Multiple Cranial Nerve Involvement Research Articles

Ramsay Hunt Syndrome: A Rare Disease of Multiple Cranial Nerve Involvement

Ramsay Hunt Syndrome (RHS) also known as herpes zoster oticus is a viral disease, a member of the human herpes virus family, is a late complication of varicella-zoster virus infection that results in inflammation of the geniculate ganglion of cranial nerve VII. Ramsay Hunt is a clinical diagnosis. The hallmark of the condition is multiple unilateral erythematous vesicles, which are distributed over the auricle and preceded by severe otalgia. If these symptoms are associated with facial nerve palsy, the condition is called RHS which is usually accompanied by vestibulocochlear abnormalities. Diagnosis is often missed or delayed, which can lead to an increased incidence of long-term complications. The condition is self-limiting, but treatment is targeted at decreasing the total duration of the illness as well as providing analgesia and preventing the complications that can occur. This activity reviews the role of the inter professional team in the diagnosis and treatment of RHS. Mugda Med Coll J. 2024; 7(1): 48-52

Atypical Herpes Zoster Ophthalmicus with Multiple Cranial Nerve Involvement in an Immunocompetent Individual (P1-13.005)

Atypical Herpes Zoster Ophthalmicus with Multiple Cranial Nerve Involvement in an Immunocompetent Individual (P1-13.005)

Challenging neurological presentation: Scrub typhus with meningoencephalitis and multiple cranial nerve involvement

AbstractA 40‐year‐old male farmer from rural West Bengal, India, presented with fever, headaches, seizure, eschar, and palsy of left cranial nerves VII, VIII, IX, and X as a rare clinical manifestation of scrub typhus. Positive IgM enzyme‐linked immunosorbent assay tests in serum and cerebrospinal fluid confirmed the diagnosis, with brain magnetic resonance imaging showing meningoencephalitis findings. Treatment with steroids and doxycycline led to neurological improvement. This case highlights the need for heightened clinician awareness toward early detection of scrub typhus's atypical presentations to ensure timely and effective treatment.

Multiple Cranial Nerve Involvement in a Complex Case of MISME Syndrome in a Paediatric Patient: A Case Report

Neurofibromatosis 2 (NF2) is characterised by numerous tumours in the central and peripheral nervous systems due to NF2 gene abnormalities that cause the tumour suppressor protein, Merlin, to disappear. Often referred to as Multiple Inherited Schwannomas, Meningiomas, and Ependymomas (MISME), a distinctive characteristic of NF2 is bilateral vestibular schwannomas manifesting in late adolescence with symptoms such as sensorineural hearing loss, tinnitus, and balance issues. Two distinct phenotypes, Wishart and Feiling-Gardner, characterise NF2. This case report discusses the case of a paediatric patient who presented with bilateral hearing loss, giddiness, and blurring of vision and sought a Magnetic Resonance Imaging (MRI) examination which revealed bilateral vestibular schwannomas, non vestibular schwannomas, left sphenoid wing meningiomas, multidirectional spinal schwannomas, spinal nerve sheath tumours, and lesions in the retroperitoneal region. Despite an absent family history, significant involvement of cranial nerves strongly indicates classical NF2. Management focuses on preserving function, and surgery is contemplated for symptomatic lesions and tumours causing cord compression. Gamma Knife radiosurgery and targeted therapies have been investigated.

Planet of the apex

A 63-year-old man with diabetes presented with unilateral ptosis and an exotropia. A diagnosis of isolated diabetic III nerve palsy was made. Subsequent neuro-ophthalmologic evaluation showed multiple cranial nerves involvement consistent with a diagnosis of orbital apex syndrome. Review of past medical history was significant for a previous nasopharyngeal carcinoma, and biopsy of the involved site was consistent with tumor recurrence. This case highlights the importance of correct medical history taking and anatomo-clinical correlation in neuro-ophthalmology.

Sinonasal mucormycosis during 2 years of COVID-19 pandemic in central Iran: incidence rate and clinical features

IntroductionAs a novel infectious disease, COVID-19 is caused by SARS-COV-2, spreading rapidly worldwide. ENT specialists have faced this challenging disease in various ways since the emergence of the COVID-19 pandemic. We are currently facing an increase in cases referred due to sinonasal mucormycosis which is a rare but invasive, rapidly progressive, and life-threatening infection. We provide an overview of this disease's incidence rate and clinical features. MethodsThis descriptive cross-sectional study was conducted on 46 sinonasal mucormycosis patients who were histopathologically confirmed after sinonasal endoscopic surgery in our educational therapeutic hospital during 2 years of the COVID-19 pandemic from March 20, 2020, to March 20, 2022. ResultsThere was an increase in the incidence of mucormycosis more than twice as much as before. All patients had a history of COVID-19 and 69.6% were diabetic. The median time to symptom onset from COVID-19 detection was 3.3 weeks. A total of 60.9% received steroids while 85.7% were prescribed during COVID-19 treatment. The most common manifestation was orbital involvement (80.4%). Of the 46 study cases, unfortunately, 17 (37%) died. An exciting point in our study was the incidence of peripheral facial palsy which is associated involvement of multiple other cranial nerves (II, III, IV, V, VI) considered to be the likely occurrence of a rare phenomenon called Garcin's syndrome. ConclusionBased on the results of this study, during 2 years of the COVID -19 pandemic, there was an increase in the incidence of sinonasal mucormycosis more than twice as much as before.

