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Neurologic Clinics May 2024Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either... (Review)
Review
Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.
Topics: Humans; Pseudotumor Cerebri; Intracranial Hypertension; Headache; Vision Disorders; Syndrome; Papilledema
PubMed: 38575259
DOI: 10.1016/j.ncl.2024.02.001 -
Headache Mar 2014Pseudotumor cerebri syndrome (PTCS) is an uncommon disorder of raised intracranial pressure of unknown etiology. The signs and symptoms have been well described but the... (Review)
Review
Pseudotumor cerebri syndrome (PTCS) is an uncommon disorder of raised intracranial pressure of unknown etiology. The signs and symptoms have been well described but the pathogenesis remains a mystery. Most of the evidence suggests increased resistance to cerebrospinal fluid outflow as being pivotal to the disorder. Any comprehensive theory on causation will have to explain the preponderance of obese women of childbearing age with primary PTCS and lack of ventriculomegaly in the disorder. It is possible that female sex hormones, along with endocrinologically active adipose tissue, directly result in the syndrome, in those genetically predisposed. Aldosterone has been proposed also as important in the development of PTCS. Vitamin A, in the form of retinoic acid, may also play a pivotal role, and is influenced by both estrogen and adipose tissue. This article reviews proposed mechanisms of PTCS.
Topics: Female; Humans; Male; Pseudotumor Cerebri
PubMed: 24433163
DOI: 10.1111/head.12291 -
Neurosurgery Clinics of North America Apr 2022Idiopathic intracranial hypertension, pseudotumor cerebri, and benign intracranial hypertension are terms used to describe a neurologic syndrome characterized by... (Review)
Review
Idiopathic intracranial hypertension, pseudotumor cerebri, and benign intracranial hypertension are terms used to describe a neurologic syndrome characterized by elevated intracranial pressure, headache, vision loss, and absence of underlying mass lesion and infection. Increased cerebrospinal fluid (CSF) production has been proposed to play a role in this condition; however, in patients with CSF hypersecretion with known causes such as choroid plexus hyperplasia, patients often develop ventriculomegaly and hydrocephalus. Classically, pseudotumor cerebri is diagnosed as a triad of headache, visual changes, and papilledema. This article discusses the role of medical and surgical management and the expanding role of venous stenting.
Topics: Constriction, Pathologic; Humans; Hydrocephalus; Intracranial Hypertension; Pseudotumor Cerebri; Vision Disorders
PubMed: 35346452
DOI: 10.1016/j.nec.2021.11.002 -
Current Opinion in Ophthalmology Sep 2016Recent findings in the literature regarding the epidemiology, diagnosis, and treatment of pediatric pseudotumor cerebri (PTC) are described. In the past, children with... (Review)
Review
PURPOSE OF REVIEW
Recent findings in the literature regarding the epidemiology, diagnosis, and treatment of pediatric pseudotumor cerebri (PTC) are described. In the past, children with PTC have been treated like adult cases; however, the literature highlights important differences between children and adults with PTC.
RECENT FINDINGS
Revised diagnostic criteria define an opening cerebrospinal fluid pressure greater than 28 cm water as elevated in the pediatric population. Obesity is an important risk factor for primary and secondary PTC in post-pubertal children. Magnetic resonance imaging shows findings suggestive of elevated intracranial pressure in children with PTC, similar to those of adults with PTC. Diamox and weight loss are effective treatments for PTC patients with mild visual field loss. Severe papilledema, decreased vision, and optical coherence tomography measures at presentation identify patients at increased risk for subsequent visual loss.
SUMMARY
Findings summarized in the recent literature may change practice patterns in the diagnosis and treatment of pediatric PTC. Although there are many similarities between the adult and pediatric populations, the differences should be considered when managing children with PTC.
