Visual field findings after a ruptured intracranial aneurysm.

BACKGROUND: Visual field defects (VFDs) negatively affect activities of daily living and rehabilitation following aneurysmal subarachnoid haemorrhage (aSAH). The aim here was to assess VFDs in patients with aSAH and their associations with age, gender, aSAH severity, and clinical outcome. METHODS: Patients admitted to Helsinki University Central Hospital and treated during 2011 were participants in this prospective study. Findings obtained with the Octopus 900 perimeter (Haag-Streit Inc, Koenic, Switzerland), the Goldmann perimeter (Haag-Streit Inc, Bern, Switzerland), or the confrontation visual field test on admission and 3 days, 14 days, 2 to 4 months, and 6 months postoperatively were assigned to 16 classes. Associations between post-chiasmal VFDs and relevant clinical, radiological, and demographic data were analysed with uni- and multivariate logistic regression. RESULTS: Of 105 survivors at 6 months, 20 (19 %) had VFDs occurring for aneurysm- or operation-related reasons; homonymous hemianopias or quadrantanopias were the most common finding, occurring in 16 patients (15 %). Posterior ischaemic optic neuropathy presented in two patients (2 %). Ten survivors (10 %) no longer fulfilled visual field requirements for driving licences. Significant associations emerged between VFDs at 6 months and the Hunt and Hess (H&H), World Federation of Neurosurgical Societies (WFNS), and Fisher grades on admission, presence of intracerebral haemorrhage (ICH), hydrocephalus, or postoperative infarction, and higher modified Rankin Scale scores at 6 months. Multivariate logistic regression showed the H&H grade and presence of ICH to independently predict VFDs. CONCLUSIONS: Assessing VFDs is advisable, especially among patients with poor-grade aSAH (H&H grade IV or V) and ICH.

Outcome of fractionated stereotactic radiotherapy in patients with pituitary adenomas resistant to conventional treatments: a 5.25-year follow-up study.

OBJECTIVE: To investigate the long-term outcome of fractionated stereotactic radiotherapy (FSRT) [45 Gy (range 45-54) in 25 fractions] in patients with pituitary adenomas characterized by tumour progression or hormonally active disease despite surgery and/or medical therapy. DESIGN: This was an observational follow-up study of 5.25 years (median; range 1.7-10.4). PATIENTS AND MEASUREMENTS: Pituitary tumour volume, visual acuity/fields, hypersecretion, hypopituitarism, cerebrovascular disease, second brain tumours and mortality were examined at regular intervals after FSRT in 30 patients with pituitary adenomas (20 nonfunctioning macroadenomas, 10 functioning). Prior to FSRT, 83% had been operated 1-3 times, 47% had visual field deficits/impaired vision and 50% pituitary dysfunction. Progressive disease, stable disease, partial and complete tumour response were defined by MRI. RESULTS: Tumour growth control was 100%. At the end of follow-up, 30% had stable disease, 60% partial and 10% complete tumour response. Visual function was preserved and 36% of patients with prior field deficits improved. GH decreased from 4.2 (range, 2.3-6.5) to 1.1 (range, 0.5-1.5) microg/l (P < 0.001) in patients with acromegaly, and medical therapy could be reduced. In large prolactinomas, partial response or complete tumour response was achieved. FSRT was well tolerated. Pituitary function remained normal in 27%, 33% of patients had stable dysfunction, 17% deteriorated further and 23% developed new dysfunction. There were no cerebrovascular events, second brain tumours or FSRT-related deaths. CONCLUSION: According to this long-term follow-up study, FSRT is an efficient and safe adjuvant therapy for pituitary adenomas refractory to conventional treatments.

[It is worthwhile to examine the optic disc]. / Näköhermon papillan tutkiminen.

Ophthalmoscopy is sometimes very difficult, especially if special ophthalmologic equipments are not available. However, the examination of the optic disc is a skill a general practitioner needs. The use of mydriatics makes it much easier. Examination of the optic disc head is essential when increased intracranial pressure is suspected. Diabetes and systemic hypertension are also diseases in which ophthalmoscopy is needed.

The clinical spectrum and prognosis of idiopathic acute optic neuritis: A longitudinal study in Southern Finland.

