Internal Carotid Artery Aneurysm Presenting as Lower Cranial Nerve Palsies.

The signs of lower cranial nerve palsies are rare and are often caused by tumors. A 49-year-old woman was admitted to our hospital with progressive right-sided atrophy of the tongue, sternocleidomastoid and trapezius, dysarthria, and dysphagia for 3 years. Brain magnetic resonance imaging revealed a circular lesion adjacent to the lower cranial nerves. Cerebral angiography confirmed that the lesion was an unruptured aneurysm in the C1 segment of the right internal carotid artery. After endovascular treatment, the symptoms of this patient had partially improved.

Remission of Mucosal Melanoma of the Middle Ear and Petrous Temporal Bone and Reversal of Cranial Nerve Paresis Following Radiation and Single Agent Nivolumab: Clinical Capsule and Review of the Literature.

OBJECTIVE: We report disease remission and recovery of fifth and seventh nerve paresis in a case of primary mucosal melanoma of the middle ear and petrous temporal bone. PATIENT: A 74-year-old man developed sudden, profound, right sided sensorineural hearing loss, disequilibrium, otalgia, and cranial nerve V and VII dysfunction. Imaging demonstrated an unresectable, osteolytic lesion involving the middle ear and anterior petrous apex. Melanoma was diagnosed via in-office biopsy; whole-body metabolic imaging revealed no other primary site. INTERVENTION: Multidisciplinary management included radiation therapy (30 Gy, 10 fractions) followed by induction (five cycles, q2w) and maintenance nivolumab (six cycles, q3w). MAIN OUTCOME MEASURE: Complete metabolic response of primary site and metastases on imaging, recovery of cranial neuropathies. RESULTS: Following palliative radiation therapy and induction nivolumab, cranial neuropathies resolved. With maintenance-dose nivolumab, primary site and metastases exhibited a complete response. Therapy was stopped at 16 months post-diagnosis. Complete remission was maintained until 22 months after diagnosis. The patient developed a solitary cerebral metastasis which was refractory to radiosurgery and biopsy confirmed melanoma. He expired 2 years, 8 months post-diagnosis. CONCLUSIONS: Mucosal melanoma of the middle ear and petrous temporal bone is exceedingly rare. Management is individualized and surgery is undertaken when possible. Key observations in this case are the complete metabolic response and reversal of cranial nerve neuropathies following radiation and anti-programed cell death receptor ligand 1 therapy. Non-surgical treatment is worthy of study as initial management for similar lesions.

Detailed analysis of recovery process of cranial nerve palsy after IMRT-based comprehensive treatment in nasopharyngeal carcinoma.

BACKGROUND: Cranial nerve (CN) palsy due to cancer involvement has been considered as an unfavorable prognostic factor for patients with nasopharyngeal carcinoma (NPC). We assessed the role of IMRT based treatment on the recovery of CN palsy and investigated the prognostic value of complete recovery of CN palsy. METHODS: A total of 115 NPC patients with cancer-related CN palsy were included in the study. We referred CTCAE version 5.0 to evaluate the grade of CN palsy. RESULTS: All patients with grade 1 CN palsy recovered completely during the 2 years of follow-up after definite treatment. Most grade 2 palsy could change gradually to grade 1 palsy or complete recovery during 2 years of follow-up. Patients with more than 2 symptoms of CN palsy had poor 3-year disease-free survival (DFS) than these with 1 or 2 symptoms (60.3% vs. 84.9%, HR 0.25, 95% CI 0.07-0.89, P = 0.001). There were no significant differences for PFS, OS, DMFS and LRFS between patients with complete recovery and non-complete recovery from CN palsy after receiving IMRT based comprehensive treatment. CONCLUSIONS: IMRT based comprehensive treatment could effectively promote the recovery of tumor-related CN palsy for NPC patient. More than 2 symptoms of CN palsy was a poor prognostic factor for DFS of NPC patients. The prognostic role of complete recovery of CN palsy was not identified in our study.

Nasopharyngeal carcinoma misdiagnosed as pituitary tumor with multiple cranial neuropathies.

