Multiple Cranial Nerve Palsies Research Articles

Multiple Cranial Nerve Palsies in the Setting of Herpes Zoster Ophthalmicus (P1-13.006)

Multiple Cranial Nerve Palsies in the Setting of Herpes Zoster Ophthalmicus (P1-13.006)

A prospective study on the incidence and outcome of cranial nerve injuries in patients with traumatic brain injuries

Background: The worldwide mortality and morbidity rates are highest for traumatic brain injury (TBI), which frequently involves damage to the cranial nerves (CNs). The occurrence of CN injury (CNI) in craniocerebral trauma ranges from 5 to 23%. Aims and Objectives: The objective of this study is to evaluate the occurrence of CNI in TBI patients within our population and determine the association between CN involvement and the severity of head injuries. In addition, we aim to assess the outcomes of patients who experience CNI in cases of head trauma. Materials and Methods: In our institution, a prospective observational analytical study was conducted from July 2022 to June 2023. The study included 100 patients aged over 1 year who had sustained head injuries. These patients were followed up for a period of 6 months. Results: Our study included a total of 100 patients, revealing that the highest number of head injuries occurred within the age range of 20–60 years, with an average age of 46 years. Among the study population, males accounted for 74%. Of these patients, 66 had mild head injuries, 22 had moderate head injuries, and 12 were admitted with severe head injuries. We discovered that 15% of TBI patients experienced CNIs. Specifically, five patients had a single CN palsy, while three patients had multiple CN palsies. Road traffic accidents accounted for 78% of the cases, making them the most common cause of injury, while low-velocity injuries only made up 22% of the cases. The facial nerve was the CN most frequently affected, followed by the olfactory nerve, optic nerve, and vestibulocochlear nerve. Among the 15 patients with CNI, seven had delayed symptoms, whereas eight patients presented with immediate symptoms. Conclusion: A substantial proportion of patients experience a delayed onset of CNI symptoms. Therefore, it is essential to perform a comprehensive neurological examination of all CNs during follow-up for all TBI patients.

Multiple cranial nerve palsies with altitudinal hemianopsia following traumatic brain injury in remote area: A case report

Multiple cranial nerve palsies with altitudinal hemianopsia following traumatic brain injury in remote area: A case report

Immunoglobulin G4-Related Hypertrophic Pachymeningitis Presenting with Multiple Lower Cranial Nerve Palsies

Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ condition that infrequently exhibits neurological involvement. Here, we describe a case of IgG4-related hypertrophic pachymeningitis with multiple lower cranial nerve palsies. A 65-year-old man presented with a 5-month history of hoarseness and dysphagia. A neurologic examination revealed multiple lower cranial nerve palsies. Brain magnetic resonance imaging indicated diffuse dural involvement along the left tentorium and temporal fossa. Serum IgG and IgG4 levels were found to be elevated. Ethmoid polyp biopsy revealed chronic inflammation with moderate lymphoplasmacytic and some eosinophilic infiltration. Up to 60 IgG4-positive cells were observed per high-power field, and the measured IgG4/IgG ratio was approximately 10% to 20%. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography indicated abnormally high FDG uptake in the left posterior fossa, tentorium, and left temporal fossa, with additional involvement of the lung, iliac artery, and renal artery. Based on these findings, we diagnosed the patient with IgG4-related hypertrophic pachymeningitis and successfully treated the condition with steroids and rituximab. IgG4-RD should be differentiated from distinct conditions presenting with multiple cranial nerve palsies accompanied by pachymeningitis.

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.

A study on etiological and clinical profile of multiple cranial nerve palsies

A study on etiological and clinical profile of multiple cranial nerve palsies

Concurrent Isolated Hypoglossal Nerve Palsy Induced by Jugular Vein Thrombosis, Cervical Plexus Masses, and Vestibular Schwannoma: A Complex Case Report Highlighting Anatomical Pathways and Clinical Challenges

