Efficacy of high dose steroid therapy in children with severe acute transverse myelitis.

No effective treatment has been demonstrated for patients with acute transverse myelopathy. In a multicentre controlled study, 12 children with severe acute transverse myelopathy were treated with intravenous methylprednisolone (IVMP) and compared with a historical group of 17 patients. The treatment had a significant effect on the proportion of patients walking independently at 1 month and on the proportion with full recovery at 1 year, with no differences in the frequency of complications between the two groups.

Multimodality evoked potentials as a prognostic tool in term asphyxiated newborns.

Hypoxic-ischemic (HI) events may cause permanent brain damage, and it is difficult to predict the long-term neurological outcome of survivors. Multimodality evoked potentials (MEPs), using flash visual (fVEPs), somatosensory (SEPs), and brain-stem auditory evoked potentials (BAEPs) may assess the cerebral function in term neonates. MEPs were recorded in 40 hypoxic-ischemic term or near-term neonates during the first week of life in order to predict the neurological outcome. A 3 point grading system registered either mild, moderate, or severe abnormalities. At 24 months of corrected age, the infants were assessed with a blind protocol to determine neurological development. Grade 0 fVEPs and SEPs were associated with a normal neurological status with 100% (P < 0.001) of the infants. Abnormal SEPs or total grade (VEPs + SEPs) > I were not associated with normal outcomes (P < 0.0001). Normal BAEPs did not predict a normal outcome, but severely abnormal BAEPs did predict an abnormal outcome. A significant correlation was found between EP (VEPs + SEPs) grade (r = 0.9, P < 0.0001), Sarnat stage (r = 0.6, P < 0.001), and clinical outcome. This study confirmed that both fVEPs and SEPs are more accurate as prognostic indicators for term neonates. EPs (VEPs + SEPs) also are more accurate in predicting the ultimate neurological outcome compared with the Sarnat scoring.

Perisylvian dysgenesis. Clinical, EEG, MRI and glucose metabolism features in 10 patients.

We studied 10 patients who had neurological disorders with a MRI-based diagnosis of perisylvian dysgenesis based on the fact that the parasagittal and centrifugal extremity of the sylvian fissure was abnormally mesial. This abnormality was bilateral in seven cases; in the other three patients, the contralateral sylvian fissure appeared either normal (two cases) or enlarged (open operculum). The perisylvian cortex had a polymicrogyric appearance in most patients. Potential aetiopathogenic factors were determined in four patients. In two of them, ischaemia at mid-gestation was ascribed to death of a co-twin in a context of monozygotic twinning. In the other two patients, who were siblings, genetic factors were suspected. Pseudobulbar palsy was found in eight patients and epilepsy in five patients. We used PET with [18F]fluorodeoxyglucose to test the hypothesis that, despite this clinical and MRI heterogeneity, regional cerebral glucose distribution could have common features in these patients. The analysis of PET data was performed by visual inspection in two cases and by using statistical parametric mapping (SPM) in eight patients compared with a control group. Segmented grey matter MRIs of seven out these patients were also analysed using SPM. We found that the abnormal perisylvian cortex had normal grey matter activity in eight patients and in the other two there was a heterogeneous pattern with areas of preserved metabolism and of decreased metabolism. Metabolic changes were also detected outside the polymicrogyric-like cortex; three patients had hypometabolic areas in cortical regions where the MRI appeared normal and had a normal intensity. When polymicrogyria extended into the white matter, this ectopic dysgenetic cortex was associated with a grey matter pattern within the white matter territory, and was detected by SPM as areas of PET hypermetabolism and MRI hyperintensity. In order to detect possible metabolic changes undetected by the individual analyses, the group of patients was compared with the control group. This comparison revealed bilateral hypometabolism in the frontal opercular cortex. We propose that these PET data be considered in light of the presumed cyto-architectonic pattern of perisylvian dysgenesis, i.e. polymicrogyria. In this malformation, two dense cell layers are separated by a necrotic sparse cell layer. We speculate that the amount of synaptic activity preserved in these dense cell layers depends on the importance and timing of the necrotic process; this hypothesis accounts for the large range of metabolic patterns found, from profoundly decreased glucose metabolism to nearly normal activity.

Brain functional imaging SPECT in agyria-pachygyria.

Agyria-pachygyria or lissencephaly type I, a diffuse cortical malformation, provides infantile spasms (IS) which are refractory and persisting after the first decade, an age at which IS have disappeared in the other causes. In order to study the functional postnatal development of the lissencephalic cortex, we measured regional cerebral blood flow (rCBF) using SPECT (Single photon emission computed tomography) and 133Xe in 14 children with lissencephaly, aged from 4 months to 12 years (mean = 40 months) compared to normal children of the same age range and to children with cryptogenic IS aged from 3 months to 3 years (mean = 13 months). rCBF was calculated in frontal (FR) and parieto-temporo-occipital (PTO) cortex as well as the ratio FR/PTO. FR/PTO was higher in lissencephalic patients than in controls (P < 0.001) due to higher FR rCBF (P < 0.001), particularly in patients aged less than 3 years. FR/PTO was also higher in lissencephalic patients than in patients with cryptogenic IS (P < 0.001) also due to higher FR rCBF (P < 0.001). The values of FR/PTO and FR rCBF remained stable during the first years of life and did not exhibit any age- or topography-related changes as they do in controls or in patients with cryptogenic IS. There results suggest that the normal process of postnatal development in the brain is lacking in agyria-pachygyria. That could play a role in determining the persistence of epileptic spasms, the specific seizure type of this malformation.

