topiramate and Pseudotumor-Cerebri

Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology that primarily affects obese women of childbearing age. Symptoms include disabling headaches, visual disturbances, and intracranial noises (pulsatile tinnitus). Currently, no standardized treatment guidelines are available and the current management focuses on weight loss and acetazolamide use. There is an increasing body of evidence suggesting that the initial use of topiramate may be considered in IIH treatment. Acetazolamide is the recommended initial treatment for IIH, with topiramate often used as a second-line agent. Topiramate has multiple benefits to indicate it would pose effective in IIH management. Through varying mechanisms, it leads to weight loss and improves migraine headache control, the most common headache phenotype in IIH. Topiramate also inhibits the carbonic anhydrase enzyme like acetazolamide to reduce intracranial pressure and treat papilledema. The safety profile of topiramate is comparable or superior to acetazolamide. To date, there are limited studies comparing topiramate to acetazolamide or other treatment modalities in IIH. Based on its varying mechanisms of action, topiramate is a strong potential treatment agent for IIH, yet acetazolamide is often chosen first-line. However, the data supporting use of acetazolamide or topiramate is inefficient to designate one agent preferred over the other. There is a need for further studies assessing topiramate use in the treatment of IIH, and comparing topiramate use to other treatment modalities.

Topics: Acetazolamide; Female; Headache; Humans; Intracranial Hypertension; Intracranial Pressure; Pseudotumor Cerebri; Topiramate; Weight Loss

Topics: Adult; Anticonvulsants; Female; Fructose; Humans; Isoxazoles; Kidney Calculi; Migraine Disorders; Pseudotumor Cerebri; Topiramate; Zonisamide

Persistent headache and loss of visual acuity combined with papilledema are the predominant symptoms of idiopathic intracranial hypertension (IIH). The clinical signs are not different from those seen in other diseases with elevated intracranial pressure. To differentiate primary and secondary forms of increased intracranial pressure neuroimaging procedures and analysis of cerebrospinal fluid (CSF) are absolutely essential according to national and international guidelines. Lumbar puncture reveals an elevated opening pressure in cases of IIH as the only pathological finding. Treatment options are serial lumbar punctures combined with body weight reduction as well as medication with carbonic anhydrase inhibitors, such as acetazolamide and topiramate or diuretic therapy with furosemide. In some patients surgical options, e.g. optic nerve sheath fenestration, CSF shunting procedures including ventriculoperitoneal and lumboperitoneal shunt systems and bariatric surgery also have to be considered. In