Multiple Lower Cranial Nerve Palsies Research Articles

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 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.

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.

Predicting Outcome in Skull Base Osteomyelitis: An Assessment of Demographic, Clinical, and Pathological Attributes.

Objective Skull base osteomyelitis (SBO) is an enigmatic clinical diagnosis which is difficult to decipher and is associated with poor outcomes. The study aims to examine the demographic and clinical characteristics of patients with SBO and its outcomes. Materials and Methods Medical records of 30 patients with diagnosis of SBO over past 5 years were assessed for demographic and clinical characteristics, type of SBO, radiological parameters, treatment received, procedure performed, microbiological profile, comorbidities, and complications including cranial nerve (CN) palsies. These factors were analyzed for prediction of outcome (death or survival). Statistical Analysis Microsoft Office Excel 2010 SAS 10.0 for Windows was used. Student's t -test for continuous variables (age, duration of symptoms, number of days of hospitalization, and treatment duration) and chi-square test for categorical variables (imaging findings, symptomatology, presence of comorbidities, surgical procedure, complications, and type of antibiotics) were utilized. Results We found SBO was the disease of elderly population (64.07 ± 6.13 years) with male predominance (83.3%) highly associated with uncontrolled diabetes status (93.3%). Headache (100%) and CN palsy (80%) were the most common neurological presenting complaints followed by stroke (17%) and encephalopathy (10%). Pathological and radiological correlation showed that fungal infection ( Aspergillus ) was associated with anterior SBO (10%), while bacteria ( Pseudomonas ) was cultured from posterior SBO (30%). Fifty per cent of patients were alive after 1 year out of which 33% had good functional outcome. The mortality rate was 33.3% in our cohort and multiple lower CN palsies ( p = 0.04), suboptimal duration of medical treatment ( p = 0.03), surgical intervention during clinical course ( p = 0.02), and development of intracranial or extracranial complications ( p = 0.03) were the predictors of mortality. Conclusion Early diagnosis including identification of pathogenic organisms and optimal duration of treatment are crucial factors for improved outcomes in SBO.

IgG4 Licks base of skull, wastes tongue: An unusual presentation

IgG4 Related disease (IgG4 RD) is a rapidly evolving clinical entity, involving multiple organs, manifesting clinically as tumefactive lesions, histopathologically characterized by an IgG4 predominant, dense lymphoplasmacytic tissue infiltrate. It might mimic malignancy, infection as well as various other inflammatory disorders. First described in literature in 2003, in the pancreas, it has been subsequently seen to involve a wide range of organs like salivary glands, paranasal sinuses, thyroid gland, retroperitoneum, lung and kidney. Rarely, it may present as a skull base osteomyelitis (SBO). Diagnosing IgG4 RD in such patients is a herculean challenge in view of absence of systemic symptoms, difficulty in obtaining adequate tissue specimens in view of densely fibrotic cranial base lesions, as well as the close proximity of the lesions to critical neurovascular structures. The standard of care, in such cases is medical, and comprises of steroids and rituximab. Herein, we discuss one such case of IgG4 RD, who presented as multiple lower cranial nerve palsies and headache, with imaging evidence of SBO, which was diagnosed as a case of IgG4RD on histopathology, and who despite receiving the standard of care, had a poor outcome. The purpose of presenting this case is to stress upon a high index of suspicion and an aggressive management approach while treating such cases.

Multiple Lower Cranial Nerve Palsies Due to Nasopharyngeal Actinomycosis Masquerading as a Tumour - A Diagnostic Conundrum.

Multiple Lower Cranial Nerve Palsies Due to Nasopharyngeal Actinomycosis Masquerading as a Tumour - A Diagnostic Conundrum.

Dysphagia and Tongue Deviation: A Rare Case of Collett–Sicard Syndrome after Blunt Head Trauma

The jugular foramen and the hypoglossal canal are both apertures located at the base of the skull. Multiple lower cranial nerve palsies tend to occur with injuries to these structures. The pattern of injuries tend to correlate with the combination of nerves damaged. Case Report: A 28-year-old male was involved in an AVP injury while crossing the highway. Exam showed a GCS of 15 AAOx3, with dysphagia, tongue deviation to the right, uvula deviation to the left and a depressed palate. Initial imaging showed B/L frontal traumatic Sub-Arachnoid Hemorrhages (tSAH), Left Frontal Epidural Hematoma and a Basilar Skull Fracture. On second look by a trained Neuroradiologist c At 3 month follow up, patient’s tongue normalized to midline and his dysphagia resolved. Discussion: Collette-Sicard syndrome is a rare condition/syndrome characterized by unilateral palsy of CN: IX, X, XII. This condition has been rarely described as a consequence of blunt head trauma. In most cases, the condition is self-limiting with patients regaining most to all of their neurological functions within 6 months. Nerve traction injuries and soft tissue edema compressing the cranial nerves are the leading two hypothesis. In conclusion, injuries with focal neurological deficits which were not apparent on initial imaging should be reviewed by relevant experts with concomitant knowledge of the patient’s history.