Multiple cranial neuropathy due to varicella zoster virus reactivation without vesicular rash: a challenging diagnosis.

Ramsay Hunt syndrome is due to reactivation of varicella zoster virus (VZV) dormant in the geniculate ganglion of the facial nerve. The diagnosis is typically based on clinical triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or the auricle. However, Ramsay Hunt syndrome may occur without skin eruption in up to onethird of patients. Moreover, the involvement of other cranial nerves in addition to the facial nerve has been also reported. Herein, we reported a case report of a man who developed a multiple cranial neuropathy caused by VZV reactivation without skin vesicular eruption. The present case underlines a possible diagnostic challenge that clinicians may hit when facing a common disorder such as peripheral facial palsy. Indeed, clinicians must be aware that Ramsay Hunt syndrome may develop without skin vesicular eruption as well it may be complicated by multiple cranial nerve involvement. Antiviral therapy is effective in VZV reactivation for recovery of nerve function.

Characteristics of facial nerve palsy in 112 children and risk factors for ocular complications

To report the ophthalmic findings and ocular complications in a large cohort of pediatric patients with facial nerve palsy (FNP). Ocular data of children (≤16 years of age) diagnosed with FNP presenting to an eye care network from 2012 to 2021 were analyzed. Study parameters were etiology of FNP, ocular and imaging findings, degree of lagophthalmos, and degree of vision loss. Clinical characteristics were compared between those with and without moderate-to-severe vision impairment (best-corrected visual acuity <20/50) and those with and without exposure keratopathy at presentation. A total of 112 patients were included. Mean age at presentation was 8.3 ± 5.0 years. The most common etiology was idiopathic (57%) followed by congenital (22.3%) and traumatic (13.4%). There was bilateral involvement in 8% of children, multiple cranial nerve involvement in 15.2%, and exposure keratopathy at presentation in 38.4%. One-fifth (20.5%) of children (29.6% of affected eyes with known visual acuity) had moderate-to-severe visual impairment. Multiple cranial nerve involvement was present in 31% of eyes with visual impairment compared with 14% of those without. Corneal scarring and strabismic amblyopia were both frequent causes of visual impairment. Most children with exposure keratopathy had lagophthalmos (76.6%), whereas it was less common in those without keratopathy (49.2%). Pediatric FNP was most commonly idiopathic, secondarily congenital. Strabismic amblyopia and corneal scarring were the most common causes of visual impairment in our cohort.

Sinonasal mucormycosis during 2 years of COVID-19 pandemic in central Iran: incidence rate and clinical features

IntroductionAs a novel infectious disease, COVID-19 is caused by SARS-COV-2, spreading rapidly worldwide. ENT specialists have faced this challenging disease in various ways since the emergence of the COVID-19 pandemic. We are currently facing an increase in cases referred due to sinonasal mucormycosis which is a rare but invasive, rapidly progressive, and life-threatening infection. We provide an overview of this disease's incidence rate and clinical features. MethodsThis descriptive cross-sectional study was conducted on 46 sinonasal mucormycosis patients who were histopathologically confirmed after sinonasal endoscopic surgery in our educational therapeutic hospital during 2 years of the COVID-19 pandemic from March 20, 2020, to March 20, 2022. ResultsThere was an increase in the incidence of mucormycosis more than twice as much as before. All patients had a history of COVID-19 and 69.6% were diabetic. The median time to symptom onset from COVID-19 detection was 3.3 weeks. A total of 60.9% received steroids while 85.7% were prescribed during COVID-19 treatment. The most common manifestation was orbital involvement (80.4%). Of the 46 study cases, unfortunately, 17 (37%) died. An exciting point in our study was the incidence of peripheral facial palsy which is associated involvement of multiple other cranial nerves (II, III, IV, V, VI) considered to be the likely occurrence of a rare phenomenon called Garcin's syndrome. ConclusionBased on the results of this study, during 2 years of the COVID -19 pandemic, there was an increase in the incidence of sinonasal mucormycosis more than twice as much as before.

