An Unusual Asymptomatic Case of Unilateral Parotid Gland Agenesis.

Congenital absence of the major salivary glands, especially of the parotid gland, is a very infrequent condition of poorly understood etiology. This condition may be unilateral or bilateral and may occur alone or in association with the absence of other salivary glands or with other developmental craniofacial deformities of maxillofacial first and second branchial arch. Only 24 cases are documented in the literature. The authors present a case of a 79-year-old female who was referred to the Neurosurgery Department for neurocognitive impairment. The brain computed tomography with contrast enhancement incidentally showed a complete absence of the left parotid gland. The medical history and physical and radiographic examinations were indicative of nonsyndromic and nonfamilial asymptomatic unilateral aplasia of the parotid gland.

Occipitocervical Fusion.

Occipitocervical fusion is a surgical technique in continuous evolution due to the innovation of devices, operative and instrumentation techniques. The aetiologies responsible for occipitocervical instability are trauma, neoplastic disease, metabolic disease or congenital disease. A variety of stabilization techniques are currently available depending on the type of patient and surgeon's experience. Each of these techniques requires thorough knowledge of the anatomy of the craniovertebral junction.

Occipital Nerve Stimulation for Refractory Pain after Occipitocervical Fusion.

Occipital nerve stimulation (ONS) is electric stimulation of the distal branches of the greater occipital nerve by cylindrical or paddle leads implanted in subcutaneous occipital tissue. This surgical option has emerged as a promising treatment for different types of disabling medical refractory headache and recently also for residual occipital and nuchal pain after previous occipitocervical fusion. The mechanisms of action have not yet been clearly explained: electrical stimulation of the occipital nerve has both peripheral and central effects on the nervous system, which may modulate nociception. ONS is a well-tolerated and safe procedure in comparison with other invasive modalities of treatment. Lead migration/dislodgement is a common complication, but use of new surgical techniques and leads may reduce the rate of this complication.

New-onset hemodialysis-related headache presenting as migraine aura.

Hemodialysis headache (HDH) is an infrequent new-onset symptom, occurring mainly in old uremic patients. Type of pain is nonspecific, occurs during hemodialysis treatment, assuming features similar to tension-type headache and representing a problem, also as regards the therapy to be taken. International Headache Society (IHS) has placed this form of headache among the headaches disorders of homeostasis. We found a case of new-onset HDH in old uremic man, presenting with migraine aura features. A similar case has not been reported in literature, placing us some questions: why and how does this happen? What are the mechanisms involved? Role of trigeminal-vascular system and cortical spreading depression as regards the aura could be considered, through the activation of neuroinflammatory events, lastly causing migraine aura. Moreover, the administration of flunarizine strongly improved migraine symptoms in our patient, as happens in migraine syndromes. Definitely, this case leads us to think that some mechanisms involved in headaches will need to be further clarified.

Anatomo-physiological basis and applied techniques of electrical neuromodulation in chronic pain.

Chronic pain, a complex and debilitating condition, poses a significant challenge to both patients and healthcare providers worldwide. Conventional pharmacological interventions often prove inadequate in delivering satisfactory relief while carrying the risks of addiction and adverse reactions. In recent years, electric neuromodulation emerged as a promising alternative in chronic pain management. This method entails the precise administration of electrical stimulation to specific nerves or regions within the central nervous system to regulate pain signals. Through mechanisms that include the alteration of neural activity and the release of endogenous pain-relieving substances, electric neuromodulation can effectively alleviate pain and improve patients' quality of life. Several modalities of electric neuromodulation, with a different grade of invasiveness, provide tailored strategies to tackle various forms and origins of chronic pain. Through an exploration of the anatomical and physiological pathways of chronic pain, encompassing neurotransmitter involvement, this narrative review offers insights into electrical therapies' mechanisms of action, clinical utility, and future perspectives in chronic pain management.

Intraoperative Neurophysiological Monitoring in Neurosurgery.

