Metabolic Profile of Cerebellum in Posterior Fossa Tumor Survivors: Correlation With Memory Impairment.

AIMS: The cerebellum is a key structure in working and procedural memory. The aim of the present prospective exploratory study was to investigate, the metabolic characteristics of the cerebellum in posterior fossa tumor (PFT) survivors using 3D proton magnetic resonance spectroscopy imaging (3D MRSI), to determine whether metabolites could be useful biomarkers of memory impairment. MATERIALS AND METHODS: Sixty participants were included in the IMPALA study, divided into three groups: 22 irradiated PFT, 17 nonirradiated PFT, and 21 healthy controls matched with irradiated PFT for age, sex, and handedness. PFT survivors were treated at least 5 years ago, either by surgery or a combination of surgery, chemotherapy, and radiotherapy. All participants underwent working and procedural memory tests and multimodal MRI including a 3D MRSI sequence. N-acetylaspartate (NAA), choline (Cho), creatine (Cr), and lactate (Lac) metabolite values were extracted from the cerebellum for comparisons between groups, correlations with neurocognitive test scores, and radiotherapy doses. RESULTS: Median (range) age at neurocognitive tests was 18 (7-26) years. Median Cho, Cr, NAA, and Lac values, and the ratio of NAA to the sum of metabolites were significantly lower for PFT survivors than for healthy controls (p < 0.05). Scores on working and procedural memory tests were significantly lower for PFT survivors (p < 0.004) and correlated with median and maximum Cho and NAA values (0.28

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.

[Asymptomatic neonatal neurocutaneous melanosis]. / Mélanose neurocutanée néonatale asymptomatique.

Giant congenital nevi in 1% of cases are associated with anomalies of the central nervous system, which are characteristic of neurocutaneous melanosis. When neurocutaneous melanosis becomes symptomatic, it is associated with a poor prognosis. With recommended neonatal screening, asymptomatic neonatal cases are being discovered more frequently. On the basis of this observation, we consider various aspects of this association.

[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.

Desmoplastic infantile astrocytoma with benign histological phenotype and multiple intracranial localizations at presentation.

Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma (DIG) are rare intracranial tumors that mostly occur in the first 2 years of life and involve superficial cerebral cortex. Despite the large size of these lesions and some worrisome histological and radiological features, prognosis is generally favorable after gross total resection. We report an original observation of a desmoplastic infantile astrocytoma in a 5-year-old boy with multiple localizations on initial presentation, including the unusual subtentorial region. Magnetic resonance imaging showed a temporal tumor with prepontine and interpeduncular extension, and two other distinct localizations in cisterna magna and left cerebellar hemisphere. Leptomeningeal enhancements were present around the basal cistern. The surgical samples, corresponding exclusively to subtentorial lesions, were devoid of anaplastic features; the temporal lesion was untouched because of the interpeduncular extension. Adjuvant chemotherapy was applied, with shrinkage of lesions. DIA and DIG are more generally unifocal at initial presentation. When the tumor is large, multilobular involvement is common, but multiple location of DIG is, on the contrary, very rare. Previously, only five cases of DIG/DIA located in two or more separate locations have been published. We report the sixth, and first noninfantile, case of DIA/DIG with multifocal initial presentation.

[Retro- and parapharyngeal infections: standardization of their management]. / Infections rétro- et parapharyngées: vers une harmonisation des pratiques.

AIM: To analyze the changes in the management of retropharyngeal and parapharyngeal infections and propose a decisional algorithm for their diagnosis and treatment. PATIENTS AND METHODS: A retrospective survey was carried out in a tertiary care pediatric hospital between January 2001 and December 2005. All children aged less than 15 years and affected by a retro- or parapharyngeal infection were included. Clinical, biological, and radiological data, medical and surgical treatment, and complications were extracted from the review of medical charts. The results of the surgical findings were correlated with a cervical computed tomographic scan (CT scan). RESULTS: Thirty-one patients were included, 64.5% during the last 2 years of the study period. All children presented fever and a stiff neck. The pharyngeal examination revealed a retropharyngeal bulge in a quarter of the population and an upper respiratory tract infection was concomitant in 68% of cases. A CT scan was carried out in 29 of 31 children (93.5%), with the radiological diagnosis of an abscess in 16 children (55.2%), presuppurative adenitis in 8 children (27.6%), and cellulitis in 5 children (17.2%). The CT scan was performed within 0.75 days of admission in 2001 and 2.3 days in 2005. All children were treated with intravenous antibiotic therapy: an association of amoxicillin/clavulanic acid and an aminoglycoside in most cases. The mean duration of intravenous antibiotic therapy was 5.2 days. Seventeen patients (93.5%) underwent surgical drainage and purulent material was found in 82.3% of cases. The accuracy of the CT scan, confirmed by surgical finding of a purulent material, was 71.4% in correctly identifying an abscess. The mean duration of surgical treatment after admission increased from 1.7 days in 2001 to 3.3 days in 2005. The number of patients who underwent surgery was divided by a factor of 3 in the second period of the study. Two groups were compared: group A (n=12) treated with antibiotic therapy and group B (n=17) treated with antibiotics and surgical drainage. No significant difference was found between the two groups considering the duration of parenteral and oral antibiotic therapy, the standardization of cervical mobility, the mean time for apyrexia, and the length of hospitalization. There was one recurrence in group B 1 month later, and one case of sepsis in group A. None of the patients with retropharyngeal infection died. CONCLUSION: Without clinical evidence of severe sepsis, parenteral antibiotic therapy is recommended as the first-line treatment for children over 6 months of age presenting with retropharyngeal and parapharyngeal infections. If the clinical and/or biological conditions do not improve within 48-72h, a CT scan is indicated to assess the extent of infection and exclude complications. The decision to initiate surgical drainage depends on the patient's clinical status and the accessibility of the abscess.

