Collet-Sicard syndrome due to cervical artery dissection disclosed by high-resolution magnetic resonance imaging.

BACKGROUND AND PURPOSE: Cervical artery dissection (CAD) represents a leading cause of unilateral lower cranial nerve IX-XII palsy, known as Collet-Sicard syndrome (CSS). High-resolution magnetic resonance imaging (HR-MRI) is widely used in the evaluation of patients with CAD, providing information regarding vessel wall abnormalities and intraluminal thrombus. METHODS: We present a patient with palsy of multiple lower cranial nerves in the context of CSS, attributed to unilateral spontaneous internal carotid artery dissection. RESULTS: We describe a 68-year-old man with unremarkable previous history, who presented with subacute, gradually worsening dysphagia and hoarse voice. Clinical examination revealed right-sided palsy of cranial nerves IX-XII. Three-dimensional fat-saturated black-blood T1-weighted high-resolution vessel wall imaging disclosed spontaneous dissection with intramural hematoma along the distal right internal carotid artery. Neck MRI showed inward displacement of right aryepiglottic fold, right pyriform sinus dilatation, and right true vocal cord in middle position, indicative of right vagus nerve palsy, atrophy of right trapezius and sternocleidomastoid muscles, due to right spinal accessory nerve palsy, and unilateral tongue atrophy with fatty infiltration, characteristic for right hypoglossal nerve palsy. CONCLUSIONS: This case highlights the utility of high-resolution vessel wall imaging and especially fat-saturated T1-weighted black-blood SPACE (sampling perfection with application-optimized contrast using different flip-angle evolutions) sequences in the accurate diagnosis of CAD, revealing the characteristic mural hematoma and intimal flap. HR-MRI is also valuable in the recognition of indirect signs of lower cranial nerve compression.

Millard-Gubler Syndrome in a Patient with Preeclampsia. Case Report and Review of Combined Intra- and Extra-Axial Facial and Abducens Nerve Injuries.

Millard-Gubler syndrome is a pontine syndrome caused by a lesion in the lower pons region. It is characterised by ipsilateral facial paralysis and VI paresis and contralateral brachiocrural palsy. We present the case of a female patient, G4P2A1, at 21 weeks of gestation, with preeclampsia, complaints of blurred vision, diplopia, and right hemiparesis, in whom a clinical diagnosis of Millard-Gubler syndrome was made. Neuroimaging showed an intraparenchymal haemorrhage towards the central portion of the bulbopontine junction. An extensive aetiological study was carried out to determine the cause of the hypertensive disorder syndrome during pregnancy. The patient improved satisfactorily from the neurological deficit after delivery of an early stillbirth.

Multiple cranial nerve palsies following COVID-19 vaccination-Case report.

BACKGROUND: The novel COVID-19 vaccines have side effects that require efficient and close monitoring. AIMS OF THE STUDY: To examine whether the Pfizer-BioNTech vaccine is associated with multiple cranial neuropathy. METHODS: We report the case of a 29-year-old male patient with no notable history who presented with left oculomotor, abducens, trigeminal and facial palsies 6 days after receiving the first dose of the Pfizer-BioNTech COVID-19 vaccine. RESULTS: Gadolinium-enhanced MRI of the brain revealed enhancement in the left facial, trigeminal and oculomotor nerves, which persisted upon repeated examination. The cerebrospinal fluid analysis showed no sign of inflammation, both initially and after 1 month from the start of the patient's symptoms. Other causes were excluded by laboratory tests. The patient received high doses of corticosteroids, with improvement of symptoms. CONCLUSIONS: In our case, the most probable etiology of the patient's multiple cranial neuropathy is the Pfizer-BioNTech vaccine, which highlights the need for prolonged surveillance of COVID-19 vaccine neurological complications.

Mucormycosis with extensive cranial nerve involvement as the first presentation of diabetes mellitus: A case report.

