ABSTRACT
PURPOSE: When operating near cranial motor nerves, transient postoperative weakness of target muscles lasting weeks to months is often observed. As nerves are typically intact at a procedure's completion, paresis is hypothesized to result from a combination of neurapraxia and axonotmesis. As both neurapraxia and axonotmesis involve Schwann cell injury and require remyelination, we developed an in vitro RSC96 Schwann cell model of injury using hydrogen peroxide (H2O2) to induce oxidative stress and investigated the efficacy of candidate therapeutic agents to promote RSC96 viability. As a first step in developing a long-term local administration strategy, the most promising of these agents was incorporated into sustained-release microparticles and investigated for bioactivity using this assay. METHODS: The concentration of H2O2 which reduced viability by 50% was determined to establish a standard for inducing oxidative stress in RSC96 cultures. Fresh cultures were then co-dosed with H2O2 and the potential therapeutics melatonin, N-acetylcysteine, resveratrol, and 4-aminopyridine. Schwann cell viability was evaluated and the most efficacious agent, N-acetylcysteine, was encapsulated into microparticles. Eluted samples of N-acetylcysteine from microparticles was evaluated for retained bioactivity. RESULTS: 100 µM N-acetylcysteine improved the viability of Schwann cells dosed with H2O2. 100 µM Microparticle-eluted N-acetylcysteine also enhanced Schwann cell viability. CONCLUSION: We developed a Schwann cell culture model of iatrogenic nerve injury and used this to identify N-acetylcysteine as an agent to promote recovery. N-acetylcysteine was packaged into microparticles and demonstrated promise as a locally administrable agent to reduce oxidative stress in Schwann cells.
Subject(s)
Acetylcysteine , Hydrogen Peroxide , Oxidative Stress , Schwann Cells , Acetylcysteine/pharmacology , Acetylcysteine/administration & dosage , Schwann Cells/drug effects , Animals , Oxidative Stress/drug effects , Rats , Resveratrol/pharmacology , Resveratrol/administration & dosage , Cranial Nerve Diseases/etiology , Cranial Nerve Diseases/drug therapy , Melatonin/pharmacology , Cell Survival/drug effects , Postoperative Complications/prevention & control , Antioxidants/pharmacologyABSTRACT
BACKGROUND: Tuberculous meningitis, a rare but severe form of extrapulmonary tuberculosis, frequently affects cranial nerves. While nerves III, VI and VII are commonly involved, involvement of caudal cranial nerves is rarely described. Here, we report a rare case of bilateral vocal cord palsy secondary to caudal cranial nerve involvement in tuberculous meningoencephalitis, that occurred in Germany, a country with low tuberculosis incidence. CASE PRESENTATION: A 71-year-old woman was transferred for further treatment of hydrocephalus as a complication of presumed bacterial meningitis with unknown pathogen at that time. Because of decreased consciousness, intubation was performed and an empiric antibiotic therapy with ampicillin, ceftriaxone and acyclovir was initiated. Upon admission to our hospital, an external ventricular drainage was placed. Cerebrospinal fluid analysis revealed Mycobacterium tuberculosis as the causative pathogen, and antitubercular treatment was initiated. Extubation was possible one week after admission. Eleven days later, the patient developed inspiratory stridor that worsened within a few hours. Flexible endoscopic evaluation of swallowing (FEES) revealed new-onset bilateral vocal cord palsy as the cause of respiratory distress, which required re-intubation and tracheostomy. The bilateral vocal cord palsy persisted despite continued antitubercular therapy on the follow-up examination. CONCLUSION: Considering the aetiology of infectious meningitis, cranial nerve palsies may be suggestive for tuberculous meningitis as underlying disease given their rarity in other bacterial forms of meningitis. Nevertheless, intracranial involvement of inferior cranial nerves is rare even in this specific entity, as only extracranial lesions of inferior cranial nerves have been reported in tuberculosis. With this report of a rare case of bilateral vocal cord palsy due to intracranial involvement of the vagal nerves, we emphasize the importance of timely initiation of treatment for tuberculous meningitis. This may help to prevent serious complications and associated poor outcome since the response to anti-tuberculosis therapy may be limited.
