, paresthesia, hyperesthesia) in the other cases. Papilledema was noted in 16 patients. At the initial phase, loss of visual acuity was documented in 6 patients and altered visual field in nine patients. All patients had a medical treatment. When recurrence occurred, each new treatment was documented, for a total of 23 treatments analyzed. Lumbar puncture was the only treatment for 2 patients. In 16 cases, first-line treatment was acetazolamide and it was the second choice in 1 case, with an average dosage of 11.2mg/kg and a mean duration of 2.5 months (15 treatments could be analyzed). This treatment was effective in 11 cases out of 15. Steroids were the initial treatment in 4 cases and second-line treatment in 4 cases (after failed acetazolamide therapy). The dosage was 1.5-2mg/kg for a mean duration of 1.5 months (6 treatments could be analyzed). This treatment was effective in 5 patients out of 6. One patient had dual therapy. No surgical procedure was necessary in this pediatric cohort. Three patients presented relapses of IIH. The outcome was good with no residual visual impairment in the 13 patients analyzed. One patient was still under medication. COMMENTS: Therapeutic management of IIH in a pediatric population is essentially medical, in some cases limited to lumbar puncture. The first-line treatment is acetazolamide, but this study shows that low doses and short duration are usually chosen. Doses must be increased and treatment prolonged to avoid the use of corticosteroids as a second-line treatment and prevent possible relapses that require close monitoring of visual function. CONCLUSION: The visual prognosis is generally better for this age group compared to adults and no risk factors for visual sequelae were identified. A standardized protocol for management of IIH was proposed.

[The outcome of children presenting with a prenatally diagnosed arachnoid cyst]. / Devenir des enfants présentant un kyste arachnoïdien de diagnostic anténatal.

AIM: Evaluate the outcome of children with prenatally diagnosed arachnoid cysts. MATERIAL AND METHODS: Retrospective study of seventeen cases of children who were diagnosed with an arachnoid cyst during prenatal MRI between July 1994 and January 2007 and followed up for a mean 6 years and 6 months. Follow-up was based on evaluation of clinical files and contacts with the physicians who were following the children. The children were divided into three groups: group 1 normal development, group 2: minor clinical signs, normal schooling, group 3: major clinical symptoms, schooling disturbed. RESULTS: Eight of the 17 patients included in this study underwent derivation surgery for the cyst. Eight of the 17 children were in group 1, and 3 in group 2. Four of the 6 children in group 3 had associated symptoms. Two of the children in group 3 present with a supratentorial cyst, and 4 with a cyst of the posterior fossa. CONCLUSION: The prenatal diagnosis of a arachnoid cyst should be accompanied by a search for associated lesions. The risk of hydroencephalitis should be explained to the parents.

[Hemorrhagic lesion of the corpus callosum in influenza-associated encephalitis]. / Lésions hémorragiques du corps calleux dans l'encéphalite liée au virus Influenza.

We describe a rare case of Influenza B-associated encephalopathy with hemorrhagic lesions of the corpus callosum. A 12-year-old Caucasian girl presented a 24-h fever followed by partial seizure, secondarily generalized, and disturbance of consciousness. Magnetic resonance imaging on Day 2 of her illness showed two hemorrhagic lesions of the corpus callosum. The Influenza B virus was found on nasopharyngeal swab. Neurologic signs had completely recovered by Day 3. A review of the literature identified a few similar cases; the common features include a relatively older age and prompt and complete recovery from clinical symptoms. This is the first report to describe hemorrhagic lesions of the corpus callosum in influenza.

Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature.

INTRODUCTION: A 14-year-old girl, followed in our department for Marfan syndrome, presented with postural headache for a month. Neurological examination was normal. The diagnosis of intracranial hypotension syndrome was suspected. DISCUSSION: Bilateral subdural hematomas were found on brain magnetic resonance imaging (MRI), and spinal MRI showed large lumbosacral arachnoid diverticula; no cerebrospinal fluid leaks could be found. Despite bed rest and hydration for 2 weeks, postural headache remained. Epidural blood patching was also performed. Subsequently, the patient became asymptomatic and could stand up after 1 day. Brain MRI did not find recurrent subdural hematoma after 1 month. Dural ectasia is one of the major criteria of Marfan syndrome, and it is often poorly symptomatic. Intracranial hypotension is a rare complication especially in children, and management is not standardized. In this case report, blood patching was sufficient. Further research into the diagnosis and management of spontaneous intracranial hypotension is required.