Mucormycosis, a rare fungal infection, mainly affects individuals with diabetes mellitus and those who were immunocompromised and has a high mortality rate. Its most common presentation is similar to that of acute bacterial sinusitis with symptoms of nasal congestion, headache, and fever. The involvement of multiple cranial nerves in mucormycosis was rarely reported in the literature and indicates severe disease. Herein, we report the case of a 56-year-old man who was referred to the ophthalmology outpatient clinic for facial nerve palsy. He was treated with systemic steroids for 10 days with no improvement. On examination, he had a loss of vision and a frozen orbit due to involvement of cranial nerves II, III, IV, V, VI, and VII. An extensive workup revealed a hemoglobin A1C of 10%. However, he was never diagnosed with diabetes mellitus previously and denied any of the classical symptoms of diabetes mellitus. He underwent ethmoidectomy, maxillectomy, and drainage of an intraorbital abscess after appropriate imaging studies. Histopathology confirmed the diagnosis of mucormycosis, and the patient was started on systemic amphotericin B. This case emphasizes the importance of screening for diabetes mellitus. Early recognition of underlying diabetes mellitus in this patient may have prevented the development of mucormycosis along with its devastating complications.

An Update on Idiopathic Hypertrophic Cranial Pachymeningitis for the Headache Practitioner.

PURPOSE OF REVIEW: We aim to review idiopathic hypertrophic cranial pachymeninigitis (IHCP), describe common head pain patterns and features associated with the disorder, suggest potential classification of head pain syndromes based on the recently published International Classification of Headache Disorders-3, explore pathophysiology found to be associated with cases of IHCP, and indicate common treatment for the disorder. RECENT FINDINGS: It is suggested that a subset of IHCP is an IgG4-related autoimmune disorder. Patients with IHCP were found to have elevated cerebrospinal fluid (CSF) protein and lymphocytic pleocytosis. Corticosteroids are a mainstay of treatment. Other immunosuppressive agents and steroid sparing agents as add-on therapy may have utility in the treatment of cases refractory to corticosteroids alone. Clinical manifestations of IHCP depend upon the location of the inflammatory lesions and compression of the adjacent nervous system structures. Headache and loss of cranial nerve function were the most common presenting features of hypertrophic cranial pachymeninigitis. Several headache diagnoses may result from IHCP. Gadolinium-enhanced MRI is the standard imaging modality for diagnosing. Although the pathophysiology is poorly understood, many cases of hypertrophic pachymeninigitis (HP) are thought to be closely related to inflammatory disorders. Cases of HP previously thought to be idiopathic may have IgG4 pathophysiology. CSF and serological studies are helpful. Treatment involves immunosuppressive agents. Advancement in neuroimaging, assays, tests, and further delineation of inflammatory disorders affecting the nervous system may provide further insight to the etiology of cases of HP previously considered and diagnosed as idiopathic.

Cranial nerve palsies due to incidental durotomy in lumbar Spine surgery: a case report.

We present a 65-year old man who underwent a partial laminectomy at L4. During surgery an incidental durotomy (ID) appeared. Postoperatively he developed cranial nerve palsies. Subsequent to surgical closure of the ID, symptoms completely resolved within three months.

Cerebrospinal fluid dissemination of anaplastic intraventricular meningioma: report of a case presenting with progressive brainstem dysfunction and multiple cranial nerve palsies.

BACKGROUND: It is extremely rare to see cerebrospinal fluid dissemination of intraventricular meningioma, particularly with the development of acute, progressive brainstem/cerebellar dysfunction with an absence of mass formation in the corresponding anatomical sites. CASE PRESENTATION: An 81-year-old man was admitted because of double vision, right facial nerve palsy and truncal ataxia. Brain magnetic resonance imaging showed normal findings except for a tumor mass in the left lateral ventricle, which had been noted over 6 months previously. The patient developed hiccups, hyperventilation, and drowsiness, which worsened progressively, and did not respond to corticosteroid or intraventricular immunoglobulin therapy. Cerebrospinal fluid study revealed a mild elevation of protein, and cytology was negative. The patient died and an autopsy was performed. Postmortem investigation disclosed a malignant transformation of benign fibroid meningioma with cerebrospinal fluid dissemination of the malignant cells, diversely involving the surface of brainstem, cerebellum, and spinal cords, secondarily resulting in extensive ischemia in the brain parenchyma by vessel occlusion. CONCLUSION: If a patient with an intraventricular tumor develops acute, progressive neurological symptoms, the possibility that it is be caused by cerebrospinal fluid dissemination of tumor cells, after malignant transformation, should be considered.

Improvement in cranial nerve palsies following treatment of intracranial aneurysms with flow diverters: Institutional outcomes, systematic review and study-level meta-analysis.

