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1.
J Headache Pain ; 25(1): 70, 2024 May 06.
Artículo en Inglés | MEDLINE | ID: mdl-38711044

RESUMEN

BACKGROUND: Recently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs). METHODS: To provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria. RESULTS: We included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively. CONCLUSION: The standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy.


Asunto(s)
Imagen por Resonancia Magnética , Seudotumor Cerebral , Humanos , Femenino , Imagen por Resonancia Magnética/normas , Imagen por Resonancia Magnética/métodos , Masculino , Adulto , Seudotumor Cerebral/diagnóstico por imagen , Seudotumor Cerebral/diagnóstico , Estudios Retrospectivos , Sensibilidad y Especificidad , Persona de Mediana Edad , Papiledema/diagnóstico por imagen , Papiledema/diagnóstico
2.
Lasers Med Sci ; 39(1): 125, 2024 May 07.
Artículo en Inglés | MEDLINE | ID: mdl-38713436

RESUMEN

AIM: To detect if we can use the reduction in the optic disc vessel density as an indicator to the reduction in intracranial tension in patients with residual optic disc elevation after shunt surgery as fundus examination in those cases is not conclusive. PATIENTS AND METHOD: 21 patients with papilledema due to idiopathic intracranial hypertension underwent shunt surgery. Full neurological and ophthalmological assessments were done. The optic disc vessel density was measured before and 3 months after surgery. Patients were then divided according to the resolution of papilledema into 2 groups: 1) Residual disc elevation group. 2) Completely resolved disc edema group. CSF pressure was measured via lumber puncture preoperative for all patients and 3 months post-operative only for patients with residual disc edema. A comparison between both groups was done. RESULTS: There was a highly statistically significant difference between the two groups as regard the papilledema grade (the residual disc elevation group had a higher grade of papilledema) with P-value=0.000. As regard the difference in the preoperative optic disc vessel density between the two groups, there were statistically significant differences (optic disc vessel density was more in the residual disc elevation group). As regard the postoperative optic disc vessel density, there were non-significant differences between the two groups in whole image, inside disc and peripapillary vessel density (either in macro or microvasculature). CONCLUSION: The optic disc vessel density decreased with normal postoperative CSF opening pressure in cases with residual disc elevation postoperatively. Thus, in cases of residual optic disc swelling after shunt surgery, we can detect the reduction of intracranial pressure by the reduction in the optic disc vessel density which is a safe non-invasive technique. That may help in cases of residual disc elevation.


Asunto(s)
Disco Óptico , Papiledema , Seudotumor Cerebral , Humanos , Disco Óptico/irrigación sanguínea , Papiledema/etiología , Papiledema/cirugía , Femenino , Masculino , Adulto , Seudotumor Cerebral/cirugía , Seudotumor Cerebral/fisiopatología , Adulto Joven , Persona de Mediana Edad , Derivaciones del Líquido Cefalorraquídeo , Adolescente
3.
BMC Ophthalmol ; 24(1): 148, 2024 Apr 02.
Artículo en Inglés | MEDLINE | ID: mdl-38566041

RESUMEN

BACKGROUND: Bilateral retinal detachment and choroidal detachment in a patient are rare occurrences. The presence of bilateral diabetic retinopathy (DR) in such a case is even rarer and complicates the condition. CASE PRESENTATION: In this study, we document a case of unconventional VKH. Manifestations in this patient included intense peripheral retinal detachment and choroidal detachment, along with vitreous opacities akin to cotton wool spots, concurrent with DR. The diagnosis was considered as probable VKH with DR. Treatment according to VKH protocols, including high-dose corticosteroids, yielded positive results. CONCLUSIONS: VKH can co-occurrence with DR. VKH manifestations vary, and early, aggressive, and long-term treatment is essential. The complexity of treatment increases with concurrent DR, necessitating the use of immunosuppressants.


