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Lakartidningen May 2019Idiopathic intracranial hypertension (IIH) is a disorder affecting both the pediatric and adult population. Investigations and treatments may differ considerably. There... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a disorder affecting both the pediatric and adult population. Investigations and treatments may differ considerably. There are no evidence-based guidelines for treatment. During a national multidisciplinary meeting in Stockholm January 2018 IIH experts from several Swedish regions met to discuss how to manage this patient group. These guidelines are based on this meeting and a review of current medical knowledge. To summarize: All patients should be investigated and treated for underlying factors that could be the cause of high intracranial pressure (ICP) (such as obesity, secondary causes such as intracranial tumors or other factors reported to affect ICP). When treating IIH the preservation of vision is crucial. Follow-up depends on visual status. In case of acute risk of visual impairment prompt surgical intervention must be considered. Symptomatic treatment of headache is recommended.
Topics: Acetazolamide; Bariatric Surgery; Carbonic Anhydrase Inhibitors; Consensus; Critical Pathways; Diagnosis, Differential; Female; Headache; Humans; Obesity; Practice Guidelines as Topic; Pregnancy; Pseudotumor Cerebri; Risk Factors; Stents; Sweden; Ventriculoperitoneal Shunt
PubMed: 31192408
DOI: No ID Found -
Headache Oct 2018Certain headache characteristics and associated symptoms are commonly attributed to increased intracranial pressure, but they have not been systematically studied among...
Headache Characteristics in Children With Pseudotumor Cerebri Syndrome, Elevated Opening Pressure Without Papilledema, and Normal Opening Pressure: A Retrospective Cohort Study.
BACKGROUND
Certain headache characteristics and associated symptoms are commonly attributed to increased intracranial pressure, but they have not been systematically studied among children in the context of revised diagnostic criteria for pseudotumor cerebri syndrome (PTCS).
METHODS
We performed a retrospective cohort study of patients treated for suspected or confirmed PTCS. Charts were reviewed for PTCS and headache diagnostic criteria and associated characteristics. Chi-squared or Fisher's exact tests were used to compare the frequency of headache characteristics between groups.
RESULTS
One hundred and twenty-seven individuals were identified: 61 had definite PTCS, 10 had probable PTCS, 31 had elevated opening pressure (OP) without papilledema, and 25 had normal OP without papilledema. Eleven children had no headache (6 with definite PTCS, 5 with probable PTCS). Headache pattern was episodic in 49% (95% CI: 34-64%) of those with definite PTCS, 18% (95% CI 6-37%) of those with elevated OP without papilledema, and 16% (5-36%) of those with normal OP without papilledema. Headache location was more likely to involve the head along with neck or shoulders in those with definite PTCS compared with elevated OP without papilledema (OR = 7.2, 95% CI: 1.9-27.6) and normal OP (OR = 4.5, 95% CI: 1.3-15.6) groups.
DISCUSSION
While missing data and small cohort size are limitations, this study suggests that headache in PTCS is more likely to involve the head along with neck/shoulders, and that headache in PTCS may be episodic or constant. Headache is occasionally absent in PTCS.
Topics: Adolescent; Child; Female; Headache; Humans; Intracranial Hypertension; Intracranial Pressure; Male; Pseudotumor Cerebri; Retrospective Studies
PubMed: 30137653
DOI: 10.1111/head.13362 -
Romanian Journal of Ophthalmology 2020Idiopathic Intracranial Hypertension (IIH) is a disease of elevated intracranial pressure without any known cause. Visual dysfunction is the major morbidity of this...
