The Pierre Wertheimer Neurological and Neurosurgical Hospital in Lyon: history of its development and contributions to neurosurgery.

At the end of the 1950s, at the direction of Pierre Wertheimer, the first French professor of neurosurgery, the treatment of neurological and neurosurgical diseases for Lyon's 2 million people was concentrated in a single center functioning as not only a hospital but also a campus for neuroscience. The ideas behind the structure revolve around concepts such as spatial unity, comprehensive specialized fields, a critical mass of patients, a structured training program, and essential cross-communication between areas in the same field. Through several generations of doctors, researchers, and professors, the Pierre Wertheimer Neurological and Neurosurgical Hospital in Lyon (NHL) has had an important impact on clinical practice, fundamental neuroscientific research, and specialist training. Under Wertheimer's stewardship, functional neurosurgery became one of the fields of excellence at the NHL with contributions in pain surgery and physiology but also epilepsy surgery and surgery for spasticity. Typically, these contributions were the result of the collaboration of separate teams, ultimately laying the groundwork for a neuroscientific doctoral school. The large mass of patients treated at the NHL provided opportunities for other, more isolated insights, such as the classification of pineal tumors and contributions to interventional neuroradiology. The present work endeavors to illustrate the contributions of the NHL to neuroscience and discuss the background allowing for their occurrence.

Management of cavernous sinus meningiomas: Consensus statement on behalf of the EANS skull base section.

Introduction: The evolution of cavernous sinus meningiomas (CSMs) might be unpredictable and the efficacy of their treatments is challenging due to their indolent evolution, variations and fluctuations of symptoms, heterogeneity of classifications and lack of randomized controlled trials. Here, a dedicated task force provides a consensus statement on the overall management of CSMs. Research question: To determine the best overall management of CSMs, depending on their clinical presentation, size, and evolution as well as patient characteristics. Material and methods: Using the PRISMA 2020 guidelines, we included literature from January 2000 to December 2020. A total of 400 abstracts and 77 titles were kept for full-paper screening. Results: The task force formulated 8 recommendations (Level C evidence). CSMs should be managed by a highly specialized multidisciplinary team. The initial evaluation of patients includes clinical, ophthalmological, endocrinological and radiological assessment. Treatment of CSM should involve experienced skull-base neurosurgeons or neuro-radiosurgeons, radiation oncologists, radiologists, ophthalmologists, and endocrinologists. Discussion and conclusion: Radiosurgery is preferred as first-line treatment in small, enclosed, pauci-symptomatic lesions/in elderly patients, while large CSMs not amenable to resection or WHO grade II-III are candidates for radiotherapy. Microsurgery is an option in aggressive/rapidly progressing lesions in young patients presenting with oculomotor/visual/endocrinological impairment. Whenever surgery is offered, open cranial approaches are the current standard. There is limited experience reported about endoscopic endonasal approach for CSMs and the main indication is decompression of the cavernous sinus to improve symptoms. Whenever surgery is indicated, the current trend is to offer decompression followed by radiosurgery.

How to Do It: Microsurgical DREZotomy for Pain After Brachial Plexus Injury: 2-Dimensional Operative Video.

More than three-quarters of victims of brachial plexus injury suffer from refractory neuropathic pain.1-6 Main putative mechanism is paroxysmal hyperactivity in the dorsal horn neurons at the dorsal root entry zone (DREZ) as demonstrated by microelectrode recordings in animal models7 and patients.8 Pain relief can be achieved by lesioning the responsible neurons in the spinal cord segments with avulsed rootlets.9,10 This video illustrates the technique for microsurgical DREZotomy.11,12 A C3-C7 hemilaminectomy is performed to access the C4-Th1 medullary segments. After opening the dura and arachnoid, and freeing the cord from arachnoid adhesions, the dorsolateral sulcus is identified. Identification can be difficult when the spinal cord is distorted and/or has a loss of substance. The dorsolateral sulcus is then opened with a microknife, so that microcoagulations are performed: 4 mm deep, at 35° angle in the axis of the dorsal horn, every millimeter in a dotted fashion along the avulsed segments. Care should be taken not to damage the corticospinal tract, laterally, and the dorsal column, medially. The patient consents to the procedure. In the presented case, surgery led to complete disappearance of the paroxysmal pain and reduced the background of burning pain to a bearable level without the need of opioid medication. There was no motor deficit or ataxia in the ipsilateral lower limb postoperatively. According to Kaplan-Meier analysis at 10 yr follow-up, in our overall series, microsurgical DREZotomy achieved total pain relief without any medication in 60% of patients, and in 85% without the need for opioids.10,13-15 Microelectrode recording at 1:26 reproduced from Guenot et al7 with permission from JNSPG.