Intraoperative neurophysiological monitoring (IONM) is a crucial advancement in neurosurgery, enhancing procedural safety and precision. This technique involves continuous real-time assessment of neurophysiological signals, aiding surgeons in timely interventions to protect neural structures. In addition to inherent limitations, IONM necessitates a detailed anesthetic plan for accurate signal recording. Given the growing importance of IONM in neurosurgery, we conducted a narrative review including the most relevant studies about the modalities and their application in different fields of neurosurgery. In particular, this review provides insights for all physicians and healthcare professionals unfamiliar with IONM, elucidating commonly used techniques in neurosurgery. In particular, it discusses the roles of IONM in various neurosurgical settings such as tumoral brain resection, neurovascular surgery, epilepsy surgery, spinal surgery, and peripheral nerve surgery. Furthermore, it offers an overview of the anesthesiologic strategies and limitations of techniques essential for the effective implementation of IONM.

A novel 3D anatomical visualization system to avoid injuries of nerves in retrosigmoid approach.

The retro-sigmoid approach (RA), widely used during different neurosurgical procedures, is burdened by the risk of injuries of the nerves that cross that region contributing to possible postoperative complications. By using, anatomage table (AT), a novel 3D anatomical visualization system, we described the nerves passing through the retromastoid area including the great occipital nerve (GON), the lesser occipital nerve (LON) and the great auricular nerve (GAN), and their courses from the origins, till terminal branches. Moreover, using dedicated software, we measured distances between the nerves and well-recognizable bony landmarks. After identifying the nerves and their distances from bony landmarks, we observed that the safest and risk-free skin incision should be made in an area delimited, superiorly from the superior nuchal line (or slightly higher), and inferiorly from a plane passing at 1-1.5 cm above the mastoid tip. The lateral aspect of such an area should not exceed 9.5-10 cm from the inion, while the medial one should be more than 7 cm far from the inion. This anatomical information has been useful in defining anatomical landmarks and reducing the risk of complications, mainly related to nerve injury, in RA. In-depth neuroanatomic knowledge of the cutaneous nerves of the retromastoid area is essential to minimize the complications related to their injury during different neurosurgical approaches. Our findings suggest that the AT is a reliable tool to enhance understanding of the anatomy, and thus contributing to the refinement of surgical techniques.

Primary Intracranial Gliosarcoma: Is It Really a Variant of Glioblastoma? An Update of the Clinical, Radiological, and Biomolecular Characteristics.

Gliosarcomas (GS) are sporadic malignant tumors classified as a Glioblastoma (GBM) variant with IDH-wild type phenotype. It appears as a well-circumscribed lesion with a biphasic, glial, and metaplastic mesenchymal component. The current knowledge about GS comes from the limited literature. Furthermore, recent studies describe peculiar characteristics of GS, such as hypothesizing that it could be a clinical-pathological entity different from GBM. Here, we review radiological, biomolecular, and clinical data to describe the peculiar characteristics of PGS, treatment options, and outcomes in light of the most recent literature. A comprehensive literature review of PubMed and Web of Science databases was conducted for articles written in English focused on gliosarcoma until 2023. We include relevant data from a few case series and only a single meta-analysis. Recent evidence describes peculiar characteristics of PGS, suggesting that it might be a specific clinical-pathological entity different from GBM. This review facilitates our understanding of this rare malignant brain tumor. However, in the future we recommend multi-center studies and large-scale metanalyses to clarify the biomolecular pathways of PGS to develop new specific therapeutic protocols, different from conventional GBM therapy in light of the new therapeutic opportunities.

Spinal Cord Stimulation in Chronic Low Back Pain Syndrome: Mechanisms of Modulation, Technical Features and Clinical Application.

