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Brain & Development Aug 2022To describe the clinical characteristics of children with pseudotumor cerebri syndrome (PTCS) who were diagnosed according to the modified Dandy criteria and to...
AIM
To describe the clinical characteristics of children with pseudotumor cerebri syndrome (PTCS) who were diagnosed according to the modified Dandy criteria and to reclassify them according to the newly proposed diagnostic criteria by Freidman.
METHODOLOGY
This retrospective study included the period from January 2016-to July 2021.
RESULTS
50 patients were included; 34 males and 16 females with a male to female ratio of 2.1:1. The average age at onset of symptoms was 8 years. Obesity was noticed in 6 (12%) patients; 34 (68%) had symptoms upon presentation. The most common presenting symptom was headache (28 patients; 56%), papilledema was present in 33 (66%) patients. Most patients (37; 74%) had an initial cerebrospinal fluid (CSF) pressure ≥280 mmHO. At last follow-up, papilledema resolved in 11/32 (34.3%) patients, and headache resolved in 17/23 (74%) patients. 22/50 (44%) patients fulfilled the definite criteria proposed by Freidman, 11/50 (22%) fulfilled the probable, 10/50 (20%) were categorized as possible, and 7 (14%) patients were categorized as unmet.
CONCLUSION
PTCS is a chronic condition. Managing patients who do not have papilledema or who do not meet the newly proposed higher CSF pressure is challenging. Although, applying the newly proposed criteria captured most of our patients, however, around one quarter were managed based on clinical experience. This study indicates a strong need for future guidelines tailored specifically for children, taking into consideration that the cut-off point of CSF pressure might not be similar for all populations.
Topics: Cerebrospinal Fluid Pressure; Child; Female; Headache; Humans; Male; Papilledema; Pseudotumor Cerebri; Retrospective Studies
PubMed: 35393129
DOI: 10.1016/j.braindev.2022.03.006 -
Otolaryngologic Clinics of North America Jun 2022Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri syndrome, is a disorder defined as elevated intracranial pressure (ICP) of unknown cause. It... (Review)
Review
Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri syndrome, is a disorder defined as elevated intracranial pressure (ICP) of unknown cause. It is a diagnosis of exclusion in most cases, and all other forms of elevated ICP must be ruled out. With its increasing prevalence, it is much more likely for physicians, otolaryngologists included, to encounter this condition. It is important to have a clear understanding of the typical and atypical presentation of this disease, along with its evaluative workup and management options. This article reviews IIH with a focus on those factors that are specifically relevant to otolaryngologic care.
Topics: Humans; Pseudotumor Cerebri
PubMed: 36803374
DOI: 10.1016/j.otc.2022.09.016 -
Current Pain and Headache Reports Feb 2018The purpose of this review is to provide an update on pediatric intracranial hypertension. (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to provide an update on pediatric intracranial hypertension.
RECENT FINDINGS
The annual pediatric incidence is estimated at 0.63 per 100,000 in the USA and 0.71 per 100,000 in Britain. The Idiopathic Intracranial Hypertension Treatment Trial found improvement in visual fields, optical coherence tomography, Frisen grade, and quality of life with acetazolamide compared to placebo in adult patients, and these findings have been translated to the pediatric population. Pediatric intracranial hypertension is a disorder that if left untreated can lead to poor quality of life and morbidity. There are no current treatment studies in pediatrics, but adult data suggests acetazolamide remains an acceptable first-line medication.
Topics: Acetazolamide; Carbonic Anhydrase Inhibitors; Child; Early Diagnosis; Humans; Pseudotumor Cerebri; Quality of Life; Terminology as Topic; Tomography, Optical Coherence; Treatment Outcome; Visual Fields
PubMed: 29441432
DOI: 10.1007/s11916-018-0665-9 -
Praxis Apr 2022CME: Idiopathic Intracranial Hypertension Idiopathic intracranial hypertension is a pressure-induced secondary headache disorder and optic neuropathy. It primarily...
CME: Idiopathic Intracranial Hypertension Idiopathic intracranial hypertension is a pressure-induced secondary headache disorder and optic neuropathy. It primarily affects obese women of childbearing age and poses an interdisciplinary challenge both diagnostically and therapeutically. The most common symptom of this disorder are headaches frequently accompanied by photo- and/or phonophobia, whose semiology often resembles that of migraine, followed by transient visual obscurations and pulsatile tinnitus. While protection of visual acuity and visual fields are of first therapeutical priority, adequate headache treatment also plays a key role. In the majority of cases, conservative treatment including weight loss and pharmacological therapy is sufficient. In case of a fulminant disease course or loss of visual function, interventional strategies can be applied additionally. Headache treatment is guided by the predominant semiology.
Topics: Female; Headache; Humans; Intracranial Hypertension; Migraine Disorders; Pseudotumor Cerebri; Vision Disorders; Weight Loss
PubMed: 35414254
DOI: 10.1024/1661-8157/a003838 -
Child's Nervous System : ChNS :... Oct 2021Pseudotumor cerebri syndrome (PTC) is characterized by increased intracranial pressure without a space-occupying lesion and a normal cerebrospinal fluid (CSF)...
PURPOSE
Pseudotumor cerebri syndrome (PTC) is characterized by increased intracranial pressure without a space-occupying lesion and a normal cerebrospinal fluid (CSF) composition without evidence of CSF infection. In this study, we aimed to compare the symptoms, signs, and clinical characteristics of patients presenting with a preliminary diagnosis of pseudotumor cerebri syndrome (PTC) who were diagnosed and not diagnosed with PTC.
METHOD
We conducted a retrospective study of patients who were referred to our clinic with signs and symptoms of PTC. We compared the patients' symptoms, signs, and clinical characteristics who were diagnosed with PTC with those who were not diagnosed with PTC using modified Dandy criteria.