Microsurgical DREZotomy for Treatment of Brachial Plexus Avulsion Pain.

A 63-year-old man with a history of motorcycle accident 42 years ago suffered a left brachial plexus avulsion (BPA). Neuropathic pain in his left upper limb was felt in the C6-C7-C8 dermatomes. The rationale for performing "DREZotomy" is to preferentially interrupt the nociceptive inputs in the lateral part of the dorsal root entry zone (DREZ).1-3 For pain with complete deafferentation, as observed in BPA, the aim is to destroy the hyperactive nociceptive neurons deep in the apex of the dorsal horn (DH).4 Surgery is performed under general anesthesia, with the patient in prone position. Once the dura mater is opened, the arachnoid needs extensive dissection to open the dorsolateral and lateral spinal cisterns.5 In cases of BPA, the dorsolateral sulcus may be difficult to identify. Three anatomic elements can facilitate its recognition. Firstly, the remaining intact rootlets (above and below the avulsed segments) allow us to roughly localize the dorsolateral sulcus by joining these cranial and caudal normal rootlets. Secondly, blood vessels running on the spinal cord penetrate into the dorsolateral sulcus and often delineate the sulcus. Thirdly, scarring can be seen along the sulcus with small holes where the rootlets used to penetrate the cord. DREZotomy is performed using a graduated sharp bipolar instrument to allow precise microcoagulations of the DH. Preoperative surgical planning helps the surgeon by giving the angle between the DH and median plane.6 In the immediate postoperative period, the patient described the complete disappearance of neuropathic pain in his left upper limb, persistent at last follow-up (1 year) (Video 1).

Usefulness of external anal sphincter EMG recording for intraoperative neuromonitoring of the sacral roots-a prospective study in dorsal rhizotomy.

BACKGROUND: In conus medullaris and cauda equina surgery, identification of the sacral nerve roots may be uncertain in spite of their anatomical/radiological landmarks. Mapping the sacral roots by recording the muscular responses to their stimulation may benefit from EMG recording of the External Anal sphincter (EAS) in addition to the main muscular groups of the lower limbs. METHOD: In a consecutive series of 27 lumbosacral dorsal rhizotomy (DRh), authors carried out a prospective study on the reliability of the EMG recording of the EAS for identification of the S1 and S2 sacral roots. RESULTS: An EAS-response was recorded in all the 27 (bilaterally) explored individuals, testifying good sensitivity and selectivity of the method. EAS-responses were obtained in 96.3% of the 54 stimulated sides of the S2 root versus in only 16.66% for the S1 root, so that an absence of response would indicate S1 rather than S2 level. Furthermore, comparison between myotomal distribution of the S1 and S2 roots showed a significant difference (p < 0.00001), so that myotomal profile may help to identify root level. CONCLUSIONS: EMG recording of the EAS can be recommended for current intraoperative neuromonitoring. This simple method also provides-indirectly by extrapolation-information on the sacral motor pathways of the external urethral sphincter (EUS), as the later has the same somatic innervation via the pudendal nerve and related S2, S3, and S4 roots. Method can be helpful not only for DRh, of all varieties, but also for spine surgery, correction of dysraphisms, lipomas and/or tethered cord, and tumor resection.

Muscle responses to radicular stimulation during lumbo-sacral dorsal rhizotomy for spastic diplegia: Insights to myotome innervation.

