Diagnosis Of Intracranial Hypertension Research Articles

Cerebrospinal fluid opening pressure in clinical practice \u2013 a prospective study

BackgroundMeasurement of CSF opening pressure (CSFOP) is valuable and much used in the investigation of several neurological conditions. However, there are different opinions regarding reference values and influence of age, gender and body mass index (BMI). We have, in a previous study, noted possible differences in CSFOP between gender and age groups. Here the aim was to collect information regarding normal distribution of CSFOP in an out-patient sample and also include BMI.MethodsWe collected CSFOP from a lumbar puncture, following a standardized procedure, performed in an ordinary neurological out-patient sample. Age, gender and BMI was also registered. Descriptive statistics and linear regression was used.Results339 patients with a normal distribution of age and BMI were included consecutively (60% females). We found a mean CSFOP of 17.5 H2O (range 4.0–30.0). In multivariable linear regression, age, gender and BMI all independently affected CSFOP. Male gender (β = 1.5, p = 0.002), lower age (β = – 0.095, p < 0.001) and higher BMI (β = 0.42, p < 0.001) were all associated with higher CSFOP.ConclusionUsing two standard deviations, we provide suggestions for CSFOP limits with respect to gender, age and BMI. Our results suggest that CSFOP cut-offs for pathological intracranial hypertension should be raised with these factors taken into consideration. As a “rule-of-thumb” we suggest the following cut-offs: for males < 30 cm H2O (< 25 if over age 70), and for females < 25 cm H2O (27.5 if over 30 BMI). A diagnosis of intracranial hypertension should not be given without such considerations.

Idiopathic Intracranial Hypertension: Contemporary Management and Endovascular Techniques.

Idiopathic intracranial hypertension (IIH) is a disease process of abnormally increased intracranial pressure in the absence of a mass lesion. Medical management, optic nerve fenestration, and surgical shunting procedures have failed to produce consistently successful results. In an unknown percentage of cases, IIH is caused by dural venous sinus obstruction which can be cured by endovascular treatment with dural venous sinus stent placement. This helps prevent progressive vision loss and worsening papilledema caused by underlying increased intracranial pressure from venous outflow obstruction. Patients are required to have an established diagnosis of IIH, preferably made by a neuroophthalmologist, with clearly documented papilledema or at minimum visual disturbance along with lumbar puncture opening pressure greater than 25 cm H 2 O. Transverse to sigmoid sinus focal narrowing (intraluminal filling defect or extrinsic compression) must be seen during the venous phase of neuroangiography (NA) along with a pressure gradient of 10 mm Hg or greater across the focal narrowing during dural venous sinus pressure monitoring. A successful reduction is defined as a pressure gradient of less than 10 mm Hg after stent placement. Neuroophthalmologic follow-up occurs within 1 to 2 months to assess for changes in papilledema. If papilledema is unchanged or worsened, NA and hemodynamic evaluation is repeated for consideration of restenting. Appropriate patient selection criteria are required for IIH venous sinus stenting. The utilization of refined endovascular techniques along with postprocedure follow-up protocols can ultimately cure IIH for a select group of patients.

Repeatability of ultrasound examination of the optic nerve sheath diameter in the adult cat: comparison between healthy cats and cats suffering from presumed intracranial hypertension.

The objectives of this study were to test: (1) the repeatability of ultrasonographic examination of the optic nerve sheath diameter (ONSD) in the cat; (2) the association between the ONSD and age, sex and body weight in healthy cats; and (3) the difference in the ONSD between healthy cats and those suffering from presumed intracranial hypertension (ICH). This study had a prospective, blinded, observational cross-sectional study design. Two groups of animals were considered: healthy cats (group A) and cats with a diagnosis of presumed ICH (group B). The ONSD was evaluated, measured and compared between the two groups via an ultrasonographic transpalpebral approach. Repeatability of the procedure was evaluated through the intraclass correlation coefficient (ICC). Data were statistically compared using the Student's t-test and linear regression analysis. A strong inter- and intraobserver ICC indicating good repeatability was observed. The interobserver ICC was 0.965 (P <0.05) for the right eye and 0.956 (P <0.05) for the left eye. The intraobserver ICC was 0.988 (P <0.05) and 0.984 (P <0.05) for the right and left eyes, respectively. In healthy cats the mean ± SD ONSD was 1.23 ± 0.11 mm (range 1-1.47 mm) and 1.23 ± 0.10 (range 1-1.4 mm) for right and left eyes, respectively. The ONSD was not related to sex or weight; a weak relationship was observed with age. In group B, the mean ONSD was 1.68 ± 0.13 mm (range 1.5-1.9 mm) and 1.61 ± 0.15 mm (range 1.4-1.9 mm) for the right and left eyes, respectively. In group B, the ONSD was statistically significantly larger than in group A, the healthy cats (P <0.001). The transpalpebral ultrasonographic technique is a non-invasive, feasible and reproducible method to measure ONSD both in healthy cats and in cats suffering from suspected ICH.

