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- Quantification of peripapillary total retinal volume in pseudopapilledema and mild papilledema using spectral-domain optical coherence tomography. [JOURNAL ARTICLE]
- Am J Ophthalmol 2014 Apr 8.
to distinguish differences in retinal nerve fiber layer (RNFL) thickness and peripapillary total retinal volume between eyes with papilledema, pseudopapilledema, and normal findings.Cohort study METHODS: Forty-two eyes with mild papilledema, 37 eyes with congenitally elevated optic disc (pseudopapilledema), and 34 normal eyes met the inclusion criteria at 1 academic institution (in Iran) and underwent neuro-ophthalmic examination. Spectral-domain optical coherence tomography scans surrounding the optic disc were performed in each eye of patients and subjects. Main outcome measures were mean RNFL thickness and peripapillary total retinal volume measurements (inner and outer rings volumes) that were compared between groups, using the generalized estimating equation approach. Area under receiver operating characteristic curves were also calculated.A statistically significant difference was found in mean RNFL thickness between both groups of patients with papilledema and pseudopapilledema and normal subjects. Average inner PTR volume in the papilledema, pseudopapilledema, and control groups were 1.95 ± 0.24 mm3, 1.81 ± 0.23 mm3, and 1.06 ± 0.10 mm3, respectively. Average outer peripapillary total retinal volume in the papilledema and pseudopapilledema groups were 2.68 ± 0.49 mm3, 2.03 ± 0.24 mm3, respectively (P=0.000). However, the outer ring peripapillary total retinal volume was not different between pseudopapilledema and normal (1.90 ± 0.11 mm3) eyes (P=0.17). Area under the curve to discriminate pseudopapilledema versus papilledema eyes for average RNFL thickness, inner and outer peripapillary total retinal volumes were 0.82, 0.68, and 0.88, respectively.Outer peripapillary total retinal ring volumes might be useful in differentiating papilledema from pseudopapilledema.
- Teaching NeuroImages: Griscelli syndrome and CNS lymphohistiocytosis. [Journal Article]
- Neurology 2014 Apr 8; 82(14):e122-3.
A 3-year-old boy developed viral illness followed by fever, altered sensorium, focal seizures, and neuroregression. Examination showed silvery-gray hair (figure 1A), bilateral papilledema, spastic quadriparesis, brisk muscle-stretch reflexes, extensor plantars, hepatosplenomegaly, and normally pigmented skin, iris, and retina. Hair microscopy confirmed Griscelli syndrome (GS) (figure 1, B-D). MRI brain was suggestive (figure 2, A-D). CSF showed 20 degenerated leukocytes. He died of an intercurrent illness 2 months later.
- Unilateral endoscopic optic nerve decompression for idiopathic intracranial hypertension: A series of ten patients. [REVIEW]
- World Neurosurg 2014 Apr 2.
Several surgical treatment modalities, including lumboperitoneal (LP) or ventriculoperitoneal (VP) shunt surgery, subtemporal decompression, endovascular venous sinus stenting, optic nerve decompression (OND) were used in the management of idiopathic intracranial hypertension (IIH). Each surgical technique has different advantages and disadvantages. Endoscopic OND is rarely used in the management of IIH. There are only four reported cases. The aim of this study is to describe the surgical results of patients treated with this less invasive surgical technique.The authors reviewed a series of ten consecutive cases of unilateral OND performed, using the endoscopic approach without nerve sheath opening between December 2008 and December 2012. Presenting symptoms, neurological examination findings, MR venography imaging results, fundoscopic and visual acuity examination findings and automated perimetry test results were recorded. Perioperative results, including complications, length of hospital stay were evaluated. Findings at the follow-up evaluations were also recorded.This report is the first series of unilateral OND performed using the endoscopic approach. The mean patient age was 34.1 years (range 9-49 years), there were nine female and one male patients. Visual impairment was the main symptom in our patient group, whereas headache was a secondary complaint in our patients. The patients were first managed with medical treatment for at least three months. Unilateral endoscopic OND was performed on the side with greater visual failure. Mean follow-up was 28.4 months (8-55 months). The visual field defects and visual acuity were improved in eight of nine patients, whereas papilloedema improved in seven of nine patients. Also headache was resolved in four of seven patients. There were no complications in this relatively small series.The surgical treatment of IIH by using unilateral endoscopic OND technique is a safe and effective method in the hands of experienced surgeons with advanced endoscopic skills. A good collaboration with the opthalmology team is needed for the follow-up. Further studies with larger patient numbers is needed to compare unilateral endoscopic OND technique with the current techniques used in the surgical management of IIH.
