Cranial nerve involvement, visual complications and headache syndromes in Lyme disease.

PURPOSE OF REVIEW: To provide a summary of the visual manifestations and cranial neuropathies seen in Lyme disease. RECENT FINDINGS: Lyme facial palsy remains the most common manifestation of Lyme neuroborreliosis. Recent investigations show likely evidence of vagal involvement in Lyme disease. SUMMARY: The literature on Lyme neuroborreliosis continues to evolve. Lyme disease can affect nearly any cranial nerve in addition to causing various headache syndromes. The most common manifestation is Lyme disease facial palsy, occurring in up to 5-10% of patients with documented Lyme disease. Headache syndromes are common in the context of facial palsy but can occur in isolation, and more specific headache syndromes including trigeminal and geniculate neuralgias can occur rarely. Signs and symptoms indicative of vestibulocochlear nerve involvement are relatively common, although it could be that these represent other vestibular involvement rather than a specific cranial neuropathy. Optic neuritis is a controversial entity within Lyme disease and is likely overdiagnosed, but convincing cases do exist. Physicians who see any cranial neuropathy, including optic neuritis, in an endemic area can consider Lyme disease as a possible cause.

Retropharyngeal abscess due to Fusobacterium necrophorum complicated by progressive internal carotid artery stenosis and multiple cranial nerve palsies.

BACKGROUND: A case of retropharyngeal abscess complicated by both artery and nerve injury has rarely been reported. METHODS: A 36-year-old woman suddenly presented with right eye visual loss, dilated pupil, reduced direct light reflex, ptosis and ocular motility disorder on the side of inflammation progression, and was diagnosed with retropharyngeal abscess due to Fusobacterium necrophorum. The patient was treated only with antibiotics and, no further surgery was necessary but tracheotomy. Four months later, MRA showed right ICA occlusion and left ICA stenosis. MRI revealed continuous spread of inflammation due to the abscess from the retropharyngeal to the intracranial space. RESULTS: These severe complications would be attributed to an endothelial damage to the arterial wall and an ischemic neuropathy caused by inflammation and thrombogenesis due to Fusobacterium necrophorum. CONCLUSIONS: This case should provide a better understanding of the mechanism of vascular and cranial nerve injury due to retropharyngeal infections, and highlights the need for early antibiotic therapy and repeated vascular evaluation.

Comparison of Perioperative Safety of Carotid Artery Stenting and Endarterectomy in the Treatment of Carotid Artery Stenosis: A Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Current management guidelines for the treatment of carotid stenosis are controversial. We performed this meta-analysis to evaluate the perioperative safety of carotid artery stenting (CAS) and endarterectomy. METHODS: We systematically searched EMBASE, PubMed, Web of Science, and the Cochrane Library from inception to November 10, 2022, for randomized controlled trials that compared CAS with carotid endarterectomy (CEA) among patients with carotid stenosis. The analyzed outcomes mainly included stroke, death, myocardial infarction (MI), cranial nerve palsy, the cumulative incidence of mortality, stroke, or MI and the cumulative incidence of death or stroke in the perioperative periods. The risk ratio (RR) and 95% confidence interval (95% CI) were calculated and pooled. Subgroup analyses were based on whether patients were symptomatic or asymptomatic. We assessed the certainty of evidence using the Grading of Recommendations Assessment, Development and Evaluation framework. RESULTS: Seventeen randomized controlled trials with 12,277 participants (6514 and 5763 in the CAS and CEA groups, respectively) were included. Pooled analysis demonstrated that compared with CEA, CAS was associated with decreased risks of perioperative MI (RR = 0.47, 95% CI = 0.29∼0.77) and perioperative cranial nerve palsy (RR = 0.02, 95% CI = 0.01∼0.06) but higher risks of perioperative stroke (RR = 1.48, 95% CI = 1.18∼1.87) and cumulative incidence of death or stroke (RR = 1.52, 95% CI = 1.20∼1.93). CONCLUSIONS: The perioperative safety was equivalent between CAS and CEA. However, CEA may be preferred when considering both procedural safety and long-term efficacy in preventing recurrent stroke.

Spinal procedures, pneumocephalus, and cranial nerve palsies: A review of the literature.

Purpose: Minimally invasive and surgical spine procedures are commonplace with various risks and complications. Cranial nerve palsies, however, are infrequently encountered, particularly after procedures such as lumbar punctures, epidural anesthesia, or intrathecal injections, and are understandably worrisome for clinicians and patients as they may be interpreted as secondary to a sinister etiology. However, a less commonly considered source is a pneumocephalus which may, in rare cases, abut cranial nerves and cause a palsy as a benign and often self-resolving complication. Here, we present the case of a patient who underwent an intrathecal methotrexate infusion for newly diagnosed non-Hodgkin's T-cell lymphoma and subsequently developed an abducens nerve palsy due to pneumocephalus. We highlight the utility of various imaging modalities, treatment options, and review current literature on spinal procedures resulting in cranial nerve palsies attributable to pneumocephalus presenting as malignant etiologies.

