[Skull base metastases with cranial nerve deficits : Clinical profile of a severe disease]. / Schädelbasismetastasen mit Hirnnervenaffektionen : Klinischer Steckbrief einer schwerwiegenden Erkrankung.

BACKGROUND AND PURPOSE: Skull base metastases are a severe complication of various malignant tumors. If cranial nerves are involved even small lesions can cause significant symptoms. Specific clinical characteristics like neurological symptoms, associated primary tumors, prognosis and optimal treatment are poorly defined and are systematically described in this article. METHODS: In a monocentric retrospective study patients with skull base metastases and cranial nerve deficits who received treatment between 2006 and 2018 were analyzed concerning clinical characteristics at initial diagnosis, treatment and course of the disease. RESULTS: In this study 45 patients with skull base metastases and cranial nerve deficits were included. The most frequent primary tumors were prostate cancer (27%), breast cancer (22%) and multiple myeloma (16%). The most involved cranial nerves were trigeminal nerve (42%), oculomomotor nerve (33%) and facial nerve (27%). Of the patients 84% had additional bone metastases outside the skull base. Dural infiltration or meningeal carcinomatosis were each observed in 13% of the patients. After radiotherapy cranial nerve deficits remained stable in 61% of all cases and in 22% symptoms improved. Median overall survival from treatment was 8 months (range 0.4-51 months). Patients with dose-escalated radiotherapy appeared to live longer (16.4 months vs. 4.7 months). This effect persisted in a multivariate analysis including the Karnofsky index, number of metastases, primary tumor and radiation dose (HR 0.37, p = 0.02). CONCLUSION: Skull base metastases with cranial nerve deficits are complex diseases with poor prognosis. Precise diagnosis and treatment are required. Further research is needed to improve treatment.

The Diagnosis and Management of Posttraumatic Headache with Associated Painful Cranial Neuralgias: a Review and Case Series.

BACKGROUND: Cranial neuralgias are common in the setting of posttraumatic headache. They may exacerbate underlying primary headache disorders and therefore may be overlooked in clinical practice. Frequently, cranial neuralgias generate neuropathic symptoms such as lancinating pain and sensory dysesthesias. Cranial neuralgias are identified based on a clinical history of focal neuropathic pain and physical exam findings including tenderness with palpation and percussion, at times eliciting radiating pain or paresthesias in the corresponding sensory nerve distribution. PURPOSE OF REVIEW: This article is a brief review of the literature and a retrospective report of 2 cases of posttraumatic headache with associated painful cranial neuralgias. RECENT FINDINGS: Two patients presented with headaches that met criteria for posttraumatic headache, but their history and physical examination suggested the presence of a focal painful cranial neuralgia. One patient was diagnosed with auriculotemporal neuralgia, which was exquisitely responsive to an auriculotemporal nerve block. The second patient was diagnosed with supratrochlear neuralgia, which was effectively treated with a supratrochlear nerve block. In both cases, adequate treatment of the painful cranial neuralgia resulted in significant improvement of the baseline PTH. Painful cranial neuralgias frequently occur within the clinical spectrum of posttraumatic headache, but are often undiagnosed. Treatment options for painful cranial neuralgias are often different than those traditionally employed for posttraumatic headache without cranial neuralgias, which can include peripheral nerve blockade, neuropathic medications, and in refractory cases, peripheral nerve decompression surgery.

Delayed-Onset Cranial Nerve Palsy After Transvenous Embolization of Indirect Carotid Cavernous Fistulas.

