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.

Effective cochlear implantation for idiopathic hypertrophic pachymeningitis with bilateral profound hearing loss: A case report.

RATIONALE: Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used. PATIENT CONCERNS: The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa. DIAGNOSES: A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss. INTERVENTIONS AND OUTCOMES: A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively. LESSONS: This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment.

Oculomotor nerve palsy as an extraintestinal manifestation of Crohn's disease.

Patients with inflammatory bowel disease (IBD) may present with extraintestinal manifestations. Neurological symptoms associated with IBD are infrequent. Thus, any unexplained neurological symptom that occurs in patients with IBD should raise the suspicion of a link between the two disorders. We report a case of a man in his 60s, who was diagnosed with Crohn's disease and developed ptosis and diplopia. Neurological examination revealed oculomotor nerve palsy, sparing the pupil. MRI and magnetic resonance angiography of the brain were insignificant and no other cause was determined. He was treated with oral corticosteroids and symptoms gradually subsided. Cranial nerve palsies associated with IBD have been rarely reported. They usually involve the optic and acoustic nerve and are attributed to a common dysimmune base. This is the first reported case of oculomotor nerve palsy (III cranial nerve) associated with IBD. Clinicians treating patients with IBD should be alert for unusual neurological complications and treat them appropriately.

A Case with Anti-ganglioside Antibodies Showing Multiple Cranial Nerve Palsies Detected on Gadolinium-enhanced Magnetic Resonance Imaging.

The anti-GQ1b IgG antibody is often accompanied by other anti-ganglioside antibodies, which induces various neurological symptoms. We herein report a patient with anti-ganglioside antibodies, including anti-GQ1b IgG and anti-GT1a IgG antibodies, showing bilateral ophthalmoplegia, facial nerve palsies, dysarthria, dysphagia, dysesthesia in both hands, and enhancement of the bilateral oculomotor, abducens, and facial nerves on gadolinium (Gd)-enhanced T1-weighted brain magnetic resonance imaging (MRI). He was first treated with intravenous immunoglobulin, which improved ophthalmoplegia, bulbar palsies, and dysesthesia of hands, but the facial nerve palsies worsened, and Gd enhancement of the brain nerves persisted. High-dose methylprednisolone therapy subsequently improved the facial nerve palsies and Gd enhancement of the cranial nerves. This is the first case with anti-ganglioside antibodies presenting with multiple cranial nerve palsies that was followed to track the changes in the Gd enhancement of cranial nerves on MRI.

Cavernous sinus and jugular thromboses, base of skull osteomyelitis and cranial nerve palsies: catastrophic complications of sphenoid sinusitis.

Sphenoid sinusitis is a clinically important entity as it may be associated with catastrophic complications including cavernous sinus thrombosis (CST), cerebral abscess or infarction, meningitis, base of skull osteomyelitis and cranial nerve palsies. We report a case of occult sphenoid sinusitis presenting as Streptococcus intermedius bacteraemia, bilateral jugular vein and CST, cranial nerve palsy and base of skull osteomyelitis.

Meningitis as a complication of Sjögren's syndrome.

Sjögren's syndrome can be complicated by several neurological manifestations, including aseptic meningitis, which can be manifested with headache, flu-like symptoms, confusion, fever, signs of meningeal irritation, with or without focal neurological symptoms and cranial nerve palsy. Neuroimaging can reveal contrast enhancement in the lepto- or pachymeninges. Therefore, Sjögren's syndrome should be considered in the differential diagnosis of lepto- or pachymeningeal enhancement.

Important aspect of hypoglossal nerve injury following gunshot wound; Can the clivus has a role? A case report.

Cranial nerve palsies after gunshot injury are not uncommon. We report the mechanism of isolated hypoglossal nerve paralysis caused by a gunshot. We report a 74 years old patient in whom a bullet entered through the right nostril and then ended up right occipital condyle. The only neurologic deficit was tongue deviation which resolved in one week. The bullet was not removed. The effect of clival slope may have an importance in this type of injury.

[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.

Varicella zoster-associated progressive lower cranial and upper cervical polyneuropathy: a case report.