BACKGROUND: Cranial nerve (CN) palsies are rare presenting symptoms of intracranial aneurysms. Our objectives were to report our institutional outcomes and study-level meta-analysis summarizing rates of improvement and identifying factors associated with recovery from CN symptoms after flow diversion. METHODS: We conducted a retrospective review of our institutional database for patients with intracranial aneurysms presenting with CN palsies who underwent treatment with flow diversion between 2015 and 2023. Systematic review of the literature was performed using Medline, EMBASE, Cochrane, as well as manual citation searches. Random effects meta-analysis was used. RESULTS: Thirteen of 136 studies were included in the meta-analysis and were combined with our institutional data. The pooled rate of improvement in any CN palsies following flow diversion was 71 % (95 %CI, 60 %-82 %, n=322). Patients presenting with CN II deficits were less likely to improve following treatment compared to other CN deficits (pooled OR [pOR] 0.32, 95 %CI, 0.16-0.63, n=224). The pooled rate of clinical improvement was 53 % in CNII deficits (95 %CI, 42 %-65 %, n=80) and 80 % in other CN deficits (95 %CI, 71 %-88 %, n=106). An increased rate of improvement was associated with acute intervention (pOR 9.12, 95 % CI, 2.26-36.73, n = 71) and radiographic aneurysm occlusion (pOR 5.29, 95 %CI, 1.66-16.90, n=118). CONCLUSIONS: Flow diversion improves CN palsy outcomes in patients with symptomatic intracranial aneurysms. The lower rate of improvement in visual acuity compared to other CN deficits may point to a different mechanism of injury or potential recoverability in these patients.

Pituitary Apoplexy Presenting with Isolated Third Nerve Palsy: A Rare Case Report.

A 46-year-old female came to neurosurgery outpatient department with sudden onset of drooping of RE upper eyelid and restriction of movements in adduction, depression and elevation in right eye. Patient was a known case of diabetes mellitus whose blood sugar levels were deranged. On examination, patient was diagnosed pituitary macroadenoma. Patient was treated for her uncontrolled diabetes mellitus following which she had underwent transsphenoidal pituitary macroadenoma removal. On treatment, patient's ptosis had subsided and restriction of movement has resolved.

Idiopathic Hypertension with Multiple Cranial Nerve Palsies: A Case Report and Literature Review.

We report a rare case of idiopathic intracranial hypertension (IIH) with multiple cranial nerve palsies involving cranial nerves VI, VII, IX, and X in a 32-year-old female who had no prior comorbidities. Her condition improved rapidly on a ten-day regimen of acetazolamide and tablet topiramate. IIH should be considered in every patient presenting with persistent headache and multiple cranial nerve abnormalities. This paper also includes a literature review of similar cases.

Pituitary Adenoma Masquerading as Diabetic Third Nerve Palsy.

A 46-year-old uncontrolled diabetic female visited the ophthalmology outpatient department with a sudden onset of drooping of the upper lid and restriction of movements in adduction, depression, and elevation in the right eye, suggestive of third nerve palsy. Initially, it was thought to be due to a vasculogenic cause due to uncontrolled diabetes, but visual fields revealed bitemporal hemianopia, characteristic of a pituitary adenoma. The diagnosis was confirmed by a CT scan. The patient then underwent a trans-nasal endoscopic removal of the pituitary macroadenoma, followed by a partial recovery of vision.

Case of Lemierre's Syndrome Presenting With Neuro-Ophthalmologic Complications That Worsened After Stopping Corticosteroids.

Lemierre's syndrome (LS) is a rare disorder that manifests as septic internal jugular thrombophlebitis following a recent oropharyngeal infection. This article details a unique case of LS, where the patient presented to the emergency room with complaints of vomiting, headache, diplopia, and left eye pain. Due to a history of sore throat, headache, neck pain, fever, and nausea five days prior to admission, the patient was initially treated with amoxicillin/clavulanate for suspected tonsillitis. A positive meningeal sign and elevated temperature were observed during the clinical examination. Lumbar puncture (LP) was deferred based on imaging indicating potential increased intracranial pressure (ICP). Nevertheless, the patient received vancomycin, ceftriaxone, and dexamethasone as an initial course of treatment for presumed bacterial meningitis. Significant improvement was observed within the first four days of admission, with no subsequent episodes of fever, nausea, or headache. However, upon discontinuation of corticosteroid therapy, the patient experienced severe headaches and frequent vomiting. An urgent brain CT scan confirmed the extension of the left internal jugular vein (IJV) thrombosis to the ipsilateral sigmoid sinuses. Metronidazole and anticoagulant medication were initiated upon LS diagnosis. There is a paucity of discussions on corticosteroid use in LS, with no definitive statistics in the current literature. This case underscores the importance of recognizing and effectively managing interconnected clinical manifestations.

