Paraneoplastic Opsoclonus-myoclonus Syndrome Research Articles

Paraneoplastic opsoclonus-myoclonus syndrome as a rare presentation of parotid adenocarcinoma

Paraneoplastic Opsoclonus-Myoclonus Ataxia Syndrome (POMA) is a rare neurological condition that affects approximately 1 in 10,000,000 people annually. This syndrome is poorly understood and can lead to long-term cognitive, behavioral, and motor complications. Opsoclonus is characterized by involuntary, rapid, repetitive, multi-vectorial oscillations of the eyes occurring in all directions of gaze. It is accompanied by diffuse or focal body myoclonus and may or may not include ataxia and other cerebellar signs. POMA is typically a paraneoplastic syndrome associated with neuroblastoma in childhood and breast carcinoma or small-cell lung carcinoma in adults. Additionally, viral or toxic agents are known to play a role in its etiology, and the immune system is involved in the pathogenesis. We report a case of a 41-year-old man with anti-Ri antibody opsoclonus-myoclonus syndrome and parotid adenocarcinoma involvement. After diagnosing opsoclonus-myoclonus syndrome, the patient underwent multimodal immunotherapy treatment, resulting in partial remission of the neurological symptoms.

Paraneoplastic Opsoclonus-Myoclonus Syndrome Associated with Non-Small Cell Lung Cancer.

Paraneoplastic Opsoclonus-Myoclonus Syndrome Associated with Non-Small Cell Lung Cancer.

Anti-SOX1 Antibody-positive Small-cell Lung Cancer That Triggered Opsoclonus.

A 72-year-old woman with opsoclonus visited our hospital and was diagnosed with small-cell lung cancer. Blood tests revealed anti-SOX1 antibodies, so the patient was diagnosed with paraneoplastic opsoclonus-myoclonus syndrome. After steroid pulse therapy was started, chemotherapy of treatment, the opsoclonus showed an improving trend. Anti-Ri and anti-Hu antibodies have been reported as autoantibodies associated with neoplastic opsoclonus-myoclonus syndrome; however, there are no such reports concerning anti-SOX1 antibody. Therefore, this is a valuable case.

Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report

BackgroundOpsoclonus-myoclonus syndrome (OMS) is a rare, immune-mediated neurological disorder. In adults, the pathogenesis can be idiopathic, post-infectious or paraneoplastic, the latter etiology belonging to the ever-expanding group of defined paraneoplastic neurological syndromes (PNS). In contrast to other phenotypes of PNS, OMS cannot be ascribed to a single pathogenic autoantibody. Here, we report the first detailed case of paraneoplastic, antibody-negative OMS occurring in association with a pancreatic neuroendocrine tumor (pNET).Case presentationA 33-year-old female presented with a two-week history of severe ataxia of stance and gait, dysarthria, head tremor, myoclonus of the extremities and opsoclonus. Her past medical history was notable for a metastatic pancreatic neuroendocrine tumor, and she was subsequently diagnosed with paraneoplastic opsoclonus-myoclonus syndrome. Further workup did not reveal a paraneoplastic autoantibody. She responded well to plasmapheresis, as she was refractory to the first-line therapy with corticosteroids.ConclusionsThis case expands current knowledge on tumors associated with paraneoplastic opsoclonus-myoclonus syndrome and the age group in which it can occur. It further adds evidence to the effectiveness of plasmapheresis in severe cases of opsoclonus-myoclonus syndrome with a lack of response to first-line therapy.

Postinfectious autoimmune encephalitis with opsoclonus-myoclonus syndrome

Opsoclonus is irregular chaotic eye movements, accompanied by impaired gaze fixation, oscillopsia and associated visual impairment. It is combined with myoclonus of the extremities, trunk, change in gait. It occurs in many pathological conditions, most often in oncological and post-infectious diseases. The rarity and uncommonness of the clinical manifestations of the opsoclonus-myoclonus syndrome, as well as the poor awareness of doctors about this pathology, cause diagnostic difficulties and errors. This report presents the case of a 49-year-old patient, who developed unsteadiness when walking, opsoclonus, myoclonus, startle syndrome, exaggerated startle response 3 weeks after an acute intestinal infection. The diagnostic errors made were analyzed. The diagnosis Post-infection autoimmune encephalitis was proposed and substantiated. Paraneoplastic opsoclonus-myoclonus syndrome was excluded by prospective observation of the patient for 6 years. High efficiency of glucocorticoid treatment was noted.

