Retinal Microvascular Changes in Internal Carotid Artery Stenosis.

Purpose: We aimed to analyze retinal microvascular parameters, measured by optical coherence tomography angiography in patients with internal carotid artery stenosis compared to healthy individuals. Materials and Methods: A total of 41 eyes from 30 patients who had varying degrees of carotid stenosis, and 42 eyes from 42 healthy controls, were enrolled in this study. Depending on the degree of stenosis evaluated by Doppler ultrasonographic imaging, the patient group was further subclassified into mild, moderate, and severe carotid artery stenosis. Superficial and deep capillary plexus vessel densities, radial peripapillary capillary vessel density, foveal avascular zone, and flow densities in the choriocapillaris and outer retina were evaluated by optical coherence tomography angiography. Results: The superficial and deep capillary plexus vessel densities were significantly reduced among the groups, only sparing the foveal region. The mean superficial plexus vessel density was 45.67 ± 4.65 and 50.09 ± 4.05 for the patient and control group, respectively (p = 0.000). The mean deep capillary plexus density was 46.33% ± 7.31% and 53.27% ± 6.31% for the patient and control group, respectively (p = 0.000). The mean superficial and deep capillary vessel densities in the foveal region did not show any statistical difference between the patient and control groups (p = 0.333 for the superficial and p = 0.195 for the deep plexus vessel density). Radial peripapillary capillary vessel density was decreased in the patient group (p = 0.004). The foveal avascular zone area was wider in the patient group but this difference did not show a significant difference (p = 0.385). Conclusions: Retinal microvascular changes are a prominent outcome of internal carotid disease, and even mild stenosis can lead to alterations in the retinal microvascular bed which could be detected by OCTA. By early detection of microvascular changes in the retina in this patient group, we might speculate the overall vascular condition.

Endoscopic Endonasal Optic Nerve Decompression in Idiopathic Intracranial Hypertension: When to Implement Optic Nerve Sheath Fenestration.

BACKGROUND AND OBJECTIVE: Idiopathic intracranial hypertension (IIH) is a syndrome characterized with increased intracranial pressure and normal cerebrospinal fluid (CSF) composition. Treatment is mainly conservative, whereas CSF diversion surgery is the most frequently used surgical intervention. Endoscopic endonasal optic nerve decompression (EOND) is a newer surgical treatment of this patient group. This study presents a single clinic's case series with comparative results of unilateral an bilateral EOND with or without optic nerve fenestration. METHODS: Sixteen patients with IIH syndrome who underwent 18 EOND procedures by a single neurosurgeon were evaluated with MRI and digitally subtracted angiography preoperatively. Both preoperative and postoperative visual acuity and perimetry and fundoscopy examinations were routinely performed. All patients underwent sphenoidotomy using the endoscopic binostril approach; however, unilateral or bilateral optic nerve decompression and accompanying optic sheath fenestration was determined on a case-by-case basis, after which all patients were also evaluated for the outcome of headaches. RESULTS: Most of the patients were female, and the mean age was 30.28 ± 9.78 years. CSF pressure was increased in all patients (406.43 ± 112.91 mm of H 2 O), and the follow-up period was 61.72 ± 21.67 months. In patients with unilateral EOND, visual fields improved in 83%, visual acuity in 70%, headache in 75%, and papilledema in 27% of cases. In patients with bilateral EOND, perimetry improved in 86%, visual acuity in 43%, headache in 50%, and papilledema in 57% of cases. CONCLUSION: EOND is an effective surgical option in the treatment of IIH. Bilateral decompression is preferable in patients with bilateral visual involvement, and optic nerve fenestration may prove to be helpful in patients with IIH whose primary complaint is headache.

Spectrum of the neurologic manifestations in childhood-onset cryopyrin-associated periodic syndrome.

OBJECTIVE: Neurologic complications of chronic infantile neurologic, cutaneous and articular syndrome (CINCA) are well-known, whereas there are scarce data regarding neurologic features of milder cryopyrin-associated periodic syndrome (CAPS) phenotypes. We aimed to review the neurologic features in detail and summarize the other CAPS-related manifestations in 12 children. METHODS: All children with CAPS that have been followed-up from pediatric rheumatology outpatient clinic, were enrolled to the study. In addition to the neurologic examination, magnetic resonance imaging (MRI) of brain, electroencephalography, eye examination, hearing test and intellectual assessment were done. Demographic, clinical features, genetic analysis and laboratory tests were noted from patient records and hospital database. RESULTS: The median age of the subjects was 7 years (range 2-19 years), with a female-to-male ratio 2/1. The phenotype was consistent with familial cold autoinflammatory syndrome in 7 patients, Muckle-Wells syndrome in 3 patients and chronic infantile neurologic, cutaneous and articular syndrome in 2 patients. Most frequently noted neurologic clinical manifestation during the entire disease course was headache (n = 4/12) followed by seizures (n = 3/12), papilledema (n = 3/12), intellectual disability (n = 2/12), aseptic meningitis (n = 2/12), hearing loss (n = 2/12) and optic atrophy (n = 1/12). MRI of the brain revealed abnormal lesions in two patients. Uveitis or conjunctivitis were seen in two children. Overall, neurological involvement was detected in 6/12 of our cohort, of which half (n = 3) was in severe form. CONCLUSION: Half of the children with CAPS exhibited neurologic manifestations with varying degrees of severity. Increased understanding and awareness of this rare but treatable syndrome among neurologists is essential. If remains untreated and unrecognized, this autoinflammatory syndrome could lead to significant morbidity and mortality. Besides complete resolution of systemic symptoms, anti-interleukin-1 treatment may also prevent progression of neurologic findings when initiated in the early stage of the disease.