Background: Hypoglossal nerve palsy is typically observed alongside other cranial nerve impairments, with isolated cases being infrequent and posing diagnostic complexities. Originating intracranially, the nerve traverses the skull and neck, governing tongue motility. Lesions are classified as supranuclear, nuclear, or infranuclear, engendering tongue deviation towards the affected side. Due to its proximity to crucial structures, hypoglossal nerve palsy often coincides with supplementary symptoms. Etiologies encompass skull base metastases, carotid artery pathologies, and trauma. This study explores less conventional triggers such as hypoglossal nerve palsy following thyroidectomy, jugular vein thrombosis, cervical plexus masses, and Vestibular Schwannoma. Case Description: A 38-year-old female presented to the Neurology outpatient clinic with leftward tongue deviation and left-sided neck and shoulder pain accompanied by stiffness that had begun several months prior. In addition to the tongue deviation, she reported minor dysphagia, blurry vision, and intermittent numbness on the left side of her scalp. MRI and duplex ultrasound verified the presence of left internal jugular vein (IJV) thrombosis. The patient was initially prescribed Eliquis and acetazolamide, with slight improvements in symptoms, though medication use ceased due to side effects. During a subsequent follow-up visit, persistent tongue deviation and worsening left shoulder pain prompted additional cervical imaging. A new cervical spine MRI revealed a 15x14 mm nodular dural enhancement with a paraspinal soft tissue mass at the C2 level. Concurrently, multiple cranial nerve palsies were detected during the physical examination. Unfortunately, the patient was subsequently diagnosed with breast cancer. To rule out brain metastases related to her breast cancer, an additional brain MRI was performed, revealing a newly formed 2.3x1.6x1.2 cm left cerebellopontine angle mass adjacent to the internal auditory canal (IAC), suggestive of a vestibular schwannoma but without evidence of metastatic disease. The patient's care involved a multidisciplinary team, including hematologist-oncologists, neurosurgeons, and neurologists. Conclusion: This case report highlights the less common causes of hypoglossal nerve palsy, such as jugular vein thrombosis, cervical plexus masses, and Vestibular Schwannoma. The persistence of the patient's palsy is attributed to the cumulative impact of these anatomical factors on the hypoglossal nerve. This underscores the diagnostic challenges posed by isolated hypoglossal nerve palsy and emphasizes the importance of comprehensive clinical evaluation and imaging for accurate diagnosis.

A Case Report: Rhino-Orbito-Cerebral Mucormycosis with visual impairment and cranial nerve palsies in a tertiary care hospital Sri Lanka

Mucormycosis is an angioinvasive infection, especially among immunocompromised patients. There are several forms of the disease and rhino-orbito-cerebral mucormycosis is the commonest presentation globally, especially among patients with diabetes mellitus with or without ketoacidosis. We report a patient with rhino-orbito-cerebral mucormycosis involving multiple cranial nerve palsies.A 70-year-old female patient with uncontrolled diabetes mellitus presented with left periorbital swelling and reduced vision on the same side along with epistaxis. On examination, she was found to have multiple cranial nerve palsies. She was managed as rhino-orbito-cerebral mucormycosis after performing several microbiological, histological, and radiological tests. The patient was discharged after 4 months of hospital stay.Despite successful medical and surgical interventions, the patient died a week after being discharged from the hospital. Though we have no strong evidence, the disease itself, her co-morbidities, sepsis, and surgical trauma could have contributed to her death.

A Study on Etiological and Clinical Profile of Multiple Cranial Nerve Palsies

A Study on Etiological and Clinical Profile of Multiple Cranial Nerve Palsies

Polyneuritis cranialis related to SARS-CoV-2 infection: a case report

Purpose: Polyneuritis cranialis (PNC) is a less known regional variant of Guillain-Barré syndrome and has rarely been described in children. Here, we report a case of an adolescent, who became perilously ill following severe acute respiratory syndrome-coronavirus 2 infection.
 Case report: A 15-year-old boy presented with acute multiple cranial nerve palsies. The presence of GQ1b antibodies and nerve conduction studies confirmed the diagnosis of PNC. The patient was treated with plasmapheresis and intravenous immunoglobulins, but clinical improvement was gradual and very slow.
 Conclusion: PNC is a rare and severe neurologic condition that can be triggered by coronavirus infection.

Antivirals vs Antivirals and Steroids for Treatment of Herpes Zoster-Related Ophthalmoplegia: A Case Series and Systematic Review.

Up to 1/3 of patients with herpes zoster ophthalmicus (HZO) may develop ophthalmoplegia. Although zoster-related ophthalmoplegia (ZO) is typically treated with antiviral agents, there is controversy regarding the therapeutic role of systemic steroids. This was a retrospective case series and case report-based systematic review. For the case series, participants were recruited from tertiary neuro-ophthalmology clinics. Eligible participants were those who developed cranial nerve palsies (CNP) within 1 month of HZO diagnosis. In the systematic review, all adults with ZO in the literature who were treated with antivirals or steroids only, or combination therapy were included. Main outcomes were initial presentation, investigations, neuroimaging, treatment regimen, and final outcomes of ophthalmoplegia. Eleven immunocompetent patients with ZO were included. The most common CNP was CN III (5/11), followed by CN VI (2/11) and CN IV (2/11). One patient had multiple CNPs. All patients were treated with antivirals, and 4 also treated with a short course of oral steroids. At 6-month follow-up, 75% of patients treated with combination therapy and 85.7% treated with antivirals alone had complete recovery of ZO. The systematic review identified 63 studies consisting of 76 cases of ZO. When comparing patients treated with antivirals with those treated with antivirals and steroids, patients on combination therapy had more severe ocular findings, including complete ophthalmoplegia (P < 0.001). Age was the only significant predictor of complete recovery of ophthalmoplegia on multivariable logistic regression (P = 0.037). The rate of complete recovery in immunocompetent patients with ZO was similar in patients treated with antivirals alone vs those treated with antivirals and oral steroids. The systematic literature review affirmed these findings. However, age may influence recovery of ophthalmoplegia.