Early rituximab therapy for paraneoplastic opsoclonus-myoclonus syndrome

Early rituximab therapy for paraneoplastic opsoclonus-myoclonus syndrome

Breast cancer-associated opsoclonus-myoclonus syndrome: a case report

BackgroundParaneoplastic neurological syndromes constitute rare neurological complications of malignant disease, manifesting in <1% of patients with cancer. Opsoclonus-myoclonus syndrome (OMS) presents with chaotic ocular saccades (opsoclonus), spontaneous muscular jerking (myoclonus) that may be accompanied by ataxia, strabismus, aphasia, or mutism. Its paraneoplastic variant in the adult is most commonly associated with small-cell lung cancer, followed by breast cancer. Importantly, neurological symptoms usually precede the diagnosis of breast cancer and tend to recure after its treatment.Case presentationA 43-year-old premenopausal Caucasian woman with a medical history of hypertension was admitted following an episode of focal seizure. This progressed to generalised tonic-clonic seizures and she was subsequently loaded with phenytoin, valproate, and levetiracetam. Initial workup included whole body CT scan, viral and autoimmune serology. The CT scan revealed an enhancing right axillary lymph node, which in combination with Anti-Ri antibody positivity raised the spectre of paraneoplastic OMS. MRI of the head revealed subtle nonspecific white matter signal change within the centrum semiovale without any mass lesions, while MRI of the spine was unremarkable. An uncomplicated right mastectomy and axillary lymph node clearance was performed: histopathology revealed a 9-mm, grade 2, oestrogen receptor-positive, progesterone receptor-negative (ER8, PR0), Her2-negative invasive ductal carcinoma, and 4/6 positive lymph nodes (T1b N2 M0). Two months later, she was readmitted with vertigo, diplopia, facial weakness, and ataxia, setting the diagnosis anti-Ri syndrome recurrence. MDT recommended mammogram and ultrasound of the left breast, which were normal. Subsequently, four months after initial discharge, she suffered another neurological recurrence; due to concomitant abdominal pain, PET-CT was performed demonstrating a hypermetabolic right ovarian focus. Bilateral salpingo-oophorectomy was performed as per gynaecology MDT and final histology showed normal tubes and ovaries. She has remained on remission since then, with a negative annual mammogram follow-up.ConclusionsIn conclusion, we report a case of OMS associated with breast cancer anti-Ri onconeural antibody. Its manifestations preceded the diagnosis of malignancy and it persisted after cancer treatment, underlining the importance for high clinical suspicion in cases of classical paraneoplastic neurological syndromes as well as the need for long-term clinical follow-up.

Anti-Ma paraneoplastic opsoclonus–myoclonus syndrome

A 73-year-old woman with hypertension, type 2 diabetes and a 25 pack-year smoking history presented to the hospital with a 2-month history of dizziness, diplopia, memory loss, ataxia, insomnia and...

Opsoclonus-myoclonus syndrome associated with anti Kelch-like protein-11 antibodies in a young female patient without cancer

Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder. The pathogenesis is thought to be immune-mediated. In adults, it may be idiopathic or paraneoplastic in origin. However, most cases of paraneoplastic OMS in adults are not associated with well-characterized antibodies, except for a small subgroup who have anti-Ri antibodies. Herein, we provide the first detailed description of a case of OMS associated with a Kelch-like protein-11 antibody, a newly discovered biomarker for paraneoplastic neurological syndromes associated with germ-cell tumors. This was a young female patient in whom no tumor was ever detected and who had an excellent response to rituximab.

Paraneoplastic Opsoclonus-Myoclonus Syndrome As Presenting Symptom of Primary Gallbladder Adenocarcinoma (2632)

Paraneoplastic Opsoclonus-Myoclonus Syndrome As Presenting Symptom of Primary Gallbladder Adenocarcinoma (2632)

Corrigendum: Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small cell carcinoma of the lung.

