Cranial Neuropathies Research Articles

CNSC-09. OTOSYPHILIS MIMICKING A VESTIBULAR SCHWANNOMA

Abstract Vestibular schwannomas are common nonmalignant primary brain tumors representing up to 80% of the cerebellopontine angle (CPA) tumors. Their diagnosis is made radiographically in most cases with the presence of an enhancing mass along the 8th nerve complex. The United States is experiencing a surge in the number of recorded syphilis cases with an approximate 80% increase since 2018 according to the CDC, after reaching an all-time low in the year 2000. Syphilis can involve the Central Nervous System (CNS) in its early, late, and latent forms, and in its early stages it typically presents in the form of meningitis which can be accompanied by cranial neuropathies especially of the nerves II, VII, and VIII. We present a case of a 35-year-old man who presented with subacute onset unilateral left-sided hearing loss and dizziness that did not respond to prescribed steroids. Magnetic resonance imaging (MRI) revealed an enhancing mass in the left cerebellopontine angle presumed to be a vestibular schwannoma. The patient was subsequently planned for radiosurgery, however, MRI done during the procedure revealed that the mass was of a smaller size. The patient was referred to neuro-oncology and work up included cerebrospinal fluid (CSF) analysis that showed 3 white blood cells, normal protein and glucose, and negative VDRL. Cytology, flow cytometry, and inflammatory markers were negative. However, the Treponema pallidum antibodies were positive confirming otoshyphilis. The patient was treated accordingly with a course of Penicillin G with near resolution of the enhancement of the 8th nerve complex. His hearing remained impaired on his initial follow up, however, his facial weakness near resolved. This case brings up an important diagnostic question in cases of presumed schwannomas where patients would be otherwise treated solely based on the MRI appearance, especially given the re-emergence of syphilis.

SURG-02. ANAPLASTIC PLEOMORPHIC XANTHOASTROCYTOMA WITH LEPTOMENINGEAL DISSEMINATION PRESENTING WITH CRANIAL NEUROPATHY IN AN ADULT PATIENT: ILLUSTRATIVE CASE AND REVIEW OF THE LITERATURE

Abstract INTRODUCTION Anaplastic pleomorphic xanthoastrocytomas (APXA) are very rare, grade III malignant astrocytic glial neoplasms first described as a distinct entity in the 2016 World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS). They are generally seen in pediatric and young adult patients as supratentorial lesions with both solid and cystic components and have a high propensity for recurrence despite local treatment. APXAs occasionally demonstrate leptomeningeal dissemination (LMD) but extremely seldom at the time of diagnosis. They are typically treated via surgical resection with adjuvant chemoradiation, although there is no standard of care for adjuvant therapy. Case Presentation: The authors describe the case of a 37-year-old male with a history of seizures since age 11 who presented with four months of worsening headache, increasingly frequent seizures, and diplopia with right cranial nerve III palsy. Imaging at the time of diagnosis of his seizure disorder as a child demonstrated a small left temporal lesion, which remained stable on imaging for over 20 years and was never biopsied, however MRI at current presentation showed significant interval enlargement with intralesional hemorrhage and leptomeningeal enhancement involving the left temporal and parietal lobes, left internal auditory canal, and right oculomotor nerve. He underwent surgical resection with gross total resection achieved and pathology revealed WHO Grade III pleomorphic xanthoastrocytoma with ATG7::RAF1 fusion, 9p21 deletion, and TERT promoter mutation on next generation sequencing. This was followed by adjuvant whole brain radiation with boost and treatment with MEK inhibitor cobimetinib. His postoperative course was remarkable for intermittent speech difficulty and improving right third nerve palsy. CONCLUSION We describe the unusual presentation of an already exceedingly rare primary CNS tumor, namely APXA in an adult patient with LMD and cranial neuropathy at the time of diagnosis, and detail tailored adjuvant therapy based on genomic profiling.