Idiopathic Intracranial Hypertension (IIH) is a disease of elevated intracranial pressure without any known cause. Visual dysfunction is the major morbidity of this disease but not much is known about the way the contrast sensitivity (CS) function is affected. This prospective, interventional study attempted to evaluate the change in central and peripheral contrast sensitivity, after treatment in patients diagnosed with IIH. Twenty eyes of 10 IIH patients underwent an internet based, Spaeth Richman Contrast Sensitivity (SPARCS) test. Average and quadrant wise SPARCS scores were compared at presentation (treatment naïve), 1-month post treatment and 3 months post treatment. The average SPARCS scores pre-treatment, 1-month post-treatment and at 3 months post treatment were 68.8 + 10.16, 74.45 + 11.17 and 75.7 + 10.81 respectively. At 3 months visit, the average SPARCS score was nearly comparable to the average score in normal Indian subjects, observed in a previous study of ours. Quadrant wise change in contrast sensitivity from first visit to third visit was significant in superonasal (p=0.003), inferonasal (p=0.029) and inferotemporal (p= 0.007) quadrants. Effect of IIH on visual system is still a relatively unexplored area, especially in the Indians. Not many studies have concentrated on its impact on central as well as peripheral CS. Previous studies have hinted at a possible role of CS as a better indicator of visual dysfunction than other parameters. IIH affects both central and peripheral contrast sensitivity and therapy results in the improvement of contrast deficit. Poor contrast can possibly be explained by relatively more involvement of Magnocellular pathway over the Parvocellular pathway. IIH = Idiopathic Intracranial Hypertension, CS = Contrast Sensitivity, SPARCS = Spaeth Richman Contrast Sensitivity Test, BMI = Body Mass Index, MC = Magnocellular pathway, PC = Parvocellular pathway.
Topics: Adult; Contrast Sensitivity; Female; Follow-Up Studies; Humans; Male; Middle Aged; Prospective Studies; Pseudotumor Cerebri; Vision Tests; Visual Acuity; Visual Fields; Young Adult
PubMed: 33367175
DOI: 10.22336/rjo.2020.59 -
Journal of Clinical Research in... Jun 2021To investigate the incidence of pseudotumor cerebri syndrome (PTCS) in children treated with growth hormone (GH) in a paediatric hospital and to identify risk factors...
OBJECTIVE
To investigate the incidence of pseudotumor cerebri syndrome (PTCS) in children treated with growth hormone (GH) in a paediatric hospital and to identify risk factors for this complication.
METHODS
Prospective pilot study of paediatric patients treated with recombinant human GH, prescribed by the Paediatric Endocrinology Department, between February 2013 and September 2017. In all these patients, a fundus examination was performed before starting treatment and 3-4 months later.
RESULTS
Two hundred and eighty-nine patients were included, of whom 244 (84.4%) had GH deficiency, 36 (12.5%) had short stature associated with small for gestational age, six (2.1%) had a mutation in the gene and three (1.0%) had Prader-Willi syndrome. Five (1.7%) developed papilledema, all were asymptomatic and had GH deficiency due to craniopharyngioma (n=1), polymalformative syndrome associated with hypothalamic-pituitary axis anomalies (n=2), a non-specified genetic disease with hippocampal inversion (n=1) and one with normal magnetic resonance imaging who had developed a primary PTCS years before.
CONCLUSION
GH treatment is a cause of PTCS. In our series, at risk patients had GH deficiency and hypothalamic-pituitary anatomic anomalies or genetic or chromosomal diseases. Fundus examination should be systematically screened in all patients in this at-risk group, irrespective of the presence or not of symptoms.
Topics: Adolescent; Child; Child, Preschool; Female; Hormone Replacement Therapy; Human Growth Hormone; Humans; Infant; Male; Papilledema; Pilot Projects; Prospective Studies; Pseudotumor Cerebri; Recombinant Proteins; Risk Factors
PubMed: 33006547
DOI: 10.4274/jcrpe.galenos.2020.2020.0007 -
Indian Journal of Ophthalmology Jul 2023To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies.
PURPOSE
To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies.