BACKGROUND: Carotid cavernous fistulas (CCF) often present with diplopia secondary to cranial nerve palsy (CNP). Immediate development of postoperative CNP has been described in the literature. This study described delayed-onset of CNP after complete and reconfirmed obliteration of the CCF and resolution of initial CNP. METHODS: A retrospective analysis was performed on patients with indirect CCF between 1987 and 2006 at 4 academic endovascular centers. Details of the endovascular procedures, embolic agents used, and complications were studied. Partial or complete obliteration was determined. Immediate and delayed cranial nerve palsies were independently assessed. RESULTS: A total of 267 patients with symptomatic indirect CCF underwent transvenous endovascular treatment. Four patients (1.5%) developed delayed abducens nerve (VI) palsy after complete resolution of presenting symptoms after embolization. Delayed presentation ranged between 3 and 13 months after complete resolution of initial double vision and cranial nerve palsies. Transvenous coil embolization through the inferior petrosal sinus was performed in all 4 affected patients. All had follow-up angiography confirming durable closure of their CCF. MRI did not show new mass lesions or abnormal soft tissue enhancement. In all 4 patients, their abducens nerve (VI) palsy remained. CONCLUSIONS: Delayed CNP can develop despite complete endovascular obliteration of the CCF. The cause of delayed CNP is not yet determined, but may represent fibrosis and ischemia. Long-term follow-up is needed even after complete neurological and radiological recovery is attained in the immediate perioperative period.

Onyx embolization of skull base paragangliomas: a single-center experience.

BACKGROUND: Skull base paragangliomas are highly vascular tumors that are often embolized before surgical resection; however, the safety and efficacy of preoperative embolization using an ethylene vinyl alcohol copolymer (Onyx; Medtronic, Dublin, Republic of Ireland) in these tumors is unknown. This retrospective cohort study evaluated patient outcomes after preoperative embolization of skull base paragangliomas using Onyx. METHODS: We retrospectively analyzed data from all patients with skull base paragangliomas who underwent preoperative Onyx embolization at our institution (January 01, 2005-December 31, 2017). Patient, tumor, embolization, and outcomes data were extracted by reviewing inpatient and outpatient clinical and imaging records. RESULTS: Seven patients were studied (5/7 [71%] female), 6 with glomus jugulares and 1 with a glomus vagale. The median age was 52 years, and the most common presenting symptom was cranial neuropathy (6/7 [86%]). The tumor vascular supply was from the ascending pharyngeal artery in all 7 cases (100%) with additional feeders including the occipital artery in 5 (71%); internal carotid artery in 3 (43%); middle meningeal, vertebral, and internal maxillary artery each in 2 (29%); and posterior auricular artery in 1 (14%). The median postembolization tumor devascularization was 80% (range, 64-95%). The only postembolization complication was a facial palsy in 1 patient. CONCLUSION: Preoperative embolization with Onyx affords excellent devascularization for the majority of skull base paragangliomas, and it may facilitate resection of these hypervascular lesions. The advantages provided by Onyx with respect to penetration of intratumoral vessels must be weighed against the risk of cranial neuropathy.

Visual dysfunction in multiple sclerosis. / Synsproblemer ved multippel sklerose.

Multiple sclerosis can give rise to signs and symptoms from the entire nervous system, including visual impairments. Visual impairments often go unreported because they are not obvious to patients, which means that doctors must ask about them specifically. Regular monitoring of vision is important, however, to provide personalised rehabilitation and assistive technologies, and thereby improve patients' functioning and quality of life.

Intracranial Aneurysms of Neuro-Ophthalmologic Relevance.

