scholarly journals Therapeutic approaches to pediatric pseudotumor cerebri: New insights from literature data

2017 ◽  
Vol 30 (1) ◽  
pp. 94-97 ◽  
Author(s):  
Giovanna Vitaliti ◽  
Piero Pavone ◽  
Nassim Matin ◽  
Omidreza Tabatabaie ◽  
Salvatore Cocuzza ◽  
...  
Keyword(s):  
Treatment Guidelines ◽  
Controlled Trial ◽  
Treatment Strategies ◽  
Signs And Symptoms ◽  
Pseudotumor Cerebri ◽  
Therapeutic Approaches ◽  
Childbearing Age ◽  
Overweight Women ◽  
Cranial Nerve Palsies ◽  
Pseudotumor Cerebri Syndrome

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.

Bilateral jugular foraminal stenosis in a patient with benign osteopetrosis associated with pseudotumor cerebri syndrome

2009 ◽  
Vol 110 (4) ◽  
pp. 804-807 ◽  
Author(s):  
Sayed Mohammad Haji Mirsadeghi ◽  
Ali Tayebi Meybodi ◽  
Farideh Nejat ◽  
Hooshang Saberi
Keyword(s):  
Disease Process ◽  
Signs And Symptoms ◽  
Pseudotumor Cerebri ◽  
Underlying Disease ◽  
Foraminal Stenosis ◽  
Laboratory Studies ◽  
Venous Outflow ◽  
Shunt Insertion ◽  
Pseudotumor Cerebri Syndrome ◽  
The Brain

One of the prominent causes of pseudotumor cerebri (PTC) syndrome is increased impedance of the venous outflow tracts of the brain. Theoretically, this is a justified mechanism for raised intracranial pressure; yet, there had been few cases of such a scenario reported in the literature, and to the authors' knowledge no case of PTC due to benign osteopetrosis has been reported to date. The present case occurred in a 19-year-old woman with a constellation of signs and symptoms compatible with PTC syndrome, whose radiological and laboratory studies confirmed the diagnosis of osteopetrosis. Magnetic resonance venography demonstrated bilateral jugular foraminal stenosis regarding the underlying disease process. The patient did well after she underwent a lumboperitoneal shunt insertion procedure.

Prevalence of Signs and Symptoms of Pseudotumor Cerebri Syndrome Before and After Transsphenoidal Surgery for Cushing’s Disease – A Prospective Consecutive Case Series

2020 ◽  
Author(s):  
Torge Huckhagel ◽  
Jörg Flitsch ◽  
Roman Rotermund ◽  
Volker Knospe
Keyword(s):  
Visual Acuity ◽  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Signs And Symptoms ◽  
Pseudotumor Cerebri ◽  
Cushing's Disease ◽  
Pseudotumor Cerebri Syndrome ◽  
Before And After ◽  
Biochemical Remission

Abstract Introduction Pseudotumor cerebri syndrome (PTCS) has anecdotally been described after successful treatment of Cushing’s disease (CD), but the prevalence following transsphenoidal surgery has not been determined so far in a prospective study. Patients and Methods 41 consecutive adult CD patients were prospectively screened for signs and symptoms of PTCS, headache-related impairment, and ophthalmological features associated with intracranial pressure elevation before surgery and at follow-up (mean 4 months). Results Biochemical remission was achieved in 36 of 41 (87.8%) patients after surgery. The most frequent preoperative complaints were visual acuity impairment (19 cases; 46.3%), headache (13 cases; 31.7%), and ear noise (9 cases; 22.0%). A significant reduction of visual disturbances was seen at follow-up. Overall, CD patients presented with fairly low headache-related emotional and functional restrictions before and after surgery. One of 34 (2.9%) patients with sufficient ophthalmological follow-up showed new-onset papilledema combined with temporary worsening of visual acuity and scotoma. Conclusion Our results suggest a very low frequency of signs and symptoms of PTCS after surgical treatment for CD in adults. This estimate is in line with previous outcomes from retrospective pediatric CD series. Further large-scale studies are needed to determine the actual prevalence of this condition following biochemical remission of CD.