Neuroborreliosis

Background Neuroborreliosis is involvement of the nervous system in Lyme disease and can often be overlooked as a diagnosis in patients presenting with cranial neuropathies and meningitis in an endemic area. There can be variability in presentation with respect to the involved cranial nerves, making the diagnosis challenging. To the best of our knowledge, there have been no reported cases of Lyme disease cranial neuropathy occurring with simultaneous periorbital skin and soft tissue inflammation in the literature. Case We present the case of a healthy 25-year-old man who presented to the emergency department with headache, diplopia, and left eye pain after experiencing fever, malaise, and a recent history of unilateral facial palsy that improved a few weeks prior. He lived in a Lyme disease endemic area and often encountered ticks. He was found to have an abduction deficit in his left eye, preseptal and postseptal orbital inflammation with bilateral optic perineuritis, and a lymphocytic pleocytosis in the cerebrospinal fluid accompanied by an elevated antibody index, consistent with a diagnosis of Lyme neuroborreliosis. He was treated with doxycycline and empiric bacterial coverage, with symptom resolution at follow-up. Conclusion This case represents an unusual combination of cranial neuropathies and is the first report of orbital inflammation driven by Lyme neuroborreliosis, thought to be caused by contiguous spread of inflammation from affected portions of the nervous system. Although neuroborreliosis is less commonly encountered in the United States than in Europe, providers should maintain a high index of suspicion for this entity when patients present with multiple cranial neuropathies in an endemic area and not exclude Lyme disease on the basis of skin and soft tissue inflammation.

When Bell's Palsy Is Cancer: Avoiding Misdiagnosis and Its Implications.

Facial paralysis due to cancer can be misdiagnosed as Bell's palsy. This study aims to clearly identify and quantify diagnostic differentiators and further evaluate the prognostic implications of misdiagnosis. Adult patients older than 18 years with facial palsy of unknown or cancerous etiology presenting between 2009 and 2023 were reviewed. Patient characteristics, examination findings, and clinical course were compared between facial paralysis patients with cancer misdiagnosed as Bell's palsy (Cancer-Bell's-Palsy group) and patients correctly diagnosed with Bell's palsy (Bell's-Palsy group). Additionally, morbidity and mortality were compared between facial paralysis patients with cancer initially misdiagnosed with Bell's palsy and facial paralysis patients initially correctly diagnosed with cancer (Cancer-Palsy group). Two-hundred and forty-three patients participated including 43 Cancer-Palsy, 18 Cancer-Bell's-Palsy, and 182 Bell's-Palsy patients. Cancer-Bell's-Palsy patients were significantly less likely than Bell's-Palsy patients to develop synkinesis (odds ratio [OR] = 0.0042; 95% confidence interval [CI]: [0.0005-0.0339]; p < 0.0001), significantly more likely to experience gradual onset facial paralysis (OR = 1,004.69; 95% CI: [54.40-18,555.77]; p < 0.0001), and significantly more likely to have additional nonfacial cranial nerve neuropathies (OR = 49.98; 95% CI: [14.61-170.98]; p < 0.0001). Cancer-Bell's-Palsy patients were more likely than Cancer-Palsy patients to have a greater than 6-month period from initial cancer-attributable symptom onset to cancer diagnosis (OR = 47.62; 95% CI: [9.26-250.00]; p < 0.001), stage IV cancer (OR: 12.36; 95% CI: 1.49-102.71; p = 0.006), and decreased duration of life after cancer diagnosis (median [interquartile range], 40.0 [87.0] vs. 12 [56.3] months, respectively; p = 0.025). Facial paralysis related to cancer must be differentiated from Bell's palsy, as misdiagnosis leads to delayed intervention and poorer prognosis. Gradual onset facial palsy, multiple cranial nerve neuropathies, lack of synkinesis, and lack of improvement were nearly definitive differentiators for underlying cancer.

Rare neurological and neuropsychiatric manifestations of scrub typhus: a case series of 10 cases