Multiple cranial neuropathies have been reported in nasopharyngeal carcinoma. Nasopharyngeal carcinoma is an uncommon cause of multiple cranial nerve palsies. However, it is difficult to diagnose at the early stage; furthermore, it can be easily misdiagnosed as inflammation, pituitary tumor, etc. A 38-year-old female patient had an ipsilateral 3rd, 5th, 6th, and 7th cranial nerves injury in this study. She was successively misdiagnosed with nonspecific inflammation and pituitary tumor. More than 1 year later, she was diagnosed with invasive nasopharyngeal carcinoma.

Cranial nerve palsies in patients with hematological malignancies: a case series.

Objectives: Cranial neuropathies (CNs) can be due to a wide spectrum of causes, and the differential diagnosis is particularly challenging in patients with positive history of hematological malignancies, when neoplastic meningitis (NM) must be excluded.Patients and Methods: We retrospectively selected a series of twelve haematological patients with isolated cranial neuropathies (ICNs) or multiple cranial neuropathies (MCNs). among 71 patients that developed neurologic symptoms during different stages of the cancer, between 1 January, 2010 and 31 December, 2017. Brain and cauda equina magnetic resonance imaging (MRI) with gadolinium, cerebrospinal fluid (CSF) analysis, including flow cytometry for cell immunophenotyping and microbiological exams were performed in all patients.Results: Patients developed signs and symptoms of involvement of isolated (n = 11) or multiple (n = 1) cranial nerves, at different stages of the primary disease, and, in 5 of these cases in complete remission after hematopoietic stem cell transplantation. Among the 5 cases that eventually were diagnosed as having NM, cerebrospinal fluid was positive for neoplastic cells in 3, and MRI gadolinium-enhancement was present in 3. The other episodes were attributed to heterogeneous pathologies that were unrelated to meningeal infiltration by neoplastic cells.Conclusions: Our observations confirm that NM in haematological malignancies can yield insidious isolated signs of cranial nerves. Only a multidisciplinary approach allows prompt recognition of these conditions through a challenging process of differential diagnosis, and proper therapies.

Herpes simplex virus type 2 meningitis as a manifestation of Good's syndrome.

Good's syndrome is a primary immunodeficiency phenocopy characterized for thymoma and immunodeficiency. The most frequent clinical presentation is recurrent or opportunistic infections, hematological alterations, and chronic diarrhea. We treated a 66-year-old man who consulted for 5 days of headache and diplopia with right sixth cranial nerve palsy at examination. Patient reported chronic diarrhea and prolonged febrile syndrome accompanied by weight loss of 23 kg in the last year. Exhaustive evaluation revealed Herpes simplex virus (HSV) type 2 meningitis, eosinophilic colitis, and type A thymoma. Severe antibody deficiency (hypogammaglobulinemia) associated with thymoma confirmed the diagnosis of Good's syndrome.

Diffuse large B-cell lymphoma recurrence presenting as multiple, progressive cranial neuropathies.

A 58-year-old man with a history of rheumatoid arthritis and stage IV diffuse large B-cell lymphoma, in complete remission with no evidence of residual disease on positron emission tomography/CT after completing six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy, presented with acute onset of dysphagia to solids and liquids. On further evaluation, his dysphagia was attributed to a vagus nerve palsy, and later during his admission, he developed rapidly progressing left facial and vestibulocochlear nerve palsies. Imaging studies displayed pathological enhancement of bilateral seventh and eighth cranial nerves, concerning for leptomeningeal recurrence of lymphoma. Cerebrospinal fluid analysis and flow cytometry were confirmatory, revealing markedly atypical monotypic CD19 positive B cells.

Vernet syndrome: intracranial extension of a slow-growing mass.

Vernet syndrome, often referred to as jugular foramen syndrome, is a rare clinical entity characterised by a set of signs and symptoms caused by dysfunction of IX, X and XI cranial nerves. Although paraganglioma of the head and neck is the most frequent aetiology, it may also be caused by meningioma, VIII cranial nerve schwannoma, pontocerebellar cistern metastases, head and neck trauma, infections and very rarely by cholesteatoma which extends to the petrous apex. The authors describe a case of a patient with a jugulotympanic paraganglioma in which evolution ends up in Vernet syndrome. The patient preferred a 'wait and scan' strategy. With the lack of data available to develop an unequivocal algorithm for paraganglioma management, we always consider not only age but also comorbidities, prior treatment and progression of the lesion. Each case has to be addressed individually and treatment should be discussed in detail with every patient.