Asunto(s)
Efusiones Coroideas , Diabetes Mellitus , Retinopatía Diabética , Papiledema , Desprendimiento de Retina , Síndrome Uveomeningoencefálico , Humanos , Síndrome Uveomeningoencefálico/complicaciones , Síndrome Uveomeningoencefálico/diagnóstico , Síndrome Uveomeningoencefálico/tratamiento farmacológico , Desprendimiento de Retina/etiología , Desprendimiento de Retina/complicaciones , Retinopatía Diabética/complicaciones , Retinopatía Diabética/diagnóstico , Papiledema/etiología
4.
BMJ Case Rep ; 17(4)2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38565226

RESUMEN

We discuss a patient who presented with bilateral VI and VII cranial nerve palsies, symmetric upper and lower limb weakness and areflexia, 2 weeks following an flu-like illness. At presentation, there was no papilloedema, and her visual function was normal. Cerebrospinal fluid analysis and electrophysiology supported the diagnosis of Guillain-Barré Syndrome (GBS). She received intravenous immunoglobulins. She subsequently developed headaches and vision loss. Funduscopy demonstrated severe papilloedema with visual acuity of 6/18 right eye, 6/12 left eye with bitemporal visual field depression. Lumbar puncture revealed elevated opening pressure with high protein and normal cell count. She received acetazolamide. There was resolution of papilloedema and normal visual function at 3 months. Of note, the patient's body mass index was 17 kg/m2Our case highlights the rare occurrence of papilloedema in GBS, reiterating the importance of performing funduscopy on patients with any neurological diagnosis. Early detection and prompt management of papilloedema can prevent permanent vision loss.


Asunto(s)
Síndrome de Guillain-Barré , Papiledema , Femenino , Humanos , Síndrome de Guillain-Barré/complicaciones , Síndrome de Guillain-Barré/diagnóstico , Papiledema/etiología , Papiledema/complicaciones , Inmunoglobulinas Intravenosas , Debilidad Muscular/complicaciones , Trastornos de la Visión/complicaciones
5.
Neurol Clin ; 42(2): 433-471, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38575259

RESUMEN

Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.


Asunto(s)
Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Humanos , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/etiología , Seudotumor Cerebral/terapia , Hipertensión Intracraneal/complicaciones , Cefalea/diagnóstico , Cefalea/etiología , Cefalea/terapia , Trastornos de la Visión/terapia , Síndrome , Papiledema/complicaciones , Papiledema/diagnóstico
6.
Cephalalgia ; 44(3): 3331024241237237, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38459955

RESUMEN

BACKGROUND: Idiopathic intracranial hypertension (IIH) occurs more frequently in obese females of childbearing age. A link between eating disorders and poor outcome has been suggested but remains unproven. METHODS: This prospective field study at two tertiary headache centers included patients with clinically suspected IIH after standardized diagnostic work-up. Eating disorders were evaluated using validated questionnaires (EDQs). Primary outcome was the impact of eating disorders on IIH severity and outcome, secondary outcome was the prevalence and type of eating disorders in IIH compared to controls. RESULTS: We screened 326 patients; 143 patients replied to the EDQs and were classified as 'IIH' or 'non-IIH' patients. The demographic profile of EDQ-respondents and non-respondents was similar. Presence of an eating disorder did not impact IIH severity (lumbar puncture opening pressure (p = 0.63), perimetric mean deviation (p = 0.18), papilledema (Frisén grad 1-3; p = 0.53)) nor IIH outcome (optic nerve atrophy (p = 0.6), impaired visual fields (p = 0.18)). Moreover, we found no differences in the prevalence and type of eating disorders when comparing IIH with non-IIH patients (p = 0.09). CONCLUSION: Eating disorders did not affect IIH severity or outcome. We found the same prevalence and distribution pattern of eating disorders in IIH and non-IIH patients advocating against a direct link between IIH and eating disorders.