Subject(s)
Cranial Nerve Diseases , Hydrocephalus , Tuberculosis, Meningeal , Vocal Cord Paralysis , Female , Humans , Aged , Vocal Cord Paralysis/complications , Vocal Cord Paralysis/drug therapy , Tuberculosis, Meningeal/complications , Cranial Nerve Diseases/drug therapy , Antitubercular Agents/therapeutic use , Hydrocephalus/etiologyABSTRACT
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.
Subject(s)
Cranial Nerve Diseases , Facial Paralysis , Miller Fisher Syndrome , Ophthalmoplegia , Male , Humans , Gadolinium , Gangliosides , Paresthesia , Cranial Nerve Diseases/diagnostic imaging , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/complications , Immunoglobulins, Intravenous/therapeutic use , Facial Paralysis/complications , Ophthalmoplegia/complications , Magnetic Resonance Imaging , Miller Fisher Syndrome/diagnosisABSTRACT
A 27-year-old woman was admitted to our hospital for fever, associated with headache, nausea, and vomiting, and she rapidly developed mild left facial nerve palsy and diplopia. Neurological examination revealed mild meningitis associated with bilateral VI cranial nerve palsy and mild left facial palsy. As central nervous system (CNS) infection was suspected, a diagnostic lumbar puncture was performed, which revealed 1,677 cells/µl, 70% of which were polymorphonuclear leukocytes. Moreover, multiplex PCR immunoassay was positive for Neisseria meningitidis, supporting the diagnosis of bacterial meningitis. Finally, IgG oligoclonal bands (IgGOB) were absent in serum and cerebrospinal fluid (CSF). Therefore, ceftriaxone antibiotic therapy was started, and in the following days, the patient's signs and symptoms improved, with complete remission of diplopia and meningeal signs within a week. On the contrary, left facial nerve palsy progressively worsened into a severe bilateral deficit. A second lumbar puncture was therefore performed: the CSF analysis revealed a remarkable decrease of pleocytosis with a qualitative modification (only lymphocytes), and oligoclonal IgG bands were present. A new brain MRI was performed, showing a bilateral gadolinium enhancement of the intrameatal VII and VIII cranial nerves bilaterally. Due to suspicion of para-infectious etiology, the patient was treated with oral steroid (prednisolone 1 mg/kg/day), with a progressive and complete regression of the symptoms. We suggest that in this case, after a pathogen-driven immunological response (characterized by relevant CSF mixed pleocytosis and no evidence of IgGOB), a para-infectious adaptive immunity-driven reaction (with mild lymphocyte pleocytosis and pattern III IgGOB) against VII and VIII cranial nerves started. Indeed, steroid administration caused a rapid and complete restoration of cranial nerve function.
Subject(s)
Cranial Nerve Diseases , Meningitis, Bacterial , Female , Humans , Adult , Oligoclonal Bands , Ceftriaxone , Gadolinium , Diplopia , Leukocytosis , Contrast Media , Cranial Nerve Diseases/diagnosis , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Prednisolone , Meningitis, Bacterial/complications , Meningitis, Bacterial/diagnosis , Meningitis, Bacterial/drug therapy , Paralysis , Immunoglobulin G , Anti-Bacterial AgentsABSTRACT
BACKGROUND: We report a rare case of ipsilateral multiple cranial neuropathy and ipsilateral lymphadenopathy following mRNA-COVID-19 vaccination. CASE PRESENTATION: A 41-year-old male visited our emergency room complaining of dysphagia and hoarseness that started a week after receiving COVID19 mRNA vaccination (in his right arm). During his hospitalization, he also complained of right side hearing loss and diplopia. Neurological examination depicted a right IV nerve palsy, ipsilateral facial paresthesia and peripheral facial paresis. Otorinolaryngological examination revealed right vocal cord paralysis. A brain magnetic resonance imaging showed enhancement of the right VII and VIII cranial nerves in the auditory canal. The lumbar puncture revealed increased protein concentration and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Additionally, a neck computed tomography (CT) scan showed a swollen right supraclavicular lymph node. We hypothesize that the ipsilateral cranial neuropathies of IV, VI, VII, VIII and X, associated with cervical lymphadenopathy, was possible caused by a post-vaccination immune-mediated reaction. The patient was treated with a 5-day course of intravenous methylprednisolone (1000 mg/day), and a gradual improvement was observed. CONCLUSIONS: Similarly, to other vaccines, it is possibly that also mRNA vaccines may act as triggers of non-specific autoimmune neurological syndromes.