METHODS
Records of 18 eyes of 15 patients who underwent optic nerve sheath fenestration for vision threatening optic disc edema were reviewed retrospectively, and results were analyzed. Improvement of visual acuity was the main measure of outcome. Improved visual fields, resolution of optic disc edema, diplopia, and headache were other benefits that were observed.
RESULTS
Fifteen patients between 13 and 54 years of age were included in the study. Three patients underwent successive bilateral surgery. Idiopathic intracranial hypertension was the most common cause for optic disc edema and was found in 80% of the patients. Mean preoperative logMAR acuity was -1.9789 ± 1.46270, which improved to -0.9022 ± 1.23181 (p < 0.005) in the operated eye, and mean logMAR acuity of contralateral eye improved from -1.3378 ± 1.50107 to -1.0667 ± 1.33813 (p < 0.05).
CONCLUSION
Early optic nerve sheath fenestration is an effective modality for treating optic disc edema due to a wide myriad of causes and helps resolve the associated symptoms.
Topics: Humans; Papilledema; Optic Nerve; Retrospective Studies; Pseudotumor Cerebri; Decompression, Surgical
PubMed: 37417132
DOI: 10.4103/IJO.IJO_3027_22 -
The Journal of Headache and Pain Jul 2021Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and...
BACKGROUND
Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and visual disturbances are frequent complaints of IIH patients, but little is known about other symptoms. In this study, we evaluated the patients' perspective on the burden of IIH.
METHODS
For this cross-sectional study, we developed an online survey for patients with IIH containing standardized evaluations of headache (HIT-6), sleep (PROMIS Sleep Disturbance Scale) and depression (MDI) in relation to BMI, lumbar puncture opening pressure (LP OP) and treatment.
RESULTS
Between December 2019 and February 2020, 306 patients completed the survey. 285 (93 %) were female, mean age was 36.6 years (± 10.8), mean BMI 34.2 (± 7.3) and mean LP OP at diagnosis was 37.8 cmHO (± 9.5). 219 (72 %) of the participants were obese (BMI ≥ 30); 251 (82 %) reported severe impacting headaches, 140 (46 %) were suffering from sleep disturbances and 169 (56 %) from depression. Higher MDI scores correlated with higher BMI and increased sleep disturbances. Patients with a normalized LP opening pressure reported less headaches, less sleep disturbances and less depression than those with a constantly elevated opening pressure.
CONCLUSIONS
In addition to headaches and visual disturbances, sleep disturbances and depression are frequent symptoms in IIH and contribute to the patients' burden. Structured questionnaires can help to identify IIH patients' needs and can lead to personalized and better treatment.
Topics: Adult; Cross-Sectional Studies; Female; Headache; Humans; Obesity; Pseudotumor Cerebri; Sleep Wake Disorders
PubMed: 34238219
DOI: 10.1186/s10194-021-01283-x -
Child's Nervous System : ChNS :... Sep 2021Alagille syndrome (AS) is a rare multisystem disease of the liver, heart, eyes, face, skeleton, kidneys, and vascular system. The occurrence of pseudotumor cerebri...
Alagille syndrome (AS) is a rare multisystem disease of the liver, heart, eyes, face, skeleton, kidneys, and vascular system. The occurrence of pseudotumor cerebri syndrome (PTCS) in patients with AS has been reported only exceptionally. Owning to its rarity and a mostly atypical presentation, the diagnosis and natural history of affected patients remain uncertain. We report an atypical case of PTCS in a 4-year-old boy with a known history of AS who presented with bilateral papilledema (PE) on a routine ophthalmological examination. Visual findings deteriorated after treatment with acetazolamide. Continuous intracranial pressure (ICP) monitoring was then utilized to investigate ICP dynamics. Successful treatment with resolution of PE was achieved after ventriculoperitoneal shunting but relapsed due to growth-related dislocation of the ventricular catheter. This report brings new insights into the ICP dynamics and the resulting treatment in this possibly underdiagnosed subgroup of PTCS patients. It also demonstrates that ventriculoperitoneal shunting can provide long-term improvement of symptoms for more than 10 years.
