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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paraneoplastic neurological syndromes (PNS) are remote effects of cancer, which are not caused by invasion of the tumour or its metastasis, but presumably immunologic mediated. They developed in less than 1% of patients with systemic cancers, most Limbic encephalitis (LE) is the most common clinical paraneoplastic syndrome attainting the CNS (Central Nervous System), and it is characterized by involvement of hippocampus and amygdala; LE is also the only one with clearly defined imaging features. We report a 64 year old man, former smoker, which presented multiple paraneoplastic syndromes, namely LE, opsoclonus-myoclonus, subacute cerebellar degeneration, brainstem encephalitis, sensory-motor neuropathy and inappropriate antidiuretic hormone secretion syndrome (SIADH); these syndromes were identifiable in the clinical and imaging examination, confirmed by the neuro-pathological study. Magnetic Resonance Imaging disclosed on T2 weighted images bilateral hyperintensity within medial temporal lobes, hypothalamus, thalamus, brain stem, upper and medium cerebellar peduncles and upper cervical spinal cord. The underlying tumour was not found in imaging studies or in the autopsy examination. A mediastinal adenopathy depicted a metastasis from low differentiated neoplasm cells, with some signs of neuroendocrine differentiation. With this case we provide a comprehensive illustration of the PNS, from a clinical, imaging and pathological point of view. This report also emphasises the importance of a diagnosis based on early clinical and imaging findings, given that, in most cases the cancer is not apparent.
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PMID:[Multiple paraneoplastic syndromes occurring in the same patient: clinical, imaging and neuro-pathological documentation]. 1758 9

The treatment and disposition of patients with neurological disorders is commonplace in the emergency setting, but atypical and uncommon presentations can prove to be especially challenging. In this article we discuss the case of a 31-year-old woman who presented with a rare disorder known as opsoclonus myoclonus syndrome (OMS). OMS is characterized by involuntary, multidirectional movement of the eyes, myoclonus, and truncal ataxia. The etiology is thought to be autoimmune, and is most commonly associated with encephalitis or paraneoplastic syndrome. After an 8-day hospital course, which included several different treatment modalities including plasmapheresis, the patient was discharged after making a complete recovery. Unusual presentations such as the one described in this article illustrate the point that it is crucial to have a systematic approach that can be applied to identify and treat potentially life-threatening neurological conditions.
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PMID:Emergency department presentation of a rare neurological disorder. 1848 9

Childhood opsoclonus-myoclonus syndrome (OMS) occurs idiopathic or, in association with a neuroblastoma, as a paraneoplastic syndrome. Since autoantibodies were identified in some patients, an autoimmune pathogenesis has been suspected. While the newly discovered B-cell activating factors BAFF and APRIL are involved in systemic autoimmune diseases, their association with neuroimmunological diseases is hardly understood. We here investigated the BAFF and APRIL levels in serum and cerebrospinal fluid (CSF) of OMS patients and their correlation with surface-binding autoantibodies. BAFF and APRIL were both determined by ELISA, and autoantibodies to cerebellar granular neurons (CGN) have been investigated by flow cytometry in 17 OMS patients, 16 neuroblastoma (NB) patients, 13 controls and 11 children with inflammatory neurological diseases (IND). BAFF, but no APRIL, was elevated in the CSF of OMS children and IND children. However, in contrast to IND patients, OMS patients did not have a blood-brain-barrier disturbance, indicating that BAFF was produced intrathecally in OMS patients, but not in IND patients. CSF BAFF levels showed a correlation with CSF CGN autoantibodies (r(2)=0.58, p<0.05). These data indicate that an activated B-cell system in the cerebrospinal fluid is involved in the pathogenesis of OMS, and BAFF may be a candidate parameter for the activation of B-cell immune system.
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PMID:Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome. 1933 60

Paraneoplastic neurologic syndrome, a rare complication of carcinoma, includes various neurologic disorders, such as encephalomyelitis, paraneoplastic cerebellar degeneration, subacute sensory neuronopathy, retinal paraneoplastic syndrome, opsoclonus-myoclonus syndrome and stiff-person syndrome. Several antibodies to malignant tumor cells and neurons are detected in sera and cerebrospinal fluids of patients with this syndrome, however, there is no direct evidence of antiYo or antiHu antibodies' causative roles in neuronal loss. Recent studies showed cytotoxic T-cell activities against peptides of an antigen protein recognized by antibodies in the peripheral blood of patients with paraneoplastic cerebellar degeneration and antiYo antibodies, as well as in patients with antiHu syndrome. Treatment of paraneoplastic neurologic syndrome with plasmapheresis, immunosuppresive drugs, or intravenous immunoglobulin therapy has been attempted. Here, we discuss previous reports and theoretical treatments based on recent etiological hypothesis of paraneoplastic cerebellar degeneration.
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PMID:Pathogenesis and treatment of paraneoplastic neurologic syndrome. 1981 Sep 23

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.
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PMID:Clonazepam responsive opsoclonus myoclonus syndrome: additional evidence in favour of fastigial nucleus disinhibition hypothesis? 2009 37