Sonographic Measurement of the Optic Nerve Sheath Diameter (ONSD) and Eyeball Transversal Diameter (ETD) in Benin Healthy African Adults

Background: Measuring optic nerve sheath diameter (ONSD), a relatively re-cent technique, allows an indirect and non-invasive diagnosis of intracranial hypertension. The ONSD ratio to eyeball transverse diameter (ETD) increases this reliability of the technique. The objective of this study was to determine the normal ONSD and its ratio with ETD in black African adults in Benin. Methods: A descriptive cross-sectional study was conducted between May 2019 and August 2019. Ultrasound ONSD and ONSD/ETD ratio were meas-ured in 210 healthy adults received in the medical imaging department of the Borgou and Alibori University Hospital Center in Benin. The ONSD corre-sponded to the average of 12 measurements (03 horizontal and 03 vertical for each eye) taken 3 mm behind the papilla. The transverse ETD corresponded to the average of 03 measurements. Results: The mean ONSD was 4.20 ± 0.28 mm (95% CI = [4.17; 4.24] mm and 95th percentile = 4.70 mm). The mean ETD was 24.25 ± 1.10 mm. The ONSD/ETD ratio was 0.17 ± 0.01 (95% CI = [0.17; 0.17] and 95th percentile = 0.19). There was no difference between ver-tical and horizontal ONSD and between right and left ONSD. The ONSD and the ONSD/ETD ratio were not correlated with any of the socio-demographic and anthropometric characteristics studied (sex, age, ethnicity, weight, height, body mass index, head circumference and waist circumference). The ONSD was associated with ETD (p < 0.001 and r = 0.55). Conclusion: An ONSD and an ONSD/ETD ratio respectively greater than 4.70 mm and 0.19 should sus-pect intracranial hypertension. The ONSD/ETD ratio may be a better indicator of HICT because of its small variation.

Psychiatric disorders are a common prognostic marker for worse outcome in patients with idiopathic intracranial hypertension

ObjectiveIdiopathic intracranial hypertension (IIH) is aetiologically unknown disorder that associates with endocrinological disturbances, including dysfunction of hypothalamic-pituitary-adrenal-axis. Neuroendocrinological dysfunctions have also been characterized in psychiatric disorders, and therefore we investigated the presence of psychiatric disorders of patients with IIH in a well-defined cohort. Patients and methodsA total of 51 patients with IIH were included. Patient demographics, symptoms, imaging data, ophthalmological and clinical findings were collected. ResultsAt the time of diagnosis the mean age was 32.5years (SD 10.7), the body mass index was 37.1 kg/m2 (SD 7.4), and the opening pressure 29.1 mmHg (SD 6.2). A total of 88.2% of patients were female and 45.1% were diagnosed with a psychiatric co-morbidity prior to IIH diagnosis. The mean follow-up time was 4.4 years (SD 5.4). The overall treatment outcome was significantly poorer on a group of patients with psychiatric diagnosis when compared to individuals without such history (p = 0.001), but there were no differences in the resolution of papilledema (p = 0.405). Patients with IIH and psychiatric disorders had more often empty sella on their imaging at diagnosis when compared to patients without psychiatric co-morbidity (p = 0.044). ConclusionPsychiatric disorders are highly prevalent in patients with IIH and associate with worse subjective outcomes. These findings advocate for monitoring the mental health of patients with IIH and warrant further multidisciplinary research to understand the potentially underlying psychosocial and neuroendocrinological mechanisms.