Traumatic cavernous sinus syndrome - A peculiar presentation of multiple cranial nerve neuropathies following a minor head injury: Case report and literature review.

BACKGROUND: In patients with traumatic head injuries, the percentage of cranial nerve injuries (CNI) range from4.3 to 17.6% in which majority are isolated CNI[1-5].In present literature, moderate to severe types of head injuries are often studied which may result in a lack of representation and description of CNI associated with minor head injuries (MHI). Alongside this peculiar case of a traumatic cavernous sinus syndrome (CSS) that is non-thrombotic and non-fistulous in nature, this paper aims to analyse traumatic CNI in non-severe head injuries and the surrounding literature. CASE REPORT: A 65-year-old man who had sustained a minor head injury was found to have CNI of III, IV and VI.Brain imaging showed scattered traumatic subarachnoid haemorrhage and a non-displaced right zygomatic arch fracture. Despite the short course of high dose dexamethasone, he showed only partial recovery of his CNI after one year. CONCLUSION: We present a case of traumatic CSS likely secondary to tractional injury from a MHI. Injury to the extraocular nerves wasfound to be one of the more commonly observed combination of CNI from the literature review conducted. In patients with MHI, multiple CNI is less common. Hence, consideration should be given to work upfor secondary causes such as tumours. There is presently no known clear identifiable pattern of CNI associated with MHI. CT brain findings of skull base fractures and early onset of cranial nerve palsies are generally associated with worse outcomes. More remains to be studied about tractional CNI in non-severe head injuries.

Cranial nerve Palsy following COVID-19 Vaccination Responsive to Plasma Exchange.

BACKGROUND: Globally, mass COVID-19 vaccine administration has revealed various adverse effects of the vaccine, such as various neurological symptoms, which are currently identified as a result of an excessive immune response. CASE REPORT: A 70-year-old woman presented with progressive unilateral oculomotor nerve palsy and decreased visual acuity 12 days after receiving the Moderna COVID-19 vaccine. In adults, such palsy is typically caused by microvascular disease (ischemia) or compressive tumors. Given the temporal relationship between vaccination and symptoms and the exclusion of other possible causative factors, the patient's oculomotor nerve palsy and optic nerve involvement was considered to be related to the vaccination. Cranial nerve palsy following COVID-19 vaccination was diagnosed, and after pulse steroid and plasma exchange, the patient showed steady recovery. CONCLUSION: Our patient with cranial nerve palsy following COVID-19 vaccination responded well to plasma exchange after limited improved toward steroid. This case highlights the importance of early identification and treatment of the immunological effects of COVID-19 vaccines on cranial nerves.

Third cranial nerve palsy due to COVID-19 infection.

We report a case of a previously healthy man in his 40s who presented with mild SARS-CoV-2 infection (COVID-19) concomitant with acute onset of left third cranial nerve palsy with restricted supraduction, adduction and infraduction. Our patient did not present any history of hypertension, hyperlipidaemia, diabetes mellitus or smoking. The patient recovered spontaneously without any antiviral treatment. To our knowledge, this is the second report of third cranial nerve palsy spontaneously resolved without any risk factors of vascular disease, specific image findings, nor any possible causes other than COVID-19. In addition, we reviewed 10 other cases of third cranial nerve palsy associated with COVID-19, which suggested that the aetiology varies greatly. As a clinician, it is important to recognise COVID-19 as a differential diagnosis for third cranial nerve palsy. Finally, we aimed to encapsulate the aetiologies and the prognosis of the third cranial nerve palsy associated with COVID-19.

The Role of Stereotactic Radiosurgery for Functioning and Nonfunctioning Pituitary Adenomas.

Pituitary adenomas are benign brain tumors that comprise 10%-20% of all central nervous system neoplasms. In recent years, stereotactic radiosurgery (SRS) has emerged as a highly effective treatment option in the management of functioning and nonfunctioning adenomas. It is associated with tumor control rates frequently ranging from 80% to 90% in published reports. While permanent morbidity is uncommon, potential side effects include endocrine dysfunction, visual field deficits, and cranial nerve neuropathies. In patients where single fraction SRS would pose an unacceptable risk (e.g. large lesion size or close proximity to the optic apparatus), hypofractionated SRS delivered in 1-5 fractions is a potential treatment option; however, available data are limited. A comprehensive literature search of PubMed/MEDLINE, CINAHL, Embase, and the Cochrane Library was conducted to identify articles reporting on the use of SRS in functioning and nonfunctioning pituitary adenomas.