- The Pseudotumor Cerebri Syndrome. [REVIEW]
- Neurol Clin 2014 May; 32(2):363-396.
The pseudotumor cerebri syndrome may be idiopathic or arise from a secondary cause. The major morbidity is visual loss, which may be severe and permanent. This article reviews the diagnosis, evaluation, and management of patients with pseudotumor cerebri syndrome in adults and children.
- Resolution of papilledema after endoscopic third ventriculostomy versus cerebrospinal fluid shunting in hydrocephalus: a comparative study. [JOURNAL ARTICLE]
- J Neurosurg 2014 Mar 28.
Object In this study the authors compare the efficacy of endoscopic third ventriculostomy (ETV) versus CSF shunting for resolution of papilledema in hydrocephalus. Methods This comparative case series study recruited 12 patients (24 eyes) with hydrocephalus who underwent either an ETV (Group 1, 6 patients [12 eyes]) or CSF shunt treatment (Group 2, 6 patients [12 eyes]). A complete ophthalmological examination including retinal nerve fiber layer (RNFL) evaluation by optical coherence tomography was provided for all patients before surgery and in the 1st week, 1st month, and 3rd month postoperatively. The 2 groups were compared for quantitative changes in RNFL thickness and, thereby, resolution of papilledema. Statistical evaluation was performed using the Mann-Whitney U-test with the aid of SPSS version 16.0. Results The mean preoperative RNFL thickness was 259.7 ± 35.8 μm in Group 1 and 244.5 ± 53.4 μm in Group 2 (p = 0.798). The mean decrease in RNFL thickness was 101.3 ± 38.8 μm, 141.2 ± 34.6 μm, and 162.0 ± 35.9 μm in Group 1 versus 97.0 ± 44.6 μm, 143 ± 45.6 μm, and 130.0 ± 59.8 μm in Group 2 for the postoperative 1st week, 1st month, and 3rd month, respectively. There was no significant difference between the two groups with respect to decrease in RNFL thickness during the 1st week, 1st month, and 3rd month (p = 0.563, p = 0.753, and p = 0.528, respectively). Conclusions This is the first study to quantitatively evaluate papilledema in assessing the success of ETV and CSF shunting. The authors' results indicated that ETV is as effective as CSF shunting with respect to decreasing intracranial pressure and resolution of papilledema.
- A173: Cerebrospinal Fluid Cytokines Correlate With Innate Immune Cells in Neonatal Onset Multisystem Inflammatory Disease (NOMID) Patients in Clinical Remission Treated With Anakinra. [Journal Article]
- Arthritis Rheumatol 2014 Mar.:S226.
Neonatal onset multisystem inflammatory disease (NOMID) is a rare autoinflammatory disease caused by NLRP3 mutations that lead to constitutive NLRP3 inflammasome activation and IL-1β release. Patients present with neutrophilic urticaria, chronic aseptic meningitis, and papilledema. IL-1β inhibitors improve the inflammatory manifestations, normalize acute phase reactants, decrease CSF WBC and protein, and proinflammatory cytokine levels. We found that cytokine levels decrease with anakinra treatment, but do not normalize during clinical remission. The higher IL-6 and IP-10 levels in CSF than in the blood suggest local production in the brain/CSF. Our objective is to examine whether CSF cytokines, particularly IL-6 and IP-10, correlate with CSF subpopulations of immune cells and CSF characteristics.Immunophenotyping and cytokine data were available on 2 baseline and 16 anakinra treated patients, 9/16, fulfilled clinical remission criteria (ESR ≤25 mm/hr, CRP ≤0.5 mg/dl, CSF WBC ≤5 cells/μl and protein ≤40 mg/dl) and control patients (n = 7). Cytokines levels (IL-1β, IL-6, IL-10, IP-10, IL-12 (p70) and (p40), IFN-γ, TNF-α, and IL-18) were correlated to CSF WBC, protein, albumin quotient (AQ), and opening pressure at baseline, post-treatment, and clinical remission. Correlation was assessed with Pearson and Spearman tests.At baseline, monocytes, granulocyte, and T cells were higher in NOMID patients versus controls. Post-treatment monocytes and granulocyte counts decreased but remained higher even in clinical remission, relative to control. Only IL-6 and IP-10 correlated with monocytes (r = .66, p = .051; r = .86, p = .002) respectively. A correlation of IP-10 but not IL-6 with granulocytes was weaker than with monocytes (r = .66, p = .058). IL-6, IP-10, and IL-18 correlated with CSF protein, AQ, and WBC (except IL-18). At baseline, IL-6 and IL-18 significantly correlated with CSF protein (r = .68, p = .027; r = .80, p = .0065) and AQ (r = .82, p = .011; r = .78, p = .029) respectively. At post-treatment, IL-18 significantly correlated and IL-6 trend towards significance with CSF protein (r = .53, p = .022; r = .40, p = .096) and AQ (r = .83, p = .0002; r = .47, p = .087) respectively.1) Inflammatory cells significantly decrease in NOMID patients, especially monocytes which remain elevated even in patients in clinical remission, suggesting residual inflammation. 2) The correlation of monocytes with IL-6 and IP-10 suggests their contribution to IL-6 and IP-10 production, which may contribute to the recruitment of other inflammatory cells including granulocytes. 3) IL-6, IP-10, and IL-18 may be a marker for blood brain barrier dysfunction in NOMID and their use in long term monitoring should be explored. 4) These results are consistent with the notion that NOMID is caused by dysfunction in innate immune cells, particularly monocytes.