Functional brain imaging in HIV-1-infected children born to seropositive mothers.

UNLABELLED: The aim of this work was to study cerebral function in vertically infected children with human immunodeficiency virus 1 (HIV-1). METHODS: PET with 18F-labeled fluorodeoxyglucose (FDG) was performed in eight children (2.5-5.5 yr): three with severe neurological symptoms and five without. Quantitative analysis was based on gray matter cortical and subcortical regions of interest for which glucose utilization was measured. RESULTS: Diffuse hypometabolism and subcortical hypermetabolism were found in the three children with severe neurological signs; the five other children had temporo-occipital cortical hypometabolism, mainly on the right side. CONCLUSION: Functional cerebral abnormalities seem to precede clinical symptoms in HIV-1infection of the brain in children.

SPECT of the brain in childhood autism: evidence for a lack of normal hemispheric asymmetry.

Autism is thought to be associated with abnormal hemispheric specialization and left-hemispheric dysfunction. Brain functional imaging using 133Xe-SPECT (single photon emission computed tomography) was used to measure left/right asymmetry and absolute values of regional cerebral blood flow (rCBF) in 18 children with autism aged from four to 17 years and 10 age-matched controls. All controls but only 10 children with autism were right-handed. The left-to-right indices, both hemispheric and regional, were positive in controls, indicating higher left than right rCBF values, but were negative in patients with autism. This inversion was statically significant for total hemispheres, sensorimotor and language-related cortex and was explained by a significant decrease of the left absolute rCBF values in these regions in the patients with autism. The inversion was independent of handedness, sex and age. These results confirm the existence of left-hemispheric dysfunction in childhood autism, especially in the cortical areas devoted to language and handedness, leading to anomalous hemispheric specialization.

Hyperinsulinism in children: diagnostic value of pancreatic venous sampling correlated with clinical, pathological and surgical outcome in 25 cases.

Neonatal hypoglycemia represents an emergency of heterogeneous etiology. The occurrence of persistent hypoglycemia caused by hyperinsulinism has not been well established. Some authors claim that it may be more common than previously suggested. The diagnostic goal is to distinguish hyperinsulinemia from other causes of hypoglycemia because management strategies differ. The diagnosis of persistent hypoglycemia attributable to hyperinsulinism is made when insulin secretion is excessive or inappropriate (> 10 microIU/ml). Medical management includes frequent feeding, high hydrocarbon intake, glucagon, diazoxide, somatostatin or steroid treatment. In case of resistance to medical intervention, surgery consisting of subtotal pancreatectomy is performed to avoid neurological sequelae. However, pediatric organic hypoglycemia secondary to hyperinsulinism can be caused by either diffuse or focal pancreatic lesions. Differentiation between these two types of lesion is necessary since partial pancreatectomy can prevent diabetes. In this prospective study, pancreatic venous sampling (PVS) was evaluated for the preoperative localization of lesions in 25 children with hyperinsulinism and correlated with surgical, pathological and clinical outcome. PVS is the most accurate preoperative technique for localizing focal lesions in children. Besides being safe and effective, it has the great advantage of detecting focal secretion, thus reducing the need for extensive surgery.

Study of regional cerebral blood flow in West syndrome.

Focal cortical disturbances are frequent sequelae in West syndrome (WS) even though it is a generalized epileptic syndrome. Functional neuroimaging was used to determine whether focal perfusion abnormalities exist at WS onset and change during evolution. We studied regional cerebral blood flow (rCBF) at different stages of WS. Mean CBF (mCBF) and rCBF were measured using SPECT (single photon emission computed tomography) and 133Xe in 13 WS patients: at onset (20 cases), just after steroids (17 cases), and after a mean follow-up of 2 years (26 cases). At WS onset, interictal mCBF was increased as the result of foci of hyper- and hypoperfusion, which were, respectively, mainly located in the frontal and posterior cortex. Just after steroid therapy, mCBF decreased without any focal predominance. During follow-up, hypoperfused foci remained unchanged whereas the frontal hyperperfused foci decreased after spasm control. Our results show that focal abnormalities are present at WS onset. Focal hypoactivity could reflect a cortical lesion responsible for WS and focal hyperactivity could play a role in the persistence of generalized epilepsy.

Clinical aspects of mitochondrial disorders.

Mitochondrial disorders have long been regarded as neuromuscular diseases only. In fact, owing to the ubiquitous nature of the oxidative phosphorylation, a broad spectrum of clinical features should be expected in mitochondrial disorders. Here, we present eight puzzling observations which give support to the view that a disorder of oxidative phosphorylation can give rise to any symptom in any organ or tissue with any apparent mode of inheritance. Consequently, we suggest giving consideration to the diagnosis of a mitochondrial disorder when dealing with an unexplained association of symptoms, with an early onset and a rapidly progressive course involving seemingly unrelated organs. Determination of lactate/pyruvate and ketone body molar ratios in plasma can help to select patients at risk for this condition.

[Tethered cord syndrome, conservative or surgical approach?]. / Syndrome du filum terminal, approche conservatrice ou chirurgicale?

The authors gave a definition of a filum terminale and discussed the symptomatology, the etiology and the pathophysiology. They stressed the importance of carrying out a full neurophysiological, orthopaedic, anal and urogenital examination. This is the only way of establishing an indication for surgical treatment that should be carried out early in order to minimize acute accidents.