Clinical Features of Pituitary or Parasellar Tumor Onset with Cranial Nerve Palsy: Surgical Intervention Considerations.

OBJECTIVE: This study aimed to clarify the symptoms of pituitary or parasellar tumor onset with cranial nerve palsy (CNP) and to improve our knowledge of this rare symptom and its most appropriate treatment. METHODS: Among 1281 patients with pituitary or parasellar tumors surgically treated from 2003 to 2020, 30 cases (2.34%; 15 men and 15 women; mean age: 55.6 years, range: 6-83 years) first presenting with CNP were reviewed to evaluate the neurological symptoms, histological diagnosis, interval from onset to surgery, and time before complete CNP recovery. RESULTS: Pathological diagnoses comprised 17 pituitary adenomas, including 10 pituitary apoplexies and 4 adrenocorticotropic hormone-positive adenomas, and 13 other tumors, including 3 chordomas, 2 xanthogranulomas, 2 malignant lymphomas, 2 metastatic tumors, 1 Rathke cleft cyst, 1 plasmacytoma, 1 craniopharyngioma, and 1 neuroendocrine carcinoma. The mechanisms causing CNP were pituitary apoplexy (n = 10), cranial nerve compression or involvement (n = 17), and inflammatory changes (n = 9). As the first manifestation, 20 (66.7%) patients presented with oculomotor nerve palsy, 2 (6.7%) with trochlear nerve palsy, and 13 (43.3%) with abducens nerve palsy. Full recovery of CNP was obtained in 25 patients (83.3%) after surgery alone and in 2 patients (6.7%) after adjuvant therapy. Early surgery provided no significant difference in full recovery rates although it reduced the time to reach full recovery. CONCLUSIONS: It is critical to determine the mechanisms of CNP and intervene surgically to improve symptoms, shorten the duration of the disorder, prevent relapses, and obtain the correct pathological diagnosis to select the proper adjuvant therapy.

[Varicella-zoster virus infection with concomitant lower cranial polyneuropathy in the absence of fever, headache and meningeal signs].

We present varicella-zoster virus (VZV) infection with concomitant lower cranial polyneuropathy in the absence of meningeal symptoms. Physical examination showed involvement of cranial nerves IX and X in Case 1 and of cranial nerves IX, X, and XI in Case 2. Cerebrospinal fluid (CSF) analysis revealed mild lymphocytic pleocytosis, normal protein levels, and absence of VZV-DNA based on polymerase chain reaction (PCR) analysis. Serum anti-VZV antibody testing showed positive results in both cases, which confirmed the diagnosis of VZV infection. VZV infection accompanied by lower cranial polyneuropathy is rare; therefore, it is important to consider VZV reactivation as an etiopathogenetic contributor to pharyngeal palsy and hoarseness. We emphasize the importance of serological analysis for precise diagnosis in VZV infection with multiple lower cranial nerve palsies because the VZV-DNA PCR test may show negative results in patients without meningitis symptoms or in those with normal CSF protein levels.

Internal Carotid Artery Aneurysm Presenting as Lower Cranial Nerve Palsies.

The signs of lower cranial nerve palsies are rare and are often caused by tumors. A 49-year-old woman was admitted to our hospital with progressive right-sided atrophy of the tongue, sternocleidomastoid and trapezius, dysarthria, and dysphagia for 3 years. Brain magnetic resonance imaging revealed a circular lesion adjacent to the lower cranial nerves. Cerebral angiography confirmed that the lesion was an unruptured aneurysm in the C1 segment of the right internal carotid artery. After endovascular treatment, the symptoms of this patient had partially improved.

Onset-to-treatment time and aneurysmal regression predict improvement of cranial neuropathy after flow diversion treatment in patients with symptomatic internal carotid artery aneurysms.