Chronic low-back pain (CLBP) is a common disease with several negative consequences on the quality of life, work and activity ability and increased costs to the health-care system. When pharmacological, psychological, physical and occupational therapies or surgery fail to reduce CLBP, patients may be a candidate for Spinal Cord Stimulation (SCS). SCS consists of the transcutaneous or surgical implantation of different types of electrodes in the epidural space; electrodes are then connected to an Implanted Pulse Generator (IPG) that generates stimulating currents. Through spinal and supraspinal mechanisms based on the "gate control theory for pain transmission", SCS reduces symptoms of CLBP in the almost totality of well-selected patients and its effect lasts up to eight years in around 75% of patients. However, the evidence in favor of SCS still remains weak, mainly due to poor trial methodology and design. This narrative review is mainly addressed to those professionals that may encounter patients with CLBP failing conventional treatments. For this reason, we report the mechanisms of pain relief during SCS, the technical features and some clinical considerations about the application of SCS in patients with CLBP.

Deep brain stimulation for Parkinson's disease in practice: results of the survey by the Italian Neurosurgery Society.

BACKGROUND: Deep brain stimulation (DBS) is a safe and effective treatment for patients with advanced Parkinson's disease (PD) and many neurosurgical centers in Italy have a DBS program. Considering the prevalence of PD and criteria for DBS implantation, about 3200-10,350 PD patients may benefit from DBS in Italy. The global management of patients underwent DBS is complex and it can be supposed that many differences exist between centers in clinical practice. The Italian Neurosurgery Society (SINch) designed this survey to investigate the state of the art of DBS for PD in Italy. METHODS: A 26-item closed-ended question survey was designed and sanded by email at all Italian Neurosurgery centers. The main topic investigated was DBS teams, anatomical target selection, surgical procedure, neuroimaging, intraoperative target localization, DBS device and patients' follow-up. RESULTS: A total of 23 neurosurgery centers completed the survey. There are mainly low-to medium-volume centers (<20 annual DBS procedures) with dedicated DBS teams. The principal anatomical target used is subthalamic nucleus (STN) and, relative to the surgical technique, it emerges that in Italy DBS are bilaterally implanted in a single-step session with awake anesthesia and with frame-based technique. Final leads positioning is defined by microelectrode recordings (MER) and microstimulation (MS), with limited role of intraoperative neuroimaging (MRI and O-Arm). The stimulation is started at 15 or 30 days from procedure. CONCLUSIONS: Many centers of neurosurgery in Italy have a well-established DBS program for patients with advanced PD and some practical differences in technique between centers exist. Further investigation is needed to investigate specific criteria for selecting one technique over another.

Deep brain stimulation for treatment-resistant depression: a safe and effective option.

Introduction: Major depressive disorder (MDD) is the leading cause of years lost to disability worldwide. Pharmacotherapy and psychotherapy are effective treatments in most depressive episodes; but, about 30% of MDD patients remain symptomatic, and relapse is a common event. Recently, deep brain stimulation (DBS) has emerged as a valid therapeutic option in treatment-resistant depression (TRD) patients.Areas covered: In this paper, the authors summarize the findings of studies focused on these pathophysiologic phenomena and specifically on the role of DBS as a therapeutic option in TRD patients. The authors simply reviewed RCTs, open-label studies, neurophysiological mechanisms of DBS in MDD, and the possible role of different targets. Finally, we suggest possible future options.Expert opinion: Depression is a systems-level disorder, involving several brain structures. Neuroimaging studies demonstrate multiple interconnected regions that modulate different neural networks. DBS can modulate different targets, and others are under investigation. Among these subcallosal cingulate gyrus (SCG), ventral capsule and ventral striatum (VC/VS) seems to be the most relevant targets. We believe that, in the next future, DBS for TRD might become a first-line of treatment, especially using directional leads, that may help us to improve therapeutic effects.

Cervical spinal cord compression from delayed epidural scar tissue formation around plate lead for SCS.