Lower cranial nerve palsy during radiotherapy for glottic cancer in a patient with Wegener's granulomatosis: An interesting case report.

The aim of the present study was to report an unusual case of multiple lower cranial nerve palsies in a patient with Wegener's granulomatosis (WG) during radiotherapy for glottic cancer. WG is an autoimmune disease characterized by necrotizing granulomas mainly in the upper and lower respiratory tract or kidneys; however, the involvement of cranial nerves is not uncommon. Prior to the use of cyclophosphamide (CYC) the 1-year mortality rate was ~82%; the introduction of rituximab (RTX) has revolutionized the course of the WG, with remission rates comparable to those of CYC and superior effectiveness in relapsing patients. Hypogammaglobulinemia and B-cell depletion are the best known monitored side effects affecting survival due to secondary infections. Immunodepression and relapse with lower cranial nerve palsy have a negative impact on prognosis. We herein present the case of a heavily pre-treated GPA patient with secondary immunosuppression, who underwent radiotherapy for glottic cancer and developed multiple low cranial nerve palsies during treatment, which was interrupted at 60 Gy. The possible related causes and the association between previous immunosuppressive treatments and radiotherapy were also analyzed to elucidate the cause of this complication.

Atypical culture-negative skull base osteomyelitis masquerading as advanced nasopharyngeal carcinoma

Skull base osteomyelitis typically arises as a complication of otogenic or sinonasal infections in immunocompromised patients. A much rarer entity, atypical skull base osteomyelitis is not associated with an obvious infective source. Atypical and culture-negative skull base osteomyelitis is even rarer and hampers diagnosis, as its clinical presentation is remarkably similar to skull base neoplasms. We report a case of extensive skull base osteomyelitis with orbital apex syndrome and multiple lower cranial nerve palsies which initially masqueraded as possible advanced nasopharyngeal carcinoma. Extensive investigations and consult with an infectious diseases specialist aided in elucidation of the correct diagnosis. Through this article, we emphasize that skull base osteomyelitis must be considered in the setting of headache, cranial neuropathies, elevated inflammatory markers and abnormal imaging findings. Early tissue sampling for histology, stainings and cultures and prompt appropriate treatment may prevent or arrest further complications.

A Case of Central Skull Base Osteomyelitis Concurrent with Multiple Lower Cranial Nerve Palsies

In most cases, skull base osteomyelitis arises from infections of external auditory canal, middle ear or paranasal sinuses. On the other hand, atypical or central skull base osteomyelitis has no evidence of external otitis or other infection sources. It is often found located on clivus and involves lower cranial nerves and brainstem through the foramina of skull base. The overall rate of mortality reaches 10% and long-term neurologic sequelae are seen in additional 31% of cases regardless of aggressive treatment. We recently encountered a 68-year-old man with right facial pain and generalized headache, and we achieved good outcome with the recovery of vocal cord palsy and disappearance of headache by surgical debridement and long-term antibiotics. We report it with a review of literature.

Madras motor neuron disease (MMND) is distinct from the riboflavin transporter genetic defects that cause Brown–Vialetto–Van Laere syndrome

IntroductionMadras motor neuron disease (MMND), MMND variant (MMNDV) and Familial MMND (FMMND) have a unique geographic distribution predominantly reported from Southern India. The characteristic features are onset in young, weakness and wasting of limbs, multiple lower cranial nerve palsies and sensorineural hearing loss. There is a considerable overlap in the phenotype of MMND with Brown–Vialetto–Van Laere syndrome (BVVL) Boltshauser syndrome, Nathalie syndrome and Fazio–Londe syndrome. Recently a number of BVVL cases and families have been described with mutations in two riboflavin transporter genes SLC52A2 and SLC52A3 (solute carrier family 52, riboflavin transporter, member 2 and 3 respectively). Methods and resultsWe describe six families and four sporadic MMND cases that have been clinically characterized in detail with history, examination, imaging and electrophysiological investigations. We sequenced the SLC52A1, SLC52A2 and SLC52A3 in affected probands and sporadic individuals from the MMND series as well as the C9ORF72 expansion. No genetic defects were identified and the C9ORF72 repeats were all less than 10. ConclusionsThese data suggest that MMND is a distinct clinical subgroup of childhood onset MND patients where the known genetic defects are so far negative. The clinico-genetic features of MMND in comparison with the BVVL group of childhood motor neuron diseases suggest that these diseases are likely to share a common defective biological pathway that may be a combination of genetic and environmental factors.

