4.Sentience and Moral Status: Comparative Study on Antonio Damasio's Feeling and Nicholas Humphrey's Sentience
Journal of the Korean Neurological Association 2025;43(1):61-68
This paper examines the neurobiology of sentience through a comparative analysis of theories proposed by Antonio Damasio and Nicholas Humphrey. Damasio's somatic marker hypothesis emphasizes the role of emotions and feelings in decision-making and consciousness, arguing that the brain's mapping of bodily states forms the basis of subjective experiences. Humphrey, conversely, approaches sentience from an evolutionary perspective, proposing that it evolved to enhance social cognition and cooperation. Both theories stress the importance of central nervous system development in the emergence of sentience, but differ in their views on its evolutionary timing and primary functions. We explore the potential applications of these theories in neuroethics and argue for continued research into sentience, emphasizing its potential impact on ethical and legal frameworks. We call for an interdisciplinary approach, integrating insights from neuroscience, philosophy, and ethics.
7.Acute Inflammatory Demyelinating Polyradiculoneuropathy in Patient with MOGAD
Journal of the Korean Neurological Association 2025;43(1):50-53
Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with central nervous system demyelination inclusive of optic neuritis and transverse myelitis. MOG antibody may rarely be associated with peripheral nervous system involvement. A 48-year-old woman presented with demyelinating polyneuropathy. She previously suffered from myelitis and optic neuritis and had diagnosed with MOG antibody-associated disease (MOGAD). Polyneuropathies, combined central and inflammatory neuropathies may be associated with MOGAD and may be immunotherapy responsive. Further studies were needed to elucidate the utility of MOG antibody testing in polyneuropathy.
8.A Genetically Confirmed Korean Case of CANVAS: Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome
Seung Hee LEE ; Hee-Jae JUNG ; Ji-Hee YOON ; Gu-Hwan KIM ; June-Young KOH ; Yuna LEE ; Young Seok JU ; Eun-Jae LEE ; Beom Hee LEE ; Young-Min LIM ; Hyunjin KIM
Journal of the Korean Neurological Association 2025;43(1):45-49
Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disorder caused by a biallelic expansion of pentanucleotide repeats in the RFC1 gene. Previous studies have reported up to 22% of patients with late-onset ataxia harbor this pathogenic repeat expansion. Despite its relatively high prevalence, CANVAS is often underdiagnosed because the disease is not well recognized and genetic testing is not performed in clinical practice. Here, we present a patient with characteristic clinical features, confirmed by genetic testing.
9.Intracranial Hypotension Associated with Meningocele
Hyunkyum CHO ; Yeo Jun YOON ; Min Kyung CHU
Journal of the Korean Neurological Association 2025;43(1):40-44
Intracranial hypotension (IH), often resulting from a cerebrospinal fluid (CSF) leak, is a notable cause of secondary headaches. Diagnosing IH through clinical assessment and neuroimaging can be challenging. Orthostatic headache (OH), characterized by neck stiffness, nausea, dizziness, phonophobia, and photophobia, is a key symptom of CSF leakage, which may stem from a variety of causes, including falling, surgery, CSF studies, or arachnoid diverticula. This study presents two OH patients with IH who were incidentally found to have an underlying meningocele.
10.Identification of de novo BSCL2 Asn88Ser Variant with Atypical Presentation of Distal Hereditary Motor Neuropathy Type 5: Clinical Challenge in Diagnosis of Motor Neuron Diseases
Hyunkee KIM ; Jung Hyun LEE ; Dongju WON ; Jong Rak CHOI ; Ha Young SHIN ; Seung Hyun KIM
Journal of the Korean Neurological Association 2025;43(1):35-39
Diagnosing amyotrophic lateral sclerosis (ALS) is challenging and requires distinguishing it from conditions like distal hereditary motor neuropathy type 5 (dHMN-V). A 21-year-old female initially diagnosed with ALS showed progressive upper limb weakness extending to the lower limbs. Trio exome sequencing revealed a de novo pathogenic Berardinelli-Seip congenital lipodystrophy 2 variant (c.263A>G, p.Asn88Ser), confirming dHMN-V. Minipolymyoclonus of small amplitudes in bilateral wrists and ankles was an atypical presentation. This case underscores the importance of considering dHMN-V as a differential diagnosis in ALS-like distal upper extremity weakness.
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