BACKGROUND: Intracranial saccular aneurysms are acquired lesions that often present with neuro-ophthalmologic symptoms and signs. Recent advances in neurosurgical techniques, endovascular treatments, and neurocritical care have improved the optimal management of symptomatic unruptured aneurysms, but whether the chosen treatment has an impact on neuro-ophthalmologic outcomes remains debated. EVIDENCE ACQUISITION: A review of the literature focused on neuro-ophthalmic manifestations and treatment of intracranial aneurysms with specific relevance to neuro-ophthalmologic outcomes was conducted using Ovid MEDLINE and EMBASE databases. Cavernous sinus aneurysms were not included in this review. RESULTS: Surgical clipping vs endovascular coiling for aneurysms causing third nerve palsies was compared in 13 retrospective studies representing 447 patients. Complete recovery was achieved in 78% of surgical patients compared with 44% of patients treated with endovascular coiling. However, the complication rate, hospital costs, and days spent in intensive care were reported as higher in surgically treated patients. Retrospective reviews of surgical clipping and endovascular coiling for all ocular motor nerve palsies (third, fourth, or sixth cranial nerves) revealed similar results of complete resolution in 76% and 49%, respectively. Improvement in visual deficits related to aneurysmal compression of the anterior visual pathways was also better among patients treated with clipping than with coiling. The time to treatment from onset of visual symptoms was a predictive factor of visual recovery in several studies. Few reports have specifically assessed the improvement of visual deficits after treatment with flow diverters. CONCLUSIONS: Decisions regarding the choice of therapy for intracranial aneurysms causing neuro-ophthalmologic signs ideally should be made at high-volume centers with access to both surgical and endovascular treatments. The status of the patient, location of the aneurysm, and experience of the treating physicians are important factors to consider. Although a higher rate of visual recovery was reported with neurosurgical clipping, this must be weighed against the potentially longer intensive care stays and increased early morbidity.

Transpetrosal approach to petro-clival meningioma.

A 38-year-old woman had a 3-week gradual onset of right-sided weakness in the upper and lower extremities. MRI showed a large left petro-clival meningioma encasing the basilar and left superior cerebellar artery and compressing the brainstem. A posterior transpetrosal approach, with a left temporal and retrosigmoid craniotomy and mastoidectomy, was performed. The tumor was removed in a gross-total resection with questionable remnants adherent to the brainstem. Intraoperative partial iatrogenic injury to the left oculomotor nerve was repaired with fibrin glue. Postoperatively, the hemiparesis improved, and the patient was discharged to the rehabilitation center with left oculomotor and abducens palsies. A postoperative MRI scan showed complete resection of tumor with no remnants on the brainstem. A 6-month follow-up examination showed complete resolution of motor symptoms and complete recovery of cranial nerve (CN) palsies affecting CN III and CN VI. The video can be found here: https://youtu.be/vOu6YFA8uoo .

[Idiopathic cranial nerve failure]. / Idiopathische Hirnnervenausfälle.

Cranial nerve lesions require a thorough diagnostic work-up and known etiologies have to be excluded before the term idiopathic can be considered. The focus of the present review is on idiopathic peripheral facial nerve paralysis (Bell's palsy) for which this terminology has been established. For all other cranial nerve lesions the typical clinical signs, established etiologies and possible diagnostic pitfalls are discussed.

Nerve block for the treatment of headaches and cranial neuralgias - a practical approach.

BACKGROUND: Several studies have presented evidence that blocking peripheral nerves is effective for the treatment of some headaches and cranial neuralgias, resulting in reduction of the frequency, intensity, and duration of pain. OBJECTIVES: In this article we describe the role of nerve block in the treatment of headaches and cranial neuralgias, and the experience of a tertiary headache center regarding this issue. We also report the anatomical landmarks, techniques, materials used, contraindications, and side effects of peripheral nerve block, as well as the mechanisms of action of lidocaine and dexamethasone. CONCLUSIONS: The nerve block can be used in primary (migraine, cluster headache, and nummular headache) and secondary headaches (cervicogenic headache and headache attributed to craniotomy), as well in cranial neuralgias (trigeminal neuropathies, glossopharyngeal and occipital neuralgias). In some of them this procedure is necessary for both diagnosis and treatment, while in others it is an adjuvant treatment. The block of the greater occipital nerve with an anesthetic and corticosteroid compound has proved to be effective in the treatment of cluster headache. Regarding the treatment of other headaches and cranial neuralgias, controlled studies are still necessary to clarify the real role of peripheral nerve block.

Cranial Neuralgias.