Unusual Presentation of Skull Base Carotid Artery Aneurysm Causing Multiple Lower Cranial Nerve Palsies

Introduction: Extracranial carotid artery dissections and pseudoaneurysms are much more common than intracranial ones, and often extend to the level of the foramen lacerum. Involvement of cranial nerve XII from extracranial dissection and pseudoaneurysm is frequent, followed by IX, X, XI, V, VII, VI, III (in order of frequency). These aneurysms have a myriad of clinical presentations, and may result in thrombosis, embolization, compression of adjacent structures, and rupture. We report a case of extracranial carotid artery dissection with a unique clinical course of evolving radiographic findings from a tongue mass to a later-diagnosed pseudoaneurysm.

ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.

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 9th, 10th, and 12th 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.

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Sir:FigureThe timing of facial reanimation surgery in congenital facial palsy syndrome has been recommended in the child at approximately 5 years of age for psychosocial rehabilitation.1 However, the dental occlusion and facial proportion actually are not yet mature, and the effects of muscle transfer on a developing facial skeleton are difficult to predict. There is a lack of long-term follow-up for those children with Möbius syndrome who underwent reanimation surgery reported in the literature. The series of Möbius and Möbius-like syndrome patients in the article by Bianchi et al.2 had rather minor severity. The absence of muscle activity in the upper lip causes excessive forward growth of the maxilla, whereas downward growth is arrested. For cases in which dental problems are corrected in the Möbius patient, we strongly recommend that orthognathic surgery precede microsurgery, especially in the patient lacking normal masticatory muscle function and with possible developmentally abnormal facial proportions. For poor dental occlusion and a longer middle and lower face, orthognathic surgery is worthwhile before “smile” reconstruction. The bony work, mainly addressed during orthognathic surgery, should be scheduled in late adolescence. Surgically, the entire maxilla can be moved forward up to 10 mm with good stability and as far as 10 to 15 mm with excellent stability. The procedure is technically exacting but not otherwise problematic.3 Orthognathic surgery should be performed first to obtain a class I dental relationship and to optimize static facial aesthetics and substantially reduce drooling, oral incompetence, and speech difficulties.4 Microsurgical functional muscle transfers may be delayed until bone maturation and reshaping by orthognathic surgery. Facial animation and nonhollowed cheeks are later achieved in a second-stage procedure. The severity of cranial nerve involvement in Möbius syndrome is variable. The classic technique of hypoglossal nerve transfer in facial nerve reconstruction with the resultant hemiglossal paralysis may lead to significant morbidity, including speech, mastication, and swallowing difficulties. These problems may be further compounded if the patient has bilateral or multiple lower cranial nerve palsies (e.g., Möbius syndrome). A weaker facial response and longer recovery time have been observed even incising the hypoglossal nerve distal to the descendens hypoglossi branch to avoid compromising adequate tongue motor innervation.5 The masseter motor branch of the fifth cranial nerve is another tempting source, because free muscle transfer can be connected directly without the need for nerve grafting. The masseter muscle has to be stripped during orthognathic surgery, and that may add to the degree of difficulty in nerve end identification later. For these reasons—risk of aspiration, speech and swallowing difficulties, and the need for bilateral motor nerves—we selected the spinal accessory nerve instead of other motor branches in contemplating functioning muscle nerve repair for patients with Möbius syndrome. By far the most often selected nerve source in the series review was the spinal accessory nerve, which is the least involved nerve reported in the literature.6 Because there are various cranial nerves involved in Möbius syndrome, no single standard procedure for dynamic restoration should be overemphasized. Sophia Chia-Ning Chang, M.D., Ph.D. Department of Plastic Surgery, China Medical University Hospital, and, School of Medicine, China Medical University, Taichung, Taiwan David Chwei-Chin Chuang, M.D. Department of Plastic Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan DISCLOSURE Neither of the authors has any commercial associations or financial disclosures.

Skull base infection presenting with multiple lower cranial nerve palsies

Diseases of the temporal bone causing lower cranial nerve palsies are uncommon. In the presence of bony erosion, they are highly suggestive of a malignant process. However, when there is a clear history of otitis externa in an immunocompromised or diabetic patient, a diagnosis of osteomyelitis and secondary inflammatory mass should be considered. We report 4 separate cases of infective skull base lesions causing multiple lower cranial nerve palsies in elderly patients who were not immunocompromised or diabetic, highlighting that this condition is not exclusive to this population.