Asunto(s)
Trastornos de Alimentación y de la Ingestión de Alimentos , Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Femenino , Humanos , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/epidemiología , Seudotumor Cerebral/diagnóstico , Papiledema/diagnóstico , Campos Visuales , Obesidad/complicaciones , Trastornos de Alimentación y de la Ingestión de Alimentos/epidemiología , Trastornos de Alimentación y de la Ingestión de Alimentos/complicaciones , Hipertensión Intracraneal/complicaciones
9.
BMJ Case Rep ; 17(2)2024 Feb 17.
Artículo en Inglés | MEDLINE | ID: mdl-38367985

RESUMEN

Idiopathic intracranial hypertension (IIH) is a condition of unknown aetiology characterised by an increase in the intracranial pressure. Familial cases of IIH are rare and not well-understood. We present two monozygotic twins who developed IIH two years apart. The case involves two monozygotic female twins developing IIH in their 50s. They presented with a history of blurry vision and headaches. The diagnosis included the neurological, radiological and ophthalmological examination, excluding other causes. Both patients received treatment with acetazolamide, successfully resolving the papilloedema and restoring a normal visual field. This case highlights the occurrence of IIH among twins presenting at similar periods, emphasising the potential genetic influence. Clinicians should alert and educate the family regarding the risk factors and potential symptoms of this condition in the unlikely occurrence that other family members are affected.


Asunto(s)
Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Femenino , Humanos , Acetazolamida/uso terapéutico , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Presión Intracraneal , Papiledema/tratamiento farmacológico , Papiledema/etiología , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/tratamiento farmacológico , Gemelos Monocigóticos , Persona de Mediana Edad
10.
Pediatr Neurol ; 152: 196-199, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38301323

RESUMEN

BACKGROUND: It is extremely rare for Lyme borreliosis to present solely with features of increased intracranial pressure. The treatment of pediatric Lyme neuroborreliosis with oral versus intravenous antibiotics remains controversial. METHODS: Case report and literature review. RESULTS: A 13-year-old male presented with five days of binocular diplopia, several weeks of headache, and a history of multiple tick bites six weeks prior. His examination showed a left eye abduction deficit and bilateral optic disc edema. Magnetic resonance imaging (MRI) of the brain with contrast showed tortuosity of the optic nerves, prominence of the optic nerve sheaths, and enhancement of the left fifth and bilateral sixth cranial nerves. Lumbar puncture showed an elevated opening pressure and a lymphocytic pleocytosis. Lyme IgM and IgG antibodies were positive in the serum and cerebrospinal fluid. The patient was treated with intravenous ceftriaxone for two days empirically followed by doxycycline by mouth for 19 days. Symptoms began improving after 48 hours. The strabismus resolved after two weeks, and the papilledema improved slowly with complete resolution at six months. CONCLUSIONS: Lyme neuroborreliosis can present as isolated intracranial hypertension in the pediatric population; it can be differentiated from idiopathic intracranial hypertension on MRI, and lumbar puncture and can be confirmed with serum antibody testing. Oral doxycycline can be considered for Lyme neuroborreliosis in children.


Asunto(s)
Hipertensión Intracraneal , Enfermedad de Lyme , Neuroborreliosis de Lyme , Papiledema , Adolescente , Humanos , Masculino , Antibacterianos/uso terapéutico , Ceftriaxona/uso terapéutico , Doxiciclina/uso terapéutico , Hipertensión Intracraneal/tratamiento farmacológico , Hipertensión Intracraneal/etiología , Enfermedad de Lyme/complicaciones , Enfermedad de Lyme/diagnóstico , Enfermedad de Lyme/tratamiento farmacológico , Neuroborreliosis de Lyme/complicaciones , Neuroborreliosis de Lyme/diagnóstico , Neuroborreliosis de Lyme/tratamiento farmacológico
11.
J Appl Physiol (1985) ; 136(4): 753-763, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38357726

