Kimura disease with optic nerve and intracranial involvement: a case description.
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ABSTRACT:
SUBMITTER: Li Y
PROVIDER: S-EPMC10784061 | biostudies-literature | 2024 Jan
REPOSITORIES: biostudies-literature
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Kimura disease with optic nerve and intracranial involvement: a case description.
Li Yuhan Y Zhao Jiamei J Ruan Xiaoling X Li Zhigao Z Li Zongfang Z Tang Zhiwei Z
Quantitative imaging in medicine and surgery 20231030 1
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Project description:Kimura Disease, an eosinophilic hyperplastic granulomatous disease of idiopathic origin is most commonly seen in young males. It mostly presents with deep subcutaneous tissue swelling along with enlargement of salivary glands and regional lymphadenopathy. Diagnosis is mainly based on histopathological findings and raised serum IgE and hypereosinophilia. The radiological investigation of choice is Magnetic Resonance Imaging. Management strategy includes surgery and steroid therapy. Chemotherapeutic agents are also frequently used by some centres. Relapse and recurrence stands to be a common problem with management of this disease process.Supplementary informationThe online version contains supplementary material available at 10.1007/s12070-023-03817-y.
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Project description:OBJECTIVE:Several studies have shown an association between intracranial pressure and the diameter of the optic nerve sheath measured by transbulbar ultrasonography. To understand the pathophysiology of this phenomenon, we aimed to measure the changes of the optic nerve, optic nerve sheath and perineural space separately with increasing intracranial pressure in a porcine model. METHODS:An external ventricular drain was placed into the third ventricle through a right paramedian burrhole in eight anesthesized pigs. The diameters of the optic nerve and the optic nerve sheath were measured while the intracranial pressure (ICP) was increased in steps of 10mmHg from baseline up to 60 mmHg. RESULTS:The median diameters of the optic nerve (ON) increased from 0.36 cm (baseline- 95% confidence interval (CI) 0.33 cm to 0.45 cm) to 0.68 cm (95% CI 0.57 cm to 0.82 cm) at ICP of 60 mmHg (p<0.0001) and optic nerve sheath (ONS) from 0.88 cm (95% CI 0.79 cm to 0.98 cm) to 1.24 cm (95% CI 1.02 cm to 1.38 cm) (p< 0.002) while the median diameter of the perineural space (PNS) (baseline diameter 95% CI 0.40 cm to 0.59 cm to diameters at ICP 60 95% CI 0.38 cm to 0.62 cm) did not change significantly (p = 0.399). Multiple comparisons allowed differentiation between baseline and values ≥40 mmHg for ON (p = 0.017) and between baseline and values ≥ 50mmHg for ONS (p = 0.006). A linear correlation between ON (R2 = 0.513, p<0.0001) and ONS (R2 = 0.364, p<0.0001) with ICP was found. The median coefficient of variation for intra- and inter-investigator variability was 8% respectively 2.3%. CONCLUSION:Unexpectedly, the increase in ONS diameter with increasing ICP is exclusively related to the increase of the diameter of the ON. Further studies should explore the reasons for this behaviour.
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Project description:BackgroundCavum septum pellucidum (CSP) and cavum vergae (CV) are normal anatomical variations present in some children, adolescents, and adults. When the cavity is larger than normal, it is called a cyst. Symptomatic cases of CSP and CV cyst are rare, and the clinical presentation is varied. A case with multiple nerve involvement is described.Case descriptionA healthy 17-year-old female presented a sudden headache, right cervicobrachialgia, right ptosis, visual changes, and left facial paralysis over 10 days. Head magnetic resonance imaging revealed CSP and CV cyst. Inflammation, infection, and vascular disorders were ruled out. We decided to perform a right transfrontal endoscopic intraventricular septostomy and a right Monro foraminoplasty, which were successful. One month after surgery, the patient had no more signs or symptoms. She has remained asymptomatic for the past year.ConclusionMultiple nerve involvement was directly related to CSP and CV cyst. The cyst bilaterally occluded the foramen of Monro causing intracranial hypertension. It was possible to obtain complete resolution of the clinical features through neuroendoscopic fenestration and foraminoplasty.
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Project description:To evaluate the results of bilateral endoscopic optic nerve decompression (EOND) with the opening nerve sheath (ONS) technique in patients with idiopathic intracranial hypertension (IIH). Between the years of 2017 and 2019, we retrospectively evaluated nine IIH patients with progressive visual impairment despite medical treatment and who were treated with the EOND and ONS techniques. We also demonstrated our surgical technique recipe on postmortem human heads in a stepwise manner. There were 9 patients (7 females and 2 males) between the ages of 21 and 72 included in this study, and the mean age was 40.8. All patients had an impairment in visual acuity and/or their visual field, with signs of papilledema and/or optic atrophy. The patients were followed up with for 9-48 months. Improvements in visual acuity were observed in 7 out of 9 patients (78%). Visual field defects improved in 5 out of 8 patients (62.5%). Papilledema was resolved in all patients (100%). Headaches improved in all symptomatic patients (100%). No intraoperative or postoperative complications were observed. EOND is a safe and effective surgical procedure in selected patients with IIH. Bilateral wide bony decompression and nerve fenestration can also be an additional benefit for headache relief. Further clinical series and long-term follow-up are needed for more precise results.