OBJECTIVE: Most of knowledge on muscle radicular innervation was from explorations in root/spinal cord pathologies. Direct and individual access to each of the lumbar-sacral -ventral and dorsal- nerve roots during dorsal rhizotomy for spastic diplegia allows precise study of the corresponding muscle innervation. Authors report the lumbo-sacral segmental myotomal organization obtained from recordings of muscle responses to root stimulation in a 20-children prospective series. METHODS: Seven key-muscles in each lower limb and anal sphincter were Electromyography (EMG)-recorded and clinically observed by physiotherapist during L2-to-S2 dorsal rhizotomy. Ventral roots (VR), for topographical mapping, and dorsal roots (DR), for segmental excitability testing, were stimulated, just above threshold for eliciting muscular response. RESULTS: In 70% of the muscles studied, VR innervation was pluri-radicular, from 2-to-4 roots, with 1 or 2 roots being dominant at each level. Overlapping was important. Muscle responses to DR stimulation were 1.75 times more extended compared to VR stimulation. Inter-individual variability was important. CONCLUSIONS: Accuracy of root identification and stimulation with the used method brings some more precise information to radicular functional anatomy. SIGNIFICANCE: Those neurophysiological findings plead for performing Intra-Operative Neuromonitoring when dealing with surgery in the lumbar-sacral roots.

Topography of the pain in classical trigeminal neuralgia: insights into somatotopic organization.

Trigeminal neuralgia is defined by its clinical characteristics of paroxysmal unilateral facial pain in a well-defined territory. Distribution of the pain may be in one or several of the cutaneous and/or mucous territories of the three divisions with V2 pain being the most frequent territory followed by V3 and V1. Factors determining the distribution of pain have not yet been systematically investigated. It is now well recognized that vascular compression factor is a predominant aetiology of classical trigeminal neuralgia. In this study we aimed to find whether there is a relation between the location of the vascular compression and the peripheral distribution of the pain. Patients with classical trigeminal neuralgia in whom microvascular decompression was performed were included. Data recorded pertained to the nature of the conflict, its degree and, most importantly, location around the root: supero-median, supero-lateral or inferior. Equally, clinical data for the distribution of pain were recorded. Most of the patients 318 (89.3%) had the compression coming from above, i.e. 220 (61.7%) had compression from a supero-medial direction and 98 (27.5%) from a supero-lateral direction; inferior compression was present in 38 patients (10.7%). Distribution of the pain was significantly different according to the location of the conflict (P = 0.0005, Fisher Exact test). Odds ratios were computed for each location of compression and painful territory involved. According to the overall distribution of pain, patients with supero-medial compression had an odds ratio of 2.7 [95% confidence interval (CI) 1.66-4.41] of manifesting with V1 pain. Conversely V3 pain was less likely to occur with supero-median compression than the other types of pain (odds ratio 0.53, 95% CI 0.34-0.83). Inferior compression on the other hand was more likely to manifest with V3 pain with an odds ratio of 2.56 (95% CI 1.21-5.45). Overall V2 pain had an odds ratio close to 1 regardless of the type of compression. These findings suggest an association between the location of the neurovascular conflict with its resulting insult and the distribution of pain supporting a somatotopic view of the organization of the trigeminal root and a role of the conflict in the clinical manifestation of trigeminal neuralgia.

Planning of Endocranial Supratentorial Basal Cistern and Skull Base Approaches Depending on Venous Patterns Using a Topogram.

BACKGROUND: Because damage or sacrifice of venous drainage during supratentorial basal cistern and skull base approaches may have severe and harmful consequences, methods to identify preoperatively veins at risk are of paramount importance. Among methods, a codified assessment with a venous topogram is helpful, with practical implications. METHODS: This technical note describes how to construct an easy-to-use topogram. Three regions of interest are defined as triangles. The anterior triangle corresponds to the anterior frontal veins draining to the superior sagittal sinus at risk during anterior cerebral fossa surgery, the middle triangle corresponds to the anterior sylvian veins draining to the cavernous sinus at risk during pterional approaches, and the inferior triangle corresponds to the inferior cerebral veins draining to the transverse sinus at risk in subtemporal approaches and temporal lobe surgery. RESULTS: Depending on predominance of the drainage, 4 situations were defined: an anterior, an inferior, and a middle predominance or equilibrium between the 3 triangles. These anatomic features have important practical implications in skull base and basal cistern approaches. CONCLUSIONS: This is, to our knowledge, the only topogram described in the scientific literature. Any well-defined approach should be adapted to the individual patient according not only to location and type of lesion but also to the venous drainage to be encountered along the way.

Microsurgical DREZ lesions for the control of cancer-related pain.