RESUMEN

Sleep and circadian temperature disturbances occur with spaceflight and may, in part, result from the chronically elevated carbon dioxide (CO2) levels on the international space station. Impaired sleep may contribute to decreased glymphatic clearance and, when combined with the chronic headward fluid shift during actual spaceflight or the spaceflight analog head-down tilt bed rest (HDTBR), may contribute to the development of optic disc edema. We determined if strict HDTBR combined with mildly elevated CO2 levels influenced sleep and core temperature and was associated with the development of optic disc edema. Healthy participants (5 females) aged 25-50 yr, underwent 30 days of strict 6° HDTBR with ambient Pco2 = 4 mmHg. Measures of sleep, 24-h core temperature, overnight transcutaneous CO2, and Frisén grade edema were made pre-HDTBR, on HDTBR days 4, 17, 28, and post-HDTBR days 4 and 10. During all HDTBR time points, sleep, core temperature, and overnight transcutaneous CO2 were not different than the pre-HDTBR measurements. However, independent of the HDTBR intervention, the odds ratios {mean [95% confidence interval (CI)]} for developing Frisén grade optic disc edema were statistically significant for each hour below the mean total sleep time (2.2 [1.1-4.4]) and stage 2 nonrapid eye movement (NREM) sleep (4.8 [1.3-18.6]), and above the mean for wake after sleep onset (3.6 [1.2-10.6]) and for each 0.1°C decrease in core temperature amplitude below the mean (4.0 [1.4-11.7]). These data suggest that optic disc edema occurring during HDTBR was more likely to occur in those with short sleep duration and/or blunted temperature amplitude.NEW & NOTEWORTHY We determined that sleep and 24-h core body temperature were unaltered by 30 days exposure to the spaceflight analog strict 6° head-down tilt bed rest (HDTBR) in a 0.5% CO2 environment. However, shorter sleep duration, greater wake after sleep onset, and lower core temperature amplitude present throughout the study were associated with the development of optic disc edema, a key finding of spaceflight-associated neuro-ocular syndrome.


Asunto(s)
Papiledema , Vuelo Espacial , Femenino , Humanos , Reposo en Cama , Duración del Sueño , Dióxido de Carbono , Inclinación de Cabeza , Temperatura , Hipercapnia , Sueño
12.
Pediatr Neurol ; 153: 92-95, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38354628

RESUMEN

BACKGROUND: Currently no guidelines for repeating a lumbar puncture to guide management in primary intracranial hypertension (PIH) exist. METHODS: An institutional database of patients 18 years and younger followed in the institution's pediatric intracranial hypertension clinic was examined for opening pressure changes in PIH at diagnosis, before medication wean, and following medication wean, as well as to examine whether measurements at the time of diagnosis differed between those with and without disease recurrence. RESULTS: Forty-two patients were included in this study; 36% were male and the mean age at diagnosis was 11.01 years. Treatment duration averaged 9.68 months in those without recurrence and 8.5 months in those with recurrence. Average body mass index percentile of patients with disease recurrence was 83.7 and 72.1 in those without recurrence (P = 0.16). Average opening pressure values of all patients at diagnosis, prewean, and postwean was 36.53 cm H2O, 30.7 cm H2O, and 31.1 cm H2O, respectively. There was no statistically significant difference in opening pressures across these time points (P = 0.14). The change in opening pressure from diagnosis to postwean was statistically significant with a reduction of 5.18 cm H2O (P = 0.04). There was no statistical difference between change in opening pressure at diagnosis versus postwean between those with and without recurrence (P = 0.17). CONCLUSIONS: This clinical observational study suggests that mean opening pressure measurements in patients with PIH remain elevated both before and after medication wean despite papilledema resolution and patient-reported PIH symptoms. Clinically, this suggests that other features such as signs of optic disc edema and symptoms should be used to inform a clinical determination of disease recurrence and treatment course.