Subject(s)
COVID-19 Vaccines , COVID-19 , Cranial Nerve Diseases , Facial Paralysis , Lymphadenopathy , Adult , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Cranial Nerve Diseases/diagnosis , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Facial Paralysis/etiology , Humans , Lymphadenopathy/complications , Male , Methylprednisolone , RNA, MessengerABSTRACT
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 (30âGy, 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 16âmonths post-diagnosis. Complete remission was maintained until 22âmonths after diagnosis. The patient developed a solitary cerebral metastasis which was refractory to radiosurgery and biopsy confirmed melanoma. He expired 2âyears, 8âmonths 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.
Subject(s)
Cranial Nerve Diseases , Melanoma , Aged , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Cranial Nerve Diseases/pathology , Cranial Nerves/pathology , Ear, Middle/pathology , Humans , Male , Nivolumab/therapeutic use , Paresis , Petrous Bone/surgeryABSTRACT
AIM: Intractable or persistent hiccups and nausea (IHN) are rarely associated with herpes zoster (HZ-IHN). We aimed to identify the clinical characteristics of HZ-IHN by comparing them with those of neuromyelitis optica spectrum disorder associated with IHN (NMOSD-IHN). METHODS: We collected 8 patients with HZ-IHN and 12 patients with NMOSD-IHN diagnosed between 2002 and 2020 from medical databases. Medical records including clinical information, laboratory data on serum anti-aquaporin 4 (AQP4) antibodies, serological or cerebrospinal fluid findings for the varicella zoster virus, medullary MRI findings, and efficacy of intravenous methylprednisolone pulse (IVMP) therapy were analyzed retrospectively. RESULTS: The age of onset (69 ± 13 years versus 46 ± 17 years, P = 0.003), percentage of men [7/8 patients (88%) versus 3/12 patients (25%), P = 0.020], serum CRP levels (1.41 ± 1.17 mg/dL versus 0.14 ± 0.33 mg/dL, P = 0.018), and frequency of hemi-cranial nerve involvement [6/8 patients (75%) versus 1/12 patients (8%), P = 0.004] were significantly higher in patients with HZ-IHN than in those with NMOSD-IHN. The hypoglossal and vagus nerves were involved in 5/8 patients (63%) with HZ-IHN. Other clinical parameters, excluding anti-AQP4 antibodies, were similar to those of NMOSD-IHN. MRI revealed ipsilateral hemi-dorsal medullar hyper-intense lesions in 5/8 patients (63%) with HZ-IHN. Acyclovir with IVMP therapy was effective for HZ-IHN. CONCLUSION: Clinicians should include HZ-IHN in the differential diagnosis for IHN, and promptly administer acyclovir and IVMP therapy. HZ-IHN is frequently accompanied by lower hemi-cranial nerve palsies and ipsilateral hemi-dorsal medullary hyper-intensity on MRI. DATA AVAILABLE STATEMENT: The authors confirm that the data supporting the findings of this study are available within the article (Tables 1 and 2), or its supplementary materials (Table S1).
