Publications by authors named "Sung-Ju Hsueh"

Controlling hypertension has become an important issue in the elderly population in whom neurological comorbidities are highly prevalent. Most of the large-scale trials focusing on hypertension management in older populations have excluded patients with comorbid neurological disorders. However, this population requires special considerations, as the benefits of antihypertensive agents are mostly uncertain and there is a higher risk of adverse events.

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Background: To investigate the peripheral nervous system involvement in  sialidosis with typical features of myoclonus, seizure, and giant waves in somatosensory evoked potentials suggesting hyperexcitability in the central nervous system.

Methods: The clinical presentation of patients with genetically confirmed sialidosis was recorded. Neurophysiological studies, including nerve conduction studies (NCSs), F-wave studies, and needle electromyography (EMG), were performed on these patients.

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Objective: Obstructive sleep apnea (OSA) is a common risk factor for stroke, and dysphagia and pneumonia are both well-known complications of stroke. The development of these conditions is related to the oropharyngeal structures. We investigated whether specific structural features of the tongue may lead to the development of these complications.

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Background And Objectives: Charcot-Marie-Tooth disease (CMT) is a syndrome of a hereditary neurodegenerative condition affecting the peripheral nervous system and is a single gene disorder. Deep phenotyping coupled with advanced genetic techniques is critical in discovering new genetic defects of rare genetic disorders such as CMT.

Methods: We applied multidisciplinary investigations to examine the neurophysiology and nerve pathology in a family that fulfilled the diagnosis of CMT2.

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Article Synopsis
  • The study investigates the link between brain imaging signatures and neurological deficits in patients with amyotrophic lateral sclerosis (ALS).
  • The researchers used MRI to analyze gray matter volume and various white matter properties in 39 ALS patients and 32 control subjects.
  • Results showed that lower gray matter volume in a specific brain area correlated with muscle strength and nerve function, suggesting a concurrent impact on both upper and lower motor neurons in ALS.
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Multiple acyl-coenzyme A dehydrogenase deficiency (MADD) is a rare metabolic disorder with a dramatic clinical presentation. It was recently discovered that MADD may present at an advanced age. The clinical and laboratory data of an index patient and patients previously diagnosed at our institution were collected.

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Aim: Senior healthcare is challenging in remote areas, particularly in an economically disadvantaged population. This study examined the benefits of a combined healthcare system (Houston-Apollo model) in improvements of physical performance and medical care utilization of local older people.

Methods: People aged ≥65 years who participated in congregate meal services were recruited.

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Background And Purpose: Disease-modifying therapies provide new horizons for hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) to slow neuropathic progression. Initiating treatment at the earliest time requires biomarkers reflecting both small- and large-fiber degeneration in carriers.

Methods: This study included examinations of pathology (intraepidermal nerve fiber [IENF] density), physiology (nerve conduction studies, autonomic function test, and nerve excitability), and psychophysics (thermal thresholds) in carriers to compare to healthy controls and asymptomatic diabetic patients.

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Background/purpose: Endovascular thrombectomy (EVT) is effective in treating acute ischemic stroke associated with large vessel occlusion. Early recurrence of ischemic stroke (ERIS) after EVT, however, is a devastating event and could worsen the condition of patient. Current study aimed to investigate the prevalence and risk factors of ERIS after EVT.

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Background: Cleidocranial dysplasia (CCD) is a rare hereditary disorder that arises from heterozygous loss of function mutations in the runt-related transcription factor 2 (RUNX2) gene. As RUNX2 is mainly expressed in osteoblasts, CCD typically affects the skeletal and dental systems. Few studies have investigated RUNX2 mutation effects on non-skeletal systems.

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