Optic Nerve Sheath Diameter Ultrasound: Optic Nerve Growth Curve and Its Application to Detect Intracranial Hypertension in Children

First, to create an optic nerve growth curve from normal optic nerve sheath diameter (ONSD) values measured by using B-scan ultrasonography in subjects 0-18 years of age. Second, to identify age-appropriate cutoff values of ONSD to be used in the diagnosis of intracranial hypertension (IHT). Prospective cross-sectional study. B-scan ocular ultrasonography was performed on both eyes of 215 subjects 0-18 years of age, divided into 3groups: 165 healthy children, 29 children with IHT (all>4 years of age), and 21 children with optic disc drusen (ODD). There were no statistically significant differences in between the ONSDs of healthy children and those in subjects with ODD. An optic nerve growth curve was created by using ONSDs measured in healthy subjects 0-18 years of age, using the equation: ONSD= ln [33.15] - (-0.18× ln [children's age]). The curve showed a progressive increase of ONSD up to 10 years of age, and it remained constant until the age of 18. For this reason, 2 different cutoff values were calculated for age groups 4-10 and 11-18. Values were 4.10mm and 4.4mm, respectively, with a 100.0% sensitivity and a specificity ranging from 83.9% to 98.8%. A sensitivity of 28.6% was reached for the population of subjects 4-18 years of age with a threshold value of 5mm, as used in published reports. The ONSD continued to enlarge gradually until the age of 10. Therefore, 2 different cutoff values for the age groups 4-10 and 11-18 were calculated, considering the ONSDs of subjects 11-18 years of age overlapping with those of adults. No patients with IHT <4 years old were found. Further studies are needed to evaluate the correct cutoff values for these ages.

Study on the feasibility of optic nerve subarachnoid width in qualitative diagnosis of intracranial hypertension

Objective To evaluate the feasibility of optic nerve subarachnoid space in non-invasive qualitatively diagnosis of intracranial hypertension. Methods In this retrospective study,patients who received lumbar puncture from October 2009 to June 2015 were enrolled and divided into normal intracranial pressure group (41 cases) and intracranial hypertension group (39 cases). Optic nerve subarachnoid space width (SASW) behind ocular 3 mm (SASW-3),9 mm (SASW-9),and 15 mm (SASW-15) were measured on MRI and compared between groups. Chi-square test, t test and ROC analyses were used. Results SASW-3,SASW-9,and SASW-15 were (1.16±0.21), (0.98±0.21) and (0.92±0.17) mm in normal group and (1.46±0.20), (1.29±0.19) and (1.17±0.20) mm in intracranial hypertension group,respectively. According to independent samples t test,SASW-3,SASW-9,and SASW-15 of intracranial hypertension group was significant larger than those at the same measured site of normal group (P<0.01). According to MedCalc analysis,Receive operating characteristic (ROC) area was 0.849 in SASW-3 and when the cut-off value was fixes as 1.19 mm,the sensibility and specificity were 94.9% and 63.4%,respectively. ROC area was 0.849 in SASW-9 and when the cut-off value was fixes as 1.10 mm,the sensibility and specificity were 84.6% and 78.0%,respectively. ROC area was 0.824 in SASW-15 and when the cut-off value was fixes as 1.06 mm,the sensibility and specificity were 69.2% and 80.5%,respectively. Conclusion SASW-9 can be used to screen and monitor the intracranial hypertension as a non-invasive tool. Key words: Optic nerve; Subarachnoid space; Intracranial pressure; Magnetic resonance imaging

Use of neuroimaging measurements of optic nerve sheath diameter to assess intracranial pressure in craniosynostosis.