Topics: Acetazolamide; Carbonic Anhydrase Inhibitors; Cerebrospinal Fluid Pressure; Child; Humans; Magnetic Resonance Imaging; Obesity; Papilledema; Pseudotumor Cerebri; Risk Factors
PubMed: 27491010
DOI: 10.1097/ICU.0000000000000300 -
Current Neurology and Neuroscience... Mar 2004Pseudotumor cerebri (PTC) is a syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal cerebrospinal fluid (CSF) composition.... (Review)
Review
Pseudotumor cerebri (PTC) is a syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal cerebrospinal fluid (CSF) composition. Although often considered to be "idiopathic," detailed investigation has revealed a high incidence of venous outflow abnormalities in PTC syndrome patients. The thought that elevated intracranial venous sinus pressure is a "universal mechanism" for PTC syndrome of varying etiologies has been called into question by a study indicating that the increased venous pressure in idiopathic PTC patients is caused by the elevated intracranial pressure and not the reverse, suggesting that "the chicken is the CSF pressure elevation and the egg is the venous sinus pressure elevation." Vitamin A toxicity may play a role in the pathogenesis of idiopathic PTC. The treatment of PTC has two major goals: the alleviation of symptoms and preservation of visual function. When medical therapy fails or when visual dysfunction deteriorates, surgical therapies for PTC should be considered. The two main procedures performed include lumboperitoneal shunt and optic nerve sheath fenestration. Because of the association of PTC with venous sinus hypertension, some authors are considering venous sinus stenting for refractory cases of PTC. It is still unclear if primary treatment of the observed venous stenosis benefits patients with idiopathic PTC. This should be no surprise, as it is not certain whether the stenoses are the cause or the result of idiopathic PTC.
Topics: Humans; Pseudotumor Cerebri; Vision Disorders
PubMed: 14984682
DOI: 10.1007/s11910-004-0024-6 -
Annals of Internal Medicine Aug 1982Pseudotumor cerebri, a disorder of intracranial pressure regulation, is characterized by elevated cerebrospinal fluid pressures. The symptoms and signs are those of... (Review)
Review
Pseudotumor cerebri, a disorder of intracranial pressure regulation, is characterized by elevated cerebrospinal fluid pressures. The symptoms and signs are those of elevated intracranial pressure alone. Extensive endocrine studies in patients with the primary disease have generally been normal. The major threat to the patient with pseudotumor cerebri is loss of vision; severe deficits occur in 4% to 12% of patients. The causative mechanism is not known. There are no controlled studies of treatment and interpretation of uncontrolled studies is limited by the high rate of spontaneous remission. A short course of steroid therapy is recommended for the patient with significant symptoms that have not remitted after 7 to 10 days of observation and serial lumbar punctures. Shunting is considered in refractory patients or when loss of vision is threatened.
Topics: Age Factors; Female; Gonadal Steroid Hormones; Humans; Male; Obesity; Pregnancy; Pseudotumor Cerebri; Sex Factors; Vision Disorders
PubMed: 7049034
DOI: 10.7326/0003-4819-97-2-249 -
Neurologic Clinics Feb 2004Pseudotumor cerebri is a perplexing syndrome of increased intra-cranial pressure without a space-occupying lesion. The terminology for the disorder has changed over the... (Review)
Review
Pseudotumor cerebri is a perplexing syndrome of increased intra-cranial pressure without a space-occupying lesion. The terminology for the disorder has changed over the years and the diagnostic criteria revised to reflect advances in diagnostic technology and insights into the disease process. The classification and nomenclature depend on the presence or absence of an underlying cause. When the diagnostic criteria are followed, a secondary etiology is unlikely. When no secondary cause is identified, the syndrome is termed "idiopathic intracranial hypertension."
Topics: Diagnosis, Differential; Headache; Humans; Papilledema; Pseudotumor Cerebri; Vision Disorders
PubMed: 15062530
DOI: 10.1016/S0733-8619(03)00096-3 -
European Journal of Ophthalmology May 2022
Topics: Humans; Lymecycline; Pseudotumor Cerebri
PubMed: 35037776
DOI: 10.1177/11206721211072373 -
Seminars in Pediatric Neurology May 2017Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS... (Review)
Review
Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS can occur in the pediatric and adult populations and, if untreated, may lead to permanent visual loss. In this review, discussion will focus on PTCS in the pediatric population and will outline its distinct epidemiology and key elements of diagnosis, evaluation and management. Finally, although the precise mechanisms are unclear, the underlying pathophysiology will be considered.
Topics: Brain; Child; Disease Management; Humans; Pseudotumor Cerebri
PubMed: 28941525
DOI: 10.1016/j.spen.2017.04.002 -
Surgical Neurology Apr 1987
Topics: Humans; Pseudotumor Cerebri; Vision Disorders
PubMed: 3824149
DOI: 10.1016/0090-3019(87)90025-5