The incidence of idiopathic intracranial hypertension in Israel from 2005 to 2007: results of a nationwide survey.

BACKGROUND AND PURPOSE: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder related to increased intracranial pressure without clinical, laboratory or radiological evidence of an intracranial space occupying lesion or cerebral sinus vein thrombosis, predominantly affecting obese women of childbearing age. Our aim was to determine the incidence and clinical features of IIH in Israel. METHODS: In a cross-sectional study, all medical records of patients discharged from the hospital with a primary diagnosis of IIH during 2005-2007 were reviewed. RESULTS: Four hundred and twenty-eight patients with a new onset of IIH were diagnosed. The average annual incidence rate was 2.02 per 100,000 with an incidence of 3.17 per 100,000 for women and 0.85 per 100,000 for men. The incidence rate in females of childbearing age (18-45) was 5.49 per 100,000. The female to male ratio for >17 years old was 6.1:1 (252 females and 41 males) and 2.1:1 (60 females and 28 males) for ages 11-17. Obesity was documented in 83.4% of patients. Body mass index (BMI) data were available for 159 (37.1%) patients; of these, 59.1% had a BMI ≥ 30. CONCLUSION: The incidence of IIH in Israel has increased during the last decade. This finding could be related to the increasing rates of obesity. The association of IIH and obesity should be further explored especially with regard to the effect of weight reduction for primary prevention.

Asymptomatic idiopathic intracranial hypertension in children.

OBJECTIVE: To define characteristics of pediatric asymptomatic idiopathic intracranial hypertension (IIH). PATIENTS AND METHODS: We retrospectively reviewed our Neuro-Ophthalmology database (2000-2006) for all cases of symptomatic and asymptomatic pediatric IIH. RESULTS: Out of 45 IIH cases, 14 (31.1%) were asymptomatic (incidental examination). When compared with children with symptomatic IIH, asymptomatic cases were younger [5.6 (1.8-15) vs 11.0 (5-17) years, P = 0.007], had lower percentage of obesity (14.3% vs 48.4%, P = 0.046), and had male predominance (71.4% vs 38.7%, P = 0.06). Asymptomatic cases required shorter duration of acetazolamide treatment [3 (0-8), vs 6 (0-20) months, P = 0.021], and resulted in complete resolution of swollen discs. CONCLUSIONS: We speculate that asymptomatic IIH may be more common in young children and could represent a milder form or a presymptomatic phase before evolving into classic symptomatic IIH. Further studies to assess the clinical significance of asymptomatic IIH are warranted.

Idiopathic intracranial hypertension after 40 years of age: clinical features in 23 patients.

PURPOSE: Idiopathic intracranial hypertension (IIH) is a well-recognized disorder of unknown etiology associated with elevated intracranial pressure (ICP), normal neuroimaging, and normal cerebrospinal fluid (CSF) composition, found mostly among obese females of childbearing age. The aim of this study is to investigate the clinical features of IIH in patients older than 40 years. METHODS: This is a retrospective chart review (1998-2007) of all consecutive patients older than 40 years who were diagnosed with IIH based on the Modified Dandy Criteria. RESULTS: Twenty three of the 200 IIH patients in the institutional database fulfilled study entry criteria. They included 22 females and one male whose mean age was 51.4 years (range 41-79). Coexisting systemic arterial hypertension was found in 13 (56.5%) patients. Seventeen patients (73.9%) had symptoms attributable to elevated ICP. The most common presenting symptoms were visual; mainly blurred vision and transient visual obscuration (15/17 patients, 88.23%). Eleven patients (64.7%) complained of headache, and another 4 (23.5%) of pulsatile intracranial noise. The average follow up period was 21.8 months. CONCLUSIONS: The findings indicate that IIH among individuals older than 40 years of age may be underreported. These patients are more likely to present with systemic hypertension and with more visual disturbances, but with fewer complaints of headache than the younger IIH population.