Neurocan is a New Substrate for the ADAMTS12 Metalloprotease: Potential Implications in Neuropathies.

BACKGROUND/AIMS: The composition of the extracellular matrix (ECM) in the central nervous system (CNS) has several features that make it unique. For instance, it is remarkable for the presence of proteoglycans such as versican, brevican, and neurocan, some of which have been identified as substrates of different members of the ADAMTS family of secreted metalloproteases. Previous studies have associated ADAMTSs with the repair of the CNS, including recovery following degradation of glial scar tissue and the stimulation of axonal growth after brain injury. However, the involvement of ADAMTSs in diseases of the CNS is complex and not understood fully, and a current challenge is unraveling the precise roles of these metalloproteases in the brain. METHODS: ADAMTS12 and neurocan gene expression was examined by quantitative PCR. Western blot analysis was employed to detect ADAMTS12 and neurocan protein expression in cell lines, and immunostaining techniques were used to detect neurocan in mouse brain tissues. Neurocan cleavage using recombinant ADAMTS1, ADAMTS4, ADAMTS5, and ADAMTS12 metalloproteases was evaluated by western blotting. Cell adhesion and migration were assessed using uncoated culture dishes or dishes coated with Matrigel or ECM components. RESULTS: We identified neurocan as a novel component of brain ECM that can be cleaved by ADAMTS12. In addition, we showed that neurocan cleavage by ADAMTS12 altered the adhesive properties of the human neuroglioma H4 cell line. Moreover, immunohistochemical analysis of Adamts12-deficient mice revealed the significant accumulation of neurocan in the brain of neonatal mice. CONCLUSION: Overall, our results suggest that ADAMTS12 could be involved in the repair of the CNS through its ability to degrade neurocan. Moreover, it can be inferred that alterations in neurocan degradation processes could be associated with the pathogenesis of neurological disorders.

MAPKs and NF-κB-mediated acrylamide-induced neuropathy in rat striatum and human neuroblastoma cells SY5Y.

Acrylamide (ACR) is a potent neurotoxin that can be produced during high-temperature food processing, but the underlying toxicological mechanism remains unclear. In this study, the detrimental effects of ACR on the striatal dopaminergic neurons and the roles of mitogen-activated protein kinases (MAPKs) and nuclear factor κB (NF-κB) in ACR-induced neuronal apoptosis were investigated. Acute ACR exposure caused dopaminergic neurons loss and apoptosis as revealed by decreased tyrosine hydroxylase (TH)-positive cells and TH protein level and increased terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL)-positive cells in the striatum. ACR-decreased glutathione content, increased levels of malondialdehyde, proinflammatory cytokines tumor necrosis factor α, and interleukin 6. In addition, nuclear NF-κB and MAPKs signaling pathway with c-Jun N-terminal kinase (JNK) and p38 were activated by ACR. Specific inhibitors were used to explore the roles of MAPKs and NF-κB pathways in ACR-induced apoptosis in SH-SY5Y cells. Pretreatment with JNK-specific inhibitors SP600125 markedly upregulated the reduced B-cell lymphoma 2 (Bcl-2) content and downregulated the increased Bcl-2-associated X protein (Bax) level and thereby eventually reduced the proportions of early and late apoptotic cells induced by ACR, while p38 suppression by SB202190 only reversed the decrease in Bcl-2 expression. Inhibition of NF-κB by BAY 11-7082 markedly upregulated Bax level and decreased Bcl-2 expression, and eventually increasing the proportions of neuronal apoptosis compared with that in ACR alone. These results suggested that JNK contributed to ACR-induced apoptosis, while NF-κB acted as a protective regulator in response to ACR-induced neuropathy. This study helps to offer a deeper insight into the mechanism of ACR-induced neuropathy.

Hypoglossal canal schwannoma causing isolated left 12th cranial nerve palsy.

A 40-year-old woman presented with insidious onset, gradually progressive dysarthria and inability to manoeuvre bolus of food in her mouth while eating. The duration of her symptoms was 3 months. On evaluation, the left half of her tongue was wasted. The tongue deviated to the left on protrusion. There were no clinical features suggestive of involvement of the ipsilateral 9th, 10th or 11th cranial nerves. MRI of the brain showed a large, fusiform lesion in the left hypoglossal canal, extending into the jugular canal. The lesion was surgically excised and found to be a schwannoma.

