Background: Damage of the optic nerve is the major complication of idiopathic intracranial hypertension. A biomarker indicative for optic nerve damage would help identifying high-risk patients requiring surgical procedures. Here, we studied the potential of cerebrospinal fluid neurofilament to predict idiopathic intracranial hypertension-induced optic nerve damage.
Methods: In two centers, serum and cerebrospinal fluid of 61 patients with clinically suspected idiopathic intracranial hypertension were prospectively collected. Neurofilament concentrations were measured and related to ophthalmological assessment.
Results: The average cerebrospinal fluid neurofilament concentration in patients with moderate and severe papilledema was increased compared to patients with minor and no papilledema (1755 ± 3507 pg/ml vs. 244 ± 102 pg/ml; < 0.001). Cerebrospinal fluid neurofilament concentrations correlated with the maximal lumbar puncture opening pressure (r = 0.67, < 0.001). In patients fulfilling the Friedman criteria for idiopathic intracranial hypertension with or without papilledema (n = 35), development of bilateral visual field defects and bilateral atrophy of the optic nerve were associated with increased average age-adjusted cerebrospinal fluid neurofilament concentrations. At last follow-up (n = 30), 8/13 of patients with increased, but only 3/17 with normal, cerebrospinal fluid neurofilament had developed bilateral visual field defects and/or bilateral optic nerve atrophy resulting in a sensitivity of 72.7% and a specificity of 73.7% of cerebrospinal fluid neurofilament to detect permanent optic nerve damage.
Conclusions: Cerebrospinal fluid neurofilament is a putative biomarker for optical nerve damage in idiopathic intracranial hypertension.
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http://dx.doi.org/10.1177/0333102420944866 | DOI Listing |
Tomography
December 2024
Department of Radiology, Nemours Children's Health, 1600 Rockland Rd., Wilmington, DE 19803, USA.
Evaluating altered mental status and suspected meningeal disorders in children often begins with imaging, typically before a lumbar puncture. The challenge is that meningeal enhancement is a common finding across a range of pathologies, making diagnosis complex. This review proposes a categorization of meningeal diseases based on their predominant imaging characteristics.
View Article and Find Full Text PDFJ Neuroophthalmol
December 2024
College of Medicine (JM, AGL), Texas A&M University, Houston, Texas; Department of Ophthalmology (SAA, OAD, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Department of Ophthalmology (SAA), The University of Jordan, Amman, Jordan; Department of Ophthalmology (OAD), Hashemite University, Amman, Jordan; Department of Ophthalmology (AL, AGL), Cullen Eye Institute, Baylor College of Medicine, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas MD Anderson Cancer Center, Houston, Texas; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa.
Background: The prevalence of idiopathic intracranial hypertension (IIH) is rising with the global obesity epidemic. Carbonic anhydrase inhibitors (CAIs), such as acetazolamide, have been shown to be effective in IIH but can also lead to kidney stone formation. This study evaluates the risk of kidney stone development in patients with IIH treated with CAIs.
View Article and Find Full Text PDFOchsner J
January 2024
The University of Queensland Medical School, Ochsner Clinical School, New Orleans, LA.
Spinal cord sarcoidosis, an uncommon manifestation of neurosarcoidosis, presents diagnostic and therapeutic challenges because the condition is rare and has diverse clinical manifestations that can mimic other conditions such as multiple sclerosis and neuromyelitis optica spectrum disorder. A middle-aged African American female with a history of idiopathic intracranial hypertension and hydrocephalus with ventriculoperitoneal shunt presented with progressive, predominantly left-sided gait instability, weakness, and paresthesia. Cerebrospinal fluid showed lymphocytosis, red blood cells, elevated oligoclonal bands, and elevated kappa free light chains, concerning for multiple sclerosis.
View Article and Find Full Text PDFFront Neurol
December 2024
Neuromuscular Imaging Research Lab, The Kolling Institute, North Sydney Local Health District, St Leonards, NSW, Australia.
Individuals with joint hypermobility and the Ehlers-Danlos Syndromes (EDS) are disproportionately affected by neuraxial dysfunction and Central Nervous System (CNS) disorders: such as Spontaneous Intracranial Hypotension (SIH) due to spinal cerebrospinal fluid (CSF) leaks, Upper Cervical Instability (UCI; including craniocervical or atlantoaxial instability (CCI/AAI)), Occult Tethered Cord Syndrome (TCS), Chiari Malformation (CM) and Idiopathic Intracranial Hypertension (IIH). The neuraxis comprises the parts of the nervous system (brain, nerves, spinal cord) along the craniospinal axis of the body. Neuraxial tissue includes all tissue structures that comprise, support, sheath, and connect along the neuraxis and peripheral nerves.
View Article and Find Full Text PDFLancet Rheumatol
December 2024
Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA; Pharming Healthcare, Warren, NJ, USA. Electronic address:
Background: Systemic juvenile idiopathic arthritis-related lung disease (sJIA-LD) is a severe complication in patients with treatment-refractory systemic juvenile idiopathic arthritis (sJIA). The objective of this study was to evaluate the effect of allogeneic haematopoietic stem-cell transplantation (HSCT) in a cohort of children with sJIA-LD.
Methods: This international, retrospective cohort study was performed in nine hospitals across the USA and Europe in children with sJIA-LD who had received allogeneic HSCT.
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