المؤلفون: Corrado Zenesini, Rocco Liguori, Vincenzo Donadio, Matteo Paolucci, Maria Pia Giannoccaro, Vitantonio Di Stasi, Patrizia Avoni

المساهمون: Giannoccaro M.P., Paolucci M., Zenesini C., Di Stasi V., Donadio V., Avoni P., Liguori R.

المصدر: Neurology. 95:e1800-e1806

مصطلحات موضوعية: IPT, medicine.medical_specialty, Ocular myasthenia, Single fiber emg, ice pack test, ocular myasthenia, Diagnostic accuracy, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Ptosis, Internal medicine, parasitic diseases, 0502 economics and business, medicine, In patient, Receiver operating characteristic, business.industry, 05 social sciences, medicine.disease, Predictive value, Confidence interval, single fiber EMG, 050211 marketing, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery

الوصف: ObjectiveTo compare the diagnostic accuracy of ice pack test (IPT) and single-fiber EMG (SF-EMG) in patients with suspected ocular myasthenia (OM) presenting with ptosis.MethodsWe studied consecutive patients referred for the clinical suspicion of OM. Patients underwent IPT and stimulated SF-EMG on the orbicularis oculi muscle. Receiver operating characteristic curve analysis was performed to determine the accuracy of IPT, SF-EMG, and their combination.ResultsWe included 155 patients, 102 with OM and 53 with other diagnosis (OD). The IPT had a sensitivity of 86% (95% confidence interval [CI] 79–93) and a specificity of 79% (95% CI 68–90). SF-EMG showed a sensitivity of 94% (95% CI 89–98) and a specificity of 79% (95% CI 68–90). Overall, IPT and SF-EMG showed discordant results in 30 cases, 16 OM and 14 OD. The combination of IPT and SF-EMG, using the positivity of at least one test for OM diagnosis, increased the sensitivity to 98% (95% CI 95–100), reducing the specificity to 66% (95% CI 53–78), whereas using the positivity of both tests, we obtained a sensitivity of 82% (95% CI 75–90) and a specificity of 92% (95% CI 85–99). The negativity of both tests had a 94% (95% CI 87–100) negative predictive value. Comparison of the areas under the curve showed no differences in the diagnostic accuracy of IPT, SF-EMG, and their combinations.ConclusionsIPT and SF-EMG have similar diagnostic accuracy in patients with OM presenting with ptosis. The negativity of both tests strongly suggests another diagnosis.Classification of evidenceThis study provides Class I evidence that both the IPT and SF-EMG accurately identify patients with OM.

وصف الملف: ELETTRONICO

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c9f2e0ecda2b52d0f3c1da248bcac784Test
https://doi.org/10.1212/wnl.0000000000010619Test

المؤلفون: Rocco Liguori, Rossella Infante, Giovanni Rizzo, Alex Incensi, Vincenzo Donadio

المساهمون: Infante R., Incensi A., Rizzo G., Donadio V., Liguori R.

المصدر: Neurology. 93:411.2-412

مصطلحات موضوعية: Dystonia, Pathology, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Parkinsonism, Language impairment, corticobasal syndrome, Limb apraxia, Lewy body disease, medicine.disease, Dysphagia, nervous system diseases, 03 medical and health sciences, 0302 clinical medicine, Parietal cortical atrophy, Skin biopsy, Medicine, 030212 general & internal medicine, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery

الوصف: We read with great interest the article by Kasanuki et al.1 that described 11 cases of pathologically confirmed diffuse Lewy body disease, antemortem clinically manifesting as corticobasal syndrome (CBS), in line with previous findings. We recently evaluated a 70-year-old woman referred to our center with possible CBS according to clinical criteria.2 The patient presented with a 3-year history of limb bradykinesia and dystonia prevalent in the right side associated with limb apraxia. Brain MRI detected bilateral parietal cortical atrophy, mainly in the left side, and 18F-fluorodeoxyglucose PET showed left parietal hypometabolism. Additional features suggested an “atypical” atypical parkinsonism3: a slow parkinsonism progression, moderate improvement of bradykinesia with levodopa therapy, and a 10-year history of memory and language impairment associated with mild dysphagia and postural instability. The patient underwent skin biopsy, unveiling abnormal deposition of phosphorylated α-synuclein (p-syn) in skin nerves, unexpected in CBS.

وصف الملف: STAMPA

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6de32c59e97d7bc1b0dbf44ca281e210Test
https://doi.org/10.1212/wnl.0000000000008018Test

المؤلفون: Masen Abdel Jaber, Alex Incensi, Vincenzo Donadio, Pietro Cortelli, Agostino Baruzzi, Rocco Liguori, Maria Pia Giannoccaro

المساهمون: V. Donadio, A. Incensi, P. Cortelli, M. P. Giannoccaro, M. A. Jaber, A. Baruzzi, R. Liguori

المصدر: Neurology. 80:725-732

مصطلحات موضوعية: Male, metabolism/pathology, Biological Marker, Pathology, medicine.medical_specialty, analysis, diagnosis, Skin, Shy-Drager Syndrome, Diagnosis, Differential, Pathogenesis, Adrenergic Fiber, diagnosis, Shy-Drager Syndrome, analysis, Diagnosi, Pure Autonomic Failure, medicine, Humans, Pure autonomic failure, Skin, medicine.diagnostic_test, business.industry, Microneurography, Middle Aged, innervation/pathology, alpha-Synuclein, medicine.disease, Immunohistochemistry, Peripheral, Differential, Female, Humans, Immunohistochemistry, Male, Middle Aged, Pure Autonomic Failure, Peripheral neuropathy, Skin biopsy, alpha-Synuclein, Biomarker (medicine), Cholinergic, Female, Neurology (clinical), Adrenergic Fibers, business, Biomarkers

الوصف: Objective: This study aimed to test whether peripheral α-synuclein staining might be useful for pure autonomic failure (PAF) diagnosis, helping to differentiate degenerative from acquired peripheral autonomic neuropathy. Methods: We studied 21 patients with chronic peripheral autonomic neuropathy showing sympathetic and parasympathetic involvement as confirmed by cardiovascular reflexes and microneurography from the peroneal nerve. Twelve patients showed a specific cause of neuropathy (acquired autonomic neuropathy) whereas 9 had no specific acquired causes fulfilling the diagnostic criteria for PAF. Fifteen matched healthy subjects served as controls. Subjects underwent skin biopsy from thigh and leg to study skin innervation and phosphorylated α-synuclein deposits in the peripheral axons. Results: Somatic and autonomic skin innervations were significantly decreased in patients with peripheral autonomic neuropathy compared to controls. No differences were found between acquired autonomic neuropathy and PAF. The deposits of α-synuclein were not found in controls but served to distinguish acquired from degenerative autonomic peripheral neuropathy: all patients with PAF showed α-synuclein deposits, which were absent in patients with acquired autonomic neuropathy. Colocalization study disclosed α-synuclein neuritic inclusions in the postganglionic sympathetic adrenergic and cholinergic nerve fibers. Conclusions: Our study demonstrated that a search for neuritic inclusions of phosphorylated α-synuclein in the skin sympathetic nerve fibers could provide a sensitive in vivo biomarker for degenerative peripheral autonomic neuropathy and may shed more light on the pathogenesis of PAF.

وصف الملف: ELETTRONICO

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::35c9391f54e1c38fc0454f2db7719f84Test
https://doi.org/10.1212/wnl.0b013e3182825127Test