Eagle syndrome: An updated review.

Background: This work aims to review the current literature and our experience on vascular Eagle syndrome (ES) that can present misleading clinical presentations and better understand the possible therapeutic strategies. Methods: We reviewed the existing literature on PubMed from January 1, 2017, to December 31, 2022, including the sequential keywords "vascular AND Eagle syndrome," "vascular AND styloid syndrome," "vascular AND elongated styloid process," "vascular AND stylocarotid syndrome," and "Eagle syndrome AND carotid artery dissection." Results: 38 vascular ES cases, including our experience, were analyzed. The most frequent clinical onset was hemiparesis (n 21, 57%), but other regular clinical presentations were aphasia, loss of consciousness, amaurosis, headache, or a combination of the latter. Massive oral bleeding was reported only once in the literature before our case. Twelve patients were treated with only antiplatelet therapy, either single or double. Nine patients were treated with anticoagulation therapy only. In 14 patients, a carotid artery stent was used, associated with anticoagulation or antiplatelet therapy. In 17 cases, a styloid process (SP) resection was performed. Conclusion: ES has many clinical presentations, and carotid artery dissection resulting in oral bleeding seems rare. Literature results and our experience make us believe that when dealing with vascular ES, the best treatment strategy is endovascular internal carotid artery stenting with antiplatelet therapy, followed by surgical removal of the elongated SP to prevent stent fracture.

Isolated intracranial myeloid sarcoma: report of a case and review of the literature.

Myeloid sarcoma is a rare malignant tumor of primitive myeloid cell origin often associated with hematologic disorders. The central nervous system is rarely involved and differentiating between myeloid sarcoma and other tumors is not possible on imaging. Here we present the rare case of an isolated intracranial myeloid sarcoma, initially misdiagnosed radiologically as a meningioma, treated with surgical total resection and subsequent chemotherapy, with no signs of any hematological disorder at follow up. Differential diagnosis and management strategies, as well as follow-up implications are discussed along with literature review, which pointed out that only five cases with no further signs of hematological disorders at follow up have been described in the literature so far and this case has the longest follow up of them at 9 years.

Deep brain stimulation of the dentato-rubro-thalamic tract in a case of Holmes tremor: a constrained spherical deconvolution (CSD)-guided procedure.

Deep brain stimulation (DBS) is an established treatment for movement disorders, including Holmes tremor (HT). HT is a rest and action tremor that occurs as a late symptom of brainstem lesions such as stroke. Unfortunately, it is frequently refractory to medical treatment, hence DBS surgery may be a good option. Due to variable results, the ideal target for DBS in HT still remains to be established, ranging from the thalamus to the globus pallidus internus, to the subthalamic nucleus. Pre-operative imaging also is very challenging, as the complexity of brain fiber architecture may prevent the correct positioning of the directional lead. Herein, we describe the case of a patient affected by a rubral tremor secondary to a brain hemorrhage, who had advanced pre-operative neuroimaging with constrained spherical deconvolution (CSD)-based tractography obtained from diffusion-weighted imaging (DWI) to identify the dentato-rubro-thalamic tract, involved in the pathophysiology of HT. The patient was then addressed to an awake DBS surgery, and with the help of intraoperative microelectrode recordings, a tailored DRTT-targeted procedure was performed. The stimulation determined an almost complete tremor suppression, with no significant side effects at a follow-up of 6 months, paving the way towards new effective techniques for the planning, i.e., CSD-based tractography and the treatment of refractory tremors.

Modified percutaneous biopsy of the spine: improvement of the technique.

PURPOSE: Biopsy of the spine can be performed by open surgery or percutaneous needle sampling. The first has the highest diagnostic yield while the second is a less invasive procedure with lower rate of complications and shorter hospitalization time. We described a modified technique of percutaneous biopsy using semi-rigid grasping forceps that may offer the advantages of both, open and minimally invasive surgery. METHODS: Thirty consecutive patients with spinal lesions requiring biopsy were admitted to Neurosurgical Unit of Belcolle Hospital (Viterbo, Italy) from January 2017 to September 2021. There was a suspicion of spondylodiscitis in 25 cases and of tumor in 5 cases. Percutanous trans-pedicular spine biopsy has been performed using this new semi-rigid grasping forceps. Combining the opening width, jaw length and full 360° rotation, the device allows a wide and precise sampling. RESULTS: Sampling was sufficient in all cases (100%); tumors was observed in 5 cases (16.7%%) with a percentage of definitive histopathologic diagnosis of 100% (n = 5); among the remaining patients histological examination yielded a diagnosis of spinal infection in 25 cases (100%), and microbiologic culture provided an aetiologic diagnosis in 23 cases (92%). All procedures were well tolerated, and no postoperative complications were observed. Levels involved included: thoracic (T5-T9) in 8 cases, thoracolumbar junction (T10-L2) in 12 cases and lumbar (L3-L5) in 10 cases. CONCLUSIONS: Percutaneous biopsy with the semi-rigid grasping forceps is a safe and effective procedure that can be used for diagnosis of both infectious and tumor lesions of the spine. It allows to obtain a larger specimen volume and to use a multidirectional trajectory for sampling, resulting in a minimally invasive technique with strong ability to yield etiologic diagnosis.

Brainstem haemorrhage as a rare complication of burr hole craniostomy.

