Intracranial primary synovial sarcoma mimicking a spontaneous cerebral hematoma-a case report and review of the literature.

BACKGROUND: Synovial sarcoma is a soft tissue sarcoma, of uncertain histological origin, usually located near large joints and concerning mainly young adults. Intracranial presentation in the form of metastasis from a primitive body sarcoma has been rarely reported. However, intracranial primitive synovial sarcoma (IPSS) is extremely rare and only a few cases have been reported in the literature. CASE DESCRIPTION: We present the case of a 48-year-old man, with no particular medical history, that was referred to our hospital for severe headache with a normal neurological exam and a CT cerebral scan showing a left frontal lobe hematoma. The initial cerebral CT scan didn't show any vascular malformation and the body CT scan was negative for a primitive lesion. A close follow-up with a cerebral MRI three months later, demonstrated a T1 enhanced lesion with an important volume progression. The patient underwent a complete surgical removal of this lesion and the first pathology diagnosis was compatible with a meningioma. After further proofreading by an expert and molecular analysis, the diagnosis of monophasic synovial sarcoma was confirmed. Nine months after the first surgery, the follow-up MRI showed the progressive recurrence of the lesion and in this context the patient underwent a second surgery with total resection of the tumor and frontal thin margin excision. Afterwards, the patient was treated with adjuvant radiotherapy, with a good clinical evolution, and till now the follow-up shows no recurrence. CONCLUSION: IPSS is an extremely rare sarcoma, with challenging diagnosis and difficult management. Specific molecular analysis is necessary. Complete resection followed by radiotherapy seem to be the most appropriate therapeutic approach. However, the prognosis is still poor. Our case is even rarer because of the initial presentation as a cerebral hematoma.

From Vesalius to tractography.

The description of an anatomical specimen may look straightforward, but it appears that it depends in fact on several intermingled factors: technical methods for conservation, dissection and vascular injection and the anatomist skills are of course important. This is especially true when the studied organ, as for instance the brain, is subject to rapid putrefaction after death without any preservation technique. Nevertheless the possibility to reject, or at least criticize, the dominant paradigm is probably as important as these technical considerations: important changes occurred in brain representation between the early Middle Ages and the Early Modern Times, without major improvements of cadaveric preservation or dissection methods; Vesalius rejected the existence of the rete mirabile in human not only because he was a talented anatomist but also because he accepted and had the courage to fight the dominant tradition inherited from Galen. Such difficulties in the scientific approach obviously remain vivid, and should not be forgotten despite the development of modern tools for studying brain morphology and function.

[Predictive factors for visual outcome after corneal collagen crosslinking treatment in progressive keratoconus: One-year refractive and topographic results]. / Facteurs prédictifs de bonne réponse au crosslinking d'un kératocône évolutif : résultats réfractifs et topographiques à un an postopératoire.

PURPOSE: To assess the effects of preoperative patient characteristics on clinical outcomes of corneal collagen crosslinking (CXL) in patients with progressive keratoconus. PATIENTS AND METHODS: Fifty-four eyes of 41 patients underwent CXL for progressive keratoconus between June 2011 and December 2012. Corneal topography (Orbscan(®)) was assessed at 1, 3, and 6 months and 1 year after CXL treatment and compared with preoperative data. RESULTS: A significant improvement in 1-year postoperative best-corrected visual acuity (BCVA) (0.16±0.21 LogMar preoperatively versus 0.09±0.16 LogMar postoperatively, P=0.007) and in 3mm topographic central irregular astigmatism (P=0.04) was demonstrated with CXL. No significant change was noted for refractive astigmatism (P=0.69), or for 1-year postoperative Kmax (48.4 D±4.1 at baseline versus 48.5 D±4.1 postoperatively, P=0.46). Predictive factors for BVCA improvement were low preoperative BCVA, high refractive astigmatism and advanced keratoconus. Predictive factors for stability of postoperative Kmax values were early keratoconus, and central cone ("nipple" morphology of the cone mainly located in the central 3mm of the cornea). CONCLUSION: This retrospective study confirms the efficacy of CXL for progressive keratoconus, from a refractive as well as topographic standpoint. While cone localization or its eccentricity seems to explain the variability of CXL efficacy reported in the literature, cone severity appears to be the main predictive factor for a lack of topographic stability after CXL treatment but must be weighted by the preferential localization of the cone (3 or 5mm central corneal zone).

Radionecrosis of malignant glioma and cerebral metastasis: a diagnostic challenge in MRI.

