Objective: To compare cardiac sympathetic adrenergic nerve activity in patients with narcolepsy type 1 (NT1) and controls using I-MIBG myocardial scintigraphy, and to determine the clinical and neurophysiological variables associated with I-MIBG scintigraphy results in NT1.
Methods: Fifty-six NT1 patients and 91 controls without neurological diseases underwent a cardiac scintigraphy. MIBG uptake was quantified by delayed heart/mediastinum (H/M) ratio. Clinical, neurophysiological and biological determinants of a low H/M were assessed in NT1.
Results: MIBG uptake did not differ between NT1 and controls in crude and adjusted associations. Five patients had low MIBG uptake (<1.42, first decile of controls), often with advanced age, cardiovascular (CV) diseases, stimulants intake, and REM sleep behavior disorder. Patients with H/M <1.62 (lowest tertile) were older, with higher BMI, microarousal index and CV comorbidities. A three-fold increase of phasic/tonic REM sleep motor activities was found in those patients, confirmed in a subanalysis of 40 drug-free patients. No association was found with CSF hypocretin levels.
Conclusion: A direct measure of the heart adrenergic nerve activity revealed no sympathetic denervation in NT1.
Significance: Our results indicate normal cardiac sympathetic innervation in NT1. However, few patients with low MIBG uptake also presented CV comorbidities and REM sleep motor deregulation, potentially at high CV risk, requiring a careful follow-up.
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http://dx.doi.org/10.1016/j.clinph.2018.11.014 | DOI Listing |
Diagnostics (Basel)
December 2024
Department of Medicine I, University Hospital Munich, Ludwig-Maximilians-University, 81377 Munich, Germany.
: Iodo-metaiodobenzylguanidine single photon emission computed tomography/computed tomography (I-MIBG SPECT/CT) is used to evaluate the cardiac sympathetic nervous system in cardiac diseases such as arrhythmogenic right ventricular cardiomyopathy (ARVC) and α-synucleinopathies such as Parkinson's diseases. A common feature of these diseases is denervation. We aimed to compare quantitative and semi-quantitative cardiac sympathetic innervation using I-MIBG imaging of ARVC and α-synucleinopathies.
View Article and Find Full Text PDFClin Nucl Med
January 2025
From the Department of Nuclear Medicine, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
A 7-year-old boy with high-risk neuroblastoma underwent 123I-MIBG SPECT/CT to evaluate the therapy response. The scan revealed abnormal 123I-MIBG uptake in the left basal ganglion, indicating the possibility of brain metastasis. Subsequent contrast-enhanced brain MRI, however, did not show any abnormal signal intensity in the left basal ganglion.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
December 2024
One Health Research Group, Universidad de las Americas, Quito, Ecuador.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and -signaling pathway variants.
View Article and Find Full Text PDFJ Neurol
December 2024
Department of Neurology, Ajou University School of Medicine, Suwon, Republic of Korea.
Introduction: Recently, "body-first" and "brain-first" subtype in Parkinson's disease (PD) was proposed based on the propagation of α-synuclein. In isolated RBD considered as a premotor stage of body-first PD, α-synuclein was supposed to originate in the enteric nervous system and spreads via autonomic nervous system. Therefore, we hypothesized that body-first PD is more likely to have a delayed gastric emptying time and reduced cardiac sympathetic denervation.
View Article and Find Full Text PDFClin Park Relat Disord
November 2024
Department of Neurology, Toho University Faculty of Medicine, Tokyo, Japan.
Introduction: Cardiac sympathetic denervation is specific to Lewy body disease (LBD). In Parkinson's disease (PD), sympathetic denervation in the major salivary glands (parotid glands [PG] and submandibular glands [SMG]) has been demonstrated by I-metaiodobenzylguanidine (MIBG) scintigraphy. We compared sympathetic denervation in the MSG between PD, dementia with Lewy bodies (DLB), and progressive supranuclear palsy (PSP).
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