Tuberculum and diaphragma sella meningioma--surgical technique and visual outcome in a series of 20 cases operated over a 2.5-year period.

BACKGROUND: A retrospective analysis of 20 cases of tuberculum sella meningioma with emphasis on the surgical technique and visual outcome. METHODS: Between 2003 and 2006 twenty patients with tuberculum and diaphragma sella meningioma were treated at the Tel Aviv medical center. There were 17 females and 3 males. The age range was 28-83. Most patients presented with visual deterioration. Surgery was performed using the subfrontal approach. The visual function before and after surgery was evaluated as the main outcome parameter of the surgical treatment of these tumours. FINDINGS: In 16 patients complete tumour resection was achieved and in 4 subtotal removal was performed. Visual acuity improved in 32% of the eyes and deterioration was observed in two eyes (5%). Visual field improved in 28% of the eyes and deteriorated in 14%. There was no complete vision loss as a result of surgery. There was no mortality in our series. CONCLUSIONS: Tuberculum and diaphragma sella meningioma can be safely resected using the subfrontal approach with preservation and even improvement of visual function after surgery. Early surgery with better pre-operation visual function and smaller tumour size were associated with a better outcome.

Automated Cross-Sectional Measurement Method of Intracranial Dural Venous Sinuses.

BACKGROUND AND PURPOSE: MRV is an important blood vessel imaging and diagnostic tool for the evaluation of stenosis, occlusions, or aneurysms. However, an accurate image-processing tool for vessel comparison is unavailable. The purpose of this study was to develop and test an automated technique for vessel cross-sectional analysis. MATERIALS AND METHODS: An algorithm for vessel cross-sectional analysis was developed that included 7 main steps: 1) image registration, 2) masking, 3) segmentation, 4) skeletonization, 5) cross-sectional planes, 6) clustering, and 7) cross-sectional analysis. Phantom models were used to validate the technique. The method was also tested on a control subject and a patient with idiopathic intracranial hypertension (4 large sinuses tested: right and left transverse sinuses, superior sagittal sinus, and straight sinus). The cross-sectional area and shape measurements were evaluated before and after lumbar puncture in patients with idiopathic intracranial hypertension. RESULTS: The vessel-analysis algorithm had a high degree of stability with <3% of cross-sections manually corrected. All investigated principal cranial blood sinuses had a significant cross-sectional area increase after lumbar puncture (P ≤ .05). The average triangularity of the transverse sinuses was increased, and the mean circularity of the sinuses was decreased by 6% ± 12% after lumbar puncture. Comparison of phantom and real data showed that all computed errors were <1 voxel unit, which confirmed that the method provided a very accurate solution. CONCLUSIONS: In this article, we present a novel automated imaging method for cross-sectional vessels analysis. The method can provide an efficient quantitative detection of abnormalities in the dural sinuses.

Pupil dilation using drops vs gel: a comparative study.

PURPOSE: To compare the efficacy in pupil dilation and degree of discomfort between topical instillation of mydriatic drops and gel. METHODS: The study included 60 patients with no previous ocular history of trauma and surgery. One eye was dilated with two drops (tropicamide 0.5% and phenylephrine 10%), and the other with one drop of gel (tropicamide 0.5%+phenylephrine 5%). Pupil size was measured by a Colvard pupillometer at baseline and 5, 15, 30, and 45 min following instillation. Pain upon instillation was measured by visual analog scale (VAS). RESULTS: There was no difference in pupil size at baseline. Use of the gel achieved greater mydriasis than drops (P=0.01), and was also associated with lower pain scores (P=0.003). In diabetic patients, pupil size was smaller at baseline and following instillation of drops and gel. Use of the gel achieved an even greater degree of pupil dilation in this subset of patients than drops (P=0.019). CONCLUSIONS: Gel formulation achieved significantly greater pupil dilation than drops, despite a lower concentration of phenylephrine, and was also associated with significantly lower patient discomfort. This study is the first report of improved mydriatic efficacy in diabetic patients.

