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A pathophysiological biomarker combination separates Lewy body from non-Lewy body neurogenic orthostatic hypotension ​.

Clin Auton Res

June 2024

Autonomic Medicine Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive MSC-1620, Building 10 Room 8N260, Bethesda, MD, 20892-1620, USA.

Purpose: Neurogenic orthostatic hypotension (nOH) results from deficient reflexive delivery of norepinephrine to cardiovascular receptors in response to decreased cardiac venous return. Lewy body (LB) forms of nOH are characterized by low F-dopamine-derived radioactivity (a measure of cardiac noradrenergic deficiency), olfactory dysfunction by the University of Pennsylvania Smell Identification Test (UPSIT), and increased deposition of alpha-synuclein (α-syn) in dermal sympathetic noradrenergic nerves by the α-syn-tyrosine hydroxylase (TH) colocalization index. This observational, cross-sectional study explored whether combinations of these biomarkers specifically identify LB forms of nOH.

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Intra-neuronal alpha-synuclein deposition is related to cardiac noradrenergic deficiency and olfactory dysfunction in neurogenic orthostatic hypotension.

Res Sq

March 2024

Autonomic Medicine Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

Purpose: Neurogenic orthostatic hypotension (nOH) results from deficient reflexive delivery of norepinephrine to cardiovascular receptors in response to decreased cardiac venous return. Lewy body (LB) forms of nOH entail low F-dopamine-derived radioactivity (a measure of cardiac noradrenergic deficiency), olfactory dysfunction by the University of Pennsylvania Smell Identification Test (UPSIT), and increased deposition of alpha-synuclein (ɑ-syn) in dermal sympathetic noradrenergic nerves by the ɑ-syn-tyrosine hydroxylase (TH) colocalization index. This observational, cross-sectional study explored whether combinations of these biomarkers specifically identify LB forms of nOH.

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Dysautonomia, Hypermobility Spectrum Disorders and Mast Cell Activation Syndrome as Migraine Comorbidities.

Curr Neurol Neurosci Rep

November 2023

Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, 955 Main Street, Buffalo, NY, 14203, USA.

Purpose Of Review: Dysautonomia refers to the dysfunction of the autonomic nervous system and encompasses a wide variety of autonomic symptoms and disorders. The most common autonomic disorders are postural orthostatic tachycardia syndrome (POTS), neurocardiogenic syncope (NCS), and orthostatic hypotension (OH), which may be encountered in clinical practice as part of a triad of dysautonomia, hypermobility spectrum disorders (HSD), and mast cell activation syndrome (MCAS). Migraine is one of the most common comorbidities of POTS, HSD, and MCAS; conversely, these conditions are also prevalent in patients with migraine, especially in those with multiple systemic symptoms, such as chronic dizziness, lightheadedness, orthostatic intolerance, joint pain, and allergic symptoms.

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Accurate and timely diagnosis of posterior circulation ischemic stroke is a challenge for emergency neurology clinicians, even MRI scan which is believed to be sensitive to acute ischemic lesions may be negative. It is particularly important to obtain the typical or characteristic symptoms and signs of the patients through comprehensive physical examination. We report a case of posterior inferior cerebellar artery (PICA) territory infarction with "episodic postural diplopia" as the initial symptom, hoping that clinicians notice the vertical diplopia caused by the disfunction of otolith gravity conduction pathway, which is characterized by the degree of diplopia being affected by postural changes.

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Orthostatic Myoclonic Jerks in a Case of Hashimoto's Encephalopathy.

J Neurosci Rural Pract

July 2022

Department of Internal Medicine, Fortis Hospital, Mohali, Punjab, India.

Hashimoto's encephalopathy (HE) is an uncommon syndrome with the characteristic triad of positive antithyroid antibodies (most commonly antibodies to thyroid peroxidase), response to steroids, and clinical picture presenting either as stroke-like pattern of focal neurological deficit or slowly progressive cognitive impairment. Myoclonus or tremors, seizures, and psychosis are other associated features which can be seen in HE. Herein, we report a girl with an uncommon presentation of orthostatic axial and myoclonic jerks in bilateral lower limbs in a case of HE.

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