BACKGROUND: Although flow diversion plays a pivotal role in treating internal carotid artery aneurysms presenting with cranial neuropathy, predictors of symptom improvement have not been established. OBJECTIVE: To investigate improvement of symptoms after flow diversion treatment in patients with internal carotid artery aneurysms causing cranial neuropathy, with sufficient follow-up period. Additionally, to examine factors associated with improvement of symptoms. METHODS: This retrospective multicenter study examined patients with unruptured internal carotid artery aneurysms presenting with cranial neuropathy who were treated using flow diversion and followed up for at least 12 months. Study outcomes were transient worsening of symptoms and symptom status 12 months after treatment. Patient and aneurysm characteristics were statistically analyzed. RESULTS: Seventy-seven patients were included. Data needed for outcome analysis were available for 66 patients. At the 1-, 3-, 6-, 12-month, and last follow-ups, the proportion of patients with resolved or improved symptoms was 26% (20/77), 51% (39/77), 74% (57/77), 83% (64/77), and 79%(62/77), respectively. Symptom onset-to-treatment time <6 months (OR=24.2; 95% CI 3.09 to 188.84; p=0.002) and aneurysmal regression (OR=23.1; 95% CI 1.97 to 271.75; p=0.012) were significantly associated with symptom improvement. Transient symptom worsening and worse symptoms at 12 months occurred in 19/77 (25%) and 2/77 (3%) patients, respectively. CONCLUSIONS: The rate of cranial neuropathy symptom improvement after flow diversion increased over the first 12 months after treatment, but not thereafter. Treatment within 6 months of symptom onset and aneurysmal regression were predictors of symptom improvement.

Modern Management of Complex Tympanojugular Paragangliomas: Systematic Review and Meta-Analysis.

OBJECTIVE: Tympanojugular paragangliomas (TJPs) are slow-growing tumors arising within the middle ear or jugular foramen. The development of modified skull base approaches and the increasing use of stereotactic radiosurgery have provided more modern techniques in the management of TJPs. Several factors dictating approach selection, and resulting clinical outcomes have been inconsistently described. METHODS: A systematic review of the literature describing modern management of complex TJPs was performed and summarized. A random-effects meta-analysis was performed to describe the rate of tumor control, complications, and symptom improvement in patients undergoing radiosurgery or surgical resection. RESULTS: Nineteen studies were identified with a total of 852 TJP patients. A minority (153 patients) underwent radiosurgery while 699 underwent surgery. On meta-analysis, there was a 3.5% (95% confidence interval [CI]: 0.5%-6.4%) tumor growth rate following radiosurgery and 3.9% (95% confidence interval [CI]: 1.8%-6.0%) recurrence rate in surgical resection, with no significant moderator effect between the 2 groups (P = 0.9046). Complication rate for radiosurgery was 7.6% (95% CI: 2.8%-12.4%), differing significantly from surgical complication rates of 29.6% (95% CI: 17.1-42.0%, P = 0.0418). CONCLUSIONS: Stereotactic radiosurgery and surgical resection for TJPs have similar rates of tumor recurrence. Radiation is associated with less risk and lower morbidity, yet there is comparably modest reduction of the tumor size. In sum, the data suggest that radiosurgery is a reasonable management option for patients with minimal symptoms who are high risk for surgery. Microsurgical resection should be reserved for patients with lower cranial neuropathies or those who have failed radiation treatment.

Is Hypertrophic Pachymeningitis Really Idiopathic?

Background: Hypertrophic pachymeningitis (HPM) is a unique disorder characterized by thickening and fibrosis of the dura mater. Clinically it presents with headache, cranial nerve palsies, and other focal neurological deficits. Two forms exist, one is primary, where all other causes have been excluded and the other is secondary where an identifiable cause exists. It is important to recognize these secondary causes as treatment depends on the etiology. Objective: To elucidate the various characteristics of HPM. To delineate clinical-radiological features that help differentiate secondary from primary causes and to understand treatment response and disease outcomes of HPM. Methods: This retrospective observational study included 33 patients who presented with radiological diagnosis of HPM from January 2014 to July 2019. Spontaneous intracranial hypotension patients were excluded. All patients were extensively evaluated for secondary causes and treatment outcomes were analyzed on follow-up. Results and Conclusions: Secondary causes of HPM were present in 48% cases. The clue for primary causes is an associated Tolosa-Hunt syndrome. Secondary causes in our series are immunological, infection, and malignancy. Clues to differentiate primary from these secondary causes are clinical like myelopathy, seizures, poor response to immunosuppression; radiological like hypertrophic cranial nerves, infarcts, bony erosion, and leptomeningeal involvement. There are case reports in literature but large Indian studies are lacking. This manuscript presents a large cohort of cases with HPM, which helps differentiate primary from secondary causes, as management and prognosis depend on etiology. An algorithm depicting the approach to the management of HPM has been presented.

Case Report: Para-infectious cranial nerve palsy after bacterial meningitis.