Multiple Cranial Nerve Palsy &amp; Choroid Tuberculoma in a Patient with Disseminated TB: A Clinic Biochemical Study

Disseminated Tuberculosis is a potentially lethal disease if not diagnosed and treated early. A high index of clinical suspicion and early diagnosis and timely institution of anti TB treatmen is life saving.Patient’s sociodemographic, clinical, labarotory investigations and treatment outcome were recorded in a case record form Here we report a case of 42 year old male, known case of diabetes mellitus, presented with the complaints of fever and headache for 1 month, dropping of both eyelids, diplopia and painful red eyes for 7 days. There was no sign of meningeal irritation. Neurological examination revealed proptosis and 3rd, 4th, 6th cranial nerve palsy as evident by bilateral ptosis, diplopia, restricted ocular movement in all direction. Fundoscopy showed choroid tubercle on left eye. CSF study suggestive of tubercular meningitis. CXR showed pleural effusion and pleural fluid study suggestive of tuberculous pleural effusion. We diagnosed him as disseminated tuberculosis (tubercular meningitis, ocular TB, pleural TB). After starting anti tubercular regimen and steroid, patient’s condition got improved. Disseminated tuberculosis usually presents with constitutional symptoms rather than respiratory features. Tubercular meningitis not always present with features of meningism. Choroid tubercle is pathognomic for tubercular meningitis. Early diagnosis and treatment can reduce the mortality, morbidity and complications of disseminated TB. J Medicine 2023; Vol. 34, No. 2(1) Supplement: 224

Coexistence of Cerebellopontine Angle Tumor and Frontal Convexity Meningioma at Distinct Location – A Rare Case Report and Review of Literature

The perioperative management of brain tumors can be significantly impacted by the presence of another intracranial tumor at a distinct location. We report a rare case of an adult who developed two primary brain tumors, a frontal convexity meningioma and a cerebellopontine angle (CPA) tumor at anatomically disparate locations. In our patient, it was unambiguously decided to address the CPA tumor first as it had led to multiple cranial nerve palsies and obstructive hydrocephalus. The relevant literature helped us better extrapolate the patient presentation and management of this disastrous presentation swiftly.

A RARE CASE OF MADRAS VARIANT OF MOTOR NEURON DISEASE

INTRODUCTION: Madras variant MND is a rare entity is characterized by multiple lower cranial nerve palsies(7-12) and atrophy of limbs. A 26 year old male presented with complaints of difculty i BACKGROUND: n hearing and hoarseness of voice since 1 year and weakness of lower limbs since 10 days without any sensory, bowel and bladder symptoms. On examination, his vitals were stable. Cranial nerves examination revealed abnormality in the form of b/l sensorineural hearing loss, weakness of b/l facial muscles with fasiculations, absent gag reex with atrophy and fasiculations of the tongue. There was signicant wasting of muscles of face, upper and lower limbs with hypotonia in upper and hypertonia in lower limbs. Reexes were feeble in upper limb and exaggerated in lower limb. On investigation EMG-NCS showed chronic partial denervation of all muscles with reduced recruitment. Very few cases of CONCLUSION: Madras variant of MND have been reported due to rare combination of features and often misdiagnosed.

Ramsay Hunt Syndrome with Pharyngolaryngeal Involvement Mimicking Acute Stroke: A Case Report.

