Pure endoscopic management of epileptogenic hypothalamic hamartomas.

Hypothalamic hamartomas (HH) are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Their usual clinical presentation is characterized by gelastic/dacrystic seizures which often become pharmaco-resistant and progress to secondary focal/generalized intractable epilepsy causing mostly in children cognitive and behavioral problems (particularly in cases of progressive epileptic encephalopathy) and precocious puberty. Whereas gelastic seizures can be surgically controlled either by resection of the lesion or disconnection (tissue-destructive) procedures, aimed at functionally prevent the spreading of the epileptic burst; generalized seizures tend to respond better to HH excision rather than isolated neocortical resections, which generally fail to control them. Prospective analysis of 14 consecutive patients harboring HH treated in an 8-year period; 12 patients had unilateral and two bilateral HH. All patients were managed by pure endoscopic excision of the HH. The mean operative time was 48 min and mean hospital stay was 2 days; perioperative blood loss was negligible in all cases. Two patients showed a transient diabetes insipidus (DI); no transient or permanent postoperative neurological deficit or memory impairment was recorded. Complete HH excision was achieved in 10/14 patients. At a mean follow-up of 48 months, no wound infection, meningitis, postoperative hydrocephalus, and/or mortality were recorded in this series of patients. Eight patients became seizure free (Engel class I), 2 other experienced worthwhile improvement of disabling seizures (Engel class II); 2 patients were cured from gelastic attacks while still experiencing focal dyscognitive seizures; and 2, having bilateral HH (both undergoing unilateral HH excision), did not experience significant improvement and required later on a temporal lobectomy coupled to amygdalohyppocampectomy. Overall, the followings resulted to be predictive factors for better outcomes in terms of seizure control: (1) cases of unilateral, Delalande class B, HH, (2) shorter history of epilepsy. Endoscopic resection of HH proved, in our series, to be effective in achieving complete control or in reducing the frequency of seizures. Furthermore, this approach has confirmed its minimally invasive nature with a very low morbidity rate: of note, it allowed to better preserve short-term memory and hypothalamic function.

[Feedback on the evaluation of clinical pharmacy activities developed in surgery]. / Retour d'expérience sur l'évaluation des activités de pharmacie clinique développées en chirurgie.

INTRODUCTION: Our current development strategy integrates clinical pharmacy activities prioritized in surgical services. Patients in these services are typically risk patients: transfers, multiple prescribers, frequent medication change, pharmacotherapeutic risk classes. PATIENTS AND METHODS: Three clinical pharmacy activities (admission reconciliation, pharmaceutical analysis, participation doctors round) have been developed in orthopaedic surgery and neurosurgery. Pharmacists prospectively recorded data describing their activities: number of reconciliations and analyzed requirements and time required to achieve them. Data on pharmaceutical interventions were recorded on the basis ActIP®. The clinical significance of interventions was retrospectively rated by a team of two pharmacists and two physicians on the scale adapted Hatoum et al. RESULTS: Four thousand five hundred pharmaceutical analysis and 248 reconciliations were conducted. One hundred and fifty-six pharmaceutical interventions were issued. The average acceptance rate was 80%. A total of 5.8% of pharmaceutical interventions have been listed with a very significant clinical importance and 48.1% with at least significant clinical importance. The activities and documentation required pharmaceutical average daily time (senior pharmacist, resident and external pharmacist) about 6 hours. DISCUSSION AND CONCLUSION: Other studies, including comparative and medico-economic, must be conducted to support these results. Nevertheless, the indicators obtained attend a better readability of the clinical importance of the activities performed by clinical pharmacists and this particularly in surgical services, both by prescribers and authorities.

Anterior trans-frontal endoscopic management of colloid cyst: an effective, safe, and elegant way of treatment. Case series and technical note from a multicenter prospective study.