Treatment of Anderson-Fabry Disease

2020 ◽  
Vol 26 (40) ◽  
pp. 5089-5099 ◽  
Author(s):  
Irene Simonetta ◽  
Antonino Tuttolomondo ◽  
Mario Daidone ◽  
Salvatore Miceli ◽  
Antonio Pinto
Keyword(s):  
Fabry Disease ◽  
Treatment Strategies ◽  
Signs And Symptoms ◽  
Current Treatment ◽  
Viral Gene ◽  
Pharmacological Chaperone ◽  
Enzyme Replacement ◽  
Cell Based Therapy ◽  
Organ Manifestations ◽  
Alpha Galactosidase

: Fabry disease is an X-linked disorder of glycosphingolipid metabolism that results in progressive accumulation of neutral glycosphingolipids, predominantly globotriaosylsphingosine (Gb3) in lysosomes, as well as other cellular compartments of several tissues, causing multi-organ manifestations (acroparesthesias, hypohidrosis, angiokeratomas, signs and symptoms of cardiac, renal, cerebrovascular involvement). Pathogenic mutations lead to a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA). In the presence of high clinical suspicion, a careful physical examination and specific laboratory tests are required. Finally, the diagnosis of Fabry’s disease is confirmed by the demonstration of the absence of or reduced alpha-galactosidase A enzyme activity in hemizygous men and gene typing in heterozygous females. Measurement of the biomarkers Gb3 and Lyso Gb3 in biological specimens may facilitate diagnosis. The current treatment of Anderson-Fabry disease is represented by enzyme replacement therapy (ERT) and oral pharmacological chaperone. Future treatments are based on new strategic approaches such as stem cell-based therapy, pharmacological approaches chaperones, mRNA therapy, and viral gene therapy. : This review outlines the current therapeutic approaches and emerging treatment strategies for Anderson-Fabry disease.

scholarly journals Study protocol: randomised controlled trial evaluating exercise therapy as a supplemental treatment strategy in early multiple sclerosis: the Early Multiple Sclerosis Exercise Study (EMSES)

BMJ Open ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. e043699
Author(s):  
Morten Riemenschneider ◽  
Lars G Hvid ◽  
Steffen Ringgaard ◽  
Mikkel K E Nygaard ◽  
Simon F Eskildsen ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Randomised Controlled Trial ◽  
Usual Care ◽  
Treatment Strategy ◽  
Controlled Trial ◽  
Treatment Strategies ◽  
Control Group ◽  
Disease Course ◽  
Disability Progression ◽  
Randomised Controlled

IntroductionIn the relapsing remitting type of multiple sclerosis (MS) reducing relapses and neurodegeneration is crucial in halting the long-term impact of the disease. Medical disease-modifying treatments have proven effective, especially when introduced early in the disease course. However, patients still experience disease activity and disability progression, and therefore, supplemental early treatment strategies are warranted. Exercise appear to be one of the most promising supplemental treatment strategies, but a somewhat overlooked ‘window of opportunity’ exist early in the disease course. The objective of this study is to investigate exercise as a supplementary treatment strategy early in the disease course of MS.Methods and analysisThe presented Early Multiple Sclerosis Exercise Study is a 48-week (plus 1-year follow-up) national multicentre single-blinded parallel group randomised controlled trial comparing two groups receiving usual care plus supervised high-intense exercise or plus health education (active control). Additionally, data will be compared with a population-based control group receiving usual care only obtained from the Danish MS Registry. The primary outcomes are annual relapse rate and MRI derived global brain atrophy. The secondary outcomes are disability progression, physical and cognitive function, MS-related symptoms, and exploratory MRI outcomes. All analyses will be performed as intention to treat.Ethics and disseminationThe study is approved by The Central Denmark Region Committees on Health Research Ethics (1-10-72-388-17) and registered at the Danish Data Protection Agency (2016-051-000001 (706)). All study findings will be published in scientific peer-reviewed journals and presented at relevant scientific conferences.Trial registration numberNCT03322761.

Challenges in Treating Chlamydia trachomatis, Including Rectal Infections: Is It Time to Go Back to Doxycycline?