Melkersson-Rosenthal syndrome treated by methylprednisolone.

Melkersson-Rosenthal syndrome is an uncommon disorder of uncertain etiology. Different therapeutic regimens have been attempted but with limited success. We report a 24-year-old woman with recurrent facial and lip swelling, lingua plicata, and peripheral facial-nerve palsy who benefited from a short course of high-dose i.v. methylprednisolone.

Kleine Levin syndrome (KLS) in young females.

During the years 1982-1998, we encountered 7 adolescents and one young woman suffering from KLS. In 4 patients, hypersomnolence was accompanied by hyperphagia and hypersexuality, while in the remaining 4, recurrent hypersomnia was the only symptom. Mean age at onset of hypersomnolent attacks was 15.1+/-3.5 yrs. The mean duration of a hypersomnolent attack was 9.9+/-5.4 days, and the number of attacks per patient was 6.2+/-3.4. Polysomnographic recordings from 3 patients inbetween attacks, and from one patient during an attack, showed relatively normal sleep structure with decreased sleep efficiency due to numerous awakenings from sleep stage 2. Besides the recurrent hypersomnia, all patients enjoyed good health, with no evidence of behavioral or endocrine dysfunction. Similarly aged males with KLS from our clinic and previously reported females, had similar clinical features.

Benign intracranial hypertension: correlation of cerebral blood flow with disease severity.

UNLABELLED: Benign intracranial hypertension (BIH) is characterized by symptoms and signs of raised intracranial pressure in the absence of an intracranial mass lesion, infection or hydrocephalus. The purpose of this study was to evaluate the effect of disease severity on cerebral blood flow in patients with BIH on acetazolamide therapy. METHODS: 11 patients (nine females, two males; mean age 30.5 years; range 22-29 years) with BIH were studied. All patients underwent CT and MRI scanning which were normal. The CSF pressure of all patients was above 200 mm H2O. All patients were under treatment with acetazolamide (1 g/day). Disease severity was determined by visual field examination and by clinical symptoms. Five patients were categorized into mild to moderate BIH (group I) and six patients had severe BIH (group II). All patients underwent perfusion brain SPECT with 740 MBq of Tc-99m-HMPAO. RESULTS: Brain perfusion abnormalities were observed in six of the 11 patients. One out of five patients in group I (20%) and five out of six patients (83%) in group II, had abnormal SPECT findings (P<0.04). In four patients of group II the left parietal lobe was involved and another patient had a right occipital abnormality. The single patient from group I with SPECT abnormalities demonstrated focal decreased perfusion in the left temporal area and decreased perfusion in the left caudate nucleus. CONCLUSION: Patients with severe degree of BIH have a higher incidence of cerebral perfusion abnormalities. This group may have an increased risk of cerebrovascular complications. The continuous administration of acetazolamide which affects the vascular autoreactivity may contribute to the regional hypoperfusion. Further studies are recommended to evaluate the natural course of disease versus iatrogenic treatment effects.

Fever in various rheumatic diseases.

In many rheumatic diseases fever may occur. This review presents data about the frequency of fever in rheumatoid arthritis, juvenile rheumatoid arthritis, systemic lupus erythematosus, crystal induced arthritis, various vasculitides and sarcoidosis. The mechanism by which body temperature rises in these situations is discussed.

Value of prothrombin fragment 1.2 (F 1.2) in the diagnosis of stroke in young patients with antiphospholipid antibodies.

