FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2736005205> ?p ?o ?g. }

• W2736005205 endingPage "888" @default.
• W2736005205 startingPage "887" @default.
• W2736005205 abstract "View Supplementary Video 1 Pseudochoreoathetosis refers to abnormal writhing movements of distal portions of the limbs occurring in association with impaired processing of proprioceptive information that alters the integration of sensory-motor signals.1 Any lesion along proprioceptive pathways from muscle spindles and joint receptors to primary somatosensory cortex may produce pseudochoreoathetosis.1 Nevertheless, pseudochoreoathetosis is most often seen in peripheral neuropathies and in dorsal column or dorsal root disorders, and it may occur in association with onconeural antibodies.1 In these disorders, pseudochoreoathetosis is frequently accompanied by gait ataxia.1 Here, we report a patient with pseudochoreoathetosis that occurred several years after the onset of Hu anti-neuronal nuclear antibody (anti-Hu) sensory neuronopathy and improved after plasma exchange (PEX). A 65-year-old woman presented with insidious onset of dysphagia, lower-limb dysesthesia, and progressive gait ataxia. She had a negative family history for neurologic disorders. Her personal medical history showed hypertension, sensory hypoacusia, esophageal achalasia, and recurrent intestinal pseudo-obstructions. At the time of the first neurologic examination (2003), the patient showed generalized areflexia, marked proprioceptive impairment, superficial sensory loss with a glove-and-stocking distribution, and preserved muscle strength. Romberg's sign was present, and the patient displayed broad-based gait and dysmetria of the 4 limbs. A nerve-conduction velocity study demonstrated severe and generalized reduction of sensory nerve action potentials associated with slight reduction of sensory nerve and normal motor nerve conduction velocity. Cerebrospinal fluid examination revealed slightly increased protein concentration. Brain magnetic resonance imaging (MRI) was normal. Dorsal column hyperintensity was found at cervical spinal cord MRI. Complete blood count, erythrocyte sedimentation rate, fasting blood glucose, hemoglobin A1c, vitamin B12, thyroid function, hepatic and kidney panel, urine analysis, serum protein electrophoresis, immunofixation, and the search for cryoglobulins were normal. Anti-human immunodeficiency virus, anti-hepatitis B virus, anti-hepatitis C virus, and venereal disease research laboratory antibodies were negative. Anti-nucleus, anti-neutrophil cytoplasmic, anti-myelin-associated glycoprotein, anti-ganglioside and anti-sulfatide antibodies were negative. A remarkably high titer of serum anti-Hu antibody (++++) was observed, indicating the diagnosis of subacute sensory neuronopathy/Denny Brown syndrome. Despite such finding, serum neoplastic markers were negative, and whole-body computed tomography and [18F]-fluorodeoxyglucose positron emission tomography scans failed to reveal primary or metastatic neoplasms. Along the 8-year follow-up, serum anti-Hu antibodies were repeatedly confirmed, and imaging studies remained negative. Based on the presence of anti-Hu antibodies, in October 2010, the patient underwent PEX with partial improvement of symptoms. PEX was chronically maintained for almost 6 years and was discontinued in January 2016 because of hypoalbuminemia. Approximately 3 months after discontinuing PEX, for the first time ever, the patient developed involuntary, mostly slow, distal movements of the legs, which were worsened by eye closure and antigravity posture, with the features of pseudochoreoathetosis (Video Segment 1; see online supporting information). Electrophysiology revealed loss of sensory nerve action potentials in median, ulnar, radial, and sural nerves but normal motor conduction velocity, action potential amplitude, and distal latency, in keeping with a severe sensory neuronopathy. On this basis, PEX was restarted. Involuntary movements markedly reduced 3 weeks after re-initiation of PEX (Video Segment 2: see online supporting information). PEX was chronically maintained for the next 12 months, with persistent reduction of pseudochoreoathetosis. However, the electrophysiological control performed 6 months after PEX restart did not show any change in nerve-conduction data. Anti-Hu antibodies (also referred to as type-1 anti-neuronal nuclear antibodies or ANNA-1) directed toward intracellular neural antigens (HuD antigen) are a marker of paraneoplastic sensory neuronopathy and also have frequently been reported in paraneoplastic encephalomyelitis (PEM).2 Sensory neuronopathy, frequently associated with autonomic involvement,2 occurs in approximately 54% of patients with anti-Hu–associated PEM. Pseudochoreoathetosis has been reported previously by others as the initial symptom in anti-Hu neuronopathy.3 Small cell lung carcinoma is the tumor most frequently associated with PEM.2 However, in approximately 16% of 200 patients with anti-Hu–related PEM, the diagnostic work-up did not reveal any cancer.4 It is worth noting that 5 patients in that sample, all diagnosed with sensory neuronopathy, improved after immunomodulating treatment.4 Cytotoxic T cells are likely the main effector of the immune response in anti-Hu neuronopathy.5 However, deposits of auto-antibodies around sensory axons have been observed in dorsal root ganglia of patients affected by anti-Hu polyneuropathy,5 and elution of immunoglobulin G from tissue lesions has revealed that they contain anti-Hu antibodies.6 Our case is characterized by peculiar features: First, anti-Hu antibodies were present despite the long-lasting negativity of both serum neoplastic markers and imaging studies in search of neoplasms. Moreover, symptoms of pseudochoreoathetosis developed 13 years after the onset of sensory neuronopathy, after the interruption of PEX. Re-initiation of PEX was followed by a marked improvement of pseudochoreoathetosis without any change of neurophysiological data. These features most likely suggest progression of deafferentation with pathologic involvement of dorsal root ganglia occurring as a consequence of the pause in immunotherapy and a rise in T cell-mediated neurotoxicity. Further studies are advised, however, to define the mechanisms that may trigger delayed symptoms in anti-Hu neuronopathies. 1. Research Project: A. Conception, B. Organization, C. Execution; 2. Statistical Analysis: A. Design, B. Execution, C. Review and Critique; 3. Manuscript Preparation: A. Writing the First Draft, B. Review and Critique. T.D.S.: 1A, 1B, 1C, 3A, 3B S.M.: 1A, 1C, 3B G.A.: 1A, 1B, 3B F.E.P.: 1A, 1B, 3B Ethical Compliance Statement: We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. Funding Sources and Conflict of Interest: The authors report no sources of funding and no conflicts of interest. Financial Disclosures for the previous 12 months: The authors report no sources of funding and no conflicts of interest. A video accompanying this article is available in the supporting information here. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article." @default.