Aim Evacuation through burr hole craniostomy is the most common type of chronic subdural hematoma surgical treatment, with a morbidity rate of 0-9%. Methods Here we present a case of 66-year-old Caucasian woman with bilateral hemispheric chronic subdural hematoma and left transtentorial uncal herniation. Bilateral burr hole craniostomy with gradual and simultaneous evacuation was performed and subdural drains were placed with daily strict monitoring of drained fluid. Results Despite immediate prompt neurological improvement, on the second postoperative day bilateral ptosis and left medial rectus weakness occurred, with no signs of consciousness deterioration. Radiological exams revealed a 9 x 6 mm haemorrhage of the tegmentum mesencephali. In the next day progressive neurological improvement occurred and a follow-up at 1 month revealed persistence of bilateral ptosis with almost complete regression of the left medial rectus weakness. Conclusion Although burr hole craniostomy is considered a minor procedure, rare but fatal complications like brainstem haemorrhage may occur. Bilateral simultaneous and gradual drainage, strict monitoring of drained fluid and blood pressure in the perioperative period and frequent neurological with prompt radiological assessment in case of clinical worsening, should be the mainstay of a correct management of chronic subdural hematoma (particularly if bilateral) in order to avoid potentially fatal complications.

Postoperative Functional Preservation of Facial Nerve in Cystic Vestibular Schwannoma.

OBJECTIVE: To retrospectively analyze clinical and surgical data of a consecutive series of 26 patients with unilateral cystic vestibular schwannomas. METHODS: Tumors were classified as type A (central cyst) and type B (peripheral cyst) and as small (tumor diameter <3 cm) and large (tumor diameter >3 cm). All patients underwent microsurgical removal via retrosigmoid approach. The course of the facial nerve (FN) was classified as anterior, anterior-inferior, anterior-superior, and dorsal to the tumor's surface. RESULTS: Mean patient age was 53.5 years. Mean tumor size was 3.2 cm. There were 22 cases classified as type A and only 4 as type B. Total or near-total resection (>95%) was achieved in 16 cases (61.5%), subtotal removal (90%-95%) was achieved in 9 cases (34.6%), and partial removal (<90%) was achieved in 1 case (3.9%). Position pattern of FN was anterior-inferior in 10 cases (38.4%), anterior-superior in 10 cases (38.4%), anterior in 23.2% of 6 cases. At hospital discharge, FN function was House-Brackmann grade I-V in 9 (36%), 10 (38%), 3 (12%), 3 (12%), and 1 (4%) patients; at final follow-up, House-Brackmann grades I, II, III, and IV accounted for 18 (72%), 6 (24%), 1, and 1 cases. During follow-up ranging from 6 months to 10 years, reoperation for growing of residue was never necessary. CONCLUSIONS: According to the literature and the results of our series, microneurosurgery of cystic vestibular schwannomas is associated with good outcomes in terms of extent of resection and FN function. In particular, long-term FN function is much more satisfactory than short-term function. In most cases, microsurgery represents the treatment of choice of cystic vestibular schwannomas.

Hypertrophic Pachymeningitis and Hydrocephalus-The Role of Neuroendoscopy: Case Report and Review of the Literature.

BACKGROUND: Hypertrophic pachymeningitis (HP) is a rare primary or secondary inflammatory disorder that manifests with thickening of dura mater involving predominantly the tentorium and falx in the focal form. Hydrocephalus has not been reported in association with secondary HP. CASE DESCRIPTION: A 61-year-old woman presented with intracranial HP of the posterior fossa secondary to Wegener granulomatosis not responsive to corticosteroid therapy. Owing to the association of noncommunicating hydrocephalus, endoscopic third ventriculostomy was performed. There was immediate improvement of neurologic symptoms and no postoperative complications. The patient had very good clinical and radiologic outcome at 1-year follow-up. CONCLUSIONS: In a case of noncommunicating hydrocephalus related to focal HP of the posterior fossa, endoscopic third ventriculostomy was effective in resolving symptoms related to increased intracranial pressure. The physiopathogenetic mechanisms and therapeutic strategies were discussed along with a review of the most relevant literature.

Heart rate variability is differentially altered in multiple sclerosis: implications for acute, worsening and progressive disability.

BACKGROUND: Sympathovagal imbalance has been associated with poor prognosis in chronic diseases, but there is conflicting evidence in multiple sclerosis. OBJECTIVES: The objective of this study was to investigate the autonomic nervous system dysfunction correlation with inflammation and progression in multiple sclerosis. METHODS: Heart rate variability was analysed in 120 multiple sclerosis patients and 60 healthy controls during supine rest and head-up tilt test; the normalised units of low frequency and high frequency power were considered to assess sympathetic and vagal components, respectively. Correlation analyses with clinical and radiological markers of disease activity and progression were performed. RESULTS: Sympathetic dysfunction was closely related to the progression of disability in multiple sclerosis: progressive patients showed altered heart rate variability with respect to healthy controls and relapsing-remitting patients, with higher rest low frequency power and lacking the expected low frequency power increase during the head-up tilt test. In relapsing-remitting patients, disease activity, even subclinical, was associated with lower rest low frequency power, whereas stable relapsing-remitting patients did not differ from healthy controls. Less sympathetic reactivity and higher low frequency power at rest were associated with incomplete recovery from relapse. CONCLUSIONS: Autonomic balance appears to be intimately linked with both the inflammatory activity of multiple sclerosis, which is featured by an overall hypoactivity of the sympathetic nervous system, and its compensatory plastic processes, which appear inefficient in case of worsening and progressive multiple sclerosis.