Pain in patients with cancer is a major problem, and sometimes it is necessary to surgically interrupt pain pathways to effectively control refractory pain. Surgical lesion of the dorsal root entry zone (DREZ) was first performed in 1972 for the treatment of pain related to a Pancoast-Tobias tumor. The rationale of DREZotomy is to preferentially interrupt the nociceptive inputs in the lateral part of the DREZ and the ventrolateral (excitatory) part of the dorsal horn. Microsurgical DREZotomy is one technique for DREZ lesioning that is suited for tailored control of pain in patients in good general condition who are experiencing pain in a well-defined territory. The video can be found here: https://youtu.be/JtLQDP7gYSQ.

Arachnoiditis as an outcome factor for microvascular decompression in classical trigeminal neuralgia.

BACKGROUND: Neurovascular conflict is considered a key element of classical trigeminal neuralgia (TN) and consequently, microvascular decompression (MVD) is an effective treatment. Nevertheless, failures of MVD are described by many authors. In some patients, the arachnoid membranes surrounding the trigeminal nerve and neighbouring vessels may be thickened and adhesive. Here we analyse the impact of such focal arachnoiditis on outcome after MVD for TN. METHODS: A cohort of prospectively followed patients after their MVD was reviewed for intraoperative, imaging and clinical data if findings of arachnoiditis during MVD were described. Long-term outcome assessment was the main endpoint. RESULTS: We reviewed data from 395 MVD procedures, performed for TN from 2001 to 2014. Intraoperative evidence of focal arachnoiditis, as described by the surgeon, has been noted in 51 patients (13%). In 35 (68.6%), neuralgia was typical and in the other 17 (31.4%) it was atypical. As expected by definition, neurovascular conflict was found in 49 interventions (96%); it was predominantly arterial in 27 (52.9%). Accompanying arachnoiditis was encountered: mild in 20 interventions (39.2%), severe in 31 (60.8%). A successful result (BNI I or II) was achieved in 29 patients (56.9%). The other 22 patients (43.1%) had persistence or recurrence of pain. Overall KM probability of being pain free at 15 years was 72%. CONCLUSIONS: Intraoperative finding of arachnoiditis during MVD for classical trigeminal neuralgia is associated with poorer outcome than that of classical trigeminal neuralgia in general. This is particularly true for low grades of conflict.

Diffusion tensor imaging abnormalities of the trigeminal nerve root in patients with classical trigeminal neuralgia: a pre- and postoperative comparative study 4 years after microvascular decompression.

BACKGROUND: As diffusion tensor imaging (DTI) is able to assess tissue integrity, authors used diffusion to detect abnormalities in trigeminal nerves (TGN) in patients with trigeminal neuralgia (TN) caused by neurovascular compression (NVC) who had undergone microvascular decompression (MVD). The authors also studied anatomical TGN parameters (cross-sectional area [CSA] and volume [V]). The study compared pre- and postoperative findings. METHODS: Using DTI sequencing on a 3-T MRI scanner, we measured the fraction of anisotropy (FA) and apparent diffusion coefficient (ADC) of the TGN in 10 patients who had undergone MVD for TN and in 6 normal subjects. We compared data between affected and unaffected nerves in patients and both nerves in normal subjects (controls). We then correlated these data with CSA and V. Data from the affected side and the unaffected side before and 4 years after MVD were compared. RESULTS: Before MVD, the FA of the affected side (0.37 ± 0.03) was significantly lower (p < 0.05) compared to the unaffected side in patients (0.48 ± 0.03) and controls (0.52 ± 0.02), and the ADC in the affected side (5.6 ± 0.34 mm2/s) was significantly higher (p < 0.05) compared to the unaffected side in patients (4.26 ± 0.25 mm2/s) and controls (3.84 ± 0.18 mm2/s). Affected nerves had smaller V and CSA compared to unaffected nerves and controls (p < 0.05). After MVD, the FA in the affected side (0.41 ± 0.02) remained significantly lower (p < 0.05) compared to the unaffected side (0.51 ± 0.02), but the ADC in the affected side (4.24 ± 0.34 mm2/s) had become similar (p > 0.05) to the unaffected side (4.01 ± 0.33 mm2/s). CONCLUSIONS: DTI revealed a loss of anisotropy and an increase in diffusivity in affected nerves before surgery. Diffusion alterations correlated with atrophic changes in patients with TN caused by NVC. After removal of the compression, the loss of FA remained, but ADC normalized in the affected nerves, suggesting improvement in the diffusion of the trigeminal root.