Cervical spinal compression is a serious and rare complication of spinal cord stimulation (SCS) that can occur using leads placed via open surgical approach. The present report describe a case of cervical plate lead implant that developed spinal and radicular compression symptoms after seven years due to the growth of fibrotic epidural mass at the level of lead. A review of literature is provided. A 59-year­old woman with 3-year history of left arm post­traumatic complex regional pain syndrome (CRPS) was treated with SCS performed with the implant of paddle lead in the epidural space from C3-C5. Seven years later she reported progressive paresthesia along the spine and the limbs, gait ataxia with sensation of weakness in the legs, increased muscle tone and tendon reflexes in the lower extremities and decrease in effectiveness of stimulation. Cervical CT showed a tissue mass into the cervical canal posteriorly to the lead. This finding was confirmed by MR performed after lead removal that also allowed to document the amount of spinal cord compression. The patient underwent C4-C5-C6 laminectomy and a thick scar was removed from the dura. After surgery there was progressive and incomplete improvement of neurological signs but symptoms related to algodystrophy recurred partly. The formation of hypertrophic epidural scar tissue at the level of lead implant must be taken into consideration in presence of the onset of progressive cervical myelopathy in patient treated with SCS using laminectomy lead.

DBS in Treatment of Post-Traumatic Stress Disorder.

Post-traumatic stress disorder (PTSD) is a debilitating psychiatric condition for which pharmacological therapy is not always solvable. Various treatments have been suggested and deep brain stimulation (DBS) is currently under investigation for patients affected by PTSD. We review the neurocircuitry and up-to-date clinical concepts which are behind the use of DBS in posttraumatic stress disorder (PTSD). The role of DBS in treatment-refractory PTSD patients has been investigated relying on both preclinical and clinical studies. DBS for PTSD is in its preliminary phases and likely to provide hope for patients with medical refractory PTSD following the results of randomized controlled studies.

A Novel Diagnostic and Prognostic Tool for Simple Decompression of Ulnar Nerve in Cubital Tunnel Syndrome.

BACKGROUND: Surgical decompression of the ulnar nerve (UN) is effective for treating cubital tunnel syndrome (CubTS). Nevertheless, the outcome is not always satisfying. Different surgical, clinical, and imaging findings have been claimed as outcome predictors, but there is no consensus in the literature. We analyzed the outcome-predicting role of ultrasonography (US) of the UN in patients with CubTS and its possible role for diagnosis and follow-up. METHODS: Patients with CubTS treated by simple UN decompression underwent US and electrodiagnotic (ED) studies of the UN at the elbow before and after surgery. Outcome was evaluated through the Bishop scale. A correlation analysis between pre- and postoperative clinical, US, and ED findings was performed. RESULTS: Thirty-six patients were enrolled. Preoperatively, we observed a negative correlation between the motor conduction velocity (MCV) and the transverse (TD) and anteroposterior diameters and cross-sectional area (CSA) of the UN at the precubital (P = 0.001, P = 0.001, P = 0.005) and cubital level (P = 0.02, P = 0.002, P = 0.001). Preoperative precubital TD and CSA were associated with outcome (P = 0.01, P = 0.006) and postoperative MCV (P = 0.004, P = 0.008). The cut-off values TD >6 mm and CSA >23.91 mm2 were predictors of poor outcome. Finally, postoperative cubital TD and CSA values were inversely correlated with outcome (P = 0.0002, P = 0.0007) and postoperative MCV (P = 0.0002, P = 0.0004). CONCLUSIONS: The US examination of the UN is useful for the management of patients with CubTS as an adjunct to clinical and ED evaluations. US measurements are correlated with pre- and postoperative ED findings and thus are useful for diagnosis and follow-up. Interestingly, specific precubital US measurements are good predictors of outcome.

An unusual association of headache, epilepsy, and late-onset Kleist's pseudodepression syndrome in frontal lobe cavernoma of the cerebral left hemisphere.