• W2736005205 created "2017-07-21" @default.
• W2736005205 creator A5015450048 @default.
• W2736005205 creator A5035489373 @default.
• W2736005205 creator A5065424394 @default.
• W2736005205 creator A5076842090 @default.
• W2736005205 date "2017-09-28" @default.
• W2736005205 modified "2023-09-26" @default.
• W2736005205 title "Plasma Exchange-responsive Tardive Delayed Pseudochoreoathetosis in a Patient with Anti-Hu Neuronopathy" @default.
• W2736005205 cites W2003555874 @default.
• W2736005205 cites W2060387369 @default.
• W2736005205 cites W2076253992 @default.
• W2736005205 cites W2090417766 @default.
• W2736005205 cites W2124472369 @default.
• W2736005205 cites W2184239107 @default.
• W2736005205 doi "https://doi.org/10.1002/mdc3.12524" @default.
• W2736005205 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6353347" @default.
• W2736005205 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30713981" @default.
• W2736005205 hasPublicationYear "2017" @default.
• W2736005205 type Work @default.
• W2736005205 sameAs 2736005205 @default.
• W2736005205 citedByCount "0" @default.
• W2736005205 crossrefType "journal-article" @default.
• W2736005205 hasAuthorship W2736005205A5015450048 @default.
• W2736005205 hasAuthorship W2736005205A5035489373 @default.
• W2736005205 hasAuthorship W2736005205A5065424394 @default.
• W2736005205 hasAuthorship W2736005205A5076842090 @default.
• W2736005205 hasBestOaLocation W27360052051 @default.
• W2736005205 hasConcept C15744967 @default.
• W2736005205 hasConcept C169760540 @default.
• W2736005205 hasConcept C171790689 @default.
• W2736005205 hasConcept C172497186 @default.
• W2736005205 hasConcept C2777154897 @default.
• W2736005205 hasConcept C2780168130 @default.
• W2736005205 hasConcept C2780906641 @default.
• W2736005205 hasConcept C2908670167 @default.
• W2736005205 hasConcept C42219234 @default.
• W2736005205 hasConcept C71924100 @default.
• W2736005205 hasConcept C94487597 @default.
• W2736005205 hasConceptScore W2736005205C15744967 @default.
• W2736005205 hasConceptScore W2736005205C169760540 @default.
• W2736005205 hasConceptScore W2736005205C171790689 @default.
• W2736005205 hasConceptScore W2736005205C172497186 @default.
• W2736005205 hasConceptScore W2736005205C2777154897 @default.
• W2736005205 hasConceptScore W2736005205C2780168130 @default.
• W2736005205 hasConceptScore W2736005205C2780906641 @default.
• W2736005205 hasConceptScore W2736005205C2908670167 @default.
• W2736005205 hasConceptScore W2736005205C42219234 @default.
• W2736005205 hasConceptScore W2736005205C71924100 @default.
• W2736005205 hasConceptScore W2736005205C94487597 @default.
• W2736005205 hasIssue "6" @default.
• W2736005205 hasLocation W27360052051 @default.
• W2736005205 hasLocation W27360052052 @default.
• W2736005205 hasLocation W27360052053 @default.
• W2736005205 hasLocation W27360052054 @default.
• W2736005205 hasOpenAccess W2736005205 @default.
• W2736005205 hasPrimaryLocation W27360052051 @default.
• W2736005205 hasRelatedWork W1963569579 @default.
• W2736005205 hasRelatedWork W2036764555 @default.
• W2736005205 hasRelatedWork W2044418492 @default.
• W2736005205 hasRelatedWork W2074580339 @default.
• W2736005205 hasRelatedWork W2094856773 @default.
• W2736005205 hasRelatedWork W2139294439 @default.
• W2736005205 hasRelatedWork W2734586193 @default.
• W2736005205 hasRelatedWork W3128622980 @default.
• W2736005205 hasRelatedWork W32370873 @default.
• W2736005205 hasRelatedWork W4242713081 @default.
• W2736005205 hasVolume "4" @default.
• W2736005205 isParatext "false" @default.
• W2736005205 isRetracted "false" @default.
• W2736005205 magId "2736005205" @default.
• W2736005205 workType "article" @default.