Detecting a new area of contrast-enhancement at MRI after irradiation of malignant brain tumor arises the problem of differential diagnosis between tumor recurrence and radiation necrosis induced by the treatment. The challenge for imaging is to distinguish the two diagnoses given: the prognostic and therapeutic issues. Various criteria have been proposed in the literature based on morphological, functional or metabolic MRI. The purpose of this study was to perform an analysis of these tools to identify MRI best criteria to differentiate radiation necrosis lesions from malignant gliomas and brain metastases recurrence. For gliomas, the morphology of the contrast-enhancement cannot guide the diagnosis and the use of perfusion techniques and spectroscopy (multivoxels if possible) are necessary. In the follow-up of metastasis, a transient increase and moderate lesion volume is possible with a good prognosis. Morphological characteristics (volume ratio T2/T1Gd) and perfusion analysis provide valuable tools for approaching the diagnosis of radionecrosis.

Surgical anatomy of the hippocampus.

BACKGROUND AND PURPOSE: Hippocampectomy is an efficient procedure for medial temporal lobe epilepsy. Nevertheless, hippocampus anatomy is complex, due to a deep location, and a complex structure. In this didactic paper, we propose a description of the hippocampus that should help neurosurgeons to feel at ease in this region. METHODS: Embryological data was obtained from the literature, whereas adult anatomy was described after dissecting 8 human hemispheres (with and without vascular injection) and slicing 3 additional ones. RESULTS: The hippocampus is C-shaped and made of 2 rolled-up laminae, the cornu Ammonis and the gyrus dentatus. Its ventricular aspect is covered by the choroid plexus of the inferior horn excepted at the head level. Its cisternal aspect faces the mesencephalon from which it is limited by the transverse fissure. Its rostral part (head) curves dorso-caudally to form the uncus, located at the medial aspect of the temporal lobe. Its caudal part (tail) splits into the fimbria and the gyrus fasciolaris that respectively run ventral and dorsal to the corpus callosum, to become the fornix and indusium griseum. CONCLUSION: Consequences of this complex anatomy are presented, and the authors stress the need for a subpial resection. Important landmarks are provided to avoid lesions of the surrounding structures.

Cerebral functional asymmetry and phonological performance in dyslexic adults.

Developmental dyslexia is a frequent language-based learning disorder characterized by difficulty in reading. The predominant etiologic view postulates that reading impairment is related to phonological and orthographic dysfunction. The aim of this fMRI study was to evaluate the neural bases of phonological processing impairment in remediated dyslexic adults (DD). We used a rhyming words judgment task contrasted with an unreadable fonts font-matching judgment task to compare patterns of activation and functional asymmetry in DD and normal-reading young adults. We found evidence of a link between asymmetry in inferior frontal gyrus and performance during the phonological processing. We also observed that DD recruit a network including regions involved in articulatory control in order to achieve rhyme judgment suggesting that, due to a lack of hemispheric specialization, DD recruit the latter network to achieve rhyme judgment.

[Evaluation of a pathway of epilepsy surgery in a polyvalent video-EEG center: retrospective analysis of a series of 295 patients]. / Évaluation d'une filière médico-chirurgicale en épileptologie au sein d'un centre de vidéo-EEG polyvalent : analyse rétrospective d'une série de 295 patients.

BACKGROUND AND PURPOSE: The supply for diagnosis and treatment is inadequate in France for epileptic patients eligible for surgery. Our institution includes a Video-electroencephalography (v-EEG) unit and a general neurosurgery department. Our objective was to evaluate the efficacy and morbidity of the surgery performed in a center non-dedicated to epilepsy surgery. METHODS: We conducted a retrospective study of a cohort of 295 patients explored with long lasting v-EEG from 1991 to 2008 in Tours in which we evaluated the efficacy and morbidity of the surgery distinguishing between hippocampal sclerosis and other epileptogenic lesions. RESULTS: Phase I directly led to surgery in 31 cases. Of them were 19 hippocampal sclerosis were operated with no surgical morbidity, and 12 other epileptogenic lesions operated with a comorbidity in two patients. Results of surgery were similar to larger studies, with 80% of patients Engel's Class I. Best results were observed in the hippocampal sclerosis group: 94.7 Class I (IA: 84%), versus 58% Class I (IA: 33%) for other etiologies. CONCLUSION: Despite the small number of operated patients, the surgical treatment keeps its efficiency without adding morbidity. v-EEG centers combined with non-specialists neurosurgical teams can contribute to improve care without reducing their quality.

Functional morphological imaging of autism spectrum disorders: current position and theories proposed.