Successful Management of Acute Streptococcal Meningoencephalitis Complicated by Bilateral Third-Nerve Palsies, Wall-Eyed Bilateral Internuclear Ophthalmoplegia, Blindness, and Deafness: Case Report.

Introduction: Streptococcal meningoencephalitis (SME) is a rare, and frequently lethal, acute infection, and inflammation of the central nervous system parenchyma, with associated meningeal involvement. Bacterial meningoencephalitis is generally associated with high rates of morbidity and mortality, despite available antimicrobial and corticosteroid treatments. While Streptococcus pneumoniae is well recognised to cause bacterial meningitis, direct extension into the central nervous system parenchyma is rare. Case Presentation: A previously well 49-year-old man presented with sudden onset severe headache, fevers, neck stiffness, and reduced consciousness. The manifestations of SME in this patient were bilateral pupil-involving third-nerve palsies, wall-eyed bilateral internuclear ophthalmoplegia (WEBINO), bilateral blindness, bilateral deafness, a right lower motor neuron facial palsy, and upper motor neuron signs in his limbs. Initially, a partial response to high dose intravenous antibiotics occurred, but with administration of intravenous corticosteroids, further substantial resolution of the patient's neurological and neuro-ophthalmological deficits occurred. Conclusion: This case highlights the benefit of multidisciplinary diagnostic and therapeutic interventions in a case of SME complicated by bilateral pupil-involving third-nerve palsies, WEBINO, bilateral blindness, bilateral deafness, a right lower motor neuron facial palsy, and upper motor neuron signs. It appears to be the first reported case of SME with this rare collection of neuro-ophthalmological abnormalities.

Bilateral abducens and facial nerve palsies following fourth ventriculoperitoneal shunt with laparoscopic-assisted abdominal catheter placement.

Introduction: Hydrocephalus, altering cerebrospinal fluid (CSF) dynamics, affects 175 per 100,000 adults worldwide. Ventriculoperitoneal shunts (VPS) manage symptomatic hydrocephalus, with 125,000 cases annually. Despite efficacy, VPS face complications, necessitating interventions. Research question: "What are the mechanisms and risk factors for bilateral VIth and VIIth lower motor neuron palsies in hydrocephalus patients with a fourth ventriculoperitoneal shunt?" Material and methods: This study details a 36-year-old female with a neonatal meningitis history, multiple shunt replacements, admitted for abdominal pain secondary to pelvic inflammatory disease. An abdominal shunt catheter removal and external ventricular drain placement occurred after consultation with a general surgeon. A cardiac atrial approach and subsequent laparoscopic abdominal approach were performed without complications. Results: After one month, the patient showed neurological complications, including decreased facial expression, gait instability, and bilateral VIth and VIIth lower motor neuron palsies, specifically upgazed and convergence restriction. Discussion: The complication's pathophysiology is discussed, attributing it to potential brainstem herniation from over-drainage of CSF. Literature suggests flexible endoscopic treatments like aqueductoplasty/transaqueductal approaches into the fourth ventricle. Conclusions: This study underscores the need for increased awareness in monitoring neurological outcomes after the fourth ventriculoperitoneal shunt, particularly in cases with laparoscopic-assisted abdominal catheter placement. The rarity of bilateral abducens and facial nerve palsies emphasizes the importance of ongoing research to understand pathophysiology and develop preventive and therapeutic strategies for this unique complication.

Multiple Cranial Nerve Palsies Without Limb Weakness: A Rare Cranial Variant of Guillain-Barré Syndrome.

We herein report a case of an unusual variant of Guillain-Barré syndrome (GBS) where the patient presented with multiple bilateral cranial nerve palsies involving nerves V, VII, IX, and X, leading to difficulties with eye closure, eyebrow-raising, chewing, swallowing, and speech. Sensation and motor examination were normal. Bilateral knee reflexes were absent. Lumbar puncture showed cerebrospinal fluid albuminoid-cytologic dissociation. Prompt initiation of plasmapheresis therapy facilitated a successful recovery. This case report underscores the significance of early identification and tailored intervention for atypical GBS presentations, highlighting the potential for improved patient outcomes through targeted management strategies.

Diplopia with a Cavernous Sinus Metastasis of a Remote Endometrial Stromal Tumour.