Morphological and functional anatomy of the trigeminal triangular plexus as an anatomical entity: a systematic review.

PURPOSE: The sensory trigeminal nerve in the trigeminal cave of Meckel-which is an individualized lodge-is classically segmented into two parts: the trigeminal ganglion (TG) and the triangular plexus (TP). The TP has been defined as the portion of the trigeminal nerve from the posterior margin of the TG to the path over the upper ridge of the petrous bone. Due to its relatively unrecognized status, its morphological and functional anatomy has been reviewed by the authors through a PRISMA systematic review of the literature. METHODS: The authors have carried out a systematic review of the TP according to the PRISMA model with various bibliographical bases. Before 1947: Medic @ Library (BIU Santé Paris, 2017); Index-Catalog of the Library of the Surgeon-General's Office (US National Library of Medicine, 2017); Gallica (French National Library, 2017). After 1947: PUBMED, PubMed Central and MEDLINE. RESULTS: 56 articles were retained for full-text examination, of which 23 were chosen and included. The TP was described as having a triangular shape (30.2%), a plexual organization (97.4%) with sensory-, motor- and sympathetic-anastomoses (96.7%) that, however, respect the somatotopic trigeminal distribution (93.3%). The direct electrical stimulation of the root at the level of the TP (during radiofrequency-thermorhizotomy procedures) confirmed a clear-cut somatotopy. CONCLUSION: An understanding of both the morphological and the functional anatomy of the triangular plexus can contribute to accuracy and safety on the surgeries performed for trigeminal neuralgia and tumor removal inside the trigeminal cave.

Veins of the Cerebellopontine Angle and Specific Complications of Sacrifice, with Special Emphasis on Microvascular Decompression Surgery. A Review.

Good knowledge of the anatomy of veins is of crucial importance for the functional surgery of cranial nerve (CN) disorders, especially microvascular decompression for trigeminal neuralgia (TN), hemifacial spasm (HFS), and vagoglossopharyngeal neuralgia (VGPN). Although controversial, veins may be involved in neurovascular conflicts and may constitute dangerous obstacles to access to the CNs. With the aim of estimating the implications of veins in those diseases and evaluating the linked surgical difficulties, we carried out a review of the literature from 2000 to the end of February 2018. For this review, articles found on PubMed that gave enough precision about veins were retained (39 articles on TN, 38 on HFS, 8 on VGPN, and 26 on complications related to venous sacrifices). Before this review, we described a simplified anatomic classification of veins, amenable to easing the surgical approach to CNs. Access to the trigeminal nerve, via the infratentorial-supracerebellar route, is almost always affected by the superficial superior petrosal venous system, whereas access to the facial and cochleovestibular complex as well as to the lower CNs, through the infrafloccular trajectory, is almost always exempt of important venous obstacles. Respective incidences of venous compression at the origin of hyperactive CN syndromes are given. The percentages of a venous conflict alone were calculated at 10.8% for TN, 0.1% for HFS, and 2.9% for VGPN. We review the complications considered in relation with venous sacrifices. Precautions to minimize these complications are given.

Reliability of MRI for predicting characteristics of neurovascular conflicts in trigeminal neuralgia: implications for surgical decision making.