This study aims to evaluate accuracy of optic nerve sheath diameter (ONSD) measurements obtained by magnetic resonance imaging (MRI) in patients with craniosynostosis (CS) for detection of high intracranial pressure (ICP) and to correlate MRI-derived ONSD measurements with those obtained by computed tomography (CT) scans in CS patients. A retrospective review was conducted on CS patients who had MRI- and age-matched controls with normal MRI. Diagnosis of intracranial hypertension was based on presence of papilledema, direct ICP monitoring, and/or lumbar puncture. The search also included patients with MRI and CT done within 30days apart. ONSDs were measured 3mm behind the globe on both modalities. The study identified 56 CS patients (mean age 3.8 ± 3.47years) and 49 controls (mean age 3.7 ± 3.62years). Mean ONSD in patients with high ICP was significantly higher than in patients without high ICP (P = 0.0001) and in controls (P < 0.0001). The optimal ONSD threshold for predicting raised ICP in patients >1year old was 6mm (71.4% sensitivity, 89.7% specificity). Nineteen patients with 38 single-eye MRI/CT pairs were identified. Substantial agreement between both modalities resulted (r = 0.959, 95% CI 0.923-0.978), and Bland and Altman Plot analysis showed that 95% of measurements fell within limits of agreement (1.96 SD; ± 0.6mm). In CS patients, ONSD measured by MRI represent indirect non-invasive means of ICP assessment. Both MRI and CT measurements of ONSD gave comparable results, and the use of CT-derived ONSD measurements may give some idea about ICP in CS patients.

Diagnostic criteria in pediatric intracranial hypertension

The modified Dandy criteria and the newer diagnostic criteria for pseudotumor cerebri syndrome (PTCS) are both used to diagnose intracranial hypertension (IH). In comparison to the modified Dandy criteria, the PTCS criteria stratify the IH diagnosis into definite, probable, and suggested categories, exclude clinical symptoms, and use radiologic evidence for diagnosis. There is a lack of consensus on which criteria should be used in the pediatric population. The purpose of this study was to compare the diagnostic criteria for PTCS to the modified Dandy criteria and to identify limitations within both sets of criteria. The PTCS criteria were retrospectively applied to 50 patients originally diagnosed with IH under the modified Dandy criteria. Of the 50 patients, 31 (62%) met diagnostic criteria for definite PTCS, 10 (20%) met criteria for probable PTCS, and 9 patients (18%) failed to meet sufficient PTCS criteria for diagnosis. Although the PTCS criteria use objective data to make the IH diagnosis, we found subjective symptoms to be useful indicators of disease in this group of patients. Additionally, distinguishing probable from definite IH may not have clinical relevance, because both groups were treated similarly. The absence of radiographic evidence of IH should not preclude a diagnosis of the condition, as it was present in a minority of patients included in this study. Further research is needed to clarify the disease process in patients who present with signs and symptoms of elevated intracranial pressure but lack ocular pathology.

Ayurvedic management of Intra Cranial Hypertension (IIH) - A Case Study

A 36 year old female patient came to P. D. Patel Ayurvedic Hospital, Nadiad with confirmed diagnosis of Intra Cranial Hypertension (IIH). The patient presented with chief complaint of headache (more over left side), transient vision loss and ear ache. Clinical findings showed bilateral papilloeodema (Lt&gt;Rt). MRI showed possibilities of Idiopathic Intracranial Hypertension. The menometry showed the pressure of CSF-360 mm of water. After clinical evaluation Ayurvedic treatment was started and significant improvement was seen. The treatment during the hospitalization of one month included Sarvanga Abhyanga, Sarvanga Swedana, Niruha Basti, Marsha Nasya and oral medication. Later on after 19 days Shirodhara was started. During the stay patient was followed up for papilloeodema. As a result of the treatment the severity of the headache was decreased and papilloeodema started resolving. The menometry showed significant reduction in the pressure of CSF. The medicines were continued after discharge.

Lumbopleural Shunting as an Alternative Cerebrospinal Fluid Diversion Modality for Management of Idiopathic Intracranial Hypertension: Is It Time for a Change?