IntroductionScrub typhus is a potentially life-threatening but curable disease that can produce multi-organ failure. Neurological manifestations in scrub typhus have gained attention recently, where the entire neural axis except the myoneural junction can be involved. Although the pathogenesis of neurological involvement has not been established, immune-mediated mechanisms are suspected. This article reports the clinicopathological features of scrub typhus cases presenting several rare neurological and neuropsychiatric manifestations. MethodsThree hundred fifty-four serologically confirmed scrub typhus cases were admitted to the Department of General Medicine of Burdwan Medical College and Hospital (West Bengal, India) between May 2018 and May 2022. There were 50 patients who had predominantly neurological manifestations. Of these 50 cases, ten patients presented with extremely rare neurological manifestations. ResultsWe report 10 cases of scrub typhus (four men and six women) who presented with complex neurological pictures (posterior reversible encephalopathy syndrome, Opalski syndrome, parkinsonism, cerebellitis, isolated opsoclonus, acute transverse myelitis, myositis, polyradiculoneuropathy with cranial neuropathy, acute transient behavioral changes, and fibromyalgia). Immune-mediated mechanisms might have mediated the pathogenesis of most cases following scrub typhus infection. ConclusionFrom a clinicopathological point of view, each case was unique in its presentation and treatment response. In any acute onset neurological disorders associated with febrile illness in the tropics or subtropics, scrub typhus infection should be included in the differential diagnosis, despite the absence of eschar and unremarkable neuroimaging findings. This otherwise curable disease may result in multi-organ dysfunction syndrome and death if the diagnosis is delayed.

Cranial neuropathy following coronavirus disease 2019 vaccination in kidney transplant recipients.

Understanding of adverse reactions to coronavirus disease 2019 vaccines remains limited. Case 1: A 52-year-old woman, post-kidney transplantation, experienced sudden vision loss in her left eye after receiving a second dose of coronavirus disease 2019 vaccine. She was diagnosed with ischemic optic neuropathy.Case 2: A 53-year-old woman, post-kidney transplantation, presented with worsening diplopia and left eye pain following the second dose of coronavirus disease 2019 vaccine. She was diagnosed with left abducens nerve palsy. Vigilance is essential for recognizing the potential for delayed cranial neuropathy in immunocompromised individuals after coronavirus disease 2019 vaccination.

Hypocalcemia-induced seizures in a peritoneal dialysis (PD) patient: A case report.

A thorough evaluation is necessary for seizures caused by hypocalcemia, both during the patient's presentation and following their recovery from the postictal seizure episode. This is because the underlying cause of hypocalcemia must be ruled out in order to ensure the best possible clinical outcome from calcium and vitamin D therapy. Patients with multiple systemic issues, including neurological involvement and seizure development, are not uncommon among nephrologists. Both the central and peripheral neural systems can be impacted by kidney disease. The main symptoms are myopathy, cranial or peripheral neuropathy, cognitive impairment, and seizures. A 22-year-old female with an unusual medical history who had been known to have end-stage kidney disease (ESKD) for a year and regularly had CAPD (continuous ambulatory peritoneal dialysis) suffered from two episodes of tonic-colonic seizures 2 weeks apart. On physical examination, symptoms of tongue biting, decreased vision in the left eye, and mild bilateral pulmonary air entry were notable. Upon examination, there was evidence of severe hypocalcemia, hyponatremia, a high renal profile (urea and creatinine), anemia, and a severe vitamin D deficiency. Her peritoneal dialysis (PD) prescription was reviewed, her seizures ceased, and she was released from the hospital after the hypocalcemia was treated with intravenous calcium and high doses of vitamin D. The issue of hypocalcemic seizures should be carefully evaluated both at the presentation and after the patient recovers from the postictal stage. By following this, seizure episodes can be prevented with good success if patients strictly adhere to the medication for which they are responsible.

Trigeminal neuralgia, demyelinating polyneuropathy, and central nervous system involvement in a patient with an SH3TC2 mutation

Abstract Background Charcot-Marie-Tooth type 4C (CMT4C) is a slowly progressive, autosomal recessive, sensorimotor polyneuropathy characterized by demyelination and distinct clinical features, including cranial nerve involvement. CMT4C is associated with pathogenic mutations in the SH3TC2 gene. Methods A patient presenting with gait instability due to demyelinating polyneuropathy and refractory trigeminal neuralgia underwent comprehensive evaluation. Nerve conduction studies, magnetic resonance imaging (MRI) of the brain, cervical spine, and thoracic spine, lumbar puncture, and genetic test through next generation sequencing were performed. Results The genetic test found an Arg1109Stop mutation in the SH3TC2 gene, associated with demyelinating polyneuropathy and cranial neuropathy. Interestingly, brain MRI showed multiple, nonenhancing white matter hyperintensities. This is the first case of CMT4C associated with white matter lesions. Conclusion Any patient with slowly progressive peripheral nervous system symptoms and disproportionally abnormal nerve conduction study findings should be tested for an inherited polyneuropathy and brain imaging for screening of possible central nervous system involvement should be performed. Further investigation is needed to elucidate the pathogenetic basis of CMT4C and a possible association with white matter lesions.