Asunto(s)
Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Humanos , Masculino , Niño , Femenino , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/terapia , Presión del Líquido Cefalorraquídeo , Estudios Retrospectivos , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/terapia , Papiledema/diagnóstico , Presión Intracraneal
13.
World Neurosurg ; 184: e743-e753, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38342171

RESUMEN

BACKGROUND: Papilledema's association with hydrocephalus (HCP)-linked larger vestibular schwannoma (VS) is established but cases lacking concurrent HCP require further investigation. METHODS: This retrospective comparative observational study, conducted from July 2018 to July 2023, examined 120 VS patients undergoing surgery. Patients were categorized into Group 1 (papilledema without HCP) and Group 2 (no papilledema or HCP), with comprehensive data analyzed. RESULTS: In this study, Group 1 (14 patients with papilledema) and Group 2 (106 patients without papilledema or HCP) were compared. Group 1 was younger (mean age 27.21 ± 11.73 years) than Group 2 (mean age 54.66 ± 11.44 years). Both groups had similar symptom durations and tumor detection times. Group 1 had increased vascularity (P = 0.001), elevated cisterna magna protein levels (P = 0.001), and a higher incidence of neurofibromatosis 2 (P = 0.003). They also experienced longer surgeries (P = 0.001) and more blood loss (P = 0.001), leading to extended postoperative complications. Group 2 showed improved postsurgery visual outcomes (P = 0.001), better Glasgow Outcome Scores (P = 0.001), enhanced facial nerve preservation (P = 0.002), and improved hearing on follow-up (P = 0.003). Logistic regression analysis highlighted prolonged surgery duration (P = 0.057) and papilledema (P = 0.0001) as significant factors influencing visual improvement. CONCLUSIONS: Patients with VS require preoperative fundoscopy evaluation due to potential visual loss and papilledema, even without HCP. Early treatment initiation enhances visual and hearing outcomes. Meticulous surgery is vital given the lesion's hypervascular nature and adherence to surrounding structures. Preoperative embolization may aid in preserving neurovascular structures. In developing countries with higher blindness rates, judicious noncontrast computed tomography brain evaluation is crucial for timely detection and treatment initiation of lesions like VS.


Asunto(s)
Hidrocefalia , Neuroma Acústico , Papiledema , Humanos , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Neuroma Acústico/complicaciones , Neuroma Acústico/diagnóstico por imagen , Neuroma Acústico/cirugía , Papiledema/diagnóstico por imagen , Papiledema/etiología , Estudios Retrospectivos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Hidrocefalia/diagnóstico , Ceguera , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Progresión de la Enfermedad , Resultado del Tratamiento
14.
J Neuroimmunol ; 388: 578312, 2024 03 15.
Artículo en Inglés | MEDLINE | ID: mdl-38364528

RESUMEN

OBJECTIVES: To describe papillitis as a clinical phenotype of IgLON5 autoimmunity. METHODS: We retrospectively reviewed patients with IgLON5 autoimmunity who had optic neuropathy, optic neuritis, or optic disc edema. Sera from patients with recurrent papillitis were tested for IgLON5 antibodies. RESULTS: We found two elderly males presenting with papillitis in the presence of IgLON5 antibodies. CSF pleocytosis was present and partial vision improvement occurred in one patient despite immunotherapy. Sera from 18 patients with recurrent papillitis were negative for IgLON5 antibodies. CONCLUSION: Papillitis could be a manifestation of IgLON5 disease, with or without accompanying cognitive, sleep, and movement disorders.