Garcin syndrome caused by sphenoid bone metastasis of lung cancer: a case study.

BACKGROUND: Garcin syndrome, which consists of unilateral palsies of almost all cranial nerves without either sensory or motor long-tract disturbances or intracranial hypertension, can be caused by malignant tumors at the skull base. The case of a patient with lung cancer that metastasized to the sphenoid bone and resulted in Garcin syndrome is presented. CASE PRESENTATION: A 76-year-old woman was diagnosed as having non-small cell lung cancer with pericardial and diaphragmatic infiltration, cT4N1M0, stage 3A. The left lower lobectomy with concomitant resection of the pericardium and diaphragm was performed. The pathological diagnosis was pleomorphic carcinoma, pT2bN0M0, stage 1B. She was then followed in the surgery clinic, and 2 months after surgery, she visited an emergency room complaining of headache and diplopia. Neurological examination showed the left IV, V1, and VI cranial nerve palsies. Metastatic tumor with bone destruction was found in the left sphenoid sinus on head computed tomography (CT) and contrast magnetic resonance imaging (MRI), and she was diagnosed with Garcin syndrome caused by sphenoid bone metastasis of lung cancer. Irradiation was performed as palliative treatment, but her neurological findings did not improve. Her general condition gradually worsened, and she died 5 months after surgery. CONCLUSIONS: Bone metastasis of lung cancer occurs frequently, but sphenoid bone metastasis is extremely rare. In this case report, Garcin syndrome caused by lung cancer is discussed in the context of the few previous reports.

Collet-Sicard Syndrome Attributable to Extramedullary Plasmacytoma of the Jugular Foramen.

BACKGROUND: Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma-multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. CASE DESCRIPTION: A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX-XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. CONCLUSION: Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion.

Disseminated Cryptococcosis With Severe Increased Intracranial Pressure Complicated With Cranial Nerve Palsy in a Child.

Cryptococcosis is less common in children than in adults but remains an important cause of pneumonia and meningoencephalitis in both immunocompromised and immunocompetent patients. Intracranial hypertension commonly complicates cryptococcal meningitis and may cause significant visual and neurologic morbidity and mortality. Early and aggressive management of intracranial hypertension in accordance with established guidelines reduces the risk of long-term complications and death. In this case report, we present a 12-year-old girl with cryptococcal meningitis, pneumonitis and dermatitis complicated with cranial nerve palsy and loss of vision. She was successfully treated with serial cerebrospinal fluid drainage, antifungal and interferon gamma therapy.

Aggressive necrotizing pseudomonal sinonasal infections.

BACKGROUND: Pseudomonas aeruginosa is a gram-negative bacterium frequently implicated in recalcitrant sinonasal infections, especially in immunocompromised hosts. We report 6 cases of rapidly progressive pseudomonal acute rhinosinusitis producing tissue necrosis and, in certain cases, cranial nerve palsies. METHODS: Retrospective review of 6 patients with aggressive necrotizing sinonasal infections treated at 4 tertiary academic medical centers with sinonasal cultures growing P. aeruginosa in the absence of other pathology. RESULTS: A total of 6 patients were identified. In all cases, there was tissue necrosis that appeared to mimic an invasive process such as mucormycosis, prompting urgent surgical intervention. Pathologic analysis revealed fibropurulent exudates in backgrounds of positive P. aeruginosa cultures without evidence of invasive fungal organisms or malignancy. Four of the 6 patients presented with cranial nerve palsies, with 3 patients having vision changes and 3 complaining of trigeminal neuropathy. Four of 6 patients improved clinically over time after surgery and antibiotic therapy; 1 remains in follow-up without complete improvement and 1 has succumbed to other causes. CONCLUSION: P. aeruginosa is a tenacious organism that is frequently associated with severe, recalcitrant sinonasal infections. We report the first case series of necrotizing sinonasal infections caused by this organism, and illustrate that, in rare cases, P. aeruginosa may mimic and behave like life-threatening conditions such as fulminant invasive fungal sinusitis or malignancy.