Autism is a pervasive disorder of childhood development. Polymorphous clinical profiles combining various degrees of communication and social interaction with restricted and stereotyped behaviour are grouped under the heading of 'autism spectrum disorders' (ASD). Many teams are trying to pick out the underlying cerebral abnormalities in order to understand the neuronal networks involved in relationships with others. Here we review the morphological, spectroscopic and functional abnormalities in the amygdala-hippocampal circuit, the caudate nuclei, the cerebellum, and the frontotemporal regions, which have been described in subjects with ASD. White matter abnormalities have also been described in diffusion tensor imaging, leading to suspected damage to the subjacent neural networks, such as mirror neurones or the social brain.

Influence of age on the dynamics of fMRI activations during a semantic fluency task.

PURPOSE: Age-related fMRI changes have not been extensively studied for language, whereas important adaptive mechanisms have been seen in other cognitive fields. This study examined age-related changes in fMRI activation during language tasks and, in particular, their dynamic course. PATIENTS AND METHODS: fMRI was performed on 22 young and 21 old healthy right-handed subjects during a silent category word-generation task. Activation and dynamics of BOLD signals were studied separately during the first and second portions of each 30-s block. RESULTS: Activation of the left frontal lobe was initially similar in young and old participants; however, it decreased after 30 s in the old participants. On the other hand, additional areas were initially involved only in old subjects and especially in the default mode network. CONCLUSION: This study showed age-related differences in the dynamics of fMRI activation during a silent word-generation task, suggesting a different pattern of language function with aging.

Acquisition and interactive 3D exploration of the internal structure of a dissected specimen.

We present a system to keep track of a destructive process such as a medical specimen dissection, from data acquisition to interactive and immersive visualization, in order to build ground truth models. Acquisition is a two-step process, first involving a 3D laser scanner to get a 3D surface, and then a high resolution camera for capturing the texture. This acquisition process is repeated at each step of the dissection, depending on the expected accuracy and the specific objects to be studied. Thanks to fiducial markers, surfaces are registered on each others. Experts can then explore data using interaction hardware in an immersive 3D visualization. An interactive labeling tool is provided to the anatomist, in order to identify regions of interest on each acquired surface. 3D objects can then be reconstructed according to the selected surfaces. We aim to produce ground truths which for instance can be used to validate data acquired with MRI. The system is applied to the specific case of white fibers reconstruction in the human brain.

[Spasmodic torticollis caused by metoclopramide: a rare etiology of C1-C2 rotatory pseudoluxation in children]. / Un torticolis spasmodique lié à la prise de métoclopramide: une cause rare de pseudoluxation rotatoire C1-C2 chez l'enfant.

Torticollis in children must always instigate a search for trauma. Many other etiologies can be found. The authors report a case of postmedicinal atlantoaxial rotatory pseudoluxation (AARP) occurring in a child. This child had fallen down in the morning with head trauma followed by headache. Clinically, a stiff neck with an irreducible right rotation of his neck, and an osteotendinous hyperreflexia were noted. There was a C1-C2 rotatory dislocation with no traumatic lesion on the cervical CT scan. After a few hours, the torticollis spontaneously reduced and then reappeared on the left side. This clinical fluctuation and the absorption of metoclopramide (Primpéran) started in the morning for acute gastroenteritis provided the diagnosis of AARP. This entity was confirmed by the good clinical and radiological follow-up and was caused by the substantial ligament laxity of the craniovertebral junction encountered in children. The analysis of medical imaging and the systematic search for a medicinal cause helped make the right diagnosis.

The persistent left superior vena cava: anatomical study, pathogenesis and clinical considerations.

The persistence of a left superior vena cava (LSVC) is an intrinsically cardiac anomaly, which can lead to serious complications during catheterization via the subclavian or internal jugular vein. We found this anomaly during dissection associated with an abnormal origin of the vertebral artery originating from the aortic arch between the left common carotid and subclavian arteries. The LSVC coursed towards the right atrium through a very dilated coronary sinus ostium. No abnormality of the azygos system was found. A thorough anatomic description was then made with external and internal morphology. The embryonic development and variations are described. Radiological and clinical implications are discussed.

[Abnormal features of the sub renal portion of the inferior vena cava]. / Anomalies de la veine cave inférieure sous rénale.

AIM: to determine the frequency of the abnormal anatomical features affecting the inferior vena cava (IVC). MATERIAL AND METHODS: we performed 161 dissections of the ICV on fresh (145) or formaldehyde-injected (16) cadavers. There were 86 men and 75 women. RESULTS: we found two abnormalities of the IVC: one left IVC in a woman and one double IVC in a man. These malformations can be explained by the embryology of the IVC. DISCUSSION: the frequency of abnormalities of the IVC is highly variable according to the studies: Richardson (1983) found 3% for the duplication of the IVC. Other malformations have been reported: double IVC, left IVC or right IVC with azygos prolongation. We never observed such associations in our study. Left IVC could be explained by the development of the left supra-cardinal vein and by that of the left sub-cardinal and intercardinal anastomoses. Double IVC corresponds either to the development of the left supra-cardinal vein or to the persistence of the left cardinal vein or the left sub-cardinal vein. CONCLUSION: abnormalities of the ICV dysplay different anatomical features due to the complexity of the embryogenesis of this vessel; their frequency is far from being rare. Unknowing these abnormalities could lead to severe haemorrhages during surgical interventions on the retroperitoneal, cardiac or oesophageal regions.