BACKGROUND: To analyse in a population-based setting the clinical features, prognostic factors, and seasonality of patients diagnosed with acute idiopathic optic neuritis (ON). METHODS: Retrospective analysis of ophthalmological records, laboratory parameters, and magnetic resonance imaging (MRI) of patients with symptoms suggestive of ON referred to the Helsinki University Hospital (serving a population of 1.53 million in Southern Finland) were analysed between May 1, 2008 and April 14, 2012. RESULTS: Of the 291 patients with suspected ON, 184 (63%) were diagnosed with ON (mean age 34 years, 76% females). Intravenous methylprednisolone treatment was administered in 131 (71%) patients. First ON was diagnosed in 123 patients (67%), 55 (30%) had a previous diagnosis of multiple sclerosis (MS) and two patients with their first ON were diagnosed with neuromyelitis optica. Evolution of best corrected visual acuity (BCVA) was analysed in 132 (72%) patients, who were reviewed median of 38 days after onset. Median and mean BCVAs in these reviewed patients were 0.4 and 0.2 at the time of diagnosis and 1.0 and 0.5 at the time of the review. Recovery was relatively good in the majority of patients; 82% (n = 108) had reached BCVA of ≥0.5 and 70% (n = 92) and BCVA of ≥0.8 at the time of the review, while thirteen (10%) had poor prognosis, BCVA ≤0.1 at review. Accessory clinical features included optic disc swelling (21%), colour vision impairment (75%), and pain with eye movements (65%). Relative afferent pupillary defect was abnormal in 76% of the patients with their first ON. Baseline visual acuity was most strongly associated with visual outcome at review (P < 0.001, linear regression). Optic disc swelling and the presence of lesions in the optic nerve on MRI had a more modest association with poorer recovery (P = 0.033 and P = 0.049, respectively), while age, sex, previous history of ON, and previous diagnosis of multiple sclerosis were not associated with outcome at review. Incidence of ON showed a clear seasonal pattern; there were two times more cases in April to June versus October to December (P = 0.03), confirming previous results from Sweden. CONCLUSIONS: Our data suggest that besides baseline visual acuity, optic disc swelling and lesions in the optic nerve on MRI are associated with poorer prognosis. As in previous studies, we observed that diagnostics of ON is difficult, accessory clinical findings such as pain and RAPD are not always present. Although the diagnosis of ON is clinical, the role of MRI should be considered in differential diagnostics and in defining potential prognostic markers.

Incidence and Mimickers of Acute Idiopathic Optic Neuritis: Analysis of 291 Consecutive Patients from Southern Finland.

PURPOSE: To estimate the population-based incidence of acute idiopathic optic neuritis (ON) and analyse its differential diagnosis in patients referred with symptoms suggestive of ON. METHODS: Patients with suspected ON referred to the Helsinki University Hospital, serving a population of 1.5 million in Southern Finland, were reviewed between 1st May 2008 and 14th April 2012. Brain and optic nerve magnetic resonance imaging (MRI) was performed within 24 hours in 83% of patients. RESULTS: Of 291 referred patients, 184 (63%; 95% confidence interval [CI], 57-69%) were diagnosed with ON whereas 107 (37%) had another condition. The estimated crude incidence of ON in Southern Finland was 3.0 (95% CI 2.8-3.3) per 100,000 (females, 4.6 and males, 1.4). Mean age was 34 years (range 15-61), 76% were female. Two (1%) were diagnosed with neuromyelitis optica. ON as the first demyelinative episode was diagnosed in 108 (59%) patients, and MRI showed demyelinating lesions (MRI+) in 82% (95% CI, 75-89) of them. MRI+ predicted the development of multiple sclerosis (MS): 54% of MRI+ vs. 5% MRI- patients were diagnosed as MS during a mean follow-up of 7.7 years. The most common differential diagnosis was non-arteritic anterior ischemic optic neuropathy (12%). Six (2%) intracranial compressive lesions were found upon MRI scan. CONCLUSIONS: More than a third of patients with symptoms suggestive of ON had another condition. Demyelinative lesions on MRI indicated higher risk of developing MS. We recommend the use of MRI to improve the differential diagnostic accuracy of ON and to identify patients with high risk of MS.

One hundred-year-old human corneal and transplanted corneal graft endothelium.