Subject(s)
Herpes Zoster/complications , Herpes Zoster/diagnosis , Hiccup/etiology , Nausea/etiology , Acyclovir/therapeutic use , Adult , Aged , Aged, 80 and over , Antiemetics/therapeutic use , Antiviral Agents/therapeutic use , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Diagnosis, Differential , Female , Herpes Zoster/drug therapy , Hiccup/drug therapy , Humans , Male , Methylprednisolone/therapeutic use , Middle Aged , Nausea/drug therapy , Neuromyelitis Optica/diagnosis , Retrospective StudiesABSTRACT
Syphilis is increasingly prevalent in the community. The protean manifestations of neurosyphilis make the recognition, diagnosis and early initiation of treatment challenging. We report a case of early syphilitic meningitis presenting with multiple cranial neuropathies. Cerebrospinal fluid (CSF) examination was inflammatory with predominant lymphocytosis. The patient was diagnosed with neurosyphilis based on serum as well as CSF testing. Intravenous benzylpenicillin treatment resulted in rapid improvement of neurological symptoms. Neurosyphilis should be considered in immunocompetent patients presenting with multiple cranial neuropathies, or isolated cranial neuropathies without vascular risk factors.
Subject(s)
Cranial Nerve Diseases , Meningitis , Neurosyphilis , Syphilis , Cranial Nerve Diseases/diagnosis , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Humans , Neurosyphilis/complications , Neurosyphilis/diagnosis , Neurosyphilis/drug therapy , Penicillin G/therapeutic use , Syphilis/complications , Syphilis/diagnosis , Syphilis/drug therapyABSTRACT
RATIONALE: Systemic lupus erythematosus (SLE) is a complex autoimmune inflammatory disease that frequently affects various organs. Neuropsychiatric manifestations in SLE patients, known as neuropsychiatric SLE, are clinically common. However, the principal manifestation of cranial neuropathy in patients with SLE and comorbidities is relatively rare. PATIENT CONCERNS: In this report, we describe a 51-year-old Chinese woman who was admitted with a chief complaint of chronic-onset facial paresthesia, dysphagia, and choking cough when drinking water, accompanied by slurred speech, salivation, and limb weakness. The blood autoantibody test results showed that many SLE-associated antibodies were positive. Meanwhile, anti-nuclear matrix protein 2 (NXP2) antibody was strongly positive in the idiopathic inflammatory myopathy (IIM) spectrum test from the serum. Muscle biopsy indicated inflammatory infiltration of the muscle fiber stroma. DIAGNOSES: Taking into account the clinical manifestations and laboratory tests of the present case, the diagnosis of SLE and probable IIM was established. INTERVENTIONS: Corticosteroids and additional gamma globulin were administered and the clinical symptoms were relieved during the treatment process. OUTCOMES: Unfortunately, the patient experienced sudden cardiac and respiratory arrest. Multiple system dysfunctions exacerbated disease progression, but in the present case, we speculated that myocardial damage resulting from SLE could explain why she suddenly died. LESSONS: To our knowledge, multiple neurological manifestations in patients with SLE and anti-NXP2-positive myositis are rare. Note that SLE is still a life-threatening disease that causes multiple system dysfunctions, which requires increasing attention.
Subject(s)
Cranial Nerve Diseases/immunology , Deglutition Disorders/immunology , Lupus Erythematosus, Systemic/diagnosis , Paresthesia/immunology , Polymyositis/diagnosis , Adenosine Triphosphatases/immunology , Autoantibodies/blood , Autoantibodies/immunology , Biopsy , Cranial Nerve Diseases/diagnosis , Cranial Nerve Diseases/drug therapy , DNA-Binding Proteins/immunology , Deglutition Disorders/diagnosis , Deglutition Disorders/drug therapy , Drug Therapy, Combination/methods , Fatal Outcome , Female , Humans , Immunologic Factors/administration & dosage , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/immunology , Middle Aged , Muscle, Skeletal/immunology , Muscle, Skeletal/pathology , Paresthesia/diagnosis , Paresthesia/drug therapy , Polymyositis/complications , Polymyositis/drug therapy , Polymyositis/immunology , Pulse Therapy, DrugABSTRACT
We herein report a case of granulomatosis with polyangiitis in a 73-year-old man. He had experienced bilateral ptosis, redness of both eyes, right facial weakness, and hearing loss in the right ear for two months. Myeloperoxidase anti-neutrophil cytoplasmic antibody was positive. Corticosteroids and intravenous cyclophosphamide pulse therapy yielded a response. After the fourth pulse of cyclophosphamide, he developed headache, diplopia, restricted left eye movement, right facial palsy, and hearing loss in the right ear. A one-year remission period was achieved by increasing the steroid and rituximab doses. Rituximab was effective against the relapse of granulomatosis with polyangiitis and cranial neuropathy.