A 27-year-old woman was admitted to our hospital for fever, associated with headache, nausea, and vomiting, and she rapidly developed mild left facial nerve palsy and diplopia. Neurological examination revealed mild meningitis associated with bilateral VI cranial nerve palsy and mild left facial palsy. As central nervous system (CNS) infection was suspected, a diagnostic lumbar puncture was performed, which revealed 1,677 cells/µl, 70% of which were polymorphonuclear leukocytes. Moreover, multiplex PCR immunoassay was positive for Neisseria meningitidis, supporting the diagnosis of bacterial meningitis. Finally, IgG oligoclonal bands (IgGOB) were absent in serum and cerebrospinal fluid (CSF). Therefore, ceftriaxone antibiotic therapy was started, and in the following days, the patient's signs and symptoms improved, with complete remission of diplopia and meningeal signs within a week. On the contrary, left facial nerve palsy progressively worsened into a severe bilateral deficit. A second lumbar puncture was therefore performed: the CSF analysis revealed a remarkable decrease of pleocytosis with a qualitative modification (only lymphocytes), and oligoclonal IgG bands were present. A new brain MRI was performed, showing a bilateral gadolinium enhancement of the intrameatal VII and VIII cranial nerves bilaterally. Due to suspicion of para-infectious etiology, the patient was treated with oral steroid (prednisolone 1 mg/kg/day), with a progressive and complete regression of the symptoms. We suggest that in this case, after a pathogen-driven immunological response (characterized by relevant CSF mixed pleocytosis and no evidence of IgGOB), a para-infectious adaptive immunity-driven reaction (with mild lymphocyte pleocytosis and pattern III IgGOB) against VII and VIII cranial nerves started. Indeed, steroid administration caused a rapid and complete restoration of cranial nerve function.

[CASE SERIES OF EARLY NEUROIMAGING IN PATIENTS WITH VASCULAR RISK FACTORS AND ISOLATED SIX CRANIAL NERVE PALSY, DEMONSTRATING THE DILEMMA OF EARLY IMAGING].

INTRODUCTION: There are various etiologies for isolated third, fourth and sixth cranial nerve palsies. The most common etiology in adults aged 50 years and older with vascular risk factors such as diabetes, hyperlipidemia, and hyperlipidemia, is microvascular ischemia. The role of early neuroimaging in older patients with vascular risk factors presenting with acute isolated ocular motor nerve palsy is controversial. AIMS: We present six cases of patients aged 50 years and older, with vascular risk factors, who suffered from acute isolated paralysis of the sixth nerve. All cases were diagnosed with a non-microvascular ischemia etiology. RESULTS: The identified etiologies included petroclival meningioma with involvement of the cavernous sinus, metastatic hepatoma, plasmacytoma, venous thrombosis, arterial-venous malformation and aneurysm. CONCLUSIONS: Despite the high cost and presumed low yield, early neuroimaging can change the clinical management in certain cases. DISCUSSION: Previous studies have found the yield of early neuroimaging in older vasculopathic patients with acute 4th or 6th cranial nerve palsies to be 1-15%. In spite of these reports, our cases demonstrate the need for early onset imaging in these cases.

A case report of unilateral cervical lymphadenopathy and multiple cranial neuropathies following mRNA-COVID-19 vaccination.

BACKGROUND: We report a rare case of ipsilateral multiple cranial neuropathy and ipsilateral lymphadenopathy following mRNA-COVID-19 vaccination. CASE PRESENTATION: A 41-year-old male visited our emergency room complaining of dysphagia and hoarseness that started a week after receiving COVID19 mRNA vaccination (in his right arm). During his hospitalization, he also complained of right side hearing loss and diplopia. Neurological examination depicted a right IV nerve palsy, ipsilateral facial paresthesia and peripheral facial paresis. Otorinolaryngological examination revealed right vocal cord paralysis. A brain magnetic resonance imaging showed enhancement of the right VII and VIII cranial nerves in the auditory canal. The lumbar puncture revealed increased protein concentration and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Additionally, a neck computed tomography (CT) scan showed a swollen right supraclavicular lymph node. We hypothesize that the ipsilateral cranial neuropathies of IV, VI, VII, VIII and X, associated with cervical lymphadenopathy, was possible caused by a post-vaccination immune-mediated reaction. The patient was treated with a 5-day course of intravenous methylprednisolone (1000 mg/day), and a gradual improvement was observed. CONCLUSIONS: Similarly, to other vaccines, it is possibly that also mRNA vaccines may act as triggers of non-specific autoimmune neurological syndromes.

Early Disseminated Lyme Disease: Cranial Neuropathy, Meningitis, and Polyradiculopathy.

Early disseminated Lyme disease can involve the peripheral or central nervous system, but with early diagnosis and treatment, prognosis for full recovery is excellent. The typical clinical presentations of neuroborreliosis are highlighted, and an approach to diagnosis and treatment is described.