BACKGROUND: Multiple cranial neuropathies carry a wide range of differential diagnoses, and when combined with cerebrospinal fluid monocytosis they often suggest an infective etiology. Reactivation of varicella zoster virus has been associated with a wide range of neurological complications. Among the cranial nerves, the upper cranial nerves (trigeminal and facial nerves) are more commonly affected; there have been some reports of lower cranial polyneuropathies resulting from varicella zoster virus reactivation. However, polyneuropathy involving both the cranial and cervical nerves is rarely reported. CASE PRESENTATION: This report highlights the case of a 64-year-old Chinese man presenting with an acute, severe dysphagia and dysphonia secondary to herpes zoster-associated progressive polyneuropathy involving the lower cranial and upper cervical nerves, without any mucocutaneous manifestations. CONCLUSIONS: To our knowledge, this is the first case of varicella zoster virus-associated cranial and cervical polyneuropathy in the literature. The report also highlights the poor sensitivity of varicella zoster virus DNA detection by polymerase chain reaction in the cerebrospinal fluid, and proposes that serology be routinely performed in such polymerase chain reaction-negative cases without a clear diagnosis.

Cerebral small vessel disease as imaging biomarker predicting ocular cranial nerve palsy of presumed ischemic origin at admission.

Ocular cranial nerve palsy of presumed ischemic origin (OCNPi) is the most common type of ocular cranial nerve palsy (OCNP) in patients aged ≥ 50 years; however, no definite diagnostic test exists. As diagnostic criteria include clinical improvement, diagnoses are often delayed. Diagnostic biomarkers for OCNPi are required; we hypothesized that cerebral small vessel disease is associated with OCNPi. We analyzed 646 consecutive patients aged ≥ 50 years with isolated unilateral OCNP who underwent work-ups at two referral hospitals. White matter hyperintensities (WMHs), silent infarctions, and cerebral microbleeds (CMBs) were assessed. In multivariate analyses, mild (grades 1-3) and moderate to severe (grades 4-6) WMHs were significantly associated with OCNPi compared to OCNP of other origins (odds ratio [OR] 3.51, 95% confidence interval [CI] 1.91-6.43, P < 0.001; OR 3.47, 95% CI 1.42-8.48, P = 0.006, respectively). Silent infarction and CMBs did not remain associated (OR 0.96, 95% CI 0.54-1.70, P = 0.870; OR 0.55, 95% CI 0.28-1.08, P = 0.080, respectively). Associations between WMH and OCNPi remained after excluding patients with vascular risk factors. In conclusion, the presence of WMH could independently predict ischemic origin in patients with isolated unilateral OCNP at early stage of admission.

Clinical, imaging features and treatment response of idiopathic hypertrophic pachymeningitis.

BACKGROUND: Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease that causes focal or diffuse thickening of the dura mater. However, longitudinal follow up studies are still lacking for these patients. OBJECTIVE: To investigate the clinical characteristics, neuroimaging findings, treatment response and outcome of IHP. METHOD: A retrospective case series of 30 patients admitted Beijing Tiantan Hospital were screened via Hospital Information System from January 1st, 2011, to January 31st, 2021. All patients' clinical symptoms, imaging, and treatment response were collected via a standardized form. We compared the effects of high-dose and low-dose corticosteroids on headache, impaired vision, and MRI remission during acute onset. The effects of different immunosuppressants on preventing relapses were also compared. RESULTS: Headache (93.3%) and multiple cranial neuropathy (66.7%) were the most common symptoms of IHP. Cerebral spinal fluid test showed that protein levels were elevated in 17 (56.7%) patients, and white blood cells were increased in seven patients. MRI demonstrated that diffuse (60%) and focal (40%) enhancement occurred in the dura mater, especially in the tentorium cerebellum (80%). High-dose and low-dose corticosteroids reduced headache and dural enhancement during the acute phase. The high dose corticosteroid significantly relieved the headache than the low dose group (p = 0.041). Patients treated with mycophenolate mofetil and cyclophosphamide might achieve longer remission (months, p = 0.428). CONCLUSION: Headache and multiple cranial neuropathy are the most common clinical manifestations of IHP. In this study, almost all patients had a good initial response to corticosteroid therapy during the acute phase. Mycophenolate mofetil and cyclosporine may be effective for preventing relapses.

Association between migraine and risk of ocular motor cranial nerve palsy.