Subject(s)
Cranial Nerve Diseases , Granulomatosis with Polyangiitis , Aged , Antibodies, Antineutrophil Cytoplasmic , Cranial Nerve Diseases/drug therapy , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/drug therapy , Humans , Male , Peroxidase , Rituximab/therapeutic useSubject(s)
Cranial Nerve Diseases/diagnosis , Herpes Zoster/diagnosis , Mononeuropathies/diagnosis , Abducens Nerve Diseases/diagnosis , Abducens Nerve Diseases/drug therapy , Abducens Nerve Diseases/physiopathology , Abducens Nerve Diseases/virology , Aged , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/physiopathology , Cranial Nerve Diseases/virology , Diagnosis, Differential , Diplopia/physiopathology , Earache/physiopathology , Edema/physiopathology , Facial Nerve Diseases/diagnosis , Facial Nerve Diseases/drug therapy , Facial Nerve Diseases/physiopathology , Facial Nerve Diseases/virology , Facial Paralysis/physiopathology , Glossopharyngeal Nerve Diseases/diagnosis , Glossopharyngeal Nerve Diseases/drug therapy , Glossopharyngeal Nerve Diseases/physiopathology , Glossopharyngeal Nerve Diseases/virology , Glucocorticoids/therapeutic use , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/drug therapy , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Sensorineural/virology , Herpes Zoster/drug therapy , Herpes Zoster/physiopathology , Humans , Male , Mononeuropathies/drug therapy , Mononeuropathies/virology , Osteomyelitis/diagnosis , Otitis Externa/diagnosis , Prednisolone/therapeutic use , Skull Base , Vagus Nerve Diseases/diagnosis , Vagus Nerve Diseases/drug therapy , Vagus Nerve Diseases/physiopathology , Vagus Nerve Diseases/virology , Vestibulocochlear Nerve Diseases/diagnosis , Vestibulocochlear Nerve Diseases/drug therapy , Vestibulocochlear Nerve Diseases/physiopathology , Vestibulocochlear Nerve Diseases/virology , Virus ActivationABSTRACT
Objective: The clinical course of the third, fourth, and sixth cranial nerve palsies varies according to etiology and onset. The effects of Ijintanggagambang in Korean patients with cranial nerve palsy and paralytic diplopia were assessed. Design: This is a retrospective observational study. Settings: The study was conducted in a Korean Medicine clinic in Seoul, Korea. Subjects: Subjects were 569 patients with cranial nerve palsy and diplopia who received Ijintanggagambang in 2009-2018. Interventions: The patients received herbal decoction, Ijintanggagambang, for 4 weeks. Main outcome measures: The baseline characteristics, including onset, etiology, type of paralysis, and deviation angle, were collected. Patients whose diplopia resolved were classified as completely recovered. Potential factors affecting the clinical effectiveness of Ijintanggagambang for this condition were explored. The rate and duration of complete recovery were narratively analyzed and compared with previous reports. Results: Sixth cranial nerve palsy was the most common type of paralysis (n = 311, 55%) and undetermined origin was the most frequent etiology (n = 281, 49%). The complete recovery rate was higher in patients who started treatment within 12 weeks of onset (95%, 503/532) than in those who started treatment later (19%, 7/37). In 532 patients who started treatment within 12 weeks of onset, the complete recovery rate of patients with vascular origin (98%, 122/124), other diseases (97%, 94/97), and undetermined etiology (96%, 252/263) was statistically high compared with those of traumatic origin (73%, 35/48, p < 0.05). The average treatment duration in the complete recovery group (n = 510) was 9.3 weeks. The treatment duration was correlated with the increase of deviation angle in all three types of cranial nerve palsies. Conclusions: Sixth cranial nerve palsy and undetermined etiology were common. There is an association between recovery of diplopia and the administration of Ijintanggagambang when started within 12 weeks of onset. Results of the study need to be interpreted cautiously due to the limited nature of narrative retrospective analysis.
