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Ocular Oncology and Pathology Dec 2023Radiation optic neuropathy may be one side effect of ionizing radiation exposure to the eye found in a minority of patients. It is generally devastating for visual... (Review)
Review
BACKGROUND
Radiation optic neuropathy may be one side effect of ionizing radiation exposure to the eye found in a minority of patients. It is generally devastating for visual function and has been the subject of a small but growing literature with respect to its pathophysiology, treatment, and expected outcomes.
SUMMARY
Clinical features include optic disc edema, peripapillary hemorrhages, cotton wool spots, and hard exudates. Visual acuity is generally significantly reduced. Treatment has been attempted with outcomes that have not been assessed by randomized trials. Observation may be indicated in addition to treatment.
KEY MESSAGES
Radiation optic neuropathy is known to generally be devastating to vision though an uncommon side effect of radiation. Treatment has been attempted with mixed results.
PubMed: 38089180
DOI: 10.1159/000533426 -
Mayo Clinic Proceedings Jan 2024Parsonage-Turner syndrome and hereditary brachial plexus neuropathy (HBPN) present with indistinguishable attacks of rapid-onset severe shoulder and arm pain, disabling... (Review)
Review
Parsonage-Turner syndrome and hereditary brachial plexus neuropathy (HBPN) present with indistinguishable attacks of rapid-onset severe shoulder and arm pain, disabling weakness, and early muscle atrophy. Their combined incidence ranges from 3 to 100 in 100,000 persons per year. Dominant mutations of SEPT9 are the only known mutations responsible for HBPN. Parsonage and Turner termed the disorder "brachial neuralgic amyotrophy," highlighting neuropathic pain and muscle atrophy. Modern electrodiagnostic and imaging testing assists the diagnosis in distinction from mimicking disorders. Shoulder and upper limb nerves outside the brachial plexus are commonly affected including the phrenic nerve where diaphragm ultrasound improves diagnosis. Magnetic resonance imaging can show multifocal T2 nerve and muscle hyperintensities with nerve hourglass swellings and constrictions identifiable also by ultrasound. An inflammatory immune component is suggested by nerve biopsies and associated infectious, immunization, trauma, surgery, and childbirth triggers. High-dose pulsed steroids assist initial pain control; however, weakness and subsequent pain are not clearly responsive to steroids and instead benefit from time, physical therapy, and non-narcotic pain medications. Recurrent attacks in HBPN are common and prophylactic steroids or intravenous immunoglobulin may reduce surgical- or childbirth-induced attacks. Rehabilitation focusing on restoring functional scapular mechanics, energy conservation, contracture prevention, and pain management are critical. Lifetime residual pain and weakness are rare with most making dramatic functional recovery. Tendon transfers can be used when recovery does not occur after 18 months. Early neurolysis and nerve grafts are controversial. This review provides an update including new diagnostic tools, new associations, and new interventions crossing multiple medical disciplines.
Topics: Humans; Brachial Plexus Neuritis; Pain; Muscular Atrophy; Steroids
PubMed: 38176820
DOI: 10.1016/j.mayocp.2023.06.011 -
Journal of Ophthalmic & Vision Research 2023This review discusses the physical examination and diagnostic tests necessary to diagnose optic neuritis (ON) and provides an update on the approach and management of... (Review)
Review
This review discusses the physical examination and diagnostic tests necessary to diagnose optic neuritis (ON) and provides an update on the approach and management of acute ON. A comprehensive search of the PubMed database was conducted, limited to English-language journals and recent publications. A total of 160 articles were initially screened by title, of which 73 articles were included in the narrative synthesis. ON is an inflammation of the optic nerve that can be caused by different systemic and neurological disorders. It is commonly presented as a subacute unilateral painful vision loss, and based on its clinical manifestation, it can be classified as typical or atypical. Atypical ON is bilateral with visual acuity of worse than 20/200 or has an atypical demographic presentation for demyelination, such as a non-Caucasian male with optic disc swelling, for which neuromyelitis optica spectrum disorder (NMOSD), myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), or other etiologies should be considered. Steroids and immunosuppressants are the main treatment options for ON, and timely treatment initiation is critical to preventing irreversible vision loss, especially in atypical cases.
PubMed: 38250236
DOI: 10.18502/jovr.v18i4.14556 -
Eye (London, England) Aug 2023Optic neuritis (ON) is one of the most frequently seen neuro-ophthalmic causes of vision loss worldwide. Typical ON is often idiopathic or seen in patients with multiple... (Review)
Review
Optic neuritis (ON) is one of the most frequently seen neuro-ophthalmic causes of vision loss worldwide. Typical ON is often idiopathic or seen in patients with multiple sclerosis, which is well described in the landmark clinical trial, the Optic Neuritis Treatment Trial (ONTT). However, since the completion of the ONTT, there has been the discovery of aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies, which are biomarkers for neuromyelitis optica spectrum disorder (NMOSD) and MOG antibody-associated disease (MOGAD), respectively. These disorders are associated with atypical ON that was not well characterised in the ONTT. The severity, rate of recurrence and overall outcome differs in these two entities requiring prompt and accurate diagnosis and management. This review will summarise the characteristic neuro-ophthalmological signs in NMOSD and MOGAD, serological markers and radiographic findings, as well as acute and long-term therapies used for these disorders.