diopathic intracranial hypertension (IIH), as its name implies, is a disorder characterized by elevated cerebrospinal fluid I (CSF) pressure of an unknown etiology. First reported by Quincke in 1893 as “meningitis serosa,” this clinical syndrome has historically been a matter of serious contention, with its nomenclature subject to substantial changes over time. In 1904, Nonne introduced the term “pseudotumor cerebri.” The syndrome was subsequently renamed “benign intracranial hypertension” by Foley in 1955. With a high proportion of patients demonstrating permanent visual impairment, the term “benign” was deemed inappropriate, and the term “idiopathic” was unanimously adopted. Often considered as a diagnosis of exclusion, IIH predominantly affects obese women of reproductive age and is best defined by myriad symptoms, most commonly headaches, transient obscuration of vision, pulsatile tinnitus, photopsia, backache, retrobulbar pain, diplopia, and severe visual impairment. Analogous to its nomenclature, diagnostic criteria for IIH have evolved considerably to integrate our enhanced understanding of the disease with developments in technology. The original diagnostic classification by Dandy in 1937 included signs of increased intracranial pressure (>25 cm H2O) with normal CSF composition, absence of any localizing signs except abducens nerve palsy, and normal or slit ventricles with no intracranial mass lesion as evident on ventriculography. This classification was initially modified by Smith in 1985 to accommodate the advent of computed tomography, used in diagnosis of IIH primarily to exclude secondary causes predisposing to intracranial hypertension, including space-occupying lesions that were likely unnoticed in the previous era. The modification of the diagnostic

The Use of Acetazolamide During Pregnancy in Intracranial Hypertension Patients

Acetazolamide is the mainstay of medical therapy for idiopathic intracranial hypertension (IIH). Its use in pregnant women has not been recommended because of reported teratogenic effects in rodents and rabbits. However, the safety of acetazolamide use during human pregnancy remains unclear. We report the pregnancy and offspring outcomes in women with intracranial hypertension (IH) treated with acetazolamide during pregnancy. Data were collected through questionnaires sent to patients with IH and their physicians. The questionnaires focused on IH diagnosis, obstetric history and outcomes, and pediatric outcomes. A total of 101 women with IH were consented (total of 158 pregnancies) and acetazolamide usage before 13 weeks of gestation was reported in 50 pregnancies. The risk of spontaneous abortion was similar to the control group and no major complication was identified in the offspring of women treated with acetazolamide. There is no convincing evidence for an adverse effect for acetazolamide use in human pregnancy, even when prescribed prior to the 13th week of gestation. While the liberal use of acetazolamide should be avoided during pregnancy, this medication should remain a treatment option in pregnant women when clinically indicated.

Imaging signs in idiopathic intracranial hypertension: Are these signs seen in secondary intracranial hypertension too?

Background:The purpose of this study was to evaluate the difference in the occurrence of the various “traditional” imaging signs of intracranial hypertension (IIH) on magnetic resonance imaging (MRI) in patients with idiopathic (IIH) and secondary intracranial hypertension.Materials and Methods:In a retrospective analysis, the MRI findings of 21 patients with IIH and 60 patients with secondary intracranial hypertension (41 with tumors; 19 with intracranial venous hypertension) were evaluated for the presence or absence of various “traditional” imaging signs of IIH (perioptic nerve sheath distention, vertical buckling of optic nerve, globe flattening, optic nerve head protrusion and empty sella) using the Fisher’s exact test. Odds ratios were also calculated. Statistical Package for the Social Sciences version 17.0 was used for statistical analysis. Subgroup analysis of the IIH versus tumors and IIH versus venous hypertension were performed.Results:Optic nerve head protrusion and globe flattening were significantly associated with IIH. There was no statistically significant difference in the occurrence of rest of the findings. On subgroup analysis, globe flattening and optic nerve head protrusion occurred significantly more often in IIH than in tumors. However, there was no statistically significant difference in the occurrence of any of these findings in patients with IIH and venous hypertension.Conclusions:IIH is a diagnosis of exclusion. While secondary causes of raised intracranial pressure (ICP) have obvious clinical findings on MRI, some conditions like cerebral venous thrombosis may have subtle signs and differentiating between primary and secondary causes may be difficult. In the absence of any evident cause of raised ICP, presence of optic nerve head protrusion or globe flattening can suggest the diagnosis of IIH.