Endoscopic endonasal approach for resection of sellar leiomyosarcoma metastasis: illustrative case.

Leiomyosarcoma (LMS) is a rare neoplasm that arises from tissues of embryonic mesodermal origin. Primary tissues of origin can include smooth muscle cells of the abdominopelvic viscera, blood vessels, or arrector pili muscles. LMS is known to metastasize to the lungs, with few reported cases of spread to the central nervous system. A 66-year-old male with cutaneous LMS of the left forearm with metastases to the lungs and kidney that had been treated with chemoradiation presented with worsening headaches. Magnetic resonance imaging revealed a sellar lesion. An endocrine workup was unremarkable. Imaging over 6 months revealed rapid interval growth. Positron emission tomography demonstrated moderate uptake. Given the rapid growth, the patient was offered an endoscopic endonasal approach for resection. Pathology confirmed LMS. To the authors' knowledge, this is the first documented case of LMS metastasis to the sella. Pituitary carcinoma or metastases to the sellar region should be in the differential among patients with sellar region tumors with a rapid growth rate, bony erosion, or findings of lesions in the upper cervical lymph nodes or soft tissue. Tumors that show significant interval growth should raise suspicion for nonadenomatous lesions, and surgical intervention should be considered even in the absence of endocrinological dysfunction or cranial neuropathies. https://thejns.org/doi/10.3171/CASE2435.

Neurosarcoidosis of the trigeminal nerve: clinical accompaniments, radiographic findings, and association with neuralgia.

Cranial neuropathy is a principal disease manifestation of neurosarcoidosis, but many forms remain poorly described, including trigeminal nerve disease despite its frequency in reported cohorts (5-12%). Herein, we characterize the clinical course of patients with neurosarcoidosis involving the trigeminal nerve. A single-center retrospective cohort analysis of patients with biopsy-proven sarcoidosis involving the trigeminal nerve was conducted between 1/1/2000-3/7/2023. The trigeminal nerve was affected in 14/245 (5.7%) patients, being clinically symptomatic in 5/245 (2.0%) and asymptomatic with radiographic involvement in 9/245 (3.7%). 14/14 (100.0%) patients had systemic sarcoidosis. In the symptomatic group, trigeminal neuropathy was an inaugural feature in 4/5 (80.0%), unilateral in 5/5 (100.0%) with the V1 subdivision most affected (4/5, 80.0%), and associated with neuralgia in 2/5 (40.0%). On MRI, the cisternal nerve roots (9/14, 64.3%), Meckel's cave (7/14, 50.0%), and cavernous sinus (5/14, 35.7%) were most commonly affected, and 14/14 (100.0%) patients had extra-trigeminal neuroinflammation on cranial MRI. CSF was abnormal in at least one dimension in 11/12 (91.7%) tested. All three treated patients with symptomatic trigeminal neuropathy responded to immunomodulatory treatment, and symptomatic treatments for trigeminal neuralgia were helpful in two patients. After a median follow-up period of 63 months, the median modified Rankin scale score was 1 for both subgroups. Neurosarcoidosis may involve any portion of the trigeminal apparatus, and when affected, it frequently demonstrates a mismatch in radiographic involvement from its clinical manifestations of facial numbness and pain, and typically occurs in association with other clinical or radiographic manifestations of neurosarcoidosis.

Acute Multiple Cranial Neuropathies in Chronic Inflammatory Demyelinating Polyneuropathy: A Case Report and Scoping Review of the Literature.