PURPOSE: To investigate and analyse one hundred-year-old endothelial cells of human transplanted corneal grafts and compare them with intact endothelium of unoperated eyes. SUBJECTS AND METHODS: Specular microscopy was performed on seven individuals (14 eyes) aged >100 years and on seven transplanted corneas with endothelial cells of at least 100 years old. All keratoplasties were performed by the same surgeon (PR) 25-35 years before the time of examination. As controls, 14 unoperated healthy individuals (26 eyes) of 90-100 years, 12 individuals (24 eyes) of 48-52 years, 12 students (24 eyes) of 19-24 years and 11 children (22 eyes) from 10 to 14 years of age were photographed and analysed. RESULTS: The mean cell density in the oldest age group, over 100 years of age (average 102 years), was very good 2365 cells/mm(2) . The mean cell density in the transplanted corneas, the endothelial cells of which were over 100 years old (mean 106 years), was 923 cells/mm(2) (range 585-1800 cells/mm(2) ). CONCLUSION: Corneal endothelial cells may remain viable and capable to perform their function over 100 years. Donor corneas up to 70-75 years of age can be approved for transplantation, provided that the endothelial cells have been thoroughly evaluated with vital staining before the operation.

Neuro-ophthalmic presentation and surgical results of unruptured intracranial aneurysms­prospective Helsinki experience of 142 patients.

OBJECTIVE: To assess prospectively neuro-ophthalmic findings associated with unruptured intracranial aneurysms and treatment morbidity and to identify factors predicting these findings. METHODS: Patients admitted to Helsinki University Central Hospital and treated surgically or endovascularly during 2011 underwent a neuro-ophthalmic examination, including formal visual field testing, before operation, at discharge, and 2-4 months and ≥6 months postoperatively. Univariate and multivariate analysis was used to identify factors predicting eye movement disorders. RESULTS: Study participants included 142 patients with 184 treated aneurysms: 7 (5%) had a third, fourth, or sixth nerve palsy or skew deviation preoperatively, and 16 (11%) had a third, fourth, or sixth nerve palsy or skew deviation postoperatively; the frequency was 8 (6%) at the last follow-up evaluation. Other findings included compressive optic neuropathy (n = 4), ischemic optic neuropathy (n = 1), Weber syndrome (n = 3), Benedikt syndrome (n = 1), and Wallenberg syndrome (n = 1). Of the 140 survivors at 6 months, 7 (5%) presented with visual field defects resulting from the aneurysm or its treatment. In the best bivariate model, factors independently predicting postoperative eye movement disorders were aneurysm location in the posterior circulation with an odds ratio of 142.02 (95% confidence interval = 20.13-1002.22) and aneurysm size (odds ratio = 1.28 for each 1-mm increase in diameter, 95% confidence interval = 1.12-1.47). CONCLUSIONS: Management of unruptured intracranial aneurysms is fairly safe from a neuro-ophthalmic perspective, with some treatment-related morbidity being transient and minor. Although rare, an irreversible deficit is possible and should be taken into account when considering preventive treatment.

Exome and regulatory element sequencing of neuromyelitis optica patients.

Neuromyelitis optica (NMO) is rare in Finland. To identify rare genetic variants contributing to NMO risk we performed whole exome, HLA and regulatory region sequencing in all ascertained cases during 2005-2013 (n=5) in a Southern Finnish population of 1.6 million. There were no rare variant shared by all patients. Four missense variants were shared by two patients in C3ORF20, PDZD2, C5ORF47 and ZNF606. Another PDZD2 variant was found in a third patient. In the non-coding sequence two predictably functional rare variants were shared by two patients. Our results do not support a homogeneous genetic etiology of NMO in Finland.

Eye movement abnormalities after a ruptured intracranial aneurysm.