Topics: Humans; Neuromyelitis Optica; Myelin-Oligodendrocyte Glycoprotein; Aquaporin 4; Optic Neuritis; Multiple Sclerosis; Autoantibodies
PubMed: 36928226
DOI: 10.1038/s41433-023-02477-0 -
Arquivos de Neuro-psiquiatria Dec 2023Precision medicine has revolutionized the field of neuroimmunology, with innovative approaches that characterize diseases based on their biology, deeper understanding of... (Review)
Review
Precision medicine has revolutionized the field of neuroimmunology, with innovative approaches that characterize diseases based on their biology, deeper understanding of the factors leading to heterogeneity within the same disease, development of targeted therapies, and strategies to tailor therapies to each patient. This review explores the impact of precision medicine on various neuroimmunological conditions, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), optic neuritis, autoimmune encephalitis, and immune-mediated neuropathies. We discuss advances in disease subtyping, recognition of novel entities, promising biomarkers, and the development of more selective monoclonal antibodies and cutting-edge synthetic cell-based immunotherapies in neuroimmunological disorders. In addition, we analyze the challenges related to affordability and equity in the implementation of these emerging technologies, especially in situations with limited resources.
Topics: Humans; Precision Medicine; Immunotherapy; Antibodies, Monoclonal; Encephalitis; Hashimoto Disease; Neuromyelitis Optica; Optic Neuritis; Myelin-Oligodendrocyte Glycoprotein; Autoantibodies; Aquaporin 4
PubMed: 38157878
DOI: 10.1055/s-0043-1777752 -
Practical Neurology Mar 2024A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss...
A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss had occurred in the same eye 1 year before, with complete recovery after high-dose intravenous methylprednisolone. She had no light perception in the left eye with a swollen optic disc, but with a normal right optic disc. There were no systemic manifestations or infections. MR scan of the brain showed extensive enlargement and enhancement of the left optic nerve and optic chiasm. After excluding infections and autoimmune markers, a left optic nerve biopsy confirmed non-caseating granulomas, leading to a diagnosis of neurosarcoidosis.
Topics: Female; Humans; Middle Aged; Optic Nerve Diseases; Optic Nerve; Sarcoidosis; Central Nervous System Diseases; Neuritis; Blindness
PubMed: 37932041
DOI: 10.1136/pn-2023-003939 -
Rinsho Shinkeigaku = Clinical Neurology May 2024Leber's hereditary optic atrophy (LHON) is a genetic optic neuropathy that is more prevalent in young males but can occur from childhood to old age. The primary cause is... (Review)
Review
Leber's hereditary optic atrophy (LHON) is a genetic optic neuropathy that is more prevalent in young males but can occur from childhood to old age. The primary cause is mitochondrial genetic mutations, which are associated with dysfunction of mitochondrial electron transport chain complex I. It manifests as acute to subacute visual impairment, often starting unilaterally but progressing to involve both eyes within weeks to months. Visual loss is severe, with many patients having corrected visual acuity below 0.1. The differential diagnosis of optic neuritis is essential, and assessments such as pupillary light reflex, fluorescein fundus angiography, and magnetic resonance imaging can be useful for differentiation. LHON should be considered as one of the differential diagnoses for optic neuritis, and collaboration between neurologists and ophthalmologists is crucial for accurate diagnosis and appropriate treatment.
Topics: Optic Atrophy, Hereditary, Leber; Humans; Magnetic Resonance Imaging; Diagnosis, Differential; Male; Mutation; Optic Neuritis; Fluorescein Angiography; Female; Electron Transport Complex I; Adult; Mitochondria; Child
PubMed: 38644210
DOI: 10.5692/clinicalneurol.cn-001924 -
Autoimmunity Dec 2024Immune-mediated demyelinating polyneuropathies (IMDPs) are rare disorders in which dysregulated adaptive immune responses cause peripheral nerve demyelinating... (Review)
Review
Immune-mediated demyelinating polyneuropathies (IMDPs) are rare disorders in which dysregulated adaptive immune responses cause peripheral nerve demyelinating inflammation and axonal injury in susceptible individuals. Despite significant advances in understanding IMDP pathogenesis guided by patient data and representative mammalian models, specific therapies are lacking. Significant knowledge gaps in IMDP pathogenesis still exist, e.g. precise antigen(s) and mechanisms that initially trigger immune system activation and identification of large population disease susceptibility factors. The initial directional cues for antigen-specific effector or autoreactive leukocyte trafficking into peripheral nerves are also unknown. An overview of current animal models, with emphasis on the experimental autoimmune neuritis and spontaneous autoimmune peripheral polyneuropathy models, is provided. Insights on the initial directional cues for peripheral nerve tissue specific autoimmunity using a novel Major Histocompatibility Complex class II conditional knockout mouse strain are also discussed, suggesting an essential research tool to study cell- and time-dependent adaptive immunity in autoimmune diseases.
Topics: Animals; Disease Models, Animal; Humans; Mice; Neuritis, Autoimmune, Experimental; Mice, Knockout; Autoimmunity; Polyneuropathies; Adaptive Immunity; Histocompatibility Antigens Class II
PubMed: 38850571
DOI: 10.1080/08916934.2024.2361745 -
Archives de Pediatrie : Organe Officiel... Oct 2023Vertigo is common in childhood and adolescence. Although children and adults share common causes of vertigo, epidemiology changes with aging. For instance, ischemic... (Review)
Review
Vertigo is common in childhood and adolescence. Although children and adults share common causes of vertigo, epidemiology changes with aging. For instance, ischemic stroke is less frequent in childhood, whereas audiovestibular disorders, such as vestibular neuritis and the migraine equivalent, are the leading causes of vertigo. However, even if severe causes of vertigo are rare, clinicians must not miss them. In this review, we discuss the neurological causes of central vertigo in children. The diagnostic approaches reviewed here are focused on the search for signs of severity, such as an abrupt onset, infectious context, or intracranial hypertension, which may subsequently require brain imaging.
Topics: Adolescent; Child; Humans; Dizziness; Vertigo; Migraine Disorders
PubMed: 37537083
DOI: 10.1016/j.arcped.2023.07.001