Cranial nerve (CN) involvement is not a common feature of typical chronic inflammatory demyelinating polyneuropathy (CIDP). Patients with acute presentation of CN palsy in CIDP may be misdiagnosed and treated as other pathologies. We report a patient with multiple cranial neuropathies at the onset of CIDP in detail. In addition, we reviewed a large cohort of patients with CN involvement in CIDP and summarized their characteristics and clinical findings. We presented a 28-year-old woman who presented with progressive weakness and involvement of CN III, VII, X, XII in the subacute phase who was diagnosed as CIDP and was treated accordingly. A scoping review of the literature resulted in a total of 59 patients with available patient-level data [61.2% men, median age of 32 (Q1-Q3; 20-51.5) years]. CN impairment was present in the acute phase of the polyneuropathy in 10 out of 43 patients (23.3%), while it took a median of 7.7 [Q1-Q3; 3-13] years for other patients to present CN palsy. Sensitivity analysis did not reveal any difference among patients with acute-phase presentation of CN symptoms (N = 11) compared with those with delayed CN palsy (N = 33) in terms of demographics, patterns of CN involvement, associated diminished sensorimotor findings, or relapse. However, patients with acute presentation of CN palsy underwent plasmapheresis approximately 4 times more than those with delayed CN presentations (45.5% vs. 12.1%, P = 0.02). In this case presentation and review study, we observed that in one-fourth of patients with CIDP and CN neuropathy, CN involvement occurred in the acute phase. This finding indicates the necessity of considering CIDP among differential diagnoses of patients with CN involvement and polyneuropathies.

Staphylococcus epidermidis isolated sphenoiditis: a rare cause of recurrent orbital apex syndrome

Isolated sphenoid sinusitis (ISS) is a rare condition that can lead to severe complications, with orbital apex syndrome (OAS) being an exceptionally rare manifestation characterized by visual loss and multiple cranial neuropathies. We report a case of a 41-year-old female presenting with recurrent OAS caused by a chronic Staphylococcus epidermidis infection of the sphenoid sinus. Despite extensive diagnostic workups and multiple surgical interventions, the patient experienced recurrent episodes of OAS, highlighting the challenges in diagnosis and treatment. This report emphasizes the pathogenic potential of S. epidermidis, typically considered a commensal organism, in chronic sinusitis and underscores the importance of a multidisciplinary approach in managing such complex cases.

Blood-based biomarkers of neuronal and glial injury in active major neuropsychiatric systemic lupus erythematosus.

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a poorly understood and heterogeneous manifestation of SLE. Common major NPSLE syndromes include strokes, seizures, myelitis, and aseptic meningitis. Easily obtainable biomarkers are needed to assist in early diagnosis and improve outcomes for NPSLE. A frequent end-result of major syndromes is neuronal or glial injury. Blood-based neurofilament light (NfL) and glial fibrillary acidic protein (GFAP) have been utilized as markers for monitoring disease activity and/or severity in other neurodegenerative and neuroinflammatory diseases; however, they have not been evaluated in active major NPSLE. This was a case-control study. We enrolled patients aged 12-60 years with active major NPSLE, SLE without active major NPSLE, and healthy controls. Active NPSLE was defined as being <6months from last new or worsening neuropsychiatric symptom. Demographics, clinical data, and serum or plasma biosamples were collected. Thirteen patients with active major NPSLE, 13age/sex/kidney function matched SLE controls without active major NPSLE, and 13age/sex matched healthy controls (mean ages 26.8, 27.3, 26.6years) were included. 92% of each group were female. Major syndromes included stroke (5), autonomic disorder (3), demyelinating disease (2), aseptic meningitis (2), sensorimotor polyneuropathy (2), cranial neuropathy (1), seizures (1), and myelopathy (2). Mean (standard deviation) blood NfL and GFAP were 3.6 pg/ml (2.0) and 50.4 pg/ml (15.0), respectively, for the healthy controls. Compared to healthy controls, SLE without active major NPSLE had mean blood NfL and GFAP levels 1.3pg/ml (p = .42) and 1.2pg/ml higher (p = .53), respectively. Blood NfL was on average 17.9 pg/ml higher (95% CI: 9.2, 34.5; p < .001) and blood GFAP was on average 3.2pg/ml higher (95% CI: 1.9, 5.5; p < .001) for cases of active major NPSLE compared to SLE without active major NPSLE. In a subset of 6 patients sampled at multiple time points, blood NfL and GFAP decreased after immunotherapy. Blood NfL and GFAP levels are elevated in persons with SLE with active major NPSLE compared to disease matched controls and may lower after immunotherapy initiation. Larger and longitudinal studies are needed to ascertain their utility in a clinical setting.

