-
RoFo : Fortschritte Auf Dem Gebiete Der... Nov 2021Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a disorder of increased intracranial pressure of unknown etiology. Main symptom is...
BACKGROUND
Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a disorder of increased intracranial pressure of unknown etiology. Main symptom is headache which has a characteristic similar to other headache diseases, therefore the idiopathic intracranial hypertension often remains undetected. The incidence is 1/100 000 with the number of unreported cases being much higher. This article highlights the essential role of MRI in the differential diagnosis.
METHODS
A literature search was carried out on idiopathic intracranial hypertension and Pseudotumor cerebri in English and German. Original and Review articles as well as case reports were taken into account. Since the main focus of the article is MRI diagnostics, some images were selected to illustrate the characteristic MRI morphological features.
RESULTS AND CONCLUSION
The diagnosis of idiopathic intracranial hypertension is based on an exclusion procedure. Most common syndromes are headache, transient visual obscurations, pulsatile tinnitus and nausea. In the presence of an underlying cause for the increased intracranial pressure one speaks of a secondary intracranial hypertension. The diagnostics include a detailed medical history, neurological and ophthalmic examination, lumbar puncture, and neuroradiological imaging procedures. MRI, in particular, has become increasingly important in recent years, since signs for changes in cerebrospinal fluid pressure are now detectable and well-defined. The therapeutic approaches are symptom-oriented and aim to lower the pressure. With a precise diagnosis and timely start of therapy, idiopathic intracranial hypertension has a good prognosis, especially with regard to the preservation of eyesight.
KEY POINTS
· The idiopathic intracranial hypertension is an important differential diagnosis for unspecific headache and impaired vision. · Overweight women in childbearing age are particularly affected. · The most important component in diagnostics is MRI.
CITATION FORMAT
· Juhász J, Hensler J, Jansen O. MRT-Befunde bei der idiopathischen intrakraniellen Hypertension (Pseudotumor cerebri). Fortschr Röntgenstr 2021; 193: 1269 - 1276.
Topics: Diagnosis, Differential; Female; Headache; Humans; Magnetic Resonance Imaging; Pseudotumor Cerebri; Vision Disorders
PubMed: 33979869
DOI: 10.1055/a-1447-0264 -
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 -
American Journal of Ophthalmology Feb 2015To examine the clinical, demographic, and anthropometric patient characteristics of secondary pseudotumor cerebri syndrome in children and adolescents based on the... (Observational Study)
Observational Study
PURPOSE
To examine the clinical, demographic, and anthropometric patient characteristics of secondary pseudotumor cerebri syndrome in children and adolescents based on the recently revised diagnostic criteria.
DESIGN
Retrospective observational case series.
METHODS
Patients seen at a tertiary children's hospital for pseudotumor cerebri syndrome were classified as having either primary idiopathic (n = 59) or secondary pseudotumor cerebri syndrome (n = 16), as rigorously defined by recently revised diagnostic criteria. Outcomes included body mass index Z-scores (BMI-Z), height and weight Z-scores, demographics, and clinical features at presentation, such as headache, sixth nerve palsy, and cerebrospinal fluid (CSF) opening pressure.
RESULTS
In this cohort, the associated conditions and exposures seen in definite secondary pseudotumor cerebri syndrome included tetracycline-class antibiotics (n = 11), chronic kidney disease (n = 3), withdrawal from chronic glucocorticoids (n = 1), and lithium (n = 1). Other associations observed in the possible secondary pseudotumor cerebri syndrome group included Down syndrome, vitamin A derivatives, and growth hormone. In comparison with primary pseudotumor cerebri syndrome, definite secondary pseudotumor cerebri syndrome patients were on average older (15.0 vs 11.6 years; P = .003, Mann-Whitney test). According to US Centers for Disease Control (CDC) classifications, 79% of children with secondary pseudotumor cerebri syndrome were either overweight or obese (36% overweight [n = 5] and 43% obese [n = 6]), as compared to 32% nationally.
CONCLUSIONS
Even when a potential inciting exposure is identified for pediatric pseudotumor cerebri syndrome, the possible contribution of overweight and obesity should be considered.
Topics: Adolescent; Body Constitution; Body Mass Index; Cerebrospinal Fluid Pressure; Child; Child, Preschool; Female; Hospitals, Pediatric; Humans; Intracranial Pressure; Male; Obesity; Overweight; Pseudotumor Cerebri; Retrospective Studies
PubMed: 25447107
DOI: 10.1016/j.ajo.2014.11.003 -
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 -
Archivos Argentinos de Pediatria Apr 2014
Topics: Child; Female; Humans; Pseudotumor Cerebri
PubMed: 24584797
DOI: 10.5546/aap.2014.198 -
Acta Neurologica Scandinavica Feb 2010Idiopathic intracranial hypertension (IIH) is a condition which affects predominantly overweight women and is characterized by raised intracranial pressure without any... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a condition which affects predominantly overweight women and is characterized by raised intracranial pressure without any identifiable pathology in the brain and with normal cerebrospinal fluid (CSF) composition. The cause of IIH is unclear and as such it remains a diagnosis of exclusion. Although the pathophysiology of IIH remains elusive, some observations have recently been added to our understanding of this, including the presence of transverse sinus stenosis on many patients and the possible role of leptin and inflammation in the disease pathogenesis. Headache is the most common symptom and papilloedema is the major clinical finding. Choices of medical treatment are limited to the use of diuretics particularly acetazolamide and encouragement of weight loss. Surgical therapies such as CSF diversion procedures and fenestration of the optic nerve may be necessary in some cases with persistent symptoms or progressive visual deterioration. While not life-threatening, IIH has a significant morbidity with up to 25% of patients developing visual impairment from optic atrophy. Visual surveillance is therefore vital. Long-term follow-up is recommended as the disease may worsen after an initial period of stability.