Cerebral cavernous angioma or cavernoma is a benign vascular malformation, usually asymptomatic. It is infrequent and often its discovery is incidental, a so-called incidentaloma. However, these lesions can be symptomatic, causing headaches, epilepsy, cerebral hemorrhage and other neurological signs depending on the brain area involved. Frontal localization is responsible for psychiatric disorders, particularly the prefrontal region, leading to prefrontal syndrome, a condition common in all frontal lobe tumors. Psychopathological syndrome can be depression-type, pseudodepression syndrome or maniac-type, pseudomaniac syndrome. Surgical treatment of lesions like this may not always be possible due to their location in eloquent areas. In this study, we describe an unusual association of migraine-like headache, epilepsy and frontal lobe pseudodepression late-onset syndrome in the same patient. We have considered this case interesting mainly for the rarity of both a headache with migraine features and for the late onset of pseudodepression syndrome. Pathophysiology underlying migraine-like headache and that concerning the late-onset pseudodepression frontal lobe syndrome seems to be unclear. This case leads to further hypotheses about the mechanisms responsible for headache syndromes and psychopathological disorders, in the specific case when caused by a cerebral frontal lobe lesion.

Minimally invasive motor cortex stimulation for Parkinson's disease.

Extra/intradural strip electrode implantation on motor cortex may be possible minimally invasive neurosurgical method for therapeutic neuromodulation in Parkinson's disease (PD). The aim of this review is to assess motor cortex stimulation (MCS) efficacy and safety in advanced PD. Sixteen published articles were included with a total of 130 PD patients treated. In almost all results are from prospective observational open labeled study, only in two studies blinded assessment was carried out. Negative results are reported in three studies. Significant improvement in motor symptoms with remarkable effect on axial symptoms, L-dopa-induced dyskinesia and quality of life are outlined in thirteen studies. Surgical technique involved implant of four-contact strip electrode over M1 in epidural space with exception of few cases in which implant was carried out in subdural space. Surgical procedure was performed contralateral to most affected side with exception of five patients in which it was carried at dominant hemisphere; in four patients electrode implant was bilateral but stimulation was carried out simultaneously on both sides only in two cases. Complications and adverse events occurred very rarely for extradural MCS whereas with higher rate for subdural MCS. Based on review of current literature extra/intradural MCS represents an alternative to deep brain stimulation (DBS) to surgically treat PD patients who are not candidate for DBS. MCS is a minimally invasive neuromodulation procedure with low morbidity-mortality that can relieve all three major symptoms of PD on both sides simultaneously and bilaterally; it has significant effectiveness on axial symptoms, gait disturbances and therapy complications.

Critical reappraisal of DBS targeting for movement disorders.

Deep brain stimulation (DBS) is used as a surgical treatment of movement disorders such as Parkinson's disease, dystonia and essential tremor. Fundamental understanding of DBS effects on the pathological neural circuitry remains insufficient. In 2002 DBS of the subthalamic nucleus (STN) and the globus pallidus internus (GPi) was approved for use in patients with PD. Next year, DBS of Gpi and STN for dystonia received a Humanitarian Device exemption from the FDA. The commonly targets for DBS are subthalamic nucleus (STN) or globus pallidus internus (GPi) for Parkinson's disease, Gpi for dystonia and ventro-intermediate (VIM) nucleus of the thalamus for essential tremor. However, VIM DBS cannot sufficiently improve akinesia and rigidity. Pedunculopontine nucleus (PPN) is currently investigated as potential target to improve gait and posture. It is determined that DBS sometimes influences not only motor functions but also the cognitive and affective functions of patients. In this article we review the present state of DBS for movement disorders, appropriate indications, practical effects and stimulation-induced adverse events established in previous studies. We discuss target selection and the effect of DBS on motor and non-motor symptoms of Parkinson's disease, dystonia and essential tremor.

Administration of palmitoylethanolamide in combination with topiramate in the preventive treatment of nummular headache.

Nummular headache has been recently described as a primary disorder characterized by head pain exclusively felt in a small rounded area typically 2-6 cm in diameter, not attributed to another disorder. Both size and shape of the painful area remain constant since the onset of symptoms. A 57-year-old woman presented with a history of focal episodic pain in a circumscribed area on the right parietal region. The administration of standard oral doses of palmitoylethanolamide and topiramate in combination showed an improvement in pain symptoms and on pain measuring scales.