Paraneoplastic Neuromyelitis Optica Research Articles

Paraneoplastic Neuromyelitis Optica Associated with Renal Cell Carcinoma Presenting with Tumefactive Demyelination Lesions

Paraneoplastic Neuromyelitis Optica Associated with Renal Cell Carcinoma Presenting with Tumefactive Demyelination Lesions

Paraneoplastic neuromyelitis optica spectrum disorder in a patient with breast cancer and adiuvant chemotherapy

Neuromyelitis optica spectrum disorder (NMOSD), is an autoimmune inflammatory disorder of the central nervous system mostly associated with antibodies to AQP-4. It is generally idiopathic, but it is frequently associated with systemic autoimmune disorders and in some cases with neoplasm. We propose a case of NMOSD associated with breast cancer and potentially with its treatment.

Paraneoplastic Neuromyelitis Optica Spectrum Disorder in a Patient with Endometrial Adenocarcinoma (P1.2-018)

Paraneoplastic Neuromyelitis Optica Spectrum Disorder in a Patient with Endometrial Adenocarcinoma (P1.2-018)

Paraneoplastic neuromyelitis optica spectrum disorder diagnosed with mantle cell lymphoma

Paraneoplastic neuromyelitis optica spectrum disorder diagnosed with mantle cell lymphoma

A case of paraneoplastic neuromyelitis optica associated with small cell lung carcinoma

Neuromyelitis optic spectrum disorders are demyelinating conditions that are typically idiopathic, though various case reports have demonstrated an association with malignancy.We present the case of a 64year old woman with NMOSD in the setting of small cell lung cancer. She had longitudinally extensive transverse myelitis and left eye optic neuritis; aquaporin-4 antibodies were elevated. Biopsy of mediastinal adenopathy was positive for SCLC.Malignancy should be considered in any patient with an atypical presentation of NMOSD, or who does not respond to traditional therapies.

Paraneoplastic Neuromyelitis Optica Spectrum Disorder: A single center cohort description with two cases of histological validation

BackgroundParaneoplastic syndromes are remote effects of cancer caused by an autoimmune response triggered by tumor cells. Paraneoplastic Neuromyelitis Optica Spectrum Disorders (NMOSD) has been previously described, but the underlying mechanism for these rare cases is not well characterized. This paper presents a newly described case series of paraneoplastic NMOSD, including 2 new histological types of cancer and histological validation. MethodsThe UBC NMO clinic database was surveyed and identified 6 patients with paraneoplastic NMOSD. In 2 cases, aquaporin-4 (AQP4) immunoreactivity was assessed on patients’ tumor specimens. ResultsThe mean age at NMOSD diagnosis was 56 years old and 5/6 patients were older than 50 years old. 4/6 patients with paraneoplastic NMOSD have positive AQP4 antibodies. The median time between NMOSD and cancer was 12 months. Two new cancer types- serous ovarian carcinoma and adrenocortical carcinoma - were found in paraneoplastic NMOSD cases. A serous ovarian carcinoma and a thymoma, found in patients with AQP4 serological evidence, showed a positive reactivity to AQP4 immunostaining. ConclusionsOur findings will increase the recognition of NMOSD as a paraneoplastic syndrome. Cancer cells can express AQP4, increasing the likelihood of a direct mechanism between cancer cells and the development of NMOSD in paraneoplastic cases.

Treatment for paraneoplastic neuromyelitis optica spectrum disorder (NMOsd): Probable effects of tocilizumab for both cancer and NMOsd

Treatment for paraneoplastic neuromyelitis optica spectrum disorder (NMOsd): Probable effects of tocilizumab for both cancer and NMOsd

Clinical profile of patients with paraneoplastic neuromyelitis optica spectrum disorder and aquaporin-4 antibodies

Background: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. Objective: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. Methods: Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG. Paraneoplastic patients were defined as those with cancer identified within 2 years of the diagnosis of NMOSD. Results: Five (3.2%) of 156 patients had paraneoplastic NMOSD, and 12 previously reported patients were identified. The most common tumors were adenocarcinoma of the lung (five patients) and breast (five). Compared with the 151 non-paraneoplastic NMOSD patients, the 17 (5 current cases and 12 previously reported) were older at symptom onset (median age = 55 (range: 17–87) vs 40 (range: 10–77) years; p = 0.006), more frequently male (29.4% vs 6.6%; p = 0.009), and presented with severe nausea and vomiting (41.2% vs 6.6%; p < 0.001). The frequency of longitudinal extensive transverse myelitis (LETM) as heralding symptom was similar in both groups, but patients with paraneoplastic NMOSD were older than those with non-paraneoplastic NMOSD (median age: 63 (range: 48–73) vs 43 (range: 14–74) years; p = 0.001). Conclusion: Patients, predominantly male, with NMOSD and AQP4-IgG should be investigated for an underlying cancer if they present with nausea and vomiting, or LETM after 45 years of age.