Topics: Humans; Pseudotumor Cerebri
PubMed: 19930211
DOI: 10.1111/j.1600-0404.2009.01172.x -
Journal of Neuro-ophthalmology : the... Mar 2022Pediatric pseudotumor cerebri syndrome (PTCS) is a vision-threatening condition that is associated with female sex and obesity in pubertal and postpubertal children. It...
BACKGROUND
Pediatric pseudotumor cerebri syndrome (PTCS) is a vision-threatening condition that is associated with female sex and obesity in pubertal and postpubertal children. It is unknown whether the increase in childhood obesity during the COVID-19 pandemic has affected the rates and characteristics of pediatric PTCS.
METHODS
We conducted a retrospective study of children evaluated for PTCS (inpatient or emergency department) at our children's hospital before (March 19, 2015 to March 19, 2020) and during (March 20, 2020 to February 20, 2021) the pandemic. We compared the monthly number of inpatient and emergency department encounters for pediatric PTCS before and during the pandemic. In addition, anthropometric and ophthalmologic characteristics of children evaluated for pediatric PTCS before and during the pandemic were compared.
RESULTS
A total of 36 encounters in the 5 years before the pandemic and 26 encounters in the 11 months during the pandemic were identified. The median monthly number of encounters for pediatric PTCS was significantly higher during the pandemic compared with the 5 years before the pandemic (2 vs 0, P = 0.0021). Compared with prepandemic patients, children evaluated during the pandemic were older (median age 16 vs 14 years, P = 0.02), with higher rates of obesity (85% vs 66%, P = 0.05) and lower likelihood of reporting Caucasian race (4% vs 31%, P = 0.02). Pandemic patients had worse presenting visual acuity (median logMAR 0.14 vs 0.05, P = 0.05) and were more likely to have fulminant presentation (23% vs 6%, P = 0.04) and require surgical intervention (23% vs 6%, P = 0.04).
CONCLUSIONS
At our children's hospital, the rate of inpatient admissions and emergency department visits for pediatric PTCS increased during the pandemic. The severity of disease and frequency of surgical treatment also increased. Racial and ethnic minorities seem to be disproportionately affected. These changes may be related to increasing rates of childhood obesity during the pandemic.
Topics: Adolescent; COVID-19; Child; Emergency Service, Hospital; Female; Humans; Inpatients; Pandemics; Pediatric Obesity; Pseudotumor Cerebri; Retrospective Studies
PubMed: 34803141
DOI: 10.1097/WNO.0000000000001480 -
BMJ (Clinical Research Ed.) Jul 2010
Topics: Adult; Diet, Reducing; Female; Humans; Intracranial Pressure; Papilledema; Prospective Studies; Pseudotumor Cerebri
PubMed: 20610513
DOI: 10.1136/bmj.c2836 -
Pediatric Rheumatology Online Journal Apr 2022Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that typically affects multiple organs and can lead to potentially fatal complications.... (Review)
Review
BACKGROUND
Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that typically affects multiple organs and can lead to potentially fatal complications. Central nervous system (CNS) involvement in SLE is common, especially in children, and can present nonspecifically with various neuropsychiatric manifestations, described as neuropsychiatric SLE (NPSLE). Chronic headache is a common feature of NPSLE, secondary to increased intracranial pressure (also called pseudotumor cerebri (PTC)) due to inflammation or medication. Here, we highlight the importance of evaluating refractory headache (HA) in SLE patients to rule out PTC as a cause of severe morbidity.
METHODS
Single tertiary care pediatric center case series of 8 children who developed NPSLE in the form of intracranial hypertension at or after SLE diagnosis.
CONCLUSION
Neurologic and ophthalmologic evaluation of refractory HA in patients with SLE, especially children, is warranted to decrease the burden of the disease and rule out treatable causes like PTC.
Topics: Child; Headache Disorders; Humans; Lupus Erythematosus, Systemic; Lupus Vasculitis, Central Nervous System; Pseudotumor Cerebri
PubMed: 35428311
DOI: 10.1186/s12969-022-00688-5 -
Journal of the Neurological Sciences Aug 2000The purpose of this theoretical study is to determine whether the absence of ventricular enlargement in pseudotumor cerebri (PTC) is consistent with the theory that PTC... (Review)
Review
The purpose of this theoretical study is to determine whether the absence of ventricular enlargement in pseudotumor cerebri (PTC) is consistent with the theory that PTC is caused by reduced absorption of cerebrospinal fluid (CSF), either from increased outflow resistance at the arachnoid villi or from obstruction of the dural venous sinuses. We model the brain as a thick spherical shell of parenchyma, enclosing a CSF-filled ventricular system, and surrounded by a thin cerebral subarachnoid space (CSAS). We treat the parenchyma as a porous solid matrix, filled with interstitial fluid and blood vessels. We subject the model to a uniform increase in CSF pressure (CSFP) and solve the equations of poroelasticity for the resulting displacements of parenchymal tissue. The effect of a rise in CSFP on ventricular size depends on the response of the cerebral blood vessels and the degree to which the pia is tethered to the dura. If the cerebral vessels decrease in caliber with increasing CSFP, a rise in CSFP causes the ventricles to contract and the CSAS to expand if the pial surface is free to move inward, but causes slight ventricular enlargement if the pia is tethered to the dura. If, instead, the vessels dilate, the ventricles contract and the CSAS becomes effaced. Small, normal, or slightly enlarged ventricles in PTC are consistent with the theory of reduced CSF absorption.
Topics: Brain Neoplasms; Cerebral Ventricles; Humans; Pseudotumor Cerebri
PubMed: 10980304
DOI: 10.1016/s0022-510x(00)00348-8