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Wilson's disease (WD) is an inherited disorder of copper metabolism in which pathological copper accumulation, mainly in the liver and the brain, leads to hepatic and/or neuropsychiatric signs and symptoms. Chelators and zinc salts can successfully induce negative copper balance in many patients; however, neurological deterioration may still be observed. This phenomenon can be divided into: (1) early 'paradoxical' neurological deterioration, which usually develops in the first 6 months of anti-copper treatment and may be commonly related to drug type, or (2) late neurological deterioration, which mostly occurs after 6 months of treatment and is often related either to non-compliance with treatment, overtreatment resulting in copper deficiency, or adverse drug reactions. Another explanation, especially for early neurological deterioration, is natural WD progression, which can be difficult to differentiate from drug-related deterioration, but usually leads to a worse outcome. There is still no consensus on how to define neurological deterioration in WD using scales or biomarkers, how to distinguish it from the natural disease progression, its risk factors, and optimal management. This narrative review, based on the current literature, aims to provide definitions, prevalence, pathological mechanisms and factors related to neurological deterioration, and also proposes schemes for diagnosis and treatment.
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http://dx.doi.org/10.24976/Discov.Med.202436183.61 | DOI Listing |
Front Neurosci
December 2024
Department of Neurology, Dongyang People's Hospital, Affiliated to Wenzhou Medical University, Dongyang, China.
Background And Aim: This study aimed to develop a predictive model for early neurological deterioration (END) in branch atheromatous disease (BAD) affecting the lenticulostriate artery (LSA) territory using machine learning. Additionally, it aimed to explore the underlying mechanisms of END occurrence in this context.
Methods: We conducted a retrospective analysis of consecutive ischemic stroke patients with BAD in the LSA territory admitted to Dongyang People's Hospital from January 1, 2018, to September 30, 2023.
Ear Nose Throat J
December 2024
Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, Jeonbuk National University, Jeonju, Republic of Korea.
Pituitary macroadenomas are benign neoplasms that can cause a range of symptoms due to a mass effect on surrounding structures. This case report describes a 51-year-old male presenting with progressive bilateral hearing impairment and significant deterioration in speech discrimination over several years. Examination revealed normal tympanic membranes and no neurological deficits, while pure tone audiometry indicated mild to moderate sensorineural hearing loss.
View Article and Find Full Text PDFJ Feline Med Surg
December 2024
Tierklinik Hofheim, Hofheim, Germany.
Objectives: The aim of this study was to describe the safety and effectiveness of thoracic or lumbar partial lateral corpectomy (PLC) in cats with spinal cord compression due to intervertebral disc herniation (IVH).
Methods: A retrospective study was conducted of 12 client-owned cats from two academic and one private referral veterinary centres. Cross-sectional imaging was available in 12 cats for evaluation of disc herniation.
Brain Spine
November 2024
Neurosurgical Department, "KAT" General Hospital of Athens, Greece.
Introduction: Klippel-Trenaunay Syndrome (KTS) is a rare congenital condition characterized by vascular malformations, bone abnormalities, and limb overgrowth. The genetic basis of KTS is not fully understood, and the diagnosis relies on clinical features. Its clinical spectrum includes several neurosurgical diagnoses, such as cavernous hemangiomas, arteriovenous fistulas, and Chiari I malformation.
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