OBJECTIVE: The cranial neuralgias are relatively rare, but recognizing these syndromes and distinguishing among them is critical to reducing unnecessary pain and disability for affected patients. Despite their distinctive features, cranial neuralgias may go undiagnosed or misdiagnosed for several years. A notable proportion of cranial neuralgia presentations are due to secondary causes and require targeted treatment. The purpose of this article is to review the diagnosis and management of cranial neuralgias encountered in clinical practice. LATEST DEVELOPMENTS: In 2020, the International Classification of Orofacial Pain was released for the first time. Modeled after the International Classification of Headache Disorders, it includes updated terminology for cranial neuralgias. The underlying pathophysiology of the cranial neuralgias is currently believed to be rooted in both peripheral and central nociceptive systems. In addition, a growing number of familial cases are being identified. Recent therapeutic advancements include a better understanding of how to utilize older therapies and procedures more effectively as well as the development of newer approaches. ESSENTIAL POINTS: Cranial neuralgia syndromes are rare but important to recognize due to their debilitating nature and greater likelihood of having potentially treatable underlying causes. While management options have remained somewhat limited, scientific inquiry is continually advancing the understanding of these syndromes and how best to address them.

Clinical features of CIDP with LM1-associated antibodies.

BACKGROUND: LM1 is the predominant glycolipid in human peripheral nerve myelin and antibodies to LM1 and LM1-containing ganglioside complexes are detected in some patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The clinical features of patients with such antibodies have not yet been investigated. METHODS: Serum antibodies to LM1, a mixture of GM1 and LM1 (GM1/LM1), and that of GD1b and LM1 (GD1b/LM1) were examined in 75 consecutive patients with CIDP. The clinical features of the CIDP patients with such antibodies in the present series and those in the previous reports were investigated and compared with those of antibody-negative patients. RESULTS: Of the 75 patients with CIDP, two had antibodies to LM1, three had anti-GM1/LM1 complex antibody, one had anti-GD1b/LM1 complex antibody and two had antibodies to both the GM1/LM1 and GD1b/LM1 complexes. Patients with the LM1-associated antibodies did not have cranial nerve deficits (p<0.05) and exhibited ataxia more frequently than the antibody-negative patients (p<0.01). CONCLUSION: In humans, LM1 is contained more in the dorsal root than in the cranial nerves. The clinical features of CIDP patients with antibodies to LM1 and LM1-containing complexes may be associated with the distribution of the LM1 antigen. LM1-associated antibodies are possible markers for a subclass of CIDP.

Biological treatments: new weapons in the management of monogenic autoinflammatory disorders.

Treatment of monogenic autoinflammatory disorders, an expanding group of hereditary diseases characterized by apparently unprovoked recurrent episodes of inflammation, without high-titre autoantibodies or antigen-specific T cells, has been revolutionized by the discovery that several of these conditions are caused by mutations in proteins involved in the mechanisms of innate immune response, including components of the inflammasome, cytokine receptors, receptor antagonists, and oversecretion of a network of proinflammatory molecules. Aim of this review is to synthesize the current experience and the most recent evidences about the therapeutic approach with biologic drugs in pediatric and adult patients with monogenic autoinflammatory disorders.

Brainstem dysgenesis during the neonatal period: diagnosis and management.

AIMS: To report our neonatal management experience in patients who received a diagnosis of brainstem dysgenesis (BSD). PATIENTS AND METHODS: This study retrospectively reviewed the medical records of 15 neonates with BSD diagnosed between 1984 and 2011. Data on the perinatal period, physical examination, laboratory findings, and management by systems were systematically analyzed. RESULTS: All cases were sporadic. Cocaine abuse and misoprostol use were recorded in two pregnancies. The reason for admission was prematurity (2 of 15), respiratory distress (8 of 15), gastroschisis (1 of 15), and abnormal neurological examination (4 of 15). Clinically, the most commonly affected cranial nerves were the 7th (13 of 15), 9th (11 of 15), 10th (8 of 15), 5th (7 of 15), 12th (7 of 15), 6th (3 of 15), 4th (1 of 15), and 3rd (1 of 15). Five patients required positive pressure ventilation during delivery room resuscitation, three had difficult airways, and two needed tracheostomy during admission. Most patients required nasogastric tube feeding shortly after birth, and four patients had a gastrostomy on discharge. Two patients died of respiratory and cardiac failure. Electromyography and nerve conduction velocity were used to exclude generalized neuromuscular disorders, and in conjunction with other neurophysiological and gastrointestinal tract studies, helped uncover the extent of brainstem involvement in most cases. Cranial magnetic resonance imaging supported the diagnosis in more than half of the patients. CONCLUSIONS: Early diagnosis of BSD is mainly clinical, difficult to establish unless suspected, and crucial to prevent complications. Neonatal care of patients with BSD requires a comprehensive approach that must take into consideration the etiological, anatomical, and pathogenic aspects contributing to the clinical manifestations of this disorder. Care should be provided by multidisciplinary teams, in which neonatologists, pediatric neurologists, nutritionists, physical therapists, and other professionals participate, depending on the associated morbidity in order to improve its management and prognosis.