OBJECTIVE: Overlooking eye movement abnormalities associated with aneurysmal subarachnoid hemorrhage (aSAH) is common, although these abnormalities may greatly affect quality of life. Their prevalence remains undetermined. The aim of the study was to assess preoperative and postoperative eye movement abnormalities and their recovery in follow-up of patients with aSAH and their association with age, gender, and aSAH severity. METHODS: Patients admitted to Helsinki University Central Hospital who underwent surgery or endovascular treatment for a ruptured intracranial aneurysm during 2011 were participants in this prospective study. A neuro-ophthalmic examination was performed on admission, and 3 days, 14 days, 2-4 months, and 6 months postoperatively. For those patients with third, fourth, or sixth nerve palsies or brainstem vascular syndromes, follow-up was 12 months. Associations between neuro-ophthalmic findings and relevant clinical, radiologic, and demographic data were studied. Two intraoperative videos were selected to show rare cases of aneurysms causing cranial nerve palsies. RESULTS: Of 121 participants, 11 (9%) presented on admission and 16 (13%) postoperatively showed signs of third, fourth, or sixth nerve palsy. Most of these palsies resolved; leaving 2.5% of all patients presenting with a partial palsy at 1 year. We also evaluated the frequencies of horizontal gaze pareses (n = 9) and Parinaud's syndromes (n = 3). No statistically significant associations emerged between neuro-ophthalmic findings and other clinical variables. CONCLUSIONS: Eye movement abnormalities are a quite common finding in the acute stage of aSAH. Within 1 year, however, marked improvement occurs. Identifying these neuro-ophthalmic findings can assist in localization of the underlying pathology.

Transition From Microscopic to Endoscopic Transsphenoidal Surgery for Nonfunctional Pituitary Adenomas.

OBJECTIVE: At our institution, a total of 320 patients were operated on between 2000 and 2010 for a newly diagnosed pituitary adenoma. In an attempt to improve quality of tumor resection, the transsphenoidal microscopic technique was replaced by the endoscopic technique in June 2008. This retrospective single center study compares the outcomes after microscopic (n = 144) and endoscopic (n = 41) tumor surgery of all patients operated on for a nonfunctional pituitary adenoma. METHODS: Tumor size and location, Knosp grade, prevalence of anterior hypopituitarism, diabetes insipidus, visual acuity/fields, complication rates, and operation time were compared between the groups. RESULTS: At the 3-month follow-up, hypopituitarism had improved in 7% of patients in the microscopic group and in 9% in the endoscopic group, and had further impaired in 13% and 9%, respectively. At the 3-month follow-up magnetic resonance imaging, a total tumor removal was achieved in 45% versus 56% of patients, respectively (P = not significant [NS]). Visual fields had normalized or improved in 90% versus 88% of patients, respectively (P = NS). Postoperative cerebrospinal fluid leak occurred in 3.5% versus 2.4% (P = NS), and diabetes insipidus (transient or permanent) in 7.6% versus 4.9% (P = NS) of cases, respectively. Larger tumor size (P < 0.0005) and endoscopic technique (P = 0.03) were independent predictors of increased mean operative time. CONCLUSIONS: Initial results with the endoscopic technique were statistically similar to those achieved with the microscopic technique. However, there was a trend toward improved outcomes and fewer complications in the endoscopic group.

Echovirus type 4 as a probable cause of meningitis associated with bilateral optic neuritis: a case report.

A 30-year-old man developed bilateral optic neuritis 1 week after acute viral meningitis caused by echovirus type 4. He received a high-dose steroid treatment combined with intravenous gamma -globulin. His visual recovery was good, and there was no sign of a primary demyelinating disease.

Comparison of CT and clinical findings of Terson's syndrome in 121 patients: a 1-year prospective study.

OBJECT: Terson's syndrome (TS) is a vitreous hemorrhage in association with subarachnoid hemorrhage (SAH). Its diagnosis is often delayed, which may result in vision loss secondary to treatable conditions. Methods to hasten early diagnosis and consequent ophthalmic referral are desirable. The aims of this study were 1) to assess the specificity and sensitivity of conventional head CT for diagnosing TS in patients with aneurysmal SAH (aSAH); and 2) to determine the incidence of TS and its association with age, sex, aSAH severity, and overall mortality. METHODS: Patients admitted to Helsinki University Central Hospital who underwent surgery or endovascular treatment for a ruptured intracranial aneurysm during 2011 were participants in this prospective study. They underwent serial dilated fundoscopic examinations during a 6-month period. Two radiologists independently reviewed ocular findings suggestive of TS on conventional CT head scans obtained in all patients as a routine diagnostic procedure. Associations between TS and relevant clinical, radiological, and demographic data were analyzed with uni- and multivariate logistic regression. RESULTS: Of 121 participants, 13 (11%) presented with TS, and another 22 (18%) with intraretinal hemorrhages. For reviewing CT head scans, the overall observed agreement between the 2 raters was 96% (116 of 121 cases), with a substantial κ of 0.69 (95% CI 0.56-0.82). The sensitivity of the CT findings for TS was 42%, and the specificity was 97%. Associations of the World Federation of Neurosurgical Societies (WFNS) and Hunt and Hess grades on admission, the presence of intracerebral hemorrhage, female sex, and aneurysm length with TS were all statistically significant. Logistic regression demonstrated that sex and WFNS grade were independently associated with TS and provided the best fit to the data. CONCLUSIONS: Routinely looking for TS findings in CT head scans may prove valuable in clinical practice. Terson's syndrome is associated with female sex and poor clinical condition on admission.