Structural lesions involving the visual pathways often require tissue diagnosis to guide therapy. However, pathology is not always definitive, and a biopsy showing poorly differentiated cells poses significant difficulty in identifying the primary tumour. We report a case of metastatic disease causing cavernous sinus syndrome, in which biopsy revealed poorly differentiated tissue. The patient reported a history of a resected uterine tumour, and it was only after obtaining slides from 7 years prior that the diagnosis was made.

Neurological Manifestations of Connective Tissue Disorders.

Connective tissue disorders (CTD) are a group of disorders affecting the connective tissues. Usually the musculoskeletal and the vascular system is impacted. Along with these systems, the nervous system is also involved in CTD, which leads to various neurological manifestations. The pathophysiology of neurological complications of CTD is caused by various factors and is complicated. Disturbed immune complexes, chronic inflammation, and autoimmunity in which the body attacks its cells are considered to be responsible for the neurological complications of CTD. Additionally, the vascular symptoms that lead to decreased blood flow to the brain are also responsible for the neurological manifestations of CTD in diseases like systemic lupus erythematosus (SLE). In SLE, vessel wall integrity is compromised, which may lead to decreased blood flow leading to neurological complications. CTD can manifest a variety of neurological complications. These neurological complications can be classified into symptoms affecting the peripheral nervous system, central nervous system, and the autonomic nervous system. Some of the common neurological complications of CTD are headaches, seizures, ataxia, neuropathies leading to cranial nerve palsies, myelopathies, tremors, encephalitis, and cerebral infarction. Cranial nerve palsies can disturb sensations, vision, hearing, and mastication. Neuropsychiatric symptoms are also commonly observed in CTD. Cognitive dysfunction can be caused due to neuropsychiatric problems. Some of the cognitive dysfunctions are lack of concentration, memory loss, confusion, and coma. In this review, we will address various neurological manifestations of CTD.

Cerebral venous thrombosis presenting as multiple lower cranial nerve palsies.

Cerebral venous thrombosis (CVT) is a well-recognized entity, but its clinical presentation is varied and often mimics many neurological disorders, making it a diagnostic challenge. Cerebral venous thrombosis has a wide spectrum of signs and symptoms, which may evolve suddenly or over weeks. It mimics many neurological conditions such as meningitis, encephalopathy, idiopathic intracranial hypertension, and stroke. Cerebral venous thrombosis presenting as multiple lower cranial nerve palsies, are rarely reported. We describe a pregnant lady who presented with sensorineural deafness of the right ear and paralysis of the 9(th), 10(th), and 12(th) cranial nerves on the right side. She was diagnosed to have thrombosis of the right transverse sinus and sigmoid sinus with extension to the jugular vein and confluence of sinuses. She improved with anticoagulant treatment.

Paget's disease of bone presenting with multiple cranial nerve palsies: A case report.

Paget's disease of bone (PDB) is a progressive monostotic or polyostotic osteopathy with unknown cause. It is associated with the involvement of the nervous system. The cranial nerves, spinal roots, cauda equina, spinal cord, and brain can be affected in PDB due to their close anatomical relation to bone. Hearing loss occurs in 12%-50% of patients with PDB. The optic nerve can be affected at the optic canal. The diagnosis of PDB is radiological by highlighting characteristic lesions like thickening of the cortical bone, hypertrophic and fibrillary bones. Progressive or chronic neurological deficits should be treated with bisphosphonates. We present a rare case of multiple cranial nerve palsies as the first manifestation of PDB.

Cranial Nerve Impairment Associated With COVID-19 Infections: A Systematic Review.

The COVID-19 pandemic has created huge economic and healthcare burdens. In most cases, the virus affects the lungs and causes respiratory symptoms. Additionally, its impact on the cranial nerves remains unclear. We thus aimed to investigate cranial nerve dysfunction in patients with COVID-19 infection.  We conducted a systematic literature search of relevant and eligible literature in five databases: PubMed, Web of Science, Medline, EBSCO, and Google Scholar.  Our sample included 21 case reports, one case series with 29 patients, and one analytical study with 135 cases. Participant ages ranged from 23 months to 72 years (mean age of 47.5 ± 19.02). The mean time from respiratory symptoms to the onset of neurological signs was (9.6 ± 7.4) days, and the mean recovery time was (16.3 ± 15.3) days.  Cranial nerve impairment associated with COVID-19 infection has affected a large population, from infants to the elderly. Facial and abducent nerves were the most commonly affected cranial nerves with reported good prognosis or complete recovery within a few days to weeks. Olfactory dysfunctions were widely detected among COVID-19 patients.