Peripheral nervous system immune-related adverse events due to checkpoint inhibition.

Immune checkpoint inhibitors have revolutionized cancer therapy and are increasingly used to treat a wide range of oncological conditions, with dramatic benefits for many patients. Unfortunately, the resulting increase in T cell effector function often results in immune-related adverse events (irAEs), which can involve any organ system, including the central nervous system (CNS) and peripheral nervous system (PNS). Neurological irAEs involve the PNS in two-thirds of affected patients. Muscle involvement (immune-related myopathy) is the most common PNS irAE and can be associated with neuromuscular junction involvement. Immune-related peripheral neuropathy most commonly takes the form of polyradiculoneuropathy or cranial neuropathies. Immune-related myopathy (with or without neuromuscular junction involvement) often occurs along with immune-related myocarditis, and this overlap syndrome is associated with substantially increased mortality. This Review focuses on PNS adverse events associated with immune checkpoint inhibition. Underlying pathophysiological mechanisms are discussed, including antigen homology between self and tumour, epitope spreading and activation of pre-existing autoreactive T cells. An overview of current approaches to clinical management is provided, including cytokine-directed therapies that aim to decouple anticancer immunity from autoimmunity and emerging treatments for patients with severe (life-threatening) presentations.

Surgical and radiosurgical outcomes for Koos grade 3 vestibular schwannomas.

This study aimed to reveal the preferred initial treatment for Koos grade 3 vestibular schwannomas (VS). We performed a two-institutional retrospective study on 21 patients with Koos grade 3 VS undergoing resection at Yokohama Medical Center and 37 patients undergoing radiosurgery at Yokohama Rosai Hospital from 2010 to 2021. Tumor control, complications, and functional preservation were compared. The median pre-treatment volume and follow-up duration were 2845 mm3 and 57.0 months, respectively, in the resection group and 2127 mm3 and 81.7 months, respectively, in the radiosurgery group. In the resection group, 16 (76.2%) underwent gross total resection, and three patients (14.3%) experienced regrowth; however, no one required additional treatment. In the radiosurgery group, the tumor control rate was 86.5%, and three cases (8.1%) required surgical resection because of symptomatic brainstem compression. Kaplan-Meier analyses revealed that tumors with delayed continuous enlargement and large thin-walled cysts were significantly associated with poor prognostic factors (p = 0.0027, p < 0.001). The pre-radiosurgery growth rate was also associated with the volume increase (p = 0.013). Two cases (9.5%) required additional operation due to complications such as post-operative hematoma and cerebrospinal fluid leaks in the resection group, whereas temporary cranial neuropathies were observed in the radiosurgery group. Two patients (9.5%) had poor facial nerve function (House-Brackmann grading grade 3) in the resection group, while no one developed facial paresis in the radiosurgery group. Trigeminal neuropathy improved only in the resection group.Radiosurgery can be considered for the treatment of Koos grade 3 VS for functional preservation. However, resection may also be considered for patients with severe trigeminal neuropathy or a high risk of volume increments, such as large thin-walled cysts and rapid pre-treatment growth.

Cerebral Venous Sinus Thrombosis and Helminthiasis. A Case-Based Review

Abstract Cerebral venous sinus thrombosis (CVST) is an uncommon and underreported neurological condition. This condition has varied aetiologies, clinical manifestations, and significant sequelae if left untreated. We report the case of a 10-year-old male with fever, altered sensorium, cranial nerve neuropathies, and left hemiplegia. His imaging findings revealed features consistent with CVST, and he was subsequently noticed to be passing live worms per rectum while on admission. He was managed nonoperatively, improved significantly, and was discharged with residual motor deficits. In addition, we present a literature review of published case reports of CVST associated with helminthiasis, highlighting its possible pathophysiology, clinical spectrum, and management peculiarities.