Paraneoplastic neuromyelitis optica spectrum disorder manifesting as intractable nausea and acute cerebellar ataxia associated with lung adenocarcinoma

AbstractWe report a 65‐year‐old woman with neuromyelitis optica spectrum disorder manifesting acute cerebellar ataxia, and intractable nausea and vomiting. She tested positive for anti‐aquaporin 4 antibodies and had a lung adenocarcinoma simultaneously. She underwent partial pulmonary resection, and tumor cells of the lung adenocarcinoma were stained with an anti‐aquaporin 4 antibody. By administering intravenous methyl prednisolone and plasma exchange, aquaporin 4 antibody titer was reduced, and neurological deficits and the lesion status on magnetic resonance imaging markedly improved. Paraneoplastic neuromyelitis optica spectrum disorder in conjunction with lung adenocarcinoma was a reasonable diagnosis.

Paraneoplastic neuromyelitis optica spectrum disorders: three new cases and a review of the literature

Neuromyelitis optica spectrum disorders (NMOSD) occasionally develop in patients with tumor in relation to aquaporin-4 IgG (AQP4-IgG), representing a new paraneoplastic phenomenon. We reported three patients with paraneoplastic NMOSD and provided a comprehensive review of the literature. A total of 34 cases with paraneoplastic NMOSD were identified from our own case database (n = 3) and the previous literature (n = 31). The median age at NMOSD-related symptom onset was 50.5 years, and 91% of the cases were female. 11 (32%) cases had breast carcinoma. In 15 (44%) cases, NMOSD-related symptoms preceded tumor detection [median, 4 (range 1–180) months], and in 19 (56%) cases, symptoms followed tumor detection [median, 12 (range 3–180) months]. 5/14 (36%) cases had hiccups and vomiting as the initial symptoms, with the involvement of medulla oblongata. In 10/14 (71%) cases, cervical spinal cord was involved. In contrast to idiopathic NMO, NMOSD is more likely to be paraneoplastic than in patients aged over 50 years at the onset of symptoms, especially for female patients. Breast carcinoma is the most common tumor associated with paraneoplastic NMOSD, accounting for nearly a third of all types of tumors. Paraneoplastic NMOSD usually involves medulla oblongata and cervical spinal cord. We recommend adding AQP4-IgG as an onconeural antibody, but its clinical utility warrants further investigations.

Paraneoplastic neuromyelitis optica spectrum disorder associated with stomach carcinoid tumor

Neuromyelitis optica (NMO), or Devic's syndrome, is an autoimmune central nervous system demyelinating disorder primarily affecting the spinal cord and the optic nerves. It is characterized by the presence of NMO antibodies, alongside clinical and radiological findings. NMO and NMO-spectrum disorders (NMO-SD) have been reported in autoimmune disorders, and are infrequently described as a paraneoplastic syndrome with cancers of lung, breast, and carcinoid tumors of the thyroid. We report a patient who presented with severe vomiting, blurring of vision, vertigo, diplopia, left hemiparesis and hemisensory loss and ataxia. She was found to have a longitudinally-extensive demyelinating lesion extending from the medulla to the upper cervical spinal cord on MRI. Her gastric endoscopy revealed carcinoid tumor of the stomach, and classic paraneoplastic antibodies in the serum were negative. She had extremely high serum gastrin level and high titer of NMO IgG autoantibody. The patient made an excellent recovery with tumor resection and immunotherapy, with both clinical and radiological improvement. On rare instances, NMO or NMO-SD may present as a paraneoplastic neurological syndrome associated with carcinoid tumor of the stomach.

Paraneoplastic neuromyelitis optica associated with stomach carcinoid tumor

Paraneoplastic neuromyelitis optica associated with stomach carcinoid tumor

Uncommon ocular and visual signs of relapsing remitting multiple sclerosis

Uncommon ocular and visual signs of relapsing remitting multiple sclerosis

Rheumatoid arthritis and amyotrophic lateral sclerosis association: A case report and literature review

Rheumatoid arthritis and amyotrophic lateral sclerosis association: A case report and literature review

A possible paraneoplastic neuromyelitis optica associated with lung cancer

Neuromyelitis optica is associated with antibodies to aquaporin-4 channels and has been classically defined as the occurrence of bilateral optic neuritis and transverse myelitis with no other neurological involvement. We describe here a 63-year-old woman who presented to our local hospital with an acute, bilateral visual loss followed by progressive paraparesis. Medical evaluations revealed a lung tumor and spinal MRI showed multiple cervical-dorsal hyperintense T(2) lesions with mild mass effect. Testing for NMO-Ig resulted positive. Patient's diagnosis was thus consistent with a possible paraneoplastic neuromyelitis optica associated with non-small cell lung cancer and evidence of NMO-Ig.