Subject(s)
Abducens Nerve Diseases/drug therapy , Diplopia/drug therapy , Drugs, Chinese Herbal/therapeutic use , Phytotherapy , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Cranial Nerve Diseases/drug therapy , Drugs, Chinese Herbal/administration & dosage , Female , Humans , Male , Medicine, Korean Traditional , Middle Aged , Retrospective Studies , Young AdultABSTRACT
PURPOSE OF REVIEW: This article provides an overview and update on the neurologic manifestations of sarcoidosis. RECENT FINDINGS: The 2018 Neurosarcoidosis Consortium diagnostic criteria emphasize that biopsy is key for diagnosis and determines the level of diagnostic certainty. Thus, definite neurosarcoidosis requires nervous system biopsy and probable neurosarcoidosis requires biopsy from extraneural tissue. Without biopsy, possible neurosarcoidosis can be diagnosed if the clinical, imaging, and laboratory picture is compatible and other causes are ruled out. Recent large retrospective studies from the United States and France established that infliximab appears to be efficacious when other treatments are inadequate. SUMMARY: Sarcoidosis is a multisystem noninfectious granulomatous disorder that is immune mediated, reflecting the response to an as-yet unidentified antigen or antigens. Neurosarcoidosis refers to neurologic involvement due to sarcoidosis that clinically manifests in 5% of cases of sarcoidosis, with asymptomatic involvement in as many as another one in five patients with sarcoidosis. Sarcoid granulomas can occur in any anatomic substrate in the nervous system, causing protean manifestations that have earned neurosarcoidosis the sobriquet the great mimic. Nevertheless, central nervous system sarcoidosis occurs in well-defined presentations that can be classified as cranial neuropathies, meningeal disease, brain parenchymal (including pituitary-hypothalamic) disease, and spinal cord disease. In addition, the peripheral nervous system is affected in the form of peripheral neuropathy and myopathy. Glucocorticoids are the cornerstone of treatment, especially in the acute stage, whereas steroid-sparing agents such as methotrexate, mycophenolate mofetil, and azathioprine are used for prolonged therapy to minimize steroid toxicity. Anti-tumor necrosis factor agents may help in refractory cases.
Subject(s)
Brain Diseases/diagnosis , Central Nervous System Diseases/diagnosis , Cranial Nerve Diseases/diagnosis , Neuromuscular Diseases/diagnosis , Peripheral Nervous System Diseases/diagnosis , Sarcoidosis/diagnosis , Spinal Cord Diseases/diagnosis , Brain Diseases/drug therapy , Brain Diseases/etiology , Central Nervous System Diseases/complications , Central Nervous System Diseases/drug therapy , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Humans , Neuromuscular Diseases/drug therapy , Neuromuscular Diseases/etiology , Peripheral Nervous System Diseases/drug therapy , Peripheral Nervous System Diseases/etiology , Sarcoidosis/complications , Sarcoidosis/drug therapy , Spinal Cord Diseases/drug therapy , Spinal Cord Diseases/etiologySubject(s)
Central Nervous System Diseases/diagnostic imaging , Cranial Nerve Diseases/diagnostic imaging , Optic Nerve Diseases/diagnostic imaging , Sarcoidosis/diagnostic imaging , Central Nervous System Diseases/drug therapy , Cranial Nerve Diseases/drug therapy , Female , Humans , Immunomodulation , Magnetic Resonance Imaging , Middle Aged , Optic Nerve Diseases/drug therapy , Positron Emission Tomography Computed Tomography , Recurrence , Sarcoidosis/drug therapy , Vision Disorders/diagnosis , Vision Disorders/physiopathology , Visual Acuity/physiologyABSTRACT
A 53-year-old immunocompromised woman developed acute left eye blindness and paraparesis suspected to be due to neuromyelitis optica (NMO). During treatment for NMO, right eye blindness and progressive multiple cranial neuropathies developed. Cerebrospinal fluid polymerase chain reaction (PCR) revealed Varicella zoster virus (VZV). This case emphasizes the importance of considering VZV in individuals, particularly the immunocompromised, presenting with a constellation of neurological signs and symptoms, even in the absence of rash.