[Orbital tumors in children: CT and MR imaging features]. / Tumeurs orbitaires chez l'enfant: revue iconographique en TDM et IRM.

The purpose of the article is to review the CT and MR imaging features of orbital tumors in children. Tumors in children are usually different than those in adults. Clinical symptoms are usually non-specific. Clinical examination combined with US may be sufficient for diagnosis and follow-up of benign and superficial lesions. CT and/or MRI are needed for deep or malignant lesions. CT is valuable for osseous and/or calcified lesions. MR is advantageous because of its superior spatial resolution and non-ionizing nature. Malignant tumors correspond to about 20% of lesions and include primary tumors (retinoblastoma, rhabdomyosarcoma) and metastases. Benign pathology is more frequent (80%) with dermoid cyst corresponding to about 50% of orbital masses.

[Optic pathway gliomas in neurofibromatosis type I. Longitudinal study of 30 cases in two multidisciplinary practices]. / Les gliomes des voies optiques dans la neurofibromatose de type 1. Etude longitudinale de 30 cas suivis dans deux consultations multidisciplinaires.

UNLABELLED: The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol. PATIENTS AND METHODS: All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination. RESULTS: Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis. CONCLUSION: Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.

[Reliability of CT scan in the diagnosis of conductive hearing loss with normal tympanic membrane]. / Fiabilité du scanner dans le diagnostic des surdités de transmission à tympan normal.

The goal of this study was to assess the reliability of CT-Scan in the cases of conductive hearing losses with normal tympanic membrane. A computed tomography of the temporal bone (CT-Scan) has been performed in a prospective manner in all patients who underwent surgery for a conductive hearing loss with a normal tympanic membrane in our department. Out of 474 cases, 437 cases (92.2%) presented with otosclerosis. In 25 cases a minor malformation (5.3%) was found at surgery, and in 12 cases (2.5%), another diagnosis was made. Sensitivity of CT-Scan was 91.3% in otosclerosis and 57% in minor malformations. In 8.7% of cases, a superficial and beginning surgical focus was put in evidence whereas CT-Scan was normal. Theses cases represent infra-radiological cases of otosclerosis. In case of radiological otosclerosis, fenestral otosclerosis was found in 83.5% of the cases. CT-Scan was found specific on the operated and deaf side, but in 11.3% of the cases, a radiologic focus did not have a clinical consequence on the controlateral side. A radiological focus is not systematically responsible for a hearing loss. Finally, CT-Scan remains a reliable, sensitive and specific exam in the diagnosis of cases of conductive hearing losses with normal tympanic membrane.

Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases.

The aim of this study was to determine if a relationship exists between bone level thresholds and the extension of otosclerotic foci within the otic capsule. The study consisted of a retrospective case review in a university hospital. We included patients who underwent surgery for otosclerosis in our department and who had a CT scan prior to surgery. We analyzed the data charts and CT scans of 437 cases (386 patients). On CT scan, we distinguished patients with fenestral otosclerosis and/or with a pericochlear focus. A pericochlear focus could be extended (Group 2) or not (Group 1) to the cochlear endosteum. Data for Groups 1 and 2 were compared with those for the control group of all patients for whom CT scan showed no cochlear focus (Group 3). Of the 437 CT scans, 399 were positive (91.3%). An anterior focus was reported in 305 cases (69.8%), a footplate thickening in 21 cases (4.8%) and both anomalies were encountered in 60 cases (13.7%). A pericochlear focus was reported in 53 examinations. This focus was extended to the endosteum in 14 cases (26.4% of the pericochlear foci). In Group 1, preoperative air conduction (AC) thresholds were significantly lower than in the control group (p < 0.05). The air--bone gap was also significantly larger in Group 1 (p < 0.05). Bone conduction (BC) thresholds were lower in Group 1 than in the control group but the difference was not significant. In Group 2, preoperative AC thresholds were significantly lower than in the control group (p < 0.05). BC thresholds were also lower in Group 2 than in the control group and the difference was significant (p < 0.05). As a result of this study, we assume that there may be a relationship between bone level thresholds and the radiological extension of otosclerosis within the otic capsule.