Different management options are available for the treatment of colloid cysts. Goals of those procedures are to achieve a complete resection avoiding potential long-term recurrence along with CSF pathways restoration with minimal morbidity and mortality. The two main surgical options are endoscopic resection or direct removal by either transfrontal or transcallosal approach. The efficacy of endoscopic technique to achieve gross total colloid cyst excision has been well documented. In the present study, authors describe a series of 29 patients who underwent surgery by a variation of the standard worldwide implemented endoscopic technique. Using a more anterior approach, it is easier to reach the roof of the cyst, its possible adherences with the tela choroidea, plexus, and the internal cerebral veins. The described approach has shown to be safe, quick, and very effective with a total cyst removal rate of 86.2%.

[Clinical pharmacy and surgery: Review]. / Pharmacie clinique et chirurgie : revue de la littérature.

Clinical pharmacy has been developed and evaluated in various medical hospital activities. Reviews conducted in this area reported a higher value of this discipline. In surgical services, evenly adverse drug events may occur, so clinical pharmacy activities must also help to optimize the management of drug's patient. The objectives of this literature review is to determine the profile of clinical pharmacy activities developed in surgical services and identify indicators. The research was conducted on Pubmed(®) database with the following keywords (2000-2013), "surgery", "pharmacy", "pharmacist", "pharmaceutical care", "impact" and limited to French or English papers. Studies dealing on simultaneously medical and surgical areas were excluded. Twenty-one papers were selected. The most frequently developed clinical pharmacy activities were history and therapeutic drug monitoring (antibiotics or anticoagulants). Two types of indicators were identified: activity indicators with the number of pharmaceutical interventions, their description and clinical signification, the acceptance rate and workload. Impact indicators were mostly clinical and economic impacts. The development of clinical pharmacy related to surgical patients is documented and appears to have, as for medical patients, a clinical and economical value.

Security and reliability of CUSTOMBONE cranioplasties: A prospective multicentric study.

BACKGROUND: Repairing bone defects generated by craniectomy is a major therapeutic challenge in terms of bone consolidation as well as functional and cognitive recovery. Furthermore, these surgical procedures are often grafted with complications such as infections, breaches, displacements and rejections leading to failure and thus explantation of the prosthesis. OBJECTIVE: To evaluate cumulative explantation and infection rates following the implantation of a tailored cranioplasty CUSTOMBONE prosthesis made of porous hydroxyapatite. One hundred and ten consecutive patients requiring cranial reconstruction for a bone defect were prospectively included in a multicenter study constituted of 21 centres between December 2012 and July 2014. Follow-up lasted 2 years. RESULTS: Mean age of patients included in the study was 42±15 years old (y.o), composed mainly by men (57.27%). Explantations of the CUSTOMBONE prosthesis were performed in 13/110 (11.8%) patients, significantly due to infections: 9/13 (69.2%) (p<0.0001), with 2 (15.4%) implant fracture, 1 (7.7%) skin defect and 1 (7.7%) following the mobilization of the implant. Cumulative explantation rates were successively 4.6% (SD 2.0), 7.4% (SD 2.5), 9.4% (SD 2.8) and 11.8% (SD 2.9%) at 2, 6, 12 and 24 months. Infections were identified in 16/110 (14.5%): 8/16 (50%) superficial and 8/16 (50%) deep. None of the following elements, whether demographic characteristics, indications, size, location of the implant, redo surgery, co-morbidities or medical history, were statistically identified as risk factors for prosthesis explantation or infection. CONCLUSION: Our study provides relevant clinical evidence on the performance and safety of CUSTOMBONE prosthesis in cranial procedures. Complications that are difficulty incompressible mainly occur during the first 6 months, but can appear at a later stage (>1 year). Thus assiduous, regular and long-term surveillances are necessary.

[Corticectomy: technical considerations]. / Cortectomies: aspects techniques.

The surgical treatment of epilepsy requires careful preparation and presents a certain number of technical specificities. The neurosurgeon must master not only the technical aspects but also the therapeutic and functional trade-off in order to modulate the procedure according to morphological and electrophysiological intraoperative data. A large number of technical variants have been developed to correspond to epileptological or functional anatomical considerations. Until this point, the choice of a particular technique does not seem to have a significant impact on the therapeutic effectiveness of surgery, and differences in results can be related to the presurgical evaluation and surgical indications. On the other hand, technical development promises to play an important role in limiting the long-term neurocognitive consequences of surgery.