[Duplication of the inferior vena cava: anatomical dissection and clinical implications]. / Dédoublement de la veine cave inférieure: dissection anatomique et applications cliniques.

The duplication of the inferior vena cava is often incidentally discovered during radiological studies. The presence of this anomaly can be mistaken for retroperitoneal adenopathy. Its knowledge allows to avoid hemorrhagic complications during retroperitoneal surgery. An anatomical dissection of a duplication of the inferior vena cava is presented. Embryogenesis and variations are described. Radiological and clinical implications are discussed.

Anatomical study of the length of the human intestine.

Although preoperative assessment of the length of the intestine may be of interest to avoid postoperative consequences of large intestinal resection, measurements of the intestine are quite rare and results variable in the literature. This anatomical study aimed to assess the length of the different intestinal segments, their variation and their correlation with sex, age, weight and height. Two hundred non-fixed adult cadavers (100 men, 100 women) who willingly gave their bodies for scientific purposes were studied. The post mortem average length of the whole intestine was 795.5+/-129 cm and was significantly longer in men and in young subjects. It was correlated with the subject's weight but not height. Multivariate analysis demonstrated that the factor showing the strongest correlation with intestinal length was body weight. This latter parameter may be useful in the preoperative assessment of intestinal length.

[Anatomical variations of the brachial plexus]. / Variations anatomiques du plexus brachial.

Important variations exist in the brachial plexus (Kaufmann, Cunningham, Kerr, Lee). Perivascular techniques of brachial plexus block used sometimes use important quantities of anaesthesia product. It seems that certain surgical treatment failures of brachial plexus lesions are related to the brachial plexus variations (Bonnel). Our aim is to study the brachial plexus variations and its relations from the cervical region to brachial region. We dissected twenty-three brachial plexus (13 women and 10 men, fresh or injected by formalin). We studied the brachial plexus anatomic variations and its relations. We found the anatomic variations at the level of different parts of brachial plexus: Trunk: 8 cases (34.8%); Terminal branch: 8 cases (34.8%); brachial plexus tract: 1 case (4.3%); collateral branch: 1 case (4.3%); in 5 cases we didn't find any abnormalities. Some studies show the frequency of 4th cervical root (C4) participation in brachial plexus. Kerr found 65.9%. It was 30.4% of C4 participation in brachial plexus in our series. Among the 8 cases of terminal branch variations in our series, two (8.7%) musculocutaneous was low source. The brachial plexus variations could fail the brachial plexus loco-regional anaesthesia. In the surgical treatment of brachial plexus lesions, the surgeon must know brachial plexus anatomical variations perfectly.

[Role of high-dose chemotherapy with hemopoietic stem-cell support in the treatment of adult patients with high-grade glioma]. / Place de la chimiothérapie intensive avec autogreffe de cellules souches hématopoïétiques dans le traitement des tumeurs gliales de haut grade chez l'adulte.

Despite surgery, post-operative irradiation and adjuvant conventional chemotherapy, prognosis of high-grade gliomas remains poor. Carmustine (BCNU) has been shown to have limited activity at conventional dosage but is still the standard chemotherapy. Activity of chemotherapy is limited by the blood-brain barrier impermeability and high levels of expression of multidrug resistance proteins on tumor and/or endothelial cells. Despite high response rates, development of intra-arterial chemotherapy remains limited because of frequent acute brain toxicity related to drug administration. High-dose intravenous chemotherapy rescued by autologous hemopoietic stem cell transplantation is an alternative that might increase drug delivery through the blood-brain barrier and tumor control. Several phase I-II trials using high-dose BCNU were published. The maximum tolerated dose seems to be 800 mg/m2 and interstitial pneumonitis and hepatitis are dose-limiting toxicities. Few phase I-II trials of high-dose therapy were published using drug combinations. High response rates in patients with progressive tumor were observed and in adjuvant setting, encouraging results in terms of median survival time and long survivors were published. No phase III trial was reported to date. Future investigations should include randomized trials comparing high-dose and conventional-dose chemotherapy and development of new high-dose regimens that incorporate new drugs such as temozolomide.