Asunto(s)
Neuritis Óptica , Papiledema , Masculino , Humanos , Anciano , Autoinmunidad , Estudios Retrospectivos , Neuritis Óptica/complicaciones , Nervio Óptico , Moléculas de Adhesión Celular Neuronal/uso terapéutico
15.
Rev Med Liege ; 79(2): 68-72, 2024 Feb.
Artículo en Francés | MEDLINE | ID: mdl-38356421

RESUMEN

Sarcoidosis is a chronic inflammatory granulomatous disease of unknown aetiology. Although the lungs and thoracic lymph nodes are the most frequently affected structures, every tissue can be affected. Ophthalmological involvement occurs in 25 % of cases. We report a clinical case in which the diagnosis of sarcoidosis was revealed by isolated unilateral optic disc edema.


La sarcoïdose est une pathologie inflammatoire granulomateuse chronique dont l'étiologie reste inconnue. Bien que les poumons et les ganglions thoraciques soient les structures les plus fréquemment atteintes, tous les tissus peuvent être affectés. L'atteinte ophtalmologique est présente dans 25 % des cas. Nous rapportons un cas clinique dont le diagnostic de sarcoïdose fut révélé par un œdème papillaire unilatéral isolé.


Asunto(s)
Papiledema , Sarcoidosis , Humanos , Papiledema/diagnóstico , Sarcoidosis/complicaciones , Sarcoidosis/diagnóstico , Diagnóstico Diferencial , Pulmón
16.
Eur J Ophthalmol ; 34(3): NP122-NP126, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38238930

RESUMEN

PURPOSE: To describe a rare case of vision loss due to bilateral edema of the optic nerve in a patient with Hereditary Angioedema, treated with prophylactic C1-esterase inhibitor. METHODS: A 60-year-old Caucasian male affected by Hereditary Angioedema with unknown genetic defect (HAE- UNK) was admitted to our hospital presenting bilateral vision loss (best corrected visual acuity of 20/32 in the right eye and hand motion in the left eye) during an HAE attack. Intravenous administration of C1- esterase inhibitor (C1-INH, 1500 IU, Berinert, CSL Behring) determined the resolution of facial and periorbital swelling, however visual impairment persisted, in contrast with previous attacks experienced by the patient. Fundus examination revealed a vital optic disc without papilledema in both eyes. Magnetic resonance imaging (MRI) of the head and orbits showed bilateral edema of the optic nerve sheath. Treatment with intravenous and oral steroids was ineffective. Subsequently, a prophylactic treatment strategy with subcutaneous C1-esterase inhibitor was started (7000 IU every four days). RESULTS: Complete regression of edema of the optic nerves was observed by imaging at two months of follow-up after chronic treatment with C1-esterase inhibitor (7000 IU every four days). Complete restoration of visual acuity was achieved (BCVA 20/20 in both eyes) and multimodal imaging of the optic nerves demonstrated the absence of anatomical and functional damage. CONCLUSION: Patients affected by HAE may show atypical presentation with edema of the optic nerves without involvement of the optic nerve head. They may significantly benefit from prophylactic and chronic treatment with C1-esterase inhibitor.


Asunto(s)
Proteína Inhibidora del Complemento C1 , Imagen por Resonancia Magnética , Papiledema , Agudeza Visual , Humanos , Masculino , Persona de Mediana Edad , Papiledema/diagnóstico , Papiledema/tratamiento farmacológico , Papiledema/etiología , Agudeza Visual/fisiología , Proteína Inhibidora del Complemento C1/uso terapéutico , Ceguera/diagnóstico , Ceguera/etiología , Angioedemas Hereditarios/diagnóstico , Angioedemas Hereditarios/complicaciones , Angioedemas Hereditarios/tratamiento farmacológico , Angioedemas Hereditarios/fisiopatología , Nervio Óptico/diagnóstico por imagen
17.
J AAPOS ; 28(1): 103806, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38216114