Effects of reduced rates of two insecticides on enzyme activity and mortality of an aphid and its lacewing predator.

By applying insecticides at lower rates of active ingredients per unit area, survival rates of the pests' natural enemies can be enhanced, whereas pest mortality can remain high. The effects of reduced application rates of the insecticides lambda-cyhalothrin and dimethoate on the mortality of bird cherry-oat aphid, Rhopalosiphon padi (L.), and lacewing Micromus tasmaniae Walker were determined in the laboratory and field. Cholinesterase (ChE) and glutathione S-transferase (GST) activities in survivors provided a measure of sublethal effects and general fitness. In the laboratory, lacewings were less sensitive than aphids to both insecticides, and dimethoate was more toxic than lambda-cyhalothrin. However, these results could not be recreated in the field, in part due to very low recapture rates. In summary, lambda-cyhalothrin seemed to have no effect on aphids, but it was toxic to lacewings. Dimethoate was far less toxic in the field, but aphids were still more sensitive than were lacewings. Cholinesterase activity was reduced by dimethoate exposure in the laboratory in both species, but there were species-specific differences. Dimethoate and lambda-cyhalothrin had no effects on GST activity in either species. The high mortality rate for lacewings and aphids exposed to dimethoate in the field suggests that the application rate could be reduced to as low as 10% of that recommended by manufacturers, and this should still be highly efficacious against aphids, while protecting the predatory lacewing. Measurement of enzyme activity could provide a useful indicator of "fitness" of survivors.

[Epileptogenic and non-epileptogenic zones: blood flow studies of temporo-limbic seizures]. / Zones épileptogènes et non-épileptogènes: imagerie de perfusion des crises temporo-limbiques.

To assess the contribution of ictal SPECT to the definition of the epileptogenic zone (EZ) prior to surgery in focal drug-resistant epilepsies, we investigated the effect of the timing of injection and seizure semiology on patterns of perfusion and cerebral blood flow changes (CBF) beyond the EZ. In the rat model of amygdala-kindled seizures, we measured CBF changes with the quantitative [(14)C]-iodoantipyrine autoradiographic method during secondary generalized (SGS, n=26 fully-kindled rats) and focal seizures (FS, n=19 partially kindled rats), according to sequential timing of injection with respect to seizure onset. During SGS, the correct lateralization and rough localization of the focus within limbic structures was only possible at the early ictal and post-ictal times, in between we observed widespread rCBF increases. The switch from hyper to hypoperfusion occurred at the time of late ictal injection. The accurate localization of the EZ was obtained in the study of the more subtle FS (stage 0). At stage 1 of the kindling, there was already a remote widespread spreading of hyperperfusion. In patients surgically cured from a mesio-temporal lobe epilepsy (mean post-operative follow-up: 66 months), we retrospectively studied 26 pairs of ictal and interictal pre-operative SPECTs, classified in 3 groups according to the progression of ictal semiology. Using visual analysis of subtracted SPECTs (SISCOM) and group comparisons with a control group (using SPM), we observed more widespread combined hyper and hypoperfusion with the increasing complexity of seizures. In simple partial seizures, the SISCOM analysis allowed a correct localization of the focus in 4/8 patients, whereas the SPM analysis failed to detect significant changes, due to individual variation, spatial normalization and small magnitude of CBF changes. In complex partial seizures with automatisms, SISCOM and SPM analysis showed antero-mesial temporal hyperperfusion (overlapping the EZ), extending to the insula, basal ganglia, and thalamus in the group of patients having dystonic posturing (DP group) in addition to automatisms. Ictal hypoperfusion involved pre-frontal and parietal regions, the anterior and posterior cingulate gyri, to a greater extent in the DP group. In both human and animals studies, we observed a correlation between the extent of composite patterns of hyper/hypoperfusion and the severity of seizures, and the recruitment of remote sub-cortical structures. Hypoperfused areas belong to neural networks involved in perceptual decision making and motor planning, whose transient disruption could support purposeless actions, i.e. motor automatisms.

Efficacy and safety in frameless robot-assisted stereo-electroencephalography (SEEG) for drug-resistant epilepsy.