To assess association between migraines and development of ocular motor cranial nerve palsy (CNP) and finding risk factors using the National Sample Cohort database from the Korea National Health Insurance Service. Data was analyzed from 4,234,341 medical screening examinees aged 20-90 years in 2009. Cox proportional hazard regression analysis was used to the adjusted hazard ratios (HR) for ocular motor CNP according to presence of migraine. Subgroup analysis was performed to evaluate effect of other factors on association of migraine with ocular motor CNP. A total of 5806 participants (0.14% of subjects) developed ocular motor CNP and were assigned to CNP group, 4,048,018 were assigned to control group, with an average of 8.22 ± 0.93 years of follow-up. Incidence of ocular motor CNP increased in migraine group compared to control. After adjusting potential confounding variables, HR for ocular motor CNP was 1.166 (confidence interval [CI] 1.013-1.343) in migraine group. Subgroups of relatively younger age less than 65 years (HR = 1.267, 95% CI 1.067-1.504), male gender (HR = 1.228, 95% CI 1.000-1.122), smokers (HR 1.426, 95% CI 1.127-1.803), and diabetes mellitus patients (HR = 1.378, 95% CI 1.045-1.378) showed a stronger association between migraines and development of ocular motor CNP. Our population-based cohort study demonstrated a significant association between presence of migraines and incidence of ocular motor CNP. Especially, relatively younger age, males, smokers, and diabetes patients with migraines could have a higher risk of developing ocular motor CNP.

Clinical outcomes and etiology of acquired sixth cranial nerve palsy.

ABSTRACT: The objective of this study was to investigate the difference in clinical features according to age and factors affecting recovery of acquired sixth cranial nerve (CN6) palsy.A total of 156 patients with acute CN6 palsy between March 2016 and August 2021 who were followed up for at least 3 months were included in this study. Etiology, rate of recovery, and factors associated with recovery were retrospectively investigated.The average age of patients with CN6 palsy was about 60years and the mean duration of recovery was about 2.5 months. Of 156 patients, 72 (46.15%) had a microvascular etiology and 25 (16.03%) patients had a brain vascular lesions. Brain neoplasm, trauma, and "others" were found in 10 (6.41%), 11 (7.05%), and 15 (9.62%) patients, respectively. Among the total of 156 patients, 28 (17.95%) failed to completely recover. Non-isolated CN6 palsy with other cranial nerve palsies were recorded in 29 (18.59%) cases. Comparison of age (<50years vs ≥50years), between recovery and non-recovery groups showed that etiology was significantly different.The recovery rate of acquired CN6 palsy was about 82% and about 27% of patients had brain lesions. Also, varying rates and duration of recovery were found according to etiology, so we should be pay attention to diagnosis of causative disease in CN6 palsy patients.

Stroke risk after ocular cranial nerve palsy - A systematic review and meta-analysis.

BACKGROUND: Isolated ischemic ocular cranial nerve palsies (OCNP) involving the 3rd, 4th and 6th cranial nerves (CN) are prevalent conditions in ophthalmic practice. However, it is not clearly established whether such patients are at increased risk of stroke after onset of OCNPs. METHODS: Medline, PubMed, Embase and Cochrane Central registers were systematically searched for eligible studies comparing isolated ischemic OCNPs against matched controls on the subsequent development of stroke with at least two years of follow up. Case reports and series were excluded. Appropriate studies were entered for meta-analysis to determine hazard ratios. Search and data extraction was completed on 22 Feb 2021. Random effect models were used to generate pooled hazard ratios (HRs) and 95% confidence intervals (CIs). RESULTS: Three studies were suitable for meta-analysis (total n = 2,756 OCNP cases and 21,239 matched controls). The meta-analysis demonstrated a hazard ratio of 5.96 (4.20-8.46 95% CI) of subsequent stroke after isolated OCNP within the first year. The hazard ratio reduced to 3.27 (2.61-4.10 95% CI) after five years although remains raised at 2.49 (1.53-4.06 95% CI) up to 12 years. The highest risk was demonstrated with 3rd cranial nerve palsies. Two additional studies assessed the risk of stroke with newly diagnosed diabetics and compared OCNPs against lacunar stroke. These studies did not demonstrate a significant increased risk of stroke, although they may be statistically underpowered. CONCLUSION: Ischemic OCNPs represent a significant risk factor for development of subsequent stroke in a similar magnitude to transient ischemic attack within the first year.

Invasive Fungal Sinusitis in Patients With Coronavirus Disease 2019 Seen in South India.

