Transient neonatal myasthenia gravis revealing a myasthenia gravis and a systemic lupus erythematosus in the mother: case report and review of the literature.

Transient neonatal myasthenia gravis (TNMG) and neonatal lupus are rare conditions due to the transplacental passage of antibodies. We describe a unique case of TNMG, revealing a myasthenia gravis (MG) associated with systemic lupus erythematosus (SLE) in the mother. J. M., 8 days of age, was admitted for jaundice. Examination revealed poor sucking, facial weakness, and hypotonicity. TNMG was confirmed with a high level of antiacetylcholine receptor antibodies in the infant and his mother. No sign of neonatal lupus was observed. Clinical recovery was obtained. The elder brother had autism. In case of previous maternal MG, a low percentage of infants develop TNMG (10 to 20%), but monitoring is required at birth. Improvement is usually obtained within 3 weeks. No correlation has been found between maternal symptoms, antibodies titer, and signs of TNMG. Most cases of neonatal lupus are associated with positive anti-SSA/SSB antibodies in the mother. Both conditions, MG and SLE, are reported, but pregnancies are very few. Autism in the brother focuses on its relationship with immune diseases.

Neurological and functional recovery in human stroke are associated with peripheral blood CD34+ cell mobilization.

BACKGROUND: A spontaneous mobilization of Peripheral Blood-Mononuclear CD34+ Cells (PB-MNC-CD34+) has recently been reported in human myocardial infarction and found to be related to improved heart function and survival. However, nothing is known regarding a possible relation between PB-MNC-CD34+ mobilization and neurological recovery in human acute cerebral ischemia. METHODS AND RESULTS: PB-MNC-CD34+ were determined daily after an acute cerebral ischemic attack for 14 days in 25 patients with acute ischemic stroke and compared with controls. Results indicated that stroke was followed by large and bursting mobilizations of PB-MNC-CD34+. The amplitude of the mobilizations was similar to those observed in Granulocyte Colony Stimulating Factor (G-CSF) conditioned aplastic patients following myeloablative therapy before leukapheresis and autologous bone graft. The extent of PB-MNC-CD34+ mobilization in each patient was directly related to neurological and functional recoveries as assessed by NIH Stroke Scale, and modified Rankin Scale respectively. CONCLUSIONS: The mobilization of PB-MNC-CD34+ cells might be predictive of neurological and functional recovery.

[Ependymal variant of dysembryoplastic neuro-epithelial tumor]. / Tumeur neuro-épithéliale dysembryoplasique à différenciation épendymaire.

Dysembryoplastic neuro-epithelial tumors (DNET) are glioneuronal tumors with a common astrocytic or oligodendroglial differentiation. We report a case of DNET with an ependymal differentiation. A 13 years-old girl had refractory focal seizures for 3 years. Successive MRI showed a stable cortical lesion of the right temporal lobe with DNET characteristics. The lesion was resected. Histological examination revealed a pure ependymal differentiation characterized by perivascular pseudorosettes containing EMA immunoreactive cells. Mib1 positive cells were absent. In spite of histologic features of ependymoma, this tumor showed clinical, radiological and behaviour characteristics of DNET. We are not aware of any other case of DNET with ependymal differentiation in the literature. Our observation suggests that the glial component of DNET may show an ependymal differentiation.

Voxel-based mapping of cortical ischemic damage using Tc 99m L,L-ethyl cysteinate dimer SPECT in acute stroke.

BACKGROUND AND PURPOSE: When performed soon after stroke onset, single-photon emission computed tomography (SPFCT) with hexamethylpropylenamine oxime or Tc 99m L,L-ethyl cysteinate dimer (ECD) has significant added predictive value compared to neurological scores. With ECD SPECT, the degree of tracer uptake reduction predicts neurological recovery, and using a 40% threshold to characterize irreversibly damaged tissue (IDT), significant correlations have been observed. However, correlations between this uptake threshold and tissue outcomes have not been assessed. The purpose of this study was to validate the 40% ECD uptake threshold for the probabilistic mapping of IDT using an automatic, voxel-based approach. METHODS: In 10 acute stroke patients, the authors first compared early ECD SPECT and late coregistered magnetic resonance imaging (MRI) data and assessed for IDT and "tissue at risk" (i.e., tissue with ECD uptake below and above 40%) the percentage of voxels ultimately infarcted and noninfarcted on late brain MRI. They then assessed the correlations between the volumes of brain tissue compartments and subsequent neurological recovery. Finally, to assess whether visual SPECT analysis is reliable compared to the more complex voxel-based approach, the authors compared the predictive value of the 2 methods for neurological recovery. RESULTS: The majority of IDT voxels (average = 84%), defined by ECD uptake < 40%, evolved toward infarction, and 51.8% to 100% of at-risk voxels (average = 89%) escaped infarction. The extent of IDT correlated significantly with neurological recovery (P = .0009). There was good agreement between visual and voxel-based analyses (P = .0004). CONCLUSION: The results support the validity of the ECD uptake thresholds chosen, suggesting that ECD uptake can reflect neuronal viability and that ECD SPECT can be useful for the early detection of potentially salvageable tissue and irreversible damage. These preliminary results encourage the use of this method in a clinical setting for fast decision making in choosing acute therapy.

[Standards, Options and Recommendations 2002 for the management of adult patients with intracranial gliomas (summary report)]. / Standards, options et recommandations 2002 pour la prise en charge des patients atteints de gliomes intracrâniens de l'adulte (rapport abrégé).

CONTEXT: The "Standards, Options and Recommendations" (SOR) project, which started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French Regional Cancer Centers, and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and the outcome of cancer patients. OBJECTIVE: To define clinical practice guidelines for the management of adult patients with intracranial glioma in collaboration with the Association of French-speaking Neuro-oncologists (Anocef) and the French society of neurosurgeons (SNCLF). These recommendations cover diagnosis, classification, treatment and follow-up of patients with these tumors. METHOD: The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines has been defined, the document is submitted for review by independent reviewers. RESULTS: This article is a summary version of the full document presenting the clinical practice guidelines with algorithms. The main recommendations concern the place of the surgery, radiotherapy, chemiotherapy, imagery and concomitant medical treatments in the specific treatment strategy of grade III and IV glioma, grade II glioma, gliomatosis cerebri, pilocytic astrocytoma, subependymoma, xanthoastrocytoma, intracranial ependymoma and brain stem glioma.