Two cases of pharyngolaryngeal zoster advanced to multiple cranial neuropathy.

Varicella zoster virus (VZV) infection of the head and neck region may present with various symptoms. I present two cases of VZV infection of the pharynx and larynx with multiple cranial nerve neuropathies. Their initial symptoms such as sore throat, odynophagia, and dysphasia were complicated by otalgia, dizziness, hearing loss, or ipsilateral facial nerve paralysis. All of these lesions tended to lateralize to the ipsilateral side and endoscopic findings suggested VZV infections, which were confirmed by serial serologic examinations.

Cranial nerve palsies in childhood parameningeal rhabdomyosarcoma.

BACKGROUND: Children with parameningeal rhabdomyosarcoma (PM RMS) and cranial nerve palsy (CNP) are at risk for permanent neurologic dysfunction. Clinicians often consider the use of emergent therapies such as expedited radiation and/or corticosteroids; however, there is a paucity of information describing the natural history of CNP in PM RMS. We sought to describe the clinical features of patients with PM RMS plus associated CNP and to evaluate the patient, disease, and treatment-related factors that impacted neurologic recovery. METHODS: We conducted a retrospective review of PM RMS cases treated at the Hospital for Sick Children between 1985 and 2010. RESULTS: Thirty-five children were treated for PM RMS, 19 (54%) of whom presented with CNP. Children with CNP were nine times more likely to have other high-risk features (cranial base bony erosion and/or intracranial extension) at the time of presentation than children without CNP (OR 9.6, 95% CI 1.69, 54.79, P = 0.013). In addition to commencing chemotherapy, 13 patients (68%) received expedited RT and corticosteroids, four (21%) corticosteroids alone, and two (11%) received only standard chemotherapy and RT. At last follow up of the 11 survivors, neurologic recovery was complete in five (45%), partial in five (45%), and absent in one (9%). CONCLUSIONS: In our cohort, recovery of PM RMS associated CNP was often incomplete despite multi-modal therapy. A larger cohort of patients is required to determine the utility of emergent initiation of radiation or corticosteroids. This study will facilitate the counseling of future families on the long-term neurologic recovery CNP in PM RMS.

Paralysis of cranial nerve and striking prognosis of cervical necrotizing fasciitis.

Necrotizing soft-tissue infection (NSTI) is a bacterial infection with necrosis of the cutaneous, subcutaneous tissue and fascia with sparing of the underlying muscle. The most frequent initiating factor reported, for necrotizing fasciitis, in the head and neck region is a primary odontogenic infection or postextraction infection, abrasion, and laceration of the face or scalp. Necrotizing fasciitis can progress rapidly to systemic toxicity and even death if not promptly diagnosed and treated. If the patient has any risk factors, this can worsen the prognosis. In this study, 2 cases of NSTI with dental pathology history (one with the spreading to mediastinum and the other spreading to suprahyoid) were discussed with a review of the literature. One of the cases had diabetes mellitus, but interestingly, she had a better prognosis, and she was discharged asymptomatic. In addition, the other case had no any risk factors, but he had a worse clinical cranial nerve paralysis (a rare complication of NSTI) and died. As a conclusion, despite the intensive therapy, large debridement, and antibiotics with large spectrum, the delay in the patient's diagnosis and treatment increased mortality. We aimed to attract attention to the importance of dental pathologies and early diagnosis.

