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- Treatment of Idiopathic Intracranial Hypertension with Gastric Bypass Surgery. [JOURNAL ARTICLE]
- Int J Neurosci 2014 Feb 28.
Abstract Idiopathic intracranial hypertension (IIH) is defined by elevated intracranial pressure and associated headaches, changes in vision, and pulsatile tinnitus, among other symptoms. It occurs most frequently in young, obese women. Gastric bypass surgery has been used to treat morbid obesity and its comorbidities, and IIH has recently been considered among these indications. We present a case report of a 29 year-old female with a maximum BMI of 50.3 and a five-year history of severe headaches and moderate papilledema due to IIH. She also developed migraine headaches. After a waxing and waning course and various medical treatments, the patient underwent laparoscopic Roux-en-Y gastric bypass surgery with anterior repair of hiatal hernia. Dramatic improvement in IIH headaches occurred by 4 months post-procedure and was maintained at 1 year, when she reached her weight plateau with a BMI of 35. Pre-surgery migraines persisted. This adds to the small number of case reports and retrospective analyses of the successful treatment of IIH with gastric bypass surgery, and brings this data from the surgical literature into the neurological domain. It offers insight into an early time course for symptom resolution, and explores the impact of weight-loss surgery on migraine headaches. This treatment modality should be further investigated prospectively to analyze the rate of headache improvement with weight loss, the amount of weight loss needed for clinical improvement, and the possible correlation with improvement in papilledema.
- Two cases of bilateral amiodarone-associated optic neuropathy. [JOURNAL ARTICLE]
- J Fr Ophtalmol 2014 Feb 24.
The widespread use of amiodarone is limited by its toxicity, notably to the optic nerve. We report two cases of bilateral optic nerve neuropathy due to amiodarone, and provide a detailed description of the disease.The first case was a 59-year-old man complaining from insidious monocular loss of vision within ten months of initiating amiodarone. Funduscopy and optical coherence tomography showed bilateral optic disc edema. The second case was a 72-year-old man presenting with a decrease in visual acuity in his left eye for a month. Funduscopy showed a left optic nerve edema, and fluorescein angiography showed bilateral papillitis. In both cases, the clinical presentation was not suggestive of ischemic neuropathy, because of the preservation of visual acuity and the insidious onset. In addition, both cardiovascular and inflammatory work-up were normal. An amiodarone-associated neuropathy was suspected, and amiodarone was discontinued with the approval of the cardiologist, with complete regression of the papilledema and a stabilization of visual symptoms.Differentiating between amiodarone-associated optic neuropathy and anterior ischemic optic neuropathy may be complicated by the cardiovascular background of such patients. The major criterion is the absence of a severe decrease in visual acuity; other criteria are the normality of cardiovascular and inflammatory work-up, and the improvement or the absence of worsening of symptoms after discontinuation of amiodarone.Amiodarone-associated neuropathy remains a diagnosis of exclusion, and requires amiodarone discontinuation, which can only be done with the approval of a cardiologist, and sometimes requires replacement therapy.
- Unilateral Optic Disc Papilloedema following Administration of Carboplatin Chemotherapy for Ovarian Carcinoma. [Journal Article]
- Case Rep Oncol 2014 Jan; 7(1):29-32.
A 48-year-old woman with a positive BRCA1 gene mutation was diagnosed with stage 3b high-grade ovarian endometrioid carcinoma. She was treated with adjuvant carboplatin at a dose of 740 mg (AUC 6) in 3-weekly cycles. Five days after her fifth cycle of carboplatin, she awoke with new-onset blurred vision in her left eye. An ophthalmology review showed left-sided disc oedema with normal optic nerve function tests and 6/24 visual acuity. A CT scan of the head and orbits was performed which showed no evidence of metastasis or raised intracranial pressure. An autoimmune screen was performed which did not reveal any explanation for her visual symptoms. Fundus fluorescein angiography showed bilateral intense late disc leakage with no evidence of vasculitis. Her chemotherapy was stopped in view of a radiological and biochemical remission and her visual symptoms were monitored. She was also started on a tapering dose of prednisolone 40 mg daily. Five months after the initial review, she has developed left optic disc atrophy with 6/18 visual acuity, while the right eye remains asymptomatic. The diagnosis was felt to be that of carboplatin-induced unilateral disc oedema, a very rare side effect of this chemotherapy.