RESULTS
Ninety-four patients with the pre-diagnosis of PTC were included in the study. LP procedure was done in all patients. After LP, 75.3% of the patients were diagnosed with PTC, but 24.7% did not meet the criteria for PTC. A statistically significant relationship was found between the increase in headache complaints when leaning forward, headache that keeps the child from playing, and the CSF pressure level (p = 0.014, p = 0.019; p < 0.05). There was no statistically significant correlation between papilledema and CSF pressure level (p > 0.05). A statistically significant relationship was found between papilledema grade and CSF pressure level (p = 0.038; p < 0.05), and the rate of high CSF pressure in the groups with Grades 2-3 and Grade 4 papilledema was higher than that in the group with Grade 1 papilledema. Cranial nerve 6 palsy (CN6) (p = 0.048) and flattening of the posterior aspect of the globe (FPS) are found independent risk factors (p = 0.004 p < 0.05).
CONCLUSIONS
PTC signs and symptoms show variability among pediatric population.
Topics: Cerebrospinal Fluid Pressure; Child; Humans; Intracranial Hypertension; Papilledema; Pseudotumor Cerebri; Retrospective Studies
PubMed: 34263340
DOI: 10.1007/s00381-021-05279-5 -
Radiologia 2020Pseudotumor cerebri is a disorder characterized by increased intracranial pressure that predominantly affects obese young women. This paper aims to define the concepts...
Pseudotumor cerebri is a disorder characterized by increased intracranial pressure that predominantly affects obese young women. This paper aims to define the concepts of primary pseudotumor cerebri, in which the cause cannot be identified (also known as idiopathic intracranial hypertension), and secondary pseudotumor cerebri, in which the cause can be identified. We review the current role of imaging techniques in diagnosing pseudotumor cerebri and describe and illustrate the most characteristic imaging findings of the disorder, some of which are included in the diagnostic criteria proposed in 2013. We also consider the fundamental role of interventional radiology in the treatment of pseudotumor cerebri because placing a stent in stenosed venous sinuses is a novel treatment option in patients who are refractory to classical treatment. Finally, we describe the imaging biomarkers that have been evaluated for diagnosing primary pseudotumor cerebri and predicting the response to treatment.
Topics: Humans; Pseudotumor Cerebri; Syndrome
PubMed: 32736880
DOI: 10.1016/j.rx.2020.05.007 -
Neurology Jul 2014
Topics: Brain; Female; Humans; Male; Neuroimaging; Pseudotumor Cerebri
PubMed: 25136709
DOI: No ID Found -
Current Opinion in Ophthalmology Nov 2018We aim to provide a comprehensive and updated review on idiopathic intracranial hypertension (IIH), including the most current studies and treatment options. Special... (Review)
Review
PURPOSE OF REVIEW
We aim to provide a comprehensive and updated review on idiopathic intracranial hypertension (IIH), including the most current studies and treatment options. Special focus will be put on recent theories about the pathophysiology, and on newer prospective studies on treatment modalities.
RECENT FINDINGS
The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) provided evidence supporting acetazolamide as a well tolerated first-line therapy in IIH patients with mild vision loss. Recent studies have shown venous sinus stenting as a well tolerated and effective surgical alternative for patients with refractory IIH.
SUMMARY
Idiopathic intracranial hypertension is a vision-threatening disorder that predominantly affects obese women of childbearing age. This disorder is becoming more prevalent as the obesity epidemic continues to increase. As our understanding of this disorder continues to evolve, diagnosis and management approaches have changed over time. However, the pathogenesis for IIH remains unclear. Several theories have been proposed, including abnormalities in cerebrospinal dynamics, metabolic causes and genetics. The diagnostic criteria are based on the revised Dandy criteria. Traditionally, treatment was based on clinical experiences and retrospective studies. However, a new, prospective, randomized, controlled trial, the IIHTT, provided evidence-based data to help guide medical therapy. Additionally new, prospective studies are underway for the different surgical alternatives to treat IIH.
Topics: Acetazolamide; Carbonic Anhydrase Inhibitors; Humans; Intracranial Pressure; Papilledema; Pseudotumor Cerebri
PubMed: 30169466
DOI: 10.1097/ICU.0000000000000518 -
Seminars in Ultrasound, CT, and MR Apr 2016A cerebrospinal fluid leak from the cranial cavity requires presence of a fistulous communication between the subarachnoid and extracranial space through the skull base.... (Review)
Review
A cerebrospinal fluid leak from the cranial cavity requires presence of a fistulous communication between the subarachnoid and extracranial space through the skull base. Imaging plays a crucial role in identifying and characterizing the skull base defect and evaluating coexisting pathologies that may alter surgical approach. The authors discuss the epidemiology, imaging, and management of cerebrospinal fluid leak, with particular reference to the imaging modalities and findings.
Topics: Cerebrospinal Fluid Leak; Humans; Image Enhancement; Magnetic Resonance Imaging; Prognosis; Pseudotumor Cerebri; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 27063664
DOI: 10.1053/j.sult.2015.12.002 -
International Journal of... Mar 2017The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial... (Review)
Review
The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial pressure such as headache and cranial nerve palsies, in the absence of any space-occupying mass. This condition commonly affects overweight women of childbearing age, even if it is also frequent in men and children. Children with PTCS may present with atypical signs and symptoms, with a different prognosis compared to adults. However, the treatment is the same for both children and adults, even if there are no strict treatment guidelines in regards. All treatment strategies in children are based on retrospective studies and none has been evaluated in prospective or randomized controlled trial studies. This review focuses on literature data on PTCS treatment in children.
Topics: Child; Humans; Pseudotumor Cerebri
PubMed: 27903844
DOI: 10.1177/0394632016681578