RESUMEN

BACKGROUND: Optic disk drusen (ODD) in pediatric patients typically presents with pseudopapilledema. Diagnosing concomitant papilledema due to idiopathic intracranial hypertension (IIH) in these patients can be challenging. The purpose of this study was to evaluate the incidence and clinical features of papilledema due to IIH among pediatric patients with a new diagnosis of ODD and to discuss the clinical and paraclinical findings that helped diagnose this group. METHODS: The medical records of children <15 years of age with ODD confirmed by B-scan ultrasound at their first visit over a 4-year period (2019-2022) were reviewed retrospectively. Patients with concurrent IIH were identified, and the demographic and clinical characteristics were reviewed. RESULTS: A total of 83 children with confirmed ODD at the initial presentation were included, of whom 4 (4.8%) were diagnosed with concomitant IIH. Patients ranged in age from 7 to 15 years; 3 of the 4 were female, and 3 had IIH-related symptoms at presentation (1 was asymptomatic). None of the 4 patients had papilledema greater than Frisen grade 2. CONCLUSIONS: We recommend that clinicians review pertinent IIH symptoms and risk factors in children with ODD and follow the standard workup for IIH in suspicious cases. In asymptomatic patients with a new diagnosis of ODD, we recommend obtaining a follow-up optic nerve evaluation and optical coherence tomography scan to detect any significant interval change that might serve as a possible indicator of concomitant papilledema.


Asunto(s)
Hipertensión Intracraneal , Drusas del Disco Óptico , Enfermedades del Nervio Óptico , Papiledema , Seudotumor Cerebral , Humanos , Femenino , Niño , Adolescente , Masculino , Papiledema/diagnóstico , Papiledema/etiología , Drusas del Disco Óptico/complicaciones , Drusas del Disco Óptico/diagnóstico , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/diagnóstico , Estudios Retrospectivos , Enfermedades del Nervio Óptico/diagnóstico , Tomografía de Coherencia Óptica/métodos
18.
J AAPOS ; 28(1): 103803, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38216117

RESUMEN

BACKGROUND: Pediatric papilledema often reflects an underlying severe neurologic disorder and may be difficult to appreciate, especially in young children. Ocular fundus photographs are easy to obtain even in young children and in nonophthalmology settings. The aim of our study was to ascertain whether an improved deep-learning system (DLS), previously validated in adults, can accurately identify papilledema and other optic disk abnormalities in children. METHODS: The DLS was tested on mydriatic fundus photographs obtained in a multiethnic pediatric population (<17 years) from three centers (Atlanta-USA; Bucharest-Romania; Singapore). The DLS's multiclass classification accuracy (ie, normal optic disk, papilledema, disks with other abnormality) was calculated, and the DLS's performance to specifically detect papilledema and normal disks was evaluated in a one-vs-rest strategy using the AUC, sensitivity and specificity, with reference to expert neuro-ophthalmologists. RESULTS: External testing was performed on 898 fundus photographs: 447 patients; mean age, 10.33 (231 patients ≤10 years of age; 216, 11-16 years); 558 normal disks, 254 papilledema, 86 other disk abnormalities. Overall multiclass accuracy of the DLS was 89.6% (range, 87.8%-91.6%). The DLS successfully distinguished "normal" from "abnormal" optic disks (AUC 0.99 [0.98-0.99]; sensitivity, 87.3% [84.9%-89.8%]; specificity, 98.5% [97.6%-99.6%]), and "papilledema" from "normal and other" (AUC 0.99 [0.98-1.0]; sensitivity, 98.0% [96.8%-99.4%]; specificity, 94.1% (92.4%-95.9%)]. CONCLUSIONS: Our DLS reliably distinguished papilledema from normal optic disks and other disk abnormalities in children, suggesting it could be utilized as a diagnostic aid for the assessment of optic nerve head appearance in the pediatric age group.