Neuroblastoma is the most common extracranial solid tumor in childhood. Its presenting signs and symptoms may be highly variable, depending on the location of the primary tumor and its local or metastatic diffusion and, rarely, with paraneoplastic syndrome such as opsoclonus-myoclonus-ataxia syndrome and gastrointestinal disturbances, due to autoantibodies or to aberrant secretion of vasoactive intestinal peptide. Herein we describe a 10-month-old child with neuroblastoma presenting with a complex clinical picture characterized by acute kidney injury manifested by renal insufficiency and signs and symptoms of tubulointerstitial damage, with polyuria, polydipsia, glucosuria, aminoaciduria and hypochloremic metabolic alkalosis, and of glomerular damage with heavy proteinuria. Imaging study documented a suprarenal mass enveloping the aorta and its abdominal and renal ramifications and bilaterally renal veins. This clinical picture shows some analogies with the hyponatremic-hypertensive syndrome concerning the renovascular disease; however, in absence of systemic arterial hypertension, the heavy proteinuria and the polyuria could be explained by sectional increased intraglomerular pressure, due to local renal blood vessels constriction. Hypochloremic metabolic alkalosis probably developed because of local production of renin, responsible of renin-angiotensin-aldosterone system activation, but above all because of chloride loss through sweating. The long lasting dehydration, due to vomiting, sweating and polyuria, caused prolonged prerenal failure evolving in proximal tubular damage manifestations.
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PMID:Neuroblastoma presenting with acute kidney injury, hyponatremic-hypertensive-like syndrome and nephrotic proteinuria in a 10-month-old child. 2194 89

Paraneoplastic cerebellar degeneration associated with anti-Ri antibodies mainly presents with opsoclonus-myoclonus-ataxia. We report here the case of a patient with anti-Ri-antibody paraneoplastic syndrome, who presented four years after treatment for small-cell lung cancer (SCLC) with oscillopsia and gait disorder. On neurological examination vertical nystagmus, ataxic gait and postural tremor of all four limbs was detected. He died one year after the onset of the symptoms because of a acute exacerbation of his severe chronic obstructive pulmonary disease. No SCLC relapse or new cancer has been detected during the one-year follow-up period.To our knowledge, our patient is the first case of anti-Ri associated disorder with oscillopsia and vertical nystagmus as the initially prominent clinical features. The findings of this case study support the variability of anti-Ri-antibody-associated paraneoplastic syndrome. Further studies must be directed to better characterize the mechanisms underlying this syndrome. Finally, paraneoplastic neurological syndromes should be kept in mind also when a neoplastic disease is not demonstrated.
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PMID:Anti-Ri-associated paraneoplastic cerebellar degeneration. Report of a case and revision of the literature. 2202 93

Opsoclonus myoclonus is a rare autoimmune condition characterized by cerebellar degeneration. It occurs most often as a paraneoplastic syndrome when a cancer remote to the brain induces cerebellar dysfunction that is unrelated to metastases. Half of all cases occur in children with a neuroblastoma. Most adults with opsoclonus myoclonus have neoplastic, infectious, metabolic, or idiopathic etiologies. Signs of cerebellar dysfunction noted at presentation include opsoclonus, myoclonus and ataxia, hence the name "dancing eyes, dancing feet syndrome." Opsoclonus is characterized by rapid, involuntary eye movements that are dysrhythmic and uncoordinated.Neuronal damage is induced by antibodies usually related to the primary pathology. Treatment targets the etiology and also employs steroids, plasmapheresis, immunosuppressive agents, or other anti-inflammatory therapies. Children with opsoclonus myoclonus resulting from a neuroblastoma often retain neurological sequelae. Adult cases of opsoclonus myoclonus with idiopathic or infectious etiologies have a more favorable prognosis than those with neoplastic origins.
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PMID:Opsoclonus myoclonus. 2224 16

A previously healthy 9-year-old girl presented with a 10-day history of slowly progressive unsteadiness, slurred speech, and behavior change. On examination there was cerebellar ataxia and dysarthria, excessive blinking, subtle perioral myoclonus, and labile mood. The finding of oligoclonal bands in the cerebrospinal fluid prompted paraneoplastic serological evaluation and search for an occult neural crest tumor. Antineuronal nuclear autoantibody type 1 (anti-Hu) and voltage-gated potassium channel complex antibodies were detected in serum. Metaiodobenzylguanidine scan and computed tomography scan of the abdomen showed a localized abdominal mass in the region of the porta hepatis. A diagnosis of occult neuroblastoma was made. Resection of the stage 1 neuroblastoma and treatment with pulsed corticosteroids resulted in resolution of all symptoms and signs. Excessive blinking has rarely been described with neuroblastoma, and, when it is not an isolated finding, it may be a useful clue to this paraneoplastic syndrome. Although voltage-gated potassium channel complex autoimmunity has not been described previously in the setting of neuroblastoma, it is associated with a spectrum of paraneoplastic neurologic manifestations in adults, including peripheral nerve hyperexcitability disorders.
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PMID:Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies. 2252 75

We analyzed the features of opsoclonus syndrome as a manifestation of post viral encephalopathy in 3 patients (one child and 2 adults). This is the first report of opsoclonus-myoclonus syndrome in the Arabian Peninsula. Symptoms appeared a few days after a viral-like illness in all patients. We excluded the possibility of paraneoplastic syndrome as investigations were carried out and follow-up did not reveal malignancy. One patient received a relatively long duration steroid treatment, another one received steroids in pulsed therapy, the third did not receive any specific treatment. The outcome was very good with improvement except in one who had severe sequelae.
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PMID:Non paraneoplastic opsoclonus - myoclonus syndrome. 2364 30


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