INTRODUCTION: Stereo-electroencephalography (SEEG) is an invasive procedure, used to identify the epileptogenic zone that can be surgically removed in order to treat drug-resistant epilepsy. Frameless robot-assisted positioning of depth electrodes permits a 3D approach with different obliquities and trajectories. The objective of the present study was to evaluate the morbidity and the accuracy related to this frameless procedure. PATIENTS AND METHODS: Sixty-six patients were managed wherein 901 electrodes were implanted during a 6-year-period. All patients had a postoperative CT-scan that was fused with preoperative MRI planning. In order to assess the accuracy of the procedure, the Euclidian distance was calculated between the coordinates of the planned trajectory and the actual position of the electrode at the entry point and at the target point for 857 electrodes. RESULTS: Among the 66 patients, one (1.5%) experienced a symptomatic brain haematoma and one (1.5%) a stroke-like migraine after radiation therapy (SMART) syndrome. There was no permanent morbidity or mortality. Compared to the classical SEEG approach, a higher rate of asymptomatic postoperative bleeding was found on the CT-scan in 8 patients (12.1%). Any infectious events were recorded. The median accuracy of frameless robotic SEEG procedure was equivalent to a 1.1mm error deviation (0.15-2.48) at the entry point and 2.09mm (1.06-3.72) at the target point respectively, with no differences for double obliquity trajectories. CONCLUSION: Frameless robot-assisted SEEG appears to be a safe procedure, providing sufficient accuracy in order to delineate the epileptogenic zone and represents a helpful tool in the pre-surgical management of refractory epilepsy.

[Selective memory impairment for public events associated with accelerated forgetting in a patient with left temporal lobe epilepsy]. / Déficit sélectif de la mémoire des faits publics associé à un oubli accéléré chez un patient atteint d'épilepsie du lobe temporal gauche.

BACKGROUND: Memory for the past has been much less explored than anterograde memory in temporal lobe epilepsy. No data are available about the course after surgical treatment. Moreover, case-reports showing isolated memory deficit for publics events with preserved autobiographical memory are notorious for their paucity. METHODS: We report the case of a patient with a left hippocampal lesion associated with intractable temporal lobe epilepsy. Comprehensive neuropsychological assessments were carried out before and after temporal lobectomy, stressing particularly memory for the past by means of standardised and tailor-made tests. RESULTS: The patient exhibited preserved memory for autobiographical events and facts but impaired scores on several public event tests, in the context of preserved general abilities. Furthermore, we found an accelerated forgetting rate for verbal and non verbal material. CONCLUSION: Our findings suggest that the patient's impaired long-term consolidation might have contributed to the impaired performance on public event memory tests. We discuss the specific features of new events processing that may contribute to its increased vulnerability relative to autobiographical memory, in the event of accelerated forgetting.

[Intradiploic encephalocele in an adult. A case report and review of the literature]. / Encéphalocèle intra-diploïque de l'adulte. A propos d'un cas et revue de la littérature.

We describe a rare case of parietal intradiploic encephalocele in a 51-year-old woman with no history of head trauma. The patient presented with a 1-month history of left hemiparesthesia. A hard indolent scalp lesion was palpable on examination in the right parietal area. Skull x-rays and cranial computed tomography examination demonstrated a lytic lesion that was consistent with a malignant osteolytic skull lesion. Magnetic resonance imaging of the head revealed an intradiploic cyst that included a round tissue mass contiguous with the parietal cortex. Surgery confirmed the diagnosis of encephalocele and the patient underwent surgical resection of the herniated brain, duraplasty, and cranioplasty. The presenting hemiparesthesia persisted at the 6-month follow-up. Encephaloceles of the cranial vault are a rare complication of skull fractures and rarely occur in adults. These lesions can be difficult to distinguish from congenital encephaloceles in patients with no history of head trauma. The pathogenesis, clinical and radiological nuances and the role of surgery are discussed.

[Adult medulloblastoma: Retrospective series of 21 patients]. / Médulloblastomes de l'adulte: étude rétrospective portant sur 21 patients.