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Project description:BackgroundPostural orthostatic tachycardia syndrome is a disorder of the autonomic nervous system. Approximately 30% of patients experience orthostatic headaches. Orthostatic headaches also are a hallmark symptom in spontaneous intracranial hypotension. While the cause of orthostatic headaches in spontaneous intracranial hypotension can be linked to the cerebrospinal fluid loss at the spinal level and consecutively reduced intracranial pressure in the upright position, the cause of orthostatic headaches in postural orthostatic tachycardia syndrome still remains unknown. The present study examined orthostatic changes of intracranial pressure using dynamic ultrasound of the optic nerve and optic nerve sheath diameter in postural orthostatic tachycardia syndrome, spontaneous intracranial hypotension and healthy subjects.MethodsData was obtained from postural orthostatic tachycardia syndrome patients with (n = 7) and without orthostatic headaches (n = 7), spontaneous intracranial hypotension patients (n = 5) and healthy subjects (n = 8). All participants underwent high-resolution transorbital ultrasound in the supine and upright position to assess optic nerve and optic nerve sheath diameter.ResultsGroup differences were found in percentage deviations when changing position of optic nerve sheath diameter (p < 0.01), but not regarding the optic nerve diameter. Pairwise comparisons indicated differences in optic nerve sheath diameter only between spontaneous intracranial hypotension and the other groups. No differences were found between postural orthostatic tachycardia syndrome patients with and without orthostatic headaches.ConclusionThis study shows that the size of the optic nerve sheath diameter dynamically decreases during orthostatic stress in spontaneous intracranial hypotension, but not in postural orthostatic tachycardia syndrome with or without orthostatic headaches, which indicates different underlying causes.
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Project description:Purpose Monitoring of raised intracranial pressure (ICP) in patients with idiopathic intracranial hypertension (IIH) is required to prevent secondary optic nerve damage. Sonographic measurement of the optic nerve sheath diameter (ONSD) is a noninvasive method to evaluate intracranial hypertension. Different ONSD cut-off values have been reported probably due to ethnic variations. Our aim was to determine optic nerve sonographic examination cut-off points to predict raised ICP in IIH patients. Methods This case-control study was conducted on 99 IIH post-pubertal female patients (both probable and definite) and 35 age- and sex-matched healthy volunteers. Sonographic ONSD and optic nerve diameter (OND) were obtained 3 mm behind the posterior edge of the globe in a horizontal plane via a 7-13 MHz linear probe. Lumbar puncture was then carried out on the patients. Results The opening cerebrospinal fluid pressure documented in the patient group was 279.64 ± 65.97 mm H2O. A statistically significant difference was found between IIH patients and controls regarding ONSD. The best ONSD cut-off value indicating raised ICP was 6.05 mm with an area under the curve of 0.850 (95% confidence interval 0.805 to 0.894, 73.2% sensitivity and 91.4% specificity). Regarding OND/ONSD ratio, there was an insignificant difference between both groups. Conclusion Sonographic ONSD but not OND/ONSD ratio could offer a bedside adjunct or alternative indicator of elevated ICP in IIH patients. Ethnic differences, however, should be noted when using this parameter.
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Project description:PurposeTo characterize newly discovered electrical synapses, formed by connexin (Cx) 36 and 45, between neighbouring axons within the optic nerve head.MethodsTwenty-five Wistar rats were killed by CO2 inhalation. Proximal and distal optic nerve (ON) stumps were collected and processed for immunostainings, electron microscopy (EM) with immunogold labelling, PCR and Western blots (WB). Additional 15 animals were deeply anaesthetized, and flash visual evoked potentials (fVEP) after retrobulbar injection of saline (negative control) or 100 μm meclofenamic acid solution (gap junctions' blocker) were recorded. Human paraffin cross-sections of eyeballs for immunostainings were obtained from the Human Eye Biobank for Research.ResultsImmunostainings of both rat and human ON revealed the presence of Cx45 and 36 colocalizing with β3-tubulin, but not with glial fibrillary acidic protein (GFAP). In WB, Cx36 content in optic nerve was approximately halved when compared with retina (0.58 ± 0.005 in proximal stump and 0.44 ± 0.02 in distal stump), Cx45 showed higher levels (0.68 ± 0.01 in proximal stump and 0.9 ± 0.07 in distal stump). In immunogold-EM of optic nerve sections, we found electric synapses (formed mostly by Cx45) directly coupling neighbouring axons. In fVEP, blocking of gap junctions with meclofenamic acid resulted in significant prolongation of the latency of P1 wave up to 160% after 30 min (p < 0.001).ConclusionsOptic nerve (ON) axons are equipped with electrical synapses composed of neuronal connexins, especially Cx45, creating direct morphological and functional connections between each other. This finding could have substantial implications for understanding of the pathogenesis of various optic neuropathies and identifies a new potential target for a therapeutic approach.
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