- Pseudopapilledema and association with idiopathic intracranial hypertension. [JOURNAL ARTICLE]
- Childs Nerv Syst 2014 Feb 27.
Diagnosing idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, can be challenging in children. Diagnosis is based on lumbar puncture, opening pressures, and appearance of the optic disk. Misdiagnosis of papilledema, a typical finding, may lead to unnecessary treatments and procedures. We report 52 children over a 6-year period to better identify the true incidence of pseudopapilledema and other factors that may confound the diagnosis of IIH.A retrospective chart review approved by the Institutional Review Board was performed. Fifty-two children under the age of 21 referred to us based on suspected IIH or papilledema from 2007 to 2013 are included in this study. Patients were assessed by a pediatric ophthalmologist and a neurosurgeon.Fifty-two children were initially diagnosed with IIH and/or papilledema; 26 diagnoses were revised to pseudopapilledema after pediatric ophthalmological review. Out of those 26 patients with pseudopapilledema, 14 had undergone lumbar punctures, 19 had MRIs, 9 had CTs, and 12 were taking medications-these medications were discontinued upon revision of the diagnoses. The difference in the CSF opening pressure between children diagnosed with true IIH (32.7 cm H2O) and children diagnosed with pseudopapilledema (24.7 cm H2O) was statistically significant.IIH diagnosis is heavily reliant on the appearance of the optic disk. Pediatric ophthalmological assessment is essential to carefully examine the optic disk and prevent further unnecessary investigation and treatments. Close communication between pediatricians, ophthalmologists, and neurosurgeons can avoid invasive procedures for children who do have pseudopapilledema, and not IIH or associated papilledema.
- Digoxin as a treatment for patients with idiopathic intracranial hypertension. [Journal Article]
- J Am Osteopath Assoc 2014 Mar; 114(3):213-5.
Idiopathic intracranial hypertension (IIH)-sometimes called pseudotumor cerebri-is a neurologic condition distinguished by any of the following symptoms: headache, increased cerebrospinal fluid pressure, papilledema, vision loss, diplopia, tinnitus, deafness, nausea and vomiting, or sixth nerve palsy. Medical and surgical management options are available for patients with IIH. The authors report a case of IIH that was successfully resolved with digoxin after standard avenues of therapy were exhausted.
- Optical Coherence Tomography Features of POEMS Syndrome and Castleman Disease-associated Papillopathy. [JOURNAL ARTICLE]
- Ocul Immunol Inflamm 2014 Feb 24.
Abstract Purpose: To report ocular symptoms and optical coherence tomography (OCT) features of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Methods: Case report of two Caucasian patients with POEMS, one of whom also had multicentric Castleman disease. Results: Both patients presented with edema on both optic disks, bilateral macular edema was seen in case 1, and peripapillary choroidal neovascularization was seen on the right side in case 2. OCT confirmed papilledema involving all retinal layers, with additional bilateral peripapillary serous retinal detachment involving the macula in both cases, and peripapillary CNV in case 2. Changes in retinal nerve fiber layer thickness, total peripapillary retinal thickness, and macular detachment were followed with OCT over the course of the disease and after administration of systemic therapy. Conclusion: OCT examination of the optic nerve head and the macula has an important additional role in the diagnosis and follow-up of POEMS syndrome and Castleman disease.
- Meningoceles in idiopathic intracranial hypertension. [Journal Article]
- AJR Am J Roentgenol 2014 Mar; 202(3):608-13.
OBJECTIVE.MRI abnormalities have been described in patients with increased intracranial pressure (ICP), including in those with idiopathic intracranial hypertension (IIH). Spontaneous CSF-filled outpouchings of the dura (meningoceles) and secondary CSF leaks can occur from elevated ICP in patients with IIH; however, few studies have evaluated these findings. Our objective was to evaluate the frequency of spontaneous intracranial meningoceles among IIH patients and determine their association with visual outcome. MATERIALS AND
METHODS.We performed a retrospective case-control study of consecutive IIH patients between 2000 and 2011 who underwent MRI that included T2-weighted imaging. Demographics, presenting symptoms, CSF opening pressure, and visual outcome were collected for the first and last evaluations. Control subjects included patients without headache or visual complaints who had normal brain MRI results. Stratified analysis was used to control for potential confounding by age, sex, race, and body mass index.