PURPOSE: Retrospective analysis of the results of 21 adults treated for medulloblastoma. PATIENTS AND METHODS: Between 1978 and 2011, 21 adults with an average age of 31 years (18.3-50) were treated with surgery then with radiotherapy (n=20) at the Comprehensive Cancer Center of Strasbourg. For some (n=12), treatment consisted of chemotherapy. RESULTS: After a mean follow-up of 122 months (19-423), six relapses and seven deaths were observed. Overall survival at 5 years and 10 years was 89.4 ± 7.1% for both. Disease-free survival at 5 years and 10 years was 79.6 ± 9.2% and 85.7 ± 7.6% and 60.6 ± 17.7%, respectively. CONCLUSION: The rarity of medulloblastoma, especially in adults and these results confirm the necessity of international protocols.

[Pediatric medulloblastoma: Retrospective series of 52 patients]. / Médulloblastomes de l'enfant: étude rétrospective portant sur 52 patients.

PURPOSE: Retrospective analysis of the results of 52 children irradiated for a medulloblastoma. PATIENTS AND METHODS: Between 1974 and 2012, 52 children with an average age of 6 years and a half (11 months-17 years and a half) were treated with surgery then with radiotherapy at the Comprehensive Cancer Centre of Strasbourg (France). For 44 children, the treatment consisted of a chemotherapy. RESULTS: After a mean follow-up of 106.6 months (7-446 months), 13 relapses and 24 deaths were observed. Overall survival at 5 years and 10 years were 62% and 57%, respectively. Disease-free survival at 5 years and 10 years were 80% and 63%, respectively. Univariate analysis found the following adverse prognostic factors: the existence of a postoperative residue, the positivity of the cerebrospinal fluid, the metastatic status and medulloblastoma of high-risk. Positivity of the cerebrospinal fluid remains a negative factor in multivariate analysis. CONCLUSION: These results confirm the survival rate obtained by a conventional approach (surgery then irradiation). Insufficiency of results and rarity of medulloblastoma require the establishment of international protocols.

[Systemic Candida albicans infection with neuromeningeal complication in a premature infant]. / Complication neuroméningée d'une infection systémique à Candida albicans chez un prématuré

INTRODUCTION: Systemic maternal-fetal Candida albicans infections are uncommon diseases with a poor outcome. An associated cerebromeningeal infection increases morbidity. We present a case of neuromeningeal candidiasis following systemic neonatal infection in a premature infant. Management and therapeutic difficulties are outlined. OBSERVATION: The patient was a male infant born preterm at 30 weeks gestation. During his first week of life, he developed a systemic infection with an associated symptomatic hydrocephalus. Systemic candidaisis with neuromeningeal complication was diagnosed five weeks later. Despite treatment including cerebrospinal fluid (CSF) shunting and antimycotic medications (flucytosin and amphotericin B), the candidal infection did not resolve. Infectious and mechanical complications of the CSF drainage were treated by several surgical interventions during the following months. At 10 months of life, there was clinical and laboratory evidence of active persistent neuromeningeal candidaisis. Finally, candidal infection was eradicated with intravenous administration of fluconazole. After five year follow-up, the intellectual and psychological status of the patient was quite satisfactory, and no neurological deficits were found on clinical examination. DISCUSSION: Management of neuromeningeal candidaisis in premature infants is a challenging task particularly because of delayed diagnosis. Candida infection should routinely be suspected in cases of systemic infection with neurological impairment in premature infants. Fluconazole may constitute an efficient therapeutic option.

[Primary meningeal intermediate grade melanocytic neoplasm: case report with radiologic-pathologic correlation]. / Tumeur mélanocytaire méningée primitive de grade intermediaire: confrontation radio-pathologique.

A case of primary meningeal intermediate grade melanocytic neoplasm involving the right C2 nerve root is presented. MRI findings may suggest this rare entity, especially when an extra-axial lesion is located in the posterior fossa or cervical spinal canal and demonstrates shortening of both T1 and T2. Eventually, definitive diagnosis relies on histology which demonstrates spindle-shaped melanocytic cells that are Fontana stained and positive for HMB:45 antigen. Cellularity, pleomorphism, mitotic rate, proliferation index and invasiveness are useful criteria to distinguish among the spectrum of primary melanocytic tumors of the central nervous system ranging from melanocytoma to malignant melanoma.