Causes and treatment outcomes of third, fourth and sixth cranial nerve palsy.

OBJECTIVE: To evaluate the causes and the treatment outcomes of third, fourth and sixth cranial nerve palsy MATERIAL AND METHOD: Medical records of 157 cases with extra-ocular muscle palsy from third, fourth or sixth cranial nerve palsy between January 1995 and December 2009 were reviewed. Demographic data, age, causes of extra-ocular muscle palsy and treatment outcomes were record and analyzed. The patients who were followed-up less than 6 months, myasthenia gravis and extra-ocular muscles fibrosis were excluded from the present study. RESULTS: One hundred and fifty-seven cases from 600 cases were included in the present study. The most common cranial nerve palsy was the sixth cranial nerve with 63 cases (40.1%). Of 157 cases, the causes were trauma 41 cases (26.1%), microvascular occlusion cause 34 cases (21.7%) and congenital cause 21 cases (13.4%). Cranial nerve palsy patients from microvascular occlusion cause spontaneously improved 25 of 34 cases (73.50%) in 6 months. All patients (21 cases)from congenital cranial nerve palsy had extra-ocular muscle surgery and 17 patients (80.9%) were successful. CONCLUSION: Sixth cranial nerve palsy was the most common cranial nerve palsy. Most patients with cranial nerve palsy from vascular cause spontaneously improved in 6 months. Congenital cranial nerve palsy patients need extra-ocular muscle surgery and most cases were successful

[Clinical vignette for the medical school student: jugular foramen syndrome]. / La vignette diagnostique de l'etudiant. Syndrome du trou déchiré postérieur (foramen jugulaire).

Cranial neuropathies are frequent and their semiological analysis is the basis of the diagnostic workup. This is even more true in the case of multiple cranial neuropathies. We here propose a diagnostic exercise in the case of a simultaneous cranial nerves IX (glossopharyngeal), X (vagus) and XI (spinal) deficit. This case exemplifies that knowledge of nervous anatomy and physiology is the basis of the semiology of the nervous system.

Middle Meningeal Artery Embolization for Chronic Subdural Hematoma Using N-Butyl Cyanoacrylate With D5W Push Technique.

BACKGROUND: Middle meningeal artery (MMA) embolization has been recognized as a promising treatment for patients with subdural hematoma (SDH). OBJECTIVE: To present the technical feasibility and efficacy of n-butyl cyanoacrylate (n-BCA) embolization in the largest consecutive cohort to date. METHODS: We retrospectively reviewed our consecutive cases of recurrent SDH treated with MMA embolization using diluted n-BCA with the "sugar rush" technique. In brief, a 2.1-Fr microcatheter was used to selectively catheterize the frontal and posterior branches of the MMA. 5% dextrose in water (D5W) was injected through an intermediate catheter while injecting n-BCA through the microcatheter. Complete obliteration of MMA and lack of SDH recurrence in a 3-6 months follow-up computed tomography scan were defined as efficacy outcomes. Cranial nerve palsy, vision loss, transient neurological deficit, and stroke were defined as safety outcomes. RESULTS: A total of 61 patients were identified with a mean (±standard deviation) age of 62.5 ± 9 years. In 6 patients (10%), coil embolization of the origin of the frontal or posterior branch was performed because super-selective catheterization of the branch was unsuccessful because of tortuous anatomy. Complete obliteration of frontal and posterior branches was achieved in 100% of the cases. Recurrent SDH was seen in 3 patients (5%). No incidence of cranial nerve palsy, vision loss, or stroke occurred. One patient suffered a transient neurological deficit. CONCLUSION: MMA embolization using diluted n-BCA with concomitant D5W injection is associated with a high degree of distal penetration and complete branch occlusion and minimal risk of cranial nerve palsy or other thromboembolic complications.