OBJECTIVEThe choice of microvascular decompression (MVD), among the several other surgical options, for treating refractory classical trigeminal neuralgia (TN) relies mostly on preoperative imaging, but the degree of reliability of MRI remains a matter of debate. The authors approached the question of predictability of neurovascular conflict (NVC) in a series of 100 protocolized MRI studies from patients with TN who underwent MVD, by reexamination of MR images, blinded to the clinical data and surgical findings, including the side of the neuralgia.METHODSPatients included in the study were those who underwent MVD after surgical indication had been determined based on a protocolized imagery workup (3D high-resolution T2-weighted cisternography centered on the trigeminal nerve, 3D time-of-flight angiography, and 3D gadolinium-enhanced T1-weighted imaging) performed at our institution. All MR images were blindly reexamined, and neurovascular relationships were described on both sides, noting the existence of compression, vessels involved, situation along the root, and degree of compression. The results of MRI evaluation were then compared with actual surgical findings. The extent of agreement and quality of the prediction were expressed with Cohen's kappa coefficient (κ) and receiver operating characteristic (ROC) statistics.RESULTSA conflict had actually been found during surgery in 94 of 100 patients. The sensitivity of MRI to detect a conflict was 97% and the specificity was 50%. Vessel type was identified with high reliability (κ = 0.80), while the grade of the conflict and its situation along the root showed poor to average reliability (κ = 0.38 and κ = 0.40, respectively). The area under the ROC curve for predicting the presence of a conflict according to the grades of conflict seen on MRI was 0.93, which is considered very good. The positive predictive value was differentiated according to the grade of conflict, with a very high value for high grades of vascular conflict.CONCLUSIONSThis study shows an overall good reliability of MRI to predict the existence of an NVC. The prediction value is excellent for high grades of compression. Some apparent low-grade compressions on MRI may be revealed as false positives in surgical exploration. This raises the question of what other imaging methods might be used to determine not only the existence of a conflict but also its degree of compression. The degree of compression is of paramount importance to predict the probability of long-term pain relief, and therefore in the decision to propose MVD as the first choice of surgical treatment.

Role of the petrous ridge and angulation of the trigeminal nerve in the pathogenesis of trigeminal neuralgia, with implications for microvascular decompression.

INTRODUCTION: Vascular compression is the main pathogenetic factor in apparently primary trigeminal neuralgia; however some patients may present with clinically classical neuralgia but no vascular conflict on MRI or even at surgery. Several factors have been cited as alternative or supplementary factors that may cause neuralgia. This work focuses on the shape of the petrous ridge at the point of exit from the cavum trigeminus as well as the angulation of the nerve at this point. METHODS: Patients with trigeminal neuralgia that had performed a complete imagery workup according to our protocol and had microvascular decompression were included as well as ten controls. In all subjects, the angle of the petrous ridge as well as the angle of the nerve on passing over the ridge were measured. These were compared from between the neuralgic and the non-neuralgic side and with the measures performed in controls. RESULTS: In 42 patients, the bony angle of the petrous ridge was measured to be 86° on the neuralgic side, significantly more acute than that of controls (98°, p = 0.004) and with a trend to be more acute than the non-neuralgic side (90°, p = 0.06). The angle of the nerve on the side of the neuralgia was measured to be on average 141°, not significantly different either from the other side (144°, p = 0.2) or from controls (142°, p = 0.4). However, when taking into account the grade of the conflict, the angle was significantly more acute in patients with grade II/III conflict than on the contralateral side, especially when the superior cerebellar artery was the conflicting vessel. CONCLUSION: This pilot study analyzes factors other than NVC that may contribute to the pathogenesis of the neuralgia. It appears that aggressive bony edges may contribute-at least indirectly-to the neuralgia. This should be considered for surgical indication and conduct of surgery when patients undergo MVD.

The tethered effect of the arachnoid in vago-glossopharyngeal neuralgia: a real associated alternative mechanism?

Vago-glossopharyngeal neuralgia (VGPN) is a rarely seen disease when compared to trigeminal neuralgia. When the pain is resistant to medical therapy, microvascular decompression can be performed if a vascular conflict is suspected on magnetic resonance imaging (MRI). In addition, arachnoid pathology may play a role in VGPN. We report two cases of VGPN caused by tethered arachnoid, associated with a vascular contact in which pain was reduced by freeing rootlets from arachnoid compression. We report two cases relating to 50-year-old and 30-year-old men with a history of electric shooting pain triggered by swallowing in the right pharyngeal and auricular regions. Preoperative MRI documented a neurovascular conflict in the first case and an arachnoid cyst in the second. Surgery was performed via a retrosigmoid craniotomy. In both cases, the intraoperative findings documented a tethered arachnoid membrane compressive to cranial nerves IX and X. Untethering was performed by liberation of the rootlets from the arachnoid with microvascular decompression. No additional rhizotomy was performed. The postoperative course was uneventful and pain was relieved in the first case and decreased in the second. In VGPN, a tethered arachnoid may play a role in causing the neuralgia, either alone or associated with a neuro-vascular conflict.