Neuroblastoma Presentation With Multiple Cranial Nerve Involvement

Objective NA. Background We report a case of neuroblastoma, a pediatric neuroendocrine tumor of the sympathetic nervous system, in a 3-year-old female with multiple cranial nerve involvement. Design/Methods A 3-year-old afebrile, lethargic female presented with bilateral eyelid droop, right head tilt, slurred speech, gagging, abnormal walking and no bowel movement. Neurological examination noted bilateral ptosis, dysarthria, left tongue deviation, proximal weakness in upper and lower extremities, areflexia in biceps and patellar tendons, dysmetria, and wide-based gait. MRI of the brain showed heterogeneous appearance of the clivus and MRI of the spine showed right adrenal mass and heterogeneous enhancement of multiple vertebrae, suggesting possible metastatic disease. Serum and cerebrospinal studies were unremarkable. Patient was treated with intravenous methylprednisolone and plasmapheresis for suspected paraneoplastic syndrome; however, she continued to clinically progress. Adrenal mass biopsy results and elevated urine VMA and HVA levels were consistent with the diagnosis of neuroblastoma. Nuclear imaging and meta-iodobenzylguanidine scan were negative. Paraneoplastic panels and Lambert Eaton panel were negative for autoantibodies. Total resection of the abdominal mass and right adrenal gland with continued steroid taper, resulted in reported near total symptom resolution. Results Our patient presented with antibody negative paraneoplastic polyneuropathy due to neuroblastoma. Cranial nerve involvement in neuroblastoma can result from tumor involvement of the sympathetic chain, paraneoplastic syndromes, or metastasis to the skull. Our patient's initial imaging suggested potential metastatic or inflammatory involvement of the clivus, suspected of causing her cranial nerve symptoms. However, nuclear studies were negative for metastatic disease and patient's symptoms resolved after resection of the main mass suggesting a paraneoplastic etiology. Although her paraneoplastic panel and Lambert-Eaton panel were negative for common associated antibodies, it is likely an unmeasured autoantibody, cytokine, hormones, or peptide was at play. Conclusions Neuroblastoma should be considered as a differential for a neurological presentation involving multiple cranial nerves in a child.

“Trigeminal Tract Sign” in Patients With Herpes Zoster Ophthalmicus: A Case Series of a Novel Imaging Finding

Objective Herein, we report three patients presenting with zoster ophthalmicus caused by varicella zoster virus (VZV) with unexpected and novel cervico-medullary findings on magnetic resonance imaging (MRI). Background Zoster opthalmicus involves reactivation of latent VZV in the ophthalmic division of the trigeminal nerve producing a dermatomal rash. In a minority of patients this leads to ophthalmic findings and damage to surrounding peripheral nerves through perineural/intraneural inflammation. MRI findings in zoster opthalmicus are often nondescript or normal leading to misdiagnosis and delays in treatment. Design/Methods This was a case-series describing patients in Calgary, Alberta who presented with zoster opthalmicus, and who were noted incidentally to have similar appearing lesions in the ipsilateral cervico-medullary region on brain MRI. Standard MRI imaging of the brain and selected high-resolution images of the orbits and globes were reviewed by neuroradiology (CW). Results Three patients, with varying phenotypes of zoster opthalmicus, are described: a 70-yo male with ipsilateral decreased visual acuity and oculomotor/abducens palsy; a 75-yo female with ipsilateral oculomotor/facial nerve palsies; and a 35-yo immunocompromised female with ipsilateral blepharoconjunctivitis and optic neuritis. In all 3 cases, MRI revealed profound T2 and fluid-attenuated inversion recovery (FLAIR) hyperintensity along the spinotrigeminal tract of the clinically affected side, extending from the posterolateral pons and medulla into the cervical region. Conclusions To our knowledge, hyperintensity within the medulla and spinal trigeminal tract in zoster opthalmicus is a rare imaging finding. We have coined this radiologic finding the “trigeminal tract sign” due to the anatomic structure affected. A thorough radiologic/laboratory workup should be considered in patients with similar presentations; however, a high index of suspicion for zoster opthalmicus should be considered in cases with the aforementioned imaging findings. Whether this radiological finding predicts disease severity or the likelihood of multiple cranial nerve involvement (as was seen in all patients in this series) requires further study.

Dysphagia and Tongue Deviation: An Unexpected Cause.

Dysphagia and Tongue Deviation: An Unexpected Cause.

Ramsay hunt syndrome with multiple cranial nerve involvement

Ramsay Hunt Syndrome (RHS) is characterized by vesicles in external auditory canal, ear discomfort, and facial nerve palsy in the periphery. RHS which affects multiple cranial nerve involvement has rarely happened. This study is about RHS case that affect right multiple cranial nerves VI, VIII, IX, X. A 46-year old male patient complaint of crust and pain from right ear lobe. There were complaints of decreased hearing ability, tinnitus, difficulty to swallow, double vision, incapability of closing the right eyelid, and a facial slight palsy to the right. Multiple yellowish crusts and erosions were identified. Tzanck and Polymerase Chain Reaction test were not performed. Patient was diagnosed RHS with right multiple cranial nerves VI, VIII, IX, X, received Acyclovir and Prednisone tablets for 7 days. Patient improved. This finding showed that a detailed history taking, physical and additional examinations are required for accurate diagnosis for RHS multiple cranial nerve involvement.