RESULTS.We included 79 IIH patients and 76 control subjects. Meningoceles were found in 11% of IIH patients versus 0% of control subjects (p < 0.003). Prominent Meckel caves without frank meningoceles were found in 9% of IIH patients versus 0% of control subjects (p < 0.003). Among IIH patients, the presence of meningocele or prominent Meckel caves was not associated with demographics, symptoms, degree of papilledema, CSF opening pressure, visual acuity, or visual field defect severity.
CONCLUSION.Meningoceles are significantly more common in IIH patients than in control subjects and can be considered an additional imaging sign for IIH. Meningoceles are not, however, associated with decreased CSF opening pressure or better visual outcome in IIH.
- POEMS syndrome: 2014 Update on diagnosis, risk-stratification, and management. [Journal Article]
- Am J Hematol 2014 Feb; 89(2):213-23.
Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the PCD is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. Risk-adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3-6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. The benefit of anti-VEGF antibodies is conflicting. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 89:214-223, 2014. © 2014 Wiley Periodicals, Inc.
- Combined Intracranial Pressure Monitoring and Cerebrospinal Fluid Infusion Study to Guide Management of Slit Ventricle Syndrome. [JOURNAL ARTICLE]
- Pediatr Neurosurg 2014 Feb 11.
Introduction: Slit ventricle syndrome remains a complex entity presenting a considerable challenge to treat successfully. This study aims to demonstrate the application of dual intracranial pressure (ICP) and infusion studies together with the novel shunt occlusion test in both a diagnostic and therapeutic role. Case Report: An 8-year-old child had aqueduct stenosis treated with a ventriculoperitoneal shunt (medium-pressure valve). The presentation was of headaches with papilloedema. Imaging with both computed tomography and magnetic resonance imaging revealed slit ventricles. Initially a shunt exploration revealed distal obstruction that was treated together with insertion of a paediatric strata II regular valve; however, the child continued to deteriorate. Overnight ICP monitoring revealed dramatically raised ICP with poor compensatory reserve. Intra-operative infusion study revealed a shunt that was patent distally but with proximal obstruction. A subtemporal decompression ipsilateral to the shunt was performed together with adjustment of the paediatric strata II regular valve to 2.5 in order to prevent overdrainage. This led to normalisation of ICP, resolution of papilloedema and symptomatic improvement. Discussion: We demonstrate how combined ICP monitoring and shunt infusion studies can be used to help guide management. Unilateral subtemporal decompressions and preventing shunt overdrainage can result in normalisation of ICP and cerebrospinal fluid dynamics. © 2014 S. Karger AG, Basel.
- Optical coherence tomography: a quantitative tool to screen for papilledema in craniosynostosis. [JOURNAL ARTICLE]
- Childs Nerv Syst 2014 Feb 12.
Our aim was to evaluate if optical coherence tomography (OCT) can be used as an alternative for fundoscopy to screen for increased intracranial pressure (ICP) in children with craniosynostosis METHODS: We performed a prospective cohort study at the Dutch Craniofacial Centre. We included 38 patients with nonsyndromic scaphocephaly and Crouzon's syndrome aged 3-8 years old, in whom we scored complaints suggestive of increased ICP and performed fundoscopy and OCT. Main outcome measures total retinal thickness (TRT) which was measured on 58 OCT scans.Forty-three percent of fundoscopies revealed pathologic changes of the papil in at least one eye. Retinal thickness was increased in patients with an abnormal fundoscopy as compared to patients with a normal papil (TRT p < 0.001). Patients with Crouzon's syndrome had a significantly increased retinal thickness as compared to patients with scaphocephaly (TRT p < 0.001).The current study demonstrates that OCT in children with craniosynostosis is feasible. It confirms that retinal thickness increases in case of papilledema. Given the quantitative character, OCT has a high potential as an alternative tool to screen for papilledema in craniosynostosis and other pediatric populations.