Subject(s)
Blindness/diagnosis , Cranial Nerve Diseases/diagnosis , Encephalitis, Varicella Zoster/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Neuromyelitis Optica/diagnosis , Retinal Necrosis Syndrome, Acute/diagnosis , Antiviral Agents/therapeutic use , Blindness/drug therapy , Blindness/immunology , Blindness/virology , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/immunology , Cranial Nerve Diseases/virology , Encephalitis, Varicella Zoster/drug therapy , Encephalitis, Varicella Zoster/immunology , Encephalitis, Varicella Zoster/virology , Female , Herpesvirus 3, Human , Humans , Immunocompromised Host , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/immunology , Lupus Erythematosus, Systemic/virology , Middle Aged , Neuromyelitis Optica/drug therapy , Neuromyelitis Optica/immunology , Neuromyelitis Optica/virology , Retinal Necrosis Syndrome, Acute/drug therapy , Retinal Necrosis Syndrome, Acute/immunology , Retinal Necrosis Syndrome, Acute/virology , Virus ActivationABSTRACT
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.
Subject(s)
Cranial Nerve Diseases/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Meningeal Neoplasms/pathology , Neoplasm Recurrence, Local/pathology , Antineoplastic Combined Chemotherapy Protocols , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/radiotherapy , Diagnosis, Differential , Facial Nerve Diseases/etiology , Humans , Lymphoma, Large B-Cell, Diffuse/drug therapy , Lymphoma, Large B-Cell, Diffuse/radiotherapy , Male , Meningeal Neoplasms/drug therapy , Meningeal Neoplasms/radiotherapy , Middle Aged , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/radiotherapy , Vestibulocochlear Nerve Diseases/etiologyABSTRACT
Hypertrophic pachymeningitis (HP) is a relatively uncommon disease associated with focal or diffuse thickening of the dura mater secondary to underlying chronic inflammation. The link between systemic lupus erythematosus (SLE) and hypertrophic pachymeningitis (HP) is extremely rare, with only six other cases reported in the literature. We, however, report the first case of SLE pachymeningitis presenting with multiple cranial nerve palsies. The patient showed good response to steroids and cyclophosphamide therapy. One should maintain a high index of suspicion to make the diagnosis in patients with SLE presenting with neurological dysfunction. Prompt therapy prevents long-term neurological sequelae.
Subject(s)
Cranial Nerve Diseases/etiology , Lupus Erythematosus, Systemic/complications , Meningitis/etiology , Cranial Nerve Diseases/drug therapy , Cyclophosphamide/administration & dosage , Female , Glucocorticoids/administration & dosage , Humans , Immunosuppressive Agents/administration & dosage , Lupus Erythematosus, Systemic/drug therapy , Meningitis/drug therapy , Middle Aged , Treatment OutcomeSubject(s)
Cat-Scratch Disease/diagnosis , Splenic Diseases/diagnosis , Adolescent , Animals , Anti-Bacterial Agents/therapeutic use , Bartonella henselae/isolation & purification , Cat-Scratch Disease/drug therapy , Cats , Cranial Nerve Diseases/diagnostic imaging , Cranial Nerve Diseases/drug therapy , Humans , Male , Optic Disk/diagnostic imaging , Pleural Effusion/diagnostic imaging , Pleural Effusion/diet therapy , Splenic Diseases/drug therapyABSTRACT
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