Alkan A, Cenikli U, Uylaş S, Yilmaz M, Çakir T, Çetinkaya H, Kiran A and Tanriverdi O (2019). Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small cell carcinoma of the lung: Journal of Oncology Pharmacy Practice. E-pub Feb 2019 (Original DOI: 10.1177/1078155219831366). In the above referenced article, the following author was missed from the title: Gülnihal Kutlu. The Correct author list should read: Alkan A, Cenikli U, Uylaş S, Yilmaz M, Çakir T, Çetinkaya H, Kiran A, Kutlu G and Tanriverdi O (2019).

A rare case of paraneoplastic opsoclonus myoclonus syndrome that developed during chronic maintenance hemodialysis

A rare case of paraneoplastic opsoclonus myoclonus syndrome that developed during chronic maintenance hemodialysis

Acute Cerebellar Ataxia, Acute Cerebellitis, and Opsoclonus-Myoclonus Syndrome

Acute cerebellar ataxia and acute cerebellitis represent a process characterized by parainfectious, postinfectious, or postvaccination cerebellar inflammation. There is considerable overlap between these entities. The mildest cases of acute cerebellar ataxia represent a benign condition that is characterized by acute truncal and gait ataxia, variably with appendicular ataxia, nystagmus, dysarthria, and hypotonia. It occurs mostly in young children, presents abruptly, and recovers over weeks. Neuroimaging is normal. Severe cases of cerebellitis represent the other end of the spectrum, presenting with acute cerebellar signs often overshadowed by alteration of consciousness, focal neurological deficits, raised intracranial pressure, hydrocephalus, and even herniation. Neuroimaging is abnormal and the prognosis is less favorable than in acute cerebellar ataxia. Acute disseminated encephalomyelitis may be confused with acute cerebellitis when the clinical findings are predominantly cerebellar, but lesions on neuroimaging are usually widespread. Paraneoplastic opsoclonus-myoclonus syndrome is often initially misdiagnosed as acute cerebellar ataxia, but has very specific features, course, and etiopathogensis.

Paraneoplastic Opsoclonus-Myoclonus Syndrome as a New and Single Manifestation of Relapsing Disease in a Patient with Small Cell Lung Cancer

A 55-year-old woman smoker with a history of cough, weight loss, and chest pain, without neurologic complaints, presented with a mass in the right lung. A bronchoscopy revealed a tumor in the right intermediate bronchus. The biopsy of the lesion showed small cell carcinoma. A FDGpositron emission tomography (PET)-computed tomography (CT) scan was performed and is displayed in Figure 1A. A brain CT was normal. After the diagnosis of limited-stage small cell lung cancer, the patient was treated with four cycles of cisplatin and etoposide combined with normofractionated thoracic radiotherapy (55 Gy) concurrent with the last two cycles. A radiological complete response was assessed, and treatment was completed with prophylactic cranial irradiation. Seven months after complete response, the patient experienced rapidly progressive dizziness and gait instability, followed by gaze disturbances and involuntary, continuous, conjugate, and chaotic saccadic eye movements. The gait disorder worsened, leaving the patient absolutely bedridden. On examination, intermittent myoclonic movements were apparent in the limbs and trunk (see video, part 1, Supplemental Digital Content 1, http://links.lww.com/JTO/A73, which shows these findings). She had neither cognitive impairment nor motor/sensory losses. Brain magnetic resonance imaging and cerebrospinal fluid examination were without noteworthy alterations. Serum antineuronal antibodies (antiHu, anti-Ri, anti-CV2, and antiamphiphysin) were negative. She was diagnosed with opsoclonus-myoclonus syndrome (OMS), and intravenous corticoids were administered for 5 days without improvement. There was no evidence of relapse on the chest CT, and the patient refused a bronchoscopy. A positron emission tomography-CT examination was strictly normal (Figure 1B). Although there was no visible tumor, we considered OMS as its herald, and second-line chemotherapy was started with carboplatin and etoposide in an attempt to reverse her devastating neurologic condition. After just the first cycle, opsoclonus and gait instability improved and the myoclonus resolved completely (see video, part 2, Supplemental Digital Content 1, http://links.lww.com/JTO/A73, which demonstrates her clinical improvement). The patient has completed three cycles of chemotherapy and continued free of recurrence for a total of 31 months, when a right supraclavicular relapse was detected and pathologically confirmed. Nevertheless, paraneoplastic neurologic symptoms did not recur, and third-line chemotherapy with oral topotecan was started.