Hypertension intracrânienne isolée révélatrice d’une maladie de Lyme

We report the case of an eight-year-old patient referred by his primary care physician for chronic headache. Bilateral papilledema was observed along with right sixth cranial nerve palsy, leading to the diagnosis of intracranial hypertension. Head CT showed no mass lesion. Lyme serology was positive by both Elisa and Western blot. Anti-Borrelia antibodies were positive in the cerebrospinal fluid, with intrathecal synthesis confirming neuroborreliosis. Clinical response to ceftriaxone and acetazolamide was favorable. Intracranial hypertension is rarely caused by Lyme disease. Ophthalmologists should be aware of this clinical presentation, since the presenting clinical signs may be purely ophthalmologic. In addition, early diagnosis may avoid optic nerve atrophy or disease spread.

Pseudotumor cerebral associado ao uso de ciclosporina após transplante renal

Pseudotumor cerebri (PC) is a syndrome characterized by the presence of intracranial hypertension (ICH) and no alteration in the ventricular system. Renal transplanted patients seem more susceptible to develop it due to immunosuppressive therapy. Cyclosporin (CsA) is a rare cause of PC, scarcely reported in the literature, and should be considered in the differential diagnosis of ICH and papilledema in those patients. We report the case of a 10-year-old boy, with a renal allograft for three years, on chronic use of mycophenolate mophetil (MMF), CsA, and low doses of prednisone. The patient presented with headache, vomiting, diplopia, and photophobia. Funduscopy showed bilateral papilledema. Cerebrospinal fluid analysis and imaging tests were normal. After excluding secondary causes, PC was diagnosed based on the chronic use of CsA, which was then replaced by sirolimus. After that, the patient progressively improved, and the papilledema resolved in three months.

Intracranial hypertension: A rare presentation of lupus nephritis

A 14-year-old male presented with bilateral papilledema, growth retardation and absent secondary sexual characters. He had a past history of fever, headache and fatigue of 6 months duration. The diagnosis of intracranial hypertension (IH) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, except for partial empty sella, prominent perioptic cerebrospinal fluid (CSF) spaces and buckling of optic nerves. Evaluation showed erythrocyte sedimentation rate (ESR) of 150 mm/hr, positive antinuclear antibody (ANA), anti dsDNA and anti ribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV S (A) confirming the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methyl prednisolone and cyclophosphamide was effective in normalizing the CSF pressure, resulting in express and dramatic resolution of symptomatology. In a case of IH, SLE must be considered. IH, growth retardation and absence of sexual characters may be presenting manifestations of a chronic systemic inflammatory disease like SLE. These manifestations may act as a pointer to associated advanced grades of LN, which can be totally asymptomatic and missed without a renal biopsy.

Isotretinoin-associated intracranial hypertension

Purpose To evaluate the association between intracranial hypertension (IH) and isotretinoin use. Design Observational case series. Methods In this retrospective study, approximately 1950 case reports of adverse ocular side effects related to isotretinoin were received from spontaneous reporting systems. Reports were evaluated as to the occurrence of IH with isotretinoin use. A survey was mailed to all members of the North American Neuro-ophthalmology Society soliciting their opinions on whether isotretinoin caused IH. Results One hundred seventy-nine reports of IH were associated with isotretinoin use. The mean time from drug exposure to IH diagnosis was 2.3 months. There were 6 cases of positive rechallenge; 5 new cases are reported here, along with 1 previously published report. Of neuro-ophthalmologists surveyed (62% response rate), 6% believed an association between IH and isotretinoin use was certain; 32%, probable; 52%, possible; and 10%, unlikely. Twelve respondents (4%) had personally seen one or more cases of positive rechallenge with isotretinoin causing IH. Conclusions Based on the number and pattern of rapid IH onsets after isotretinoin exposure and the 6 cases of positive rechallenge, along with the probable similarity in metabolic pathways of this agent and vitamin A (a known cause of IH), it seems certain that there is a direct correlation between IH and isotretinoin use.

The use of transcranial Doppler ultrasonography (TCD) in the diagnosis of intracranial hypertension

In patients with severe head injury the main complication is cerebral edema and intracranial hypertension. TCD is a noninvasive, bedside technique, which detects the blood flow velocities in the great intracranial arteries. The aim of our study was to investigate whether TCD is a useful technique for the diagnosis of intracranial hypertension.

Treatable intracranial hypertension in patients with lupus nephritis.