Recovery of cranial nerve neuropathies after LINAC-based stereotactic radiosurgery for benign cavernous sinus meningioma

PurposeCranial Nerve Neuropathies (CNNs) often accompany Cavernous Sinus Meningioma (CSM), for which Stereotactic Radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSR) are established treatments. This study assesses CNNs recovery in CSM patients treated with LINAC, offering insight into treatment effectiveness.MethodsThis study was conducted on 128 patients with CSM treated with LINAC-based SRS/FSR between 2005 and 2020 at a single institution. 46 patients presented with CNNs. The study analyzed patients’ demographics, clinical parameters, SRS/FSR treatment characteristics, post-treatment CNNs recovery duration, status, and radiological control on their last follow-up.ResultsThe median follow-up duration was 53.4 months. Patients were treated with SRS (n = 25) or FSR (n = 21). The mean pretreatment tumor volume was 9.5 cc decreasing to a mean end-of-follow-up tumor volume was 5.1 cc. Radiological tumor control was achieved in all cases. CNN recovery was observed in 80.4% of patients, with specific nerve recoveries documented as follows: extra-ocular nerves (43.2%), trigeminal nerve (32.4%), and optic nerve (10.8%). A higher CNNs recovery rate was associated with a smaller pre-treatment tumor volume (p < 0.001), and the median time-to-improvement was 3.7 months. Patients with tumor volumes exceeding 6.8 cc and those treated with FSR exhibited prolonged time-to-improvement (P < 0.03 and P < 0.04 respectively).ConclusionsThis study suggests that SRS/FSR for CSM provides good and sustainable CNNs recovery outcomes with excellent long-term radiological control. A higher CNNs recovery rate was associated with a smaller pre-treatment tumor volume. while shorter time-to-improvement was identified in patients treated with SRS compared to FSR, particularly in those with small pre-treatment tumor volume.

Multiple Cranial Neuropathy with Anti-Ma2 Antibody

Multiple Cranial Neuropathy with Anti-Ma2 Antibody

Lyme Disease and Papilledema: A Retrospective Study on Clinical Characteristics and Outcomes.

Describe the clinical characteristics, treatment strategies, and outcome data of children with papilledema associated with Lyme disease at a large tertiary care pediatric hospital. Retrospective cohort study of children 1-18 years old who received care at our institution between 1995 and 2019 with concurrent diagnoses of papilledema and Lyme disease. Data were abstracted from records and prospective family surveys. Among 44 children included (median age 9.7 years), 66% (29/44) had additional cranial neuropathies, and 78% (32/41) had cerebrospinal fluid pleocytosis. All children were treated with antibiotics (39% oral, 55% intravenous, 7% both); 61% (27/44) were also treated with oral acetazolamide. Symptoms fully resolved in 86% (30/35) of children with follow-up data. Proportion recovered did not significantly differ by antibiotic administration route or presence/absence of cerebrospinal fluid pleocytosis. Papilledema in Lyme disease may occur with or without cerebrospinal fluid pleocytosis. Most children recover without residual deficits following treatment, although exceptions exist.

Unravelling the Acute, Chronic and Steroid-Refractory Management of High-Grade Neurological Immune-Related Adverse Events: A Call to Action.

Rare side effects of immune-checkpoint inhibitors (ICIs) are known as neurological immune-related adverse events (n-irAEs). Typically, n-irAEs affect the peripheral nervous system, primarily presenting as myositis, polyradiculoneuropathy, or cranial neuropathy. Less commonly, they impact the central nervous system, resulting in encephalitis, meningitis, or myelitis. High-grade n-irAEs managing and recognizing remains challenging, considering the risk of mortality and long-term disability. To date, strong scientific data are lacking to support the management of high-grade clinical forms. We performed a systematic literature search, selecting all articles describing high-grade steroid-resistance n-irAEs. and we reported them in a practical review. Specifically, current recommendations advise stopping ICI use and beginning corticosteroid treatment. Our findings highlighted that in steroid-resistant n-irAEs, it should be recommended to quickly escalate to plasma exchange (PLEX) and/or intravenously immunoglobulins (IVIg), usually in association with other immunosuppressants. Furthermore, newer evidence supports the use of drugs that may specifically block inflammation without reducing the anti-tumour effect of ICIs. In this practical review, we provide new evidence regarding the therapeutic approach of high-grade n-irAEs, particularly in steroid-resistant cases. We would also stress the importance of informing the scientific community of the discrepancy between current guidelines and clinical evidence in these rare forms of pathology.