Ramsay Hunt syndrome is a clinical manifestation of the reactivation of latent varicella zoster virus in the geniculate ganglion after acute infection. It is commonly associated with an acute peripheral facial nerve paralysis, auricular vesicular eruption and acute sensorineural hearing loss. However, in some rare cases the involvement of other cranial nerves, especially the glossopharyngeal and vagal is described. We present a 56-year-old male patient who was diagnosed with acute pharyngitis. Two days later, the patient developed dysarthria, hoarseness, and left side facial weakness. Considering a patient's history of cardiovascular events, the acute stroke was suspected. Magnetic resonance imaging ruled out an acute cerebrovascular accident. The patient's otalgia aggravated and he reported hearing loss. Clinical examination revealed facial paralysis affecting the left side of the face, erythematous rash on the left auricle, multiple vesicles present on patient's left tympanic membrane, ulcerous lesion on the left side of the soft palate and ipsilateral velopharyngeal palsy. Indirect laryngoscopy revealed ulcerous lesions in the left side of the epiglottis and left vocal cord paralysis. The patient was diagnosed with Ramsay Hunt syndrome with cranial polyneuropathy. Oral acyclovir and oral prednisolone were administered. Seven months after discharge, facial paralysis improved to lower grade and flexible laryngoscopy showed that the left vocal cord had resumed normal movement. Ramsay Hunt syndrome can be accompanied by involvement of other cranial nerves. Previous history of hypertensive crises and myocardial infarction may influence a diagnosis of Ramsay Hunt syndrome, especially when it has atypical presentation. However, this diagnosis should be considered in patients with unilateral multiple cranial nerve palsies.

Secondary Malignancy In Giant Cell Tumor Of The Skull Base After Denosumab Treatment: Case Report

Abstract Giant cell tumor of bone is a rare neoplasm characterized by its unpredictable behavior, possible malignant transformations, and/or lung metastases. Surgery is the treatment of choice. In unresectable or metastatic cases, treatment with denosumab is a new treatment option. In October 2015, a 14-year-old female presented with cachexia, dysphagia, diplopia, discoordination, strabismus, and multiple cranial nerve palsies. An MRI examination revealed an intra-extracranial mass arising from C2 vertebrae, compressing the medulla oblongata and the left cerebellar hemisphere, invading the sphenoid bone and nasopharynges. The biopsy results revealed the presence of a giant cell tumor of bone. The first surgical resection was incomplete because of tumor location (cranial nerve and vertebral artery involvement). The patient received local radiotherapy with 50.4Gy, but the patient’s condition worsened during this period and subsequent MRI examination showed disease progression. In March 2016, the administration of denosumab at a dosage of 120 mg every 4 weeks was initiated, and induced rapid clinical improvement and radiographically proven partial response. Disease was under control for three years until March 2019, when she returned with clinical symptoms of diplopia and severe headache. MRI showed local tumor progression. Repeated biopsy revealed an undifferentiated pleomorphic sarcoma. Two cycles of chemotherapy with Ifosfamide/Doxorubicin were administered, but MRI after chemotherapy showed marked tumor progression. The patient received palliative care and died due to disease progression in December 2019 – 4 years after initial diagnosis. To our knowledge, this is the youngest patient ever reported with a skull base Giant cell tumor initially responding to denosumab for 3 years but progressing to chemotherapy resistant undifferentiated pleomorphic sarcoma.

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.

Syringobulbia with Syringomyelia Presenting as Unilateral Multiple Cranial Nerve Palsies with Ipsilateral Hemiparesis in an Adult: A Rare Case and Literature Review.

Syringobulbia with Syringomyelia Presenting as Unilateral Multiple Cranial Nerve Palsies with Ipsilateral Hemiparesis in an Adult: A Rare Case and Literature Review.

Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases

BackgroundDetecting immunoglobulin G4 (IgG4)-related intracranial arteriopathy, a rare neurovascular complication of IgG4-related disease, is challenging. While magnetic resonance (MR) vessel wall imaging (VWI) can visualize various neurovascular pathologies, its application to this arteriopathy has not been reported as of this writing.Case presentationA 74-year-old male and a 65-year-old female manifested multiple cranial nerve palsy and neck pain, respectively. Both cases exhibited multiorgan masses with markedly elevated serum IgG4 levels and were clinically diagnosed with IgG4-related disease. Three-dimensional T1-weighted black blood VWI with and without contrast agent identified intracranial vascular lesions characterized as nearly-circumferential mural thickening with homogeneous contrast enhancement in the internal carotid and vertebral arteries; some of the lesions had been unrecognized with screening MR angiography due to expansive remodeling. The former patient underwent corticosteroid therapy, and VWI after treatment revealed decreased mural thickening and enhancement.ConclusionFurther studies to elucidate characteristic findings of VWI might contribute to early detection of this treatable pathology.

A Rare Presentation of CVT

Cerebral Venous Thrombosis (CVT) is an uncommon condition presenting with varying clinical symptoms, posing difficulty in diagnosis. Patients present with headaches, focal neurological deficits, seizures, and altered consciousness. CVT does not have a single clinical presentation, and it is important to consider CVT in all brain syndromes, especially young patients, and confirm it through imaging studies. Other uncommon presentations of CVT include single or multiple cranial nerve palsies.