Idiopathic intracranial hypertension is a disorder of intracerebral pressure regulation and patients run the risk of permanent visual loss. Intracranial hypertension (IH) has been reported rarely in systemic lupus erythematosus (SLE). We reviewed the medical records of 127 patients with lupus nephritis (LN) who were followed up from 1987 to 1996 in our unit. There were six patients with IH which gave a disease prevalence of 4.7% in those with LN. All were females giving a disease prevalence of 5.2% for that sex, a high rate of occurrence of IH in patients with LN. Their age ranged from 22 to 34 y (27.8 +/- 3.6 y). Headache, vomiting and diplopia were the common presenting symptoms and had started 7.3 +/- 4.4 weeks prior to the diagnosis of IH. The cerebrospinal (CSF) opening pressure (413.3 +/- 77.0 mmH2O) was raised in all cases. Biochemical and cytological analyses of CSF were normal. The only abnormal radiological finding was partially empty sella in one patient on magnetic resonance imaging (MRI) (performed in three patients) or computed tomography (CT) (performed in all patients). All patients had serological evidences of active lupus disease at the time of diagnosis of IH. The renal histology was WHO type IV in four cases and III and V in one each indicating severe renal involvement. Laboratory evidences of procoagulant activity were found in the form of positive anticardiolipin antibody (aCL) in two patients, lupus anticoagulant (LA) in two and an otherwise unexplained isolated prolongation of activated partial thromboplastin time (APTT) in the other two. Clinically, one or more episodes of symptomatic venous or arterial thrombosis had occurred in all subjects. In addition to symptomatic measures, all subjects were treated with prednisolone, azathioprine, cyclophosphamide and plasmapheresis according to the protocol of our unit. One patient who did not receive plasmapheresis and cyclophosphamide had a relapse while all others recovered completely. None received anticoagulant therapy. Young females with serologically active lupus, severe forms of renal lesions, past history of venous or arterial thrombosis and laboratory evidences of procoagulant activity, appear to be at increased risk of IH. Thrombotic occlusion of the cerebral arteriolar or venous vascular bed eventually affecting the arachnoid villi and impeding CSF absorption is favoured compared to cerebral venous or sinus thrombosis as the pathogenic mechanism. Combined immunosuppression and plasmapheresis appeared to be beneficial in short and long term follow-up. We propose that patients with SLE and IH have definable risk and pathogenetic factors and are no more to be considered 'idiopathic'. The conditions calls for aggressive intervention which leads to an excellent outcome.

Benign intracranial hypertension in children with growth hormone deficiency treated with growth hormone

We report 13 cases of benign intracranial hypertension (IH) in children with growth hormone (GH) deficiency treated with GH in the United States. The group consisted of eight boys and five girls, 3 to 16 years of age (median, 9 years). The interval from starting GH therapy to diagnosis of IH was 2 weeks or less in six patients, between 2 and 12 weeks in four, 8 months in one, 5 years in one, and unknown in one. Seven patients were not known to have previously described IH risk factors; the other six had at least one factor each. All patients but one had headache, nausea, vomiting, and visual changes. All had papilledema, and cerebrospinal fluid pressures were elevated (>250 mm H2O) in all nine patients tested. The GH dosage range was 0.17 to 0.35 mg per kilogram body weight per week (median, 0.30 mg/kg per week) for the 11 patients with dosage data. After discontinuation of GH and treatment with lumbar punctures and/or medications, signs and symptoms resolved in eight children; in two of these children signs and symptoms reappeared when GH therapy was restarted. In four patients signs and symptoms resolved while GH therapy was continued; one child was treated with a ventriculoperitoneal shunt because of an arachnoid cyst, after which GH was restarted without subsequent IH. In the 12 patients with idiopathic GH deficiency the course of IH was benign, with complete resolution of all signs and symptoms. Because doses and scheduling of GH administration have changed since the introduction of recombinant GH, higher doses and increased frequency of administration may be contributing to the development of IH in some patients. We suggest beginning therapy at the lowest recommended dose, with gradual titration to higher doses, and the performance of routine funduscopic examinations during initiation of GH therapy and whenever signs or symptoms of IH develop. (J PEDIATR 1995;126:996-9)