Asunto(s)
Aprendizaje Profundo , Papiledema , Adulto , Humanos , Niño , Preescolar , Papiledema/diagnóstico , Fondo de Ojo , Inteligencia Artificial , Nervio Óptico , Encéfalo
19.
Transl Vis Sci Technol ; 13(1): 12, 2024 01 02.
Artículo en Inglés | MEDLINE | ID: mdl-38224329

RESUMEN

Purpose: Optical coherence tomography (OCT) has been used to monitor papilledema. This study aims to determine which OCT-derived measures of the optic nerve head (ONH) detect resolving papilledema in children faster than standard OCT measures. Methods: Children (≤18 years of age) with papilledema who completed optic nerve SD-OCT pretreatment and had evidence of treatment response on one or more follow-up OCTs within 4 months were included. Standard (mean circumpapillary retinal nerve fiber layer [cpRNFL] thickness), device-derived (per-quadrant cpRNFL) and custom (ONH height, maximum Bruch's membrane displacement [BMD], ONH volume [ONHV], and BMD volume) OCT measures were calculated. Per-eye generalized estimating equations (GEEs) modelled changes in device-derived and custom measures as a function of mean cpRNFL to identify those measures that resolved faster during early (0-2 months) follow-up. Mean cpRNFL coefficients of greater than 1 indicated faster resolving papilledema. Results: We included 52 eyes of 29 children (mean age, 12.8 years; 72.4% female). In analysis of early follow-up visits (38 eyes from 22 children), nasal cpRNFL and maximum BMD in each quadrant resolved faster than mean cpRNFL (GEE coefficients range, 1.14-3.37). Inferior cpRNFL, superior, nasal, and inferior ONH heights and ONHV resolved slower than mean cpRNFL (GEE coefficients range, 0.67-0.87). Conclusions: Nasal cpRNFL is a promising device-derived OCT measure for the early detection of resolving papilledema in children compared with mean cpRNFL. Maximum BMD, a custom measure, also shows promise, but its calculation has not yet been incorporated into commercial OCT devices. Translational Relevance: This study guides the optimal use of OCT in capturing resolving papilledema in children.


Asunto(s)
Disco Óptico , Papiledema , Niño , Femenino , Humanos , Lactante , Masculino , Disco Óptico/diagnóstico por imagen , Papiledema/diagnóstico , Tomografía de Coherencia Óptica , Retina
20.
Transl Vis Sci Technol ; 13(1): 13, 2024 01 02.
Artículo en Inglés | MEDLINE | ID: mdl-38231498

RESUMEN

Purpose: To visualize and quantify structural patterns of optic nerve edema encountered in papilledema during treatment. Methods: A novel bi-channel deep-learning variational autoencoder (biVAE) model was trained using 1498 optical coherence tomography (OCT) scans of 125 subjects over time from the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) and 791 OCT scans of 96 control subjects from the University of Iowa. An independent test dataset of 70 eyes from 70 papilledema subjects was used to evaluate the ability of the biVAE model to quantify and reconstruct the papilledema spatial patterns from input OCT scans using only two variables. Results: The montage color maps of the retinal nerve fiber layer (RNFL) and total retinal thickness (TRT) produced by the biVAE model provided an organized visualization of the variety of morphological patterns of optic disc edema (including differing patterns at similar thickness levels). Treatment effects of acetazolamide versus placebo in the IIHTT were also demonstrated in the latent space. In image reconstruction, the mean signed peripapillary retinal nerve fiber layer thickness (pRNFLT) difference ± SD was -0.12 ± 17.34 µm, the absolute pRNFLT difference was 13.68 ± 10.65 µm, and the RNFL structural similarity index reached 0.91 ± 0.05. Conclusions: A wide array of structural patterns of papilledema, integrating the magnitude of disc edema with underlying disc and retinal morphology, can be quantified by just two latent variables. Translational Relevance: A biVAE model encodes structural patterns, as well as the correlation between channels, and may be applied to visualize individuals or populations with papilledema throughout treatment.


Asunto(s)
Aprendizaje Profundo , Papiledema , Humanos , Papiledema/diagnóstico por imagen , Papiledema/tratamiento farmacológico , Nervio Óptico/diagnóstico por imagen , Retina/diagnóstico por imagen , Edema
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