VIIIth nerve cavernous hemangioma mimicking a stage 1 acoustic schwannoma.

OBJECTIVE: To report a case of VIIIth nerve cavernous hemangioma, a very rare differential diagnosis among the various pathologies presenting as small enhancing entities into the internal auditory canal. It is one of the most challenging when imaging is not able to differentiate it from an intrameatal vestibular schwannoma. METHODS AND RESULTS: We report a cavernous hemangioma extruding from the internal auditory canal, diagnosed after a left translabyrinthine resection in a 45-year-old man complaining of profound sensorineural hearing loss, with no facial paresis or dizziness. The preoperative differential diagnosis of a vestibular schwannoma was impossible, due to the absence of calcifications that usually characterize temporal bone hemangiomas. Clinical presentation, radiological features and treatment considerations are discussed along with up-to-date review of pertinent literature. CONCLUSIONS: When considering an apparent small intra-auditory canal schwannoma, otoneurologists should be aware of the rare possibility of a cavernous hemangioma. Early diagnosis and surgical treatment may improve the functional outcome, possibly preserving neural integrity.

[A rare case of caudal appendage]. / Appendice caudal dans l'espèce humaine : à propos d'un cas.

Caudal appendage is a rare malformation which has since ever been interesting. We present the case of a girl in which a caudal appendage was discovered before birth. A throughout checking found an extension from this abnormality to the cordal spine. The baby is operated when 2months old. Differential diagnosis are spina bifida, teratoma and pilonidal sinus. Caudal appendage is one of the typical cutaneous finding with underlying spinal dysraphism, such as hair tuft or pigmented macule. The risk is a tethered cord syndrome that can lead to severe complication. The treatment is either surgery or expectation.

Interest and limits of endoscopic approaches for pineal region tumours.

Endoscopy of pineal region tumours has been developed since the year 2000 either via a transventricular or extracerebral approach. The initial purpose of applying neuroendoscopy in the management of pineal region tumours was to resolve the obstructive hydrocephalus, and identify the pathological characteristics of the tumour. Based on this approach, a piecemeal resection of the tumour can be performed. The approaches, derived from the microsurgical pathway using an endoscope to expose the operative field, have been proposed either via an infratentorial supracerebellar approach or posterior transtentorial interhemispheric approach. Neuroendoscopic procedures can be considered as a therapeutic alternative to the microsurgical approach when CSF markers are negative. This procedure is considered mini-invasive for the approach along the surgical corridor access but extensive and in depth at the interface between the tumour and the surrounding neurological parenchyma. The limitations and complications are related to the type of procedure (mono- or bimanual) as well as the tumoral characteristics. Different approaches are presented in detail in order to avoid the occurrence of any surgical complications.

[Evolution of the management of pediatric and adult medulloblastoma]. / Évolution de la prise en charge des médulloblastomes de l'enfant et de l'adulte.

Medulloblastoma are cerebellar tumours belonging to the group of primitive neuroectodermal tumours (PNET) and are the most common malignant brain tumours of childhood. These tumours are rare and heterogeneous, requiring some multicentric prospective studies and multidisciplinary care. The classical therapeutic approaches are based on clinical, radiological and surgical data. They involve surgery, radiation therapy and chemotherapy. Some histological features were added to characterize risk. More recently, molecular knowledge has allowed to devise risk-adapted strategies and helped to define groups with good outcome and reduce long-term sequelae, improve the prognostic of high-risk medulloblastoma and develop new therapeutic tools.

Language functional neuro-imaging changes following focal left thalamic infarction.

The involvement of the left thalamus in language function has been largely demonstrated through the effects of thalamic lesion and/or stimulation upon language. However, the pathophysiological mechanisms underlying thalamic aphasias remain a matter of debate. We report here on changes in the pattern of brain activity in auditory word processing, verb generation and visual lexical decision in a patient who sustained left thalamic infarct following the surgery of intractable mesio-temporal epilepsy with left hippocampal sclerosis. To some extent, our findings exemplify the way the left thalamus brings on line the cortical networks involved in language processing, suggested in the term 'selective engagement'.