BACKGROUND: Coronavirus disease 2019 (COVID-19) has a vast array of presentations and associations with neuro-ophthalmic diseases. There has been a recent surge in ophthalmic manifestations secondary to fungal sinus infections in India especially in diabetic patients who were given systemic steroids. We present our COVID-19-related cranial neuropathies presenting in our clinic. METHODS: This is a retrospective case series of 10 patients affected with COVID-19 disease and who presented with cranial nerve palsies at the neuro-ophthalmic department of a tertiary eye care hospital in South India. An analysis of electronic medical records data was performed, including their comorbidities, symptoms, cranial nerves involved, ocular and neuroimaging findings, site of lesion, etiology, and prognosis. RESULTS: Most of the patients (7 of 10) presented with multiple cranial nerve palsies (MCNP) with poor visual acuity. 2 of the 10 cases succumbed to death due to the intracranial involvement. All MCNP cases had uncontrolled diabetes with a history of systemic steroids, and neuroimaging of these cases showed sinusitis of varying severity most of which were suggestive of fungal invasive type. CONCLUSION: Our study emphasizes the need to screen for fungal involvement in COVID-19 cases presenting with MCNP especially on diabetic patients on systemic steroids so that an early diagnosis may reduce visual loss and mortality. Physicians treating COVID-19 cases need to be aware of this dreadful complication.

The spectrum and differential diagnosis of acquired ocular motor nerve palsies: a clinical study of 502 patients.

BACKGROUND: Ocular motor nerve palsies (OMNP) frequently cause patients to present in an emergency room. In the following study, we report the differential diagnosis of OMNP by use of magnetic resonance imaging (MRI) and CSF examination as a standard. METHOD: We performed a data analysis of N = 502 patients who presented with oculomotor, trochlear, and/or abducens nerve palsy in the emergency room of the Department of Neurology, University of Ulm, between January 2006 and December 2019. We report clinical and MRI scan findings in all patients; furthermore, the CSF of 398 patients has been analysed. RESULTS: Abducens nerve palsies were most common (45%), followed by palsies of the oculomotor (31%) (CNP III) and trochlear nerve (15%). Multiple OMNPs were seen in 9% of our cohort. The most common causes included inflammations (32.7%), space-occupying lesions, such as aneurysms or neoplasms (17.3%), diabetes mellitus (13.3%), and brainstem infarctions (11%). Still 23.4% of the patients could not be assigned to any specific cause after differential diagnostic procedures and were described as idiopathic. One of three patients with an inflammation and 39% of the patients with space-occupying lesions showed additional cranial nerve deficits. CONCLUSION: Inflammation and space-occupying processes were the most frequent causes of OMNP, although brainstem infarctions also play a significant role, in particular in CNP III. The presence of additional CNPs increases the probability of an inflammatory or space-occupying cause.

Liver enzymes and risk of ocular motor cranial nerve palsy: a nationwide population-based study.

This study aimed to assess the associations between liver enzymes including γ-glutamyl transferase (GGT) and the development of ocular motor cranial nerve palsy (CNP) using the National Sample Cohort database from Korea's National Health Insurance Service. We analyzed data from 4,233,273 medical screening examinees aged 20 years or more in 2009. Study participants were followed up until December 31, 2018. A Cox proportional hazard regression analysis was performed for quartiles of liver enzymes to determine the linkage between each value and ocular motor CNP using quartile 1 as a reference after adjusting for potential confounders. A total of 5,807 (0.14%) patients developed ocular motor CNP during the follow-up period of 8.22 ± 0.94 years. The incidence of ocular motor CNP gradually increased as the GGT levels increased. The highest quartile of the GGT group had hazard ratio (HR) of 1.245 (95% confidence interval [CI], 1.136-1.365). Regarding alanine aminotransferase (ALT), the highest quartile of the ALT group had HR of 1.141 (95% CI, 1.049-1.241). However, the incidence of ocular motor CNP did not gradually increase as the ALT levels increased. The coexistence of the increased level of GGT, metabolic syndrome, and obesity showed a stronger association with ocular motor CNP development (HR, 1.331; 95% CI, 1.173, 1.511) compared to having a single factor or two factors. In conclusion, our population-based cohort study demonstrated a significant association between serum GGT level and the incidence of ocular motor CNP, suggesting that GGT could be a new clinical marker for predicting the occurrence of ocular motor CNP.