Topics: Alagille Syndrome; Child, Preschool; Humans; Intracranial Pressure; Male; Papilledema; Pseudotumor Cerebri; Treatment Outcome
PubMed: 33555437
DOI: 10.1007/s00381-021-05043-9 -
Journal of Neurology, Neurosurgery, and... May 2012Idiopathic intracranial hypertension is a disease of unknown aetiology, typically affecting young obese women, producing a syndrome of increased intracranial pressure... (Review)
Review
Idiopathic intracranial hypertension is a disease of unknown aetiology, typically affecting young obese women, producing a syndrome of increased intracranial pressure without identifiable cause. Despite a large number of hypotheses and publications over the past decade, the aetiology is still unknown. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue and cerebral venous abnormalities are areas of active study regarding the pathophysiology of idiopathic intracranial hypertension. There continues to be no evidence based consensus or formal guidelines regarding management and treatment of the disease. Treatment studies show that the diagnostic lumbar puncture is a valuable intervention beyond its diagnostic importance, and that weight management is critical. However, many questions remain regarding the efficacy of acetazolamide, CSF shunting procedures and cerebral transverse venous sinus stenting.
Topics: Bariatric Surgery; Carbonic Anhydrase Inhibitors; Humans; Magnetic Resonance Imaging; Obesity; Prevalence; Pseudotumor Cerebri
PubMed: 22423118
DOI: 10.1136/jnnp-2011-302029 -
Agri : Agri (Algoloji) Dernegi'nin... 2015Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure of unknown cause, leading to severe headache, papilledema and... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure of unknown cause, leading to severe headache, papilledema and visual disturbances. Its former name, pseudotumor cerebri, has gained popularity recently. The strongest and most consistent risk factors of IIH are obesity and female gender. Infrequently, IIH may present in the absence of papilledema showing a headache profile similar to chronic daily headache with migrainous features. There have been several proposed mechanisms to explain the etiology of this disorder associated with various clinical conditions. In recent years, some inflammatory factors, natriuretic peptides and aquaporins have been proposed as possible contributors of the pathogenesis. On the other hand, some investigators have reported that bilateral transverse sinus stenosis is seen in the majority of IIH patients; therefore, dural sinus stent placement is used in some patients. No single theory has been able to provide a comprehensive answer, and there is no consensus about the exact cause of IIH. The aim of this review was to discuss the new insights on the mysterious pathogenesis of IIH.
Topics: Headache; Humans; Pseudotumor Cerebri; Transverse Sinuses
PubMed: 25944131
DOI: 10.5505/agri.2015.22599 -
JNMA; Journal of the Nepal Medical... Feb 2021Idiopathic Intracranial Hypertension is a rare occurrence in young, physically fit male and a diagnosis of exclusion among most patients presenting with signs and...
Idiopathic Intracranial Hypertension is a rare occurrence in young, physically fit male and a diagnosis of exclusion among most patients presenting with signs and symptoms of raised intracranial pressure. Here we describe a case of a young male in the ideal weight range with no previous exposure to offending chemicals presented with a history of headache, obscuration of vision, and photopsia. On examination, there were no positive neurological findings. Increased opening pressure was found on the lumbar puncture. Ophthalmological examination revealed bilateral papilledema. Humphrey’s Visual field test showed peripheral field loss. Magnetic resonance imaging scan of the brain and orbits were normal. The patient was diagnosed and managed in primary care setting after neurosurgical consultation. Though rare, we should suspect idiopathic intracranial hypertension in ideal body weighted male if the headache is persistent after other causes of headache have been ruled out.
Topics: Headache; Humans; Intracranial Hypertension; Male; Papilledema; Pseudotumor Cerebri; Vision Disorders
PubMed: 34506475
DOI: 10.31729/jnma.5176