P06.5 Diagnosis of paraneoplastic opsoclonus-myoclonus syndrome in children

P06.5 Diagnosis of paraneoplastic opsoclonus-myoclonus syndrome in children

Paraneoplastic opsoclonus–myoclonus syndrome secondary to immature ovarian teratoma

Paraneoplastic neurologic complications from gynecologic tumors are rare, but can be a major cause of morbidity and mortality. In most cases, the neurologic symptoms precede the diagnosis of malignancy, prompting an evaluation that identifies the tumor. Paraneoplastic opsoclonus–myoclonus syndrome, characterized by saccadic eye movements caused by lesions in the brain stem, has in particular been linked to ovarian teratomas.

Clonazepam responsive opsoclonus myoclonus syndrome: additional evidence in favour of fastigial nucleus disinhibition hypothesis?

Opsoclonus myoclonus syndrome is a rare paraneoplastic syndrome seen in 50% of children with neuroblastoma. Neural generator of opsoclonus and myoclonus is not known but evidences suggest the role of fastigial nucleus disinhibition from the loss of function of inhibitory (GABAergic) Purkinje cells in the cerebellum. We present a child with paraneoplastic opsoclonus myoclonus syndrome who responded well to clonazepam. Response to clonazepam is an evidence for the involvement of GABAergic neural circuits in the genesis of opsoclonus myoclonus syndrome and is in agreement with fastigial nucleus disinhibition hypothesis.

めまいで発症した傍腫瘍性オプソクローヌス・ミオクローヌス症候群

A case of paraneoplastic opsoclonus-myoclonus syndrome with an onset of vertigo is reported. A 72-year-old man complaining of vertigo, nausea and vomiting consulted our hospital on March 13, 2006. Since neurological examination showed opsoclonus of the eye movement, ataxia and adiadochokinesis, disequilibrium due to a central nervous system disorder was strongly suggested. Brain MRI did not demonstrate any evidence of tumor or ischemic lesion. CSF analysis did not show any malignant cells. However, lung X-ray image showed an abnormal shadow in the upper lung field. Therefore, a biopsy was performed under bronchoscopy and small cell lung carcinoma was diagnosed. Based on these findings, the patient was diagnosised as having paraneoplastic opsoclonus-myoclonus syndrome from small cell lung carcinoma. Anti-Ri and anti-Hu antibodies were unremarkable. He was treated with antidinic, psychomimetic and antineoplastic therapies. Vertiginous sensation and nausea decreased but opsoclonus persisted. He died of pneumonia on June 30th, 2006.

Paraneoplastic opsoclonus-myoclonus ataxia associated with non-small-cell lung carcinoma

We report a case of the paraneoplastic opsoclonus-myoclonus ataxia syndrome in a patient with non-small cell lung carcinoma, which represents a highly unusual association. The patient simultaneously manifested both severe neurologic symptoms and widely metastatic disease, complicating her treatment.

Analysis of antibodies to neuronal surface antigens in adult opsoclonus–myoclonus

Antibodies against neuronal surface antigens (NSA-ab) have been described in pediatric opsoclonus–myoclonus syndrome (OMS). We analyzed the presence of NSA-ab by flow cytometry and immunocytochemistry of live cerebellar granular neurons (CGN) in the serum of 25 adult patients with idiopathic (14) and paraneoplastic (11) OMS. Paraneoplastic, but not idiopathic, OMS sera showed a CGN surface binding by flow cytometry higher than that of controls (mean MFI (median fluorescence intensity): 29 ± 6.9 vs. 20 ± 5.8; p = 0.001) but only one serum had a binding greater than three standard deviations of controls. OMS sera did not label live CGN by immunocytochemistry. Unlike pediatric OMS, NSA-ab were not detected in adult cases suggesting that the immunity to NSA in OMS is heterogeneous.