- [Headache patient at the emergency call service]. [English Abstract, Journal Article]
- Duodecim 2014; 130(4):391-8.
Headache is a common symptom, sometimes concealing a severe illness. It is the primary task of the doctor on call to find out whether the headache is a symptom of serious incidence needing diagnosis urgently to prevent death or permanent disability. Dangerous headaches are associated with easily remembered warning signs: sudden onset, the first or worst headache in life, disturbance of consciousness in connection with the headache, onset at age over 50, worsening of headache in the lying position, a neurological deficit associated with the headache, or papilledema.
- Fifteen-minute consultation: the child with idiopathic intracranial hypertension. [JOURNAL ARTICLE]
- Arch Dis Child Educ Pract Ed 2014 Mar 25.
Idiopathic intracranial hypertension (IIH) is a rare condition where intracranial hypertension is found in the context of normal brain parenchyma and no mass lesion, ventriculomegaly, underlying infection, or malignancy. Our understanding of this condition has greatly improved in the recent years with neuroimaging features and normal values for lumbar puncture opening pressure now well defined. This article provides a review of IIH in children and revised diagnostic criteria based on recent evidence and published opinion. We have also presented an algorithmic approach to the child with possible IIH.
- Ocular Color-Coded Sonography - A Promising Tool for Neurologists and Intensive Care Physicians. [JOURNAL ARTICLE]
- Ultraschall Med 2014 Mar 19.
Ocular color-coded duplex sonography (OCCS), when performed within the safety limits of diagnostic ultrasonography, is an easy noninvasive technique with high potential for diagnosis and therapy in diseases with raised intracranial pressure and vascular diseases affecting the eye. Despite the capabilities of modern ultrasound systems and its scientific validation, OCCS has not gained widespread use in neurological practice. In this review, the authors describe the technique and main parameter settings of OCCS systems to reduce potential risks as thermal or cavitational effects for sensitive orbital structures. Applications of OCCS are the determination of intracranial pressure in emergency medicine, and follow-up evaluations of idiopathic intracranial hypertension and ventricular shunting by measuring the optic nerve sheath diameter. A diameter of 5.7 - 6.0 mm corresponds well with symptomatically increased intracranial pressure (> 20 cmH2O). OCCS also helps to discriminate between different etiologies of central retinal artery occlusion - by visualization of a "spot sign" and Doppler flow analysis of the central retinal artery - and aids the differential diagnosis of papilledema. At the end perspectives are illustrated that combine established ultrasound methods such as transcranial color-coded sonography with OCCS.
- Optical coherence tomography shows retinal abnormalities associated with optic nerve disease. [JOURNAL ARTICLE]
- Br J Ophthalmol 2014 Mar 13.
Optical coherence tomography (OCT) of the macula in patients with primary optic neuropathy has revealed the presence of structural changes in the neurosensory retina in addition to the nerve fibre layer. Subretinal fluid has been documented in papilloedema and non-arteritic ischaemic optic neuropathy, and may account for decreased visual acuity in affected patients. Subretinal fluid has also been described from other causes of optic nerve head swelling including diabetic papillopathy and papillitis. Drugs used in the treatment of multiple sclerosis, such as corticosteroids and fingolimod can cause decreased vision due to central serous and cystoid macular oedema sometimes confused with recurrent optic neuritis. A subset of patients with various types of optic atrophy show microcystic changes in the inner nuclear layer on spectral domain OCT imaging. The pathophysiology and visual significance of these retinal changes remain unclear, but may affect the diagnosis and management of optic nerve disorders.