[Septic arthritis of a lumbar facet joint associated with epidural and paravertebral soft tissue abscess]. / Arthrite septique interapophysaire postérieure lombaire avec abcès épidural et des parties molles paravertébrales.

Septic arthritis of the lumbar facet joint is rare, probably underdiagnosed, often associated with complications such as epidural abscess and paraspinal muscles abscess. Diagnosis is based on imaging evaluation. Plain radiographs of the lumbar spine are not helpful because often nomal. Bone scintigraphy is very sensitive but non-specific. CT scan can confirm the diagnosis and guide the needle biopsy. MRI is the preferred imaging modality for diagnosis. MRI shows early bone and joint involvement and it is helpful in detecting epidural and paravertebral soft tissue lesions.

Cervicofacial angioma and the Kasabach-Merritt syndrome.

We present a neonate with a cervicofacial haemangioma complicated by the Kasabach-Merritt syndrome, respiratory distress due to airway compression and high-output heart failure. This haemangioma and intravascular disseminated coagulation, treated initially by aspirin, ticlopidine and corticosteroids, required more invasive treatment with superselective embolisation and interferon alpha-2a. The clinical outcome was good.

Functional magnetic resonance imaging may avoid misdiagnosis of cochleovestibular nerve aplasia in congenital deafness.

OBJECTIVE: To investigate a narrow internal auditory canal (IAC) syndrome using functional magnetic resonance imaging (fMRI) of the auditory cortex. STUDY DESIGN: The study design was a case report. The follow-up period lasted 18 months. SETTING: The study was carried out in the audiology clinic of an ear, nose, and throat department and in the department of pediatric neuroradiology at a university hospital. MAIN OUTCOME MEASURES: Age-appropriate observational audiometry, objective audiovestibular tests, computed tomography (CT), magnetic resonance imaging (MRI), and (fMRI) of the auditory cortex were performed to analyze in detail the profound deafness of a young child. RESULTS: Audiovestibular examination demonstrated both measurable hearing and normal vestibulo-ocular reflex, and CT showed narrow IACs combined with normal labyrinths. Axial MR images completed by sagittal sections perpendicular to the IAC delineated a single nerve that was initially supposed to be the facial nerve. No cochleovestibular nerve was identified. However, fMRI performed with the patient under general anesthesia demonstrated activation of the primary auditory cortex during 1-kHz monaural stimulation on the left side. CONCLUSIONS: The absence of cochleovestibular nerve on MR studies cannot exclude connections between the inner ear and the central auditory pathways. This might be caused by a lack of spatial resolution of anatomical MR studies. The single nerve delineated within the IAC might also carry both facial and cochleovestibular fibers. Functional MRI can assess the cortical response to acoustic stimuli when aplasia of the cochleovestibular nerve is suspected. This case study illustrates a novel and atypical presentation of cochlear nerve dysplasia.

Clear-cell meningioma of the cauda equina.

Meningiomas are rare tumours in children and lumbar lesions are exceptional. We report a clear-cell meningioma (CCM) of the cauda equina in a 10-year-old girl. The tumour was diagnosed by MRI, showing an enhancing intradural mass extending from L1 to L4. Pathology and immunohistochemical study demonstrated a CCM. The patient had a recurrence 6 months after the operation requiring further surgery. CCM are rare lesions, characterised by abundant cytoplasmic glycogen particles. Complete surgical removal is necessary because, despite their benign histological appearance, CCM are potentially aggressive and may recur, spread locally and even metastasize.

[Clear cell meningioma: recurrent intraspinal tumor in a child]. / Méningiome à cellules claires: tumeur rachidienne récidivante chez un enfant.

BACKGROUND: Meningiomas represent 1.5% to 4.3% of cerebral and medullar primary tumors in children. CASE REPORT: A 9-year-old girl had a history of thoracolumbar scoliosis. An intracanalar and extramedullar tumor was confined to the lumbar region. Resection identified a clear cell meningioma. A symptomatic and tumoral recurrence occurred 5 months later in the same region. After a second resection, the patient received radiotherapy. At 8 months follow-up, no recurrence was documented. CONCLUSIONS: A stiff and painful scoliosis can be predictive of expansive intracanalar tumor. The recurrent or multifocal evolution of clear cell meningioma show the "aggressive behavior" of this histological type. A preventive radiotherapy could be proposed, depending on the age of the patient and the localization of the tumor.