Madras motor neuron disease (MMND): Clinical description and survival pattern of 116 patients from Southern India seen over 36 years (1971–2007)

Madras motor neuron disease (MMND), MMND variant (MMNDV) and Familial MMND (FMMND) have a unique geographic distribution predominantly reported from Southern India. The characteristic features are onset in young, weakness and wasting of limbs, multiple lower cranial nerve palsies and sensorineural hearing loss. We describe the clinical features and survival pattern in 116 patients with Sporadic MMND, MMND variant and FMMND. A retrospective review of patients' medical records for clinical manifestations, electromyography, imaging, audiological and histopathology findings was performed. Over 36 years (1971 to 2007) 116 patients (men: 59; women: 57) particularly hailing from Southern India were seen. Mean age of onset was 15.8+/-7.9 years. Predominant initial manifestations were impaired hearing with wasting and weakness of distal limb muscles and pyramidal dysfunction. All patients had clinical and/or audiological evidence of hearing impairment. Patients with MMNDV in addition had optic atrophy. The overall mean survival duration was 334.9+/-27.9 months. Thus, Madras motor neuron disease is clinically a distinct entity with features of amyotrophic lateral sclerosis but with young age of onset and presence of auditory neuropathy. Studies to look for environmental and genetic basis of this intriguing disease are necessary to find the causation of this rare disorder.

술전 혈관색전술 및 하측두와 접근법을 이용한 경정맥구 종양 치험 1례

Glomus jugulare tumor is slow growing, hypervascular neoplasm. Although glomus jugulare tumor is benign, it invades temporal bone, skull base, and lower cranial nerves and may extend further either intracranially or extracranially. Glomus jugulare tumor is difficult tumor to resect by virtue of its location, locally infiltrative behavior, and vascular nature. Surgical removal is especially complicated when cranial extension encountered. Treatment methods for glomus tumors have developed rapidly over the past two decades. Preoperative arterial embolization results in decrease in tumor size and significantly decreases blood loss. Surgically, advanced tumor is best managed by infratemporal fossa approach. We report a case of glomus jugular tumor in a 32-year old male who had had facial palsy, deafness, and other multiple lower cranial nerve palsies. The tumor was treated by preoperative embolization and infratemporal fossa approach. The patient had CSF leakage and it was managed by dural repair. Facial nerve and other lower cranial nerve palsies have not been improved until now. (J Clinical Otolaryngol 1999;10:291–296)

Stereotactic radiosurgery for jugular foramen schwannomas

BACKGROUND Jugular foramen schwannomas pose difficult management problems because of the surgical risk of lower cranial neuropathy. The indications and results of stereotactic radiosurgery are not well documented. METHODS We reviewed our 10-year experience in the management of 17 patients who had jugular foramen schwannomas managed with the gamma knife. Thirteen patients previously had undergone surgery (range, 1–6 resections). Four patients had multiple cranial nerve deficits before microsurgical resection; 12 developed multiple lower cranial nerve palsies after resection. Four patients underwent radiosurgery based on imaging criteria alone. Conformal dose planning (tumor margin dose of 12–18 Gy) successfully encompassed the irregular tumor volumes in all patients. RESULTS Follow-up varied from 6 to 74 months. Tumor size decreased in eight patients, remained stable in eight, and increased in one patient during the average follow-up interval of 3.5 years. Six patients improved and 10 others retained their preradiosurgery clinical status. One patient had an increase in tumor size and clinical deterioration 6 months after radiosurgery and underwent microsurgical resection. No patient developed new cranial nerve or other neurological deficits after radiosurgery. CONCLUSIONS We believe that gamma knife radiosurgery is an effective alternative to microsurgical resection for patients who have small tumors and intact lower cranial nerve function. It is also effective for patients who have residual or recurrent tumors after microsurgical resection.

A new technique for hypoglossal-facial nerve repair.

Hypoglossal reinnervation of the facial nerve may be required after a proximal facial nerve injury. The classic hypoglossal-facial graft procedure involves transection of the donor hypoglossal nerve, resulting in hemiglottic paralysis that, in association with paralysis of other cranial nerves, may cause speech and swallowing difficulties. Multiple lower cranial nerve palsies in conjunction with facial paralysis, as may occur after procedures such as skull base surgery, contraindicate the use of such techniques. The successful use of XII-VII "interposition jump grafts" without hemiglossal weakness has been described However, a prolonged recovery period and weaker facial reanimation have been seen. In order to attain maximum facial reinnervation while preserving hypoglossal function, we have developed a new technique of XII-VII repair. This method involves mobilization of the intratemporal portion of the facial nerve remnant, achieving a single anastomosis with the hypoglossal nerve, which has been partially incised. This technique has been used in three patients to date, with 6 to 11 months follow-up. In all cases facial tone and symmetry have been restored and voluntary facial expression accomplished. The authors conclude that by employing the techniques described highly satisfactory cosmetic and functional results may be expected, without compromising hypoglossal nerve function.