Involvement of cranial nerves in ATTR Ile127Val amyloidosis

The involvement of cranial nerves is rare in ATTR amyloidosis. However, involvement has occasionally been reported in the p.Val50Met variant, the most commonly studied worldwide. On the other hand, in ATTR p.Ile127Val, an uncommon variant, the cranial nerves IX, X, and XII, are frequently involved.Here, we present a series of cases of ATTR Ile107Val amyloidosis, in which the involvement of multiple cranial nerves V, VII, IX, X, XI, and XII seems to be routinely included in phenotypic manifestations in different phases of clinical evolution, contributing significantly to the resulting disabilities caused by this variant. The recognition of these manifestations enables earlier diagnoses and reduces complications from the involvement of cranial nerves, such as dysphagia and respiratory impairment, which greatly increase the burden caused by the disease and significantly limit the quality of life.

COVID-19 associated multiple cranial neuropathies

The polymorphism of the clinical manifestations of coronavirus infection often creates great diagnostic difficulties for the practitioners, especially in patients with a predominance of neurological symptoms. Of the latter, cranial neuropathies take a particular place, as they may be either one of the first symptoms or appear during the disease progression. Impairment of smell and taste is considered an early manifestation of SARS-CoV2 infection. Similar to the involvement of the olfactory bulb, multiple cranial nerves involvement (for example, VII, VI, and III) has been described. Patients with COVID-19 associated multiple cranial neuropathies are more likely to experience partial recovery of impaired function. We present and discuss a rare case of COVID-19 associated multiple cranial neuropathies with a sequential involvement of the olfactory (I), right visual (II), frontal branch of the trigeminal (V), facial (VII), and hypoglossal (XII) nerves. Data on clinical manifestations and diagnostic criteria for this form of pathology are presented.

Oculomotor Cranial Neuropathies: Diagnosis and Management.

Ocular nerve palsies are among the most common cranial neuropathies in neurological practice. Nerves can get affected anywhere along their path from the brainstem to the orbit. There can be isolated involvement of multiple cranial nerves together. The etiologies differ according to the type of presentation. The steps toward the diagnosis need to be strategically planned and must be based on clinical localization. It is crucial to make proper localization to plan further investigations and thus treatment of the etiology. This review covers the approach toward the diagnosis, etiologies involved, and management of ocular cranial neuropathies.

Multiple cranial nerve involvement as a presentation of primary central nervous system lymphoma: A case-based review

Central nervous system (CNS) lymphoma is of two types: primary and secondary (more common). Primary CNS lymphoma usually presents as parenchymal lesions having characteristic imaging findings and may be associated with leptomeningeal involvement. Involvement of multiple cranial nerves as the initial manifestation of primary CNS lymphoma with the development of typical parenchymal lesions on follow-up is a rare entity. This nerve involvement is termed as neurolymphomatosis. We present the magnetic resonance imaging features of five patients presenting with neurolymphomatosis due to non-Hodgkin's lymphoma.

Persistent hiccups as a rare presentation of Ramsey Hunt syndrome in a renal transplant recipient

Ramsay Hunt syndrome type 2, also known as RHS and herpes zoster oticus, is a disorder that is caused by the reactivation of varicella zoster virus in the geniculate ganglion, a nerve cell bundle of the facial nerve. RHS typically presents with inability to move many facial muscles, pain in the ear, taste loss on the front of the tongue, dry eyes and mouth, and a vesicular rash. However, the association of RHS with multiple cranial nerve involvement and persistent hiccups is less well reported.

Herpes Zoster Oticus With Delayed Facial Palsy and Multiple Cranial Nerve Involvement

Herpes zoster oticus with multiple cranial nerve (CN) involvement and delayed facial paralysis is a rare occurrence, and there is limited information regarding the disease. We herein report two cases of RHS with delayed facial palsy involving multiple lower CNs. The degree of facial paralysis was not severe as grade III or less, and recovery was observed after treatment. To our knowledge, these are the first cases in the literature to report RHS with delayed facial paralysis and its favorable recovery. It will be a good example for expanding the possibility of treatment in RHS with delayed facial paralysis and multiple CN involvement.

Tuberculosis of the parotid gland associated with lower cranial neuropathy

Introduction: Parotid gland involvement as a form of extra pulmonary tuberculosis is a very rare manifestation. Extension of tuberculosis to skull base with multiple cranial nerve involvement is even rarer. We report a case of atypical presentation of tuberculosis with multiple cranial nerve palsies.