The presence in the serum of antiphospholipid antibodies (aPL) is associated with venous and arterial thrombosis. This observation has led to the search for these antibodies in young patients with ischemic neurologic syndromes. However, 1% to 5% of healthy people may be found to have circulating aPL without necessarily being at increased risk of thromboembolism. Thus, the finding of APLA in a patient with cerebral ischemia does not necessarily provide an explanation for the etiology of the clinical syndrome. The aim of this study was to determine whether the presence of aPL in young patients with stroke or transient ischemic attacks represents a possible cause of hypercoagulability as defined by ongoing thrombin formation with resultant elevation of prothrombin fragment 1.2 (F1.2) levels. This was a retrospective, case-control study involving 57 subjects. Twenty-seven patients had a recent cerebrovascular ischemic event--either TIA or a stroke. Fifteen were positive for aPL, and 12 were aPL-negative. Thirty subjects, matched for age and sex with no history of cerebrovascular disease, served as controls. Of this group, 20 were aPL-positive and 10 were aPL-negative. Causes of hypercoagulability other than aPL were excluded by laboratory testing. A positive test for aPL was repeated after a 6-week interval and two positive tests were required for a patient to be regarded as being aPL-positive. Levels of F1.2 were measured by an ELISA technique. There was a significant difference (p < 0.05) in the mean F1.2 levels between the aPL-positive group with a history of cerebrovascular disease (mean F1.2 = 2.3733) and each of the other study groups. There was no statistically significant difference between any of the other study groups. Our findings suggest that F1.2 levels are elevated in young patients with cerebrovascular syndromes who have aPL and in whom other causes of hypercoagulability and atherosclerotic vascular disease are absent. Elevated F1.2 in these patients may be a potential marker of the hypercoagulable state associated with aPL.

Pupil size in Jewish theological seminary students.

PURPOSE: To investigate the authors' clinical impression that pupil size among myopic Jewish theological seminary students is different from pupil size of similar secular subjects. METHODS: This cross-sectional study was conducted on 28 male Jewish theological seminary students and 28 secular students or workers who were matched for age and refraction. All participants were consecutively enrolled. Scotopic and photopic pupil size was measured by means of a Colvard pupillometer. Comparisons of various parameters between the groups were performed using the two-sample t-test, Fisher exact test, a paired-sample t-test, a two-way analysis of variance, and Pearson correlation coefficients as appropriate. RESULTS: The two groups were statistically matched for age, refraction, and visual acuity. The seminary students were undercorrected by an average of 2.35 diopters (D), while the secular subjects were undercorrected by only 0.65 D (p<0.01). The average pupil size was larger in the religious group under both scotopic and photopic luminance. This difference was maintained when the two groups were compared according to iris color under both conditions, reaching a level of statistical significance (p<0.0001). There was a significant difference in photopic pupil size between dark and light irises (p=0.049), but this difference was not maintained under scotopic conditions. CONCLUSIONS: The average pupil size of young ultraorthodox seminary students was significantly larger than that of matched secular subjects. Whether this is the result of intensive close-up work or of apparently characteristic undercorrection of the myopia is undetermined.

[Optic nerve sheath enlargement and reversal of optic nerve head in pseudotumor cerebri].

Using standard cerebral computerized tomography (CT), we diagnosed pseudotumor cerebri (PTC) and correlated the CT findings with CSF pressure and severity of visual impairment. 13 patients with a clinical diagnosis of PTC were compared with 20 age-matched controls with headache, but without papilledema or other neurologic signs. Cerebral CT consisted of axial sections of the posterior fossa, including the orbits. In all subjects the diameter of the optic nerve sheath, reversal of the optic nerve head, presence of empty sella, and size of the ventricles, cisterns and sulci were evaluated. There were no differences in basal cisterns and ventricles between those with PTC and control subjects. Empty sella was found in 6 of 13 PTC patients, compared with 1 of the 20 controls. Optic nerve sheath diameter in controls ranged from 3.5-5.0 mm (average 4.2 +/- 0.54 mm) but from 4.5-9.0 mm (average 6.8 +/- 1.54 mm) in those with PTC. Reversal of the optic nerve head was seen in 4 cases of PTC but in none of the controls. In PTC patients with opening CSF pressure greater than 270 mm water, the diameter of the optic nerve was wider than 7.5 mm. Thus, in most cases of PTC, bilateral enlarged optic nerves can be measured by standard cerebral CT and intracranial space-occupying lesions can be excluded as well. Moreover, reversal of optic nerve head, and empty sella can frequently be seen on CT in those with PTC.