Neuromyelitis optica and aquaporin-4 (AQP4) autoantibodies in consecutive optic neuritis patients in Southern Finland.

PURPOSE: To analyse the frequency of neuromyelitis optica (NMO) among consecutive optic neuritis (ON) patients in Southern Finland and the feasibility of Aquaporin-4 (AQP4) autoantibody assay in the diagnosis of NMO. METHODS: Consecutive patients with symptoms suggestive of acute ON and managed in the Helsinki University Central Hospital were evaluated critically screened for AQP4 autoantibody during a 47.5-month period. The antibodies were determined using radioimmunoprecipitation method. AQP4 index >15 was considered positive, 10-15 borderline and <10 normal. Brain magnetic resonance imaging (MRI) was performed for all patients. RESULTS: Of the 300 patients with suspected ON, 191 were eventually diagnosed as ON, and 66 (35%) of them had a previous diagnosis or were diagnosed with multiple sclerosis (MS). Of the 125 patients without MS diagnosis, 62 (50%) had demyelinative lesions in MRI, which is a risk factor for developing MS. Two patients (1.1%; 95% CI 0.3-4.5) fulfilled the criteria of NMO. Positive AQP4 antibodies were found in three patients (1.6% 95% CI 0.3-4.5), one of them had NMO, one had MS and one became diagnosed with MS a month later. Borderline autoantibody levels were found in 10 patients, 7 of whom had MS. CONCLUSIONS: NMO is rare among ON patients in the population of Southern Finland. In this small cohort, the sensitivity and positive predictive values of the AQP4 autoantibody index for NMO were low, 1/2 and 1/3 respectively, and do not support initiating routine screening.

Long-term visual outcome after microsurgical removal of occipital lobe cavernomas.

OBJECT: Cavernomas in the occipital lobe are relatively rare. Because of the proximity to the visual cortex and incoming subcortical tracts, microsurgical removal of occipital cavernomas may be associated with a risk of visual field defects. The goal of the study was to analyze long-term outcome after operative treatment of occipital cavernomas with special emphasis on visual outcome. METHODS: Of the 390 consecutive patients with cavernomas who were treated at Helsinki University Central Hospital between 1980 and 2011, 19 (5%) had occipital cavernomas. Sixteen patients (4%) were surgically treated and are included in this study. The median age was 39 years (range 3-59 years). Seven patients (56%) suffered from hemorrhage preoperatively, 5 (31%) presented with visual field deficits, 11 (69%) suffered from seizures, and 4 (25%) had multiple cavernomas. Surgery was indicated for progressive neurological deterioration. The median follow-up after surgery was 5.25 years (range 0.5-14 years). RESULTS: All patients underwent thorough neuroophthalmological assessment to determine visual outcome after surgery. Visual fields were classified as normal, mild homonymous visual field loss (not disturbing the patient, driving allowed), moderate homonymous visual field loss (disturbing the patient, driving prohibited), and severe visual field loss (total homonymous hemianopia or total homonymous quadrantanopia). At the last follow-up, 4 patients (25%) had normal visual fields, 6 (38%) had a mild visual field deficit, 1 (6%) complained of moderate visual field impairment, and 5 (31%) had severe homonymous visual field loss. Cavernomas seated deeper than 2 cm from the pial surface carried a 4.4-fold risk of postoperative visual field deficit relative to superficial ones (p = 0.034). Six (55%) of the 11 patients presenting with seizures were seizure-free postoperatively. Eleven (69%) of 16 patients had no disability during the long-term follow-up. CONCLUSIONS: Surgical removal of occipital cavernomas may carry a significant risk of postoperative visual field deficit, and the risk is even higher for deeper lesions. Seizure outcome after removal of these cavernomas appeared to be worse than that after removal in other supratentorial locations. This should be taken into account during preoperative planning.

Efficacy and safety of orbital decompression in treatment of thyroid-associated ophthalmopathy: long-term follow-up of 78 patients.

OBJECTIVE: To study the long-term results of decompression surgery in patients with thyroid-associated ophthalmopathy (TAO). DESIGN AND METHODS: Retrospective analysis and clinical re-examination of TAO patients with comparison to healthy controls in Helsinki University Central Hospital. PATIENTS: Seventy-eight patients who had undergone orbital decompression due to TAO by transantral or endonasal technique between the years 1985 and 2000 were invited for re-examination. For comparison, 79 healthy age- and sex-matched controls underwent a similar examination. RESULTS: In comparison to the patients' preoperative state, proptosis was reduced by 4.7 (2.6) mm (mean (SD)) in the right and 4.4 (2.9) mm in the left globe (P < 0.0001) but did not reach the level of the controls' globe (P < 0.0001). In the right and left eye of patients, visual acuity improved in 44-55%, remained stable in 27-36% and worsened in 18-20%, but remained significantly worse than in controls (P =0.02-0.001). Prevalence of diplopia was high at the re-examination (N = 39, 50%), but 54% of those patients considered it less difficult than before the surgery. Among patients and controls, maxillary sinusitis and facial neuralgias were equally common. Sensory disturbances were significantly more frequent in patients (P < 0.0001). In assessment of overall satisfaction with present eye status, the median of the visual analogue scale (VAS) was lower for the patients than the controls (P < 0.0001). The majority of the patients considered the operation very helpful regardless of the technique used. CONCLUSION: For patients with TOA, orbital decompression seems to be an effective and safe treatment.

Neuroimaging supports the clinical diagnosis of methanol poisoning.

In addition to visual loss, methanol intoxication can cause brain damage that is revealed by neuroimaging. We report on a 34-year-old man whose visual acuity deteriorated dramatically during his journey round the world, shortly after an evening with excessive alcohol consumption. Two months after the start of visual disturbances he returned to Finland. At examination, poor visual acuity and glaucomatously cupped pale optic discs were detected. The history and clinical findings indicated possible methanol intoxication, which was supported by both CT and MRI. The MRI findings are discussed in the light of the MRI appearance of the pallidum nuclei among patients of different age groups.

High dose intravenous methylprednisolone pulse therapy versus oral prednisone for thyroid-associated ophthalmopathy.

PURPOSE: To compare the effectiveness of intravenous (i.v.) methylprednisolone pulse therapy and oral prednisone when used as the initial treatment of patients with mild or moderate thyroid-associated ophthalmopathy. METHODS: Thirty-three consecutive patients with thyroid-associated ophthalmopathy in Helsinki and Turku University Hospitals were randomly assigned either i.v. methylprednisolone pulse therapy (group A, n = 18) or oral prednisone (group B, n = 15). Treatment outcomes were measured by subjective changes in the grade of diplopia and quantitatively in several ophthalmic variables at 3 and 12 months. Any decision to proceed with additional treatment at 3 months was made on clinical grounds. The study was open in respect of both the initial treatment and the need for additional therapy. RESULTS: No significant differences in the grade of diplopia, proptosis or soft tissue activity scores were noted between groups A and B from 0 to 3 months. However, group A required additional forms of therapy at 3 months less frequently than did group B (p = 0.038). CONCLUSIONS: Our data suggest that i.v. methylprednisolone pulse therapy and oral prednisone are equally effective as initial treatments for thyroid-associated ophthalmopathy where diplopia, proptosis and signs of soft tissue inflammation are concerned. When additional treatment is required, i.v. methylprednisolone pulse therapy may be more effective than oral prednisone. However, the study's limitations meant that any decision to give additional treatment after the initial therapy was made on clinical grounds.