2021 ◽  
pp. 106002802110299
Author(s):  
S. Lena Kang-Birken
Keyword(s):  
Chlamydia Trachomatis ◽  
Treatment Failure ◽  
English Language ◽  
Treatment Guidelines ◽  
Case Reports ◽  
Controlled Trial ◽  
Absolute Difference ◽  
Cochrane Library ◽  
Increased Risk ◽  
Meta Analyses

Objective: To evaluate recent publications on efficacy of single-dose azithromycin and 7-day doxycycline when treating Chlamydia trachomatis. Data Sources: A literature search of MEDLINE, EMBASE, PubMed, and Cochrane library was conducted (1990 to June 13, 2021) using the terms: Chlamydia trachomatis, genital chlamydia, rectal chlamydia, extragenital chlamydia, azithromycin, doxycycline, and treatment guidelines. ClinicalTrials.gov was searched to identify ongoing trials. Study Selection and Data Extraction: English language studies, including controlled studies, retrospective analyses, systematic reviews, meta-analyses, and case reports, reporting microbiological or clinical outcomes in adolescents and adults were considered. Data Synthesis: Systemic reviews and meta-analyses of randomized trials reported azithromycin efficacy of 96% to 97% in genital chlamydia. However, reports of treatment failure have emerged, especially among symptomatic males, with an increased risk of microbiological failure after azithromycin than doxycycline (relative risk = 2.45; 95% CI = 1.36-4.41). Retrospective analyses and prospective observational cohort studies reported lower efficacy range following azithromycin than doxycycline (74%-87% vs 92%-100%, respectively) in rectal chlamydia. First randomized controlled trial comparing azithromycin and doxycycline reported significantly higher microbiological cure following doxycycline, with absolute difference of 26% (95% CI = 16%-36%; P < 0.001). The proposed 2021 Centers for Disease Control and Prevention treatment guidelines designate doxycycline as the preferred agent for treatment at any site. Relevance to Patient Care and Clinical Practice: A growing body of evidence for treatment failure following azithromycin, especially in rectal chlamydia supports updating current practice. Conclusions: Doxycycline continues to achieve high efficacy in genital and rectal chlamydia. Clinicians should consider efficacy with convenience of dosing regimen, medication compliance, and sexual behavior risks when treating chlamydia infections.

Pseudotumor cerebri syndrome in a patient with narcolepsy type 1

2018 ◽  
Vol 22 (1) ◽  
pp. 194-198 ◽  
Author(s):  
Thomas Rossor ◽  
Ming Lim ◽  
Kirandeep VanDenEshof ◽  
Paul Gringras
Keyword(s):  
Pseudotumor Cerebri ◽  
Pseudotumor Cerebri Syndrome

Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement

2003 ◽  
Vol 98 (5) ◽  
pp. 1045-1055 ◽  
Author(s):  
Brian K. Owler ◽  
Geoffrey Parker ◽  
G. Michael Halmagyi ◽  
Victoria G. Dunne ◽  
Verity Grinnell ◽  
...  
Keyword(s):  
Stent Placement ◽  
Pseudotumor Cerebri ◽  
Venous Sinus ◽  
Stent Insertion ◽  
Benign Intracranial Hypertension ◽  
Content Type ◽  
Venous Sinuses ◽  
Pseudotumor Cerebri Syndrome ◽  
New Treatment ◽  
Fine Print

Object. Pseudotumor cerebri, or benign intracranial hypertension, is a condition of raised intracranial pressure in the absence of a mass lesion or cerebral edema. It is characterized by headache and visual deterioration that may culminate in blindness. Pseudotumor cerebri is caused by venous sinus obstruction in an unknown percentage of cases. The purpose of this study was to investigate the role of cerebral venous sinus disease in pseudotumor cerebri and the potential of endoluminal venous sinus stent placement as a new treatment. Methods. Nine consecutive patients in whom diagnoses of pseudotumor cerebri had been made underwent examination with direct retrograde cerebral venography (DRCV) and manometry to characterize the morphological features and venous pressures in their cerebral venous sinuses. The cerebrospinal fluid (CSF) pressure was measured simultaneously in two patients. If patients had an amenable lesion they were treated using an endoluminal venous sinus stent. Five patients demonstrated morphological obstruction of the venous transverse sinuses (TSs). All lesions were associated with a distinct pressure gradient and raised proximal venous sinus pressures. Four patients underwent stent insertion in the venous sinuses and reported that their headaches improved immediately after the procedure and remained so at 6 months. Vision was improved in three patients, whereas it remained poor in one despite normalized CSF pressures. Conclusions. Patients with pseudotumor cerebri should be evaluated with DRCV and manometry because venous TS obstruction is probably more common than is currently appreciated. In patients with a lesion of the venous sinuses, treatment with an endoluminal venous sinus stent is a viable alternative for amenable lesions.

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