[Post-shock optic neuropathy].

A case of rare, post-shock optic neuropathy is presented. In most cases, it occurs after blood loss or decrease in blood pressure. We present a woman, aged 47, with this condition in whom 3 possible causes, hypoxia, hypercoagulability and vasodilatation, coexisted. Neurologists and ophthalmologists should be aware of this rare condition, because prompt treatment with steroids and hemodynamic stabilization may significantly improve outcome.

Optic nerve sheath diameter on MR imaging: establishment of norms and comparison of pediatric patients with idiopathic intracranial hypertension with healthy controls.

BACKGROUND AND PURPOSE: IIH is a disorder associated with increased intracranial pressure with no clinical, laboratory, or radiologic evidence of an intracranial space-occupying lesion. The aim of this study was to establish ONSD standards of healthy pediatric subjects and compare the normal measurements with those of patients with IIH. MATERIALS AND METHODS: One hundred fifteen MR imaging studies of children 4 months to 17 years of age were blinded and reviewed by a pediatric neuroradiologist. A total of 230 optic nerves were measured. Eighty-six MR imaging examinations were performed in apparently healthy subjects. This control group included subjects who underwent MR imaging for various reasons, and their MR imaging findings were interpreted as normal. Twenty-nine MR imaging examinations were performed in patients with documented IIH. The ONSD was measured 1 cm anterior to the optic foramina on an axial T2 sequence. For statistical analysis, both patients and controls were stratified into 4 age groups (I, 0-3 years; II, 3-6 years; III, 6-12 years; IV, 12-18 years). RESULTS: The mean ONSD of the control group in all age groups (I, 3.1 mm; II, 3.41 mm; III, 3.55 mm; IV, 3.56 mm) was significantly smaller than the mean ONSD of patients (I, 4.35 mm; II, 4.37 mm; III, 4.25 mm; IV, 4.69 mm). A positive correlation between age and ONSD (r = 0.414, P < .01) was found in the control group. CONCLUSIONS: According to our study, in pediatric patients with IIH, the ONSD is significantly larger than that in healthy controls regardless of age group and sex. This measurement might prove to be an auxiliary tool in the diagnosis of increased intracranial pressure in pediatric patients.

Macular findings on optical coherence tomography in cat-scratch disease neuroretinitis.

PURPOSE: To describe the macular findings on optical coherence tomography (OCT) in patients with cat-scratch disease (CSD) neuroretinitis. METHODS: Medical records of all patients diagnosed with CSD neuroretinitis at the Tel Aviv Medical Center between April 2006 and May 2010 were retrospectively reviewed. All patients underwent Stratus OCT macular examination. RESULTS: Eight eyes of seven patients with confirmed CSD neuroretinitis, (mean age 33 ± 9.9 years, range 6-48 years) were included in the study. All patients presented clinically with optic nerve swelling and macular edema or macular exudates. OCT demonstrated flattening of the foveal contour, thickening of the neurosensory retina, and accumulation of subretinal fluid (SRF) in all studied eyes. Retinal exudates appeared as multiple hyper-reflective foci in the outer plexiform layer. The average central macular thickness was 460 µm (range 170-906 µm) and the average maximal retinal thickness was 613 µm (range 387-1103 µm), at presentation. The macula appeared normal on repeated exams during follow-up. CONCLUSION: Similar OCT findings were demonstrated in patients with CSD neuroretinitis. SRF was found in all eyes, although was not visible on clinical examination or fluorescein angiography. OCT may be used as an adjunct imaging tool in the diagnosis and follow-up of patients with CSD neuroretinitis.