Spinal cord abscess in a heroin addict: case report.

Spinal cord abscesses are extremely rare, even in intravenous drug abusers. They usually have a poor prognosis unless diagnosed and treated promptly. MRI is the best imaging modality for diagnosis and follow-up. We report a 42-year-old man, an active intravenous drug user, HIV negative, who developed subacute tetraplegia from an intramedullary abscess caused by Staphylococcus aureus. Immediate decompressive surgery and antibiotic treatment led to progressive recovery.

[Pituitary stalk transection syndrome]. / Syndrome d'interruption de la tige pituitaire.

BACKGROUND: Pituitary stalk transection is a non-negligible cause of growth hormone (GH) deficiency. POPULATION AND METHODS: We studied 22 children (13 boys, nine girls) aged at the first clinical manifestations from 2 days to 10 years (average = 5.33 +/- 2 years). Pituitary stalk transection was assessed by the means of magnetic resonance imaging (MRI). The children's past history showed fetal distress in 12 cases (54.5%), cranial trauma in three (13%) and a midline anomaly in three (13%). The first clinical manifestations were neonatal hypoglycemia (two cases), decreased growth velocity (18 cases) and diabetes insipidus (two cases). RESULTS: GH deficiency was complete, present from the onset in 19 of 22 cases and isolated in four. Fifteen of 22 cases had adreno-corticotrophic hormone (ACTH) and thyroid stimulating hormone (TSH) deficiency. Diabetes insipidus was present in six cases and revealed the syndrome in two. All children older than normal age of puberty (n = 10) had gonadotropin deficiency. In our study, these hormonal anomalies progressed from isolated GH deficiency to multiple hormonal deficiencies. CONCLUSION: The recently described stalk transection syndrome is relatively frequent and should be suspected after cranial trauma or fetal distress syndrome. The outcome is progressive evolution towards panhypopituitarism and these patients require regular clinical survey and hormonal controls.

[Acute orbital myositis and idiopathic inflammatory pseudotumor in children: three cases]. / Myosite orbitaire aiguë et pseudotumeur inflammatoire idiopathique de l'enfant: trois cas.

UNLABELLED: Orbital pseudo-tumors account for approximately 8% of the orbital tumors. They are scarcely described in children and raise diagnostic dilemas, especially when they present as an isolated extra-ocular muscle swelling called idiopathic orbital myositis (IOM). CASE REPORTS: A diagnosis of inflammatory orbital pseudotumors (IOPT) was made in three children aged 7, 13 and 14 years from clinical and CT scan and/or MRI findings associated with histological data in two of them. Two of these IOPT presented as IOM. Analysis was made in a effort to eliminate intra-orbital tumors, especially rhabdomyosarcoma. CONCLUSION: The abrupt onset of the orbital signs, often related in the literature, was not a specific diagnostic criterion regarding to embryonic rhabdomyosarcoma. The inconstant presence of inflammatory signs and the absence of local osseous defect could be more discriminating. Although diagnostic procedure, including histological documentation, was difficult, even dangerous, surgical biopsy should be recommended. Eventually, considering the mandatory multidisciplinary care, steroid therapy response should be evaluated as a diagnostic test.

[Pseudotumor forms of neurosarcoidosis in children. Diagnostic difficulties and therapeutic management]. / Formes pseudo-tumorales de la neurosarcoïdose chez l'enfant. Difficultés diagnostiques et prise en charge thérapeutique.

In systemic sarcoidosis, neurologic manifestations are possible and occurred in 5 p. 100 of cases. The diagnosis is easier when sarcoiddosis is known, but in half case neurologic symptoms are inaugural. In these cases, involvement of other sites (liver, lung, eyes or skin) by the disease is needed to confirm sarcoidosis. Main manifestations in neurosarcoidosis are cranial nerve palsy, diabetes insipidus or chronic aseptic meningitis, but sometimes sarcoidosis can present as an intracranial mass. The diagnosis of neurosarcoidosis may be difficult and could be confused with infection (such tuberculosis or mycosis), with inflammatory diseases (such multiple sclerosis) or with neoplasm, particularly in case of oedema. Brain biopsy may be necessary in isolated neurosarcoidosis if no peripheral histology evidence of noncaseating granulomas has been obtained. Treatment with steroids alone is the best choice for initial therapy but must be prolonged. It is difficult to predict accurately the response to steroids and relapse may be possible after a long time. Other immunosuppressive therapeutics or radiotherapy have been used in patient with refractory neurosarcoidosis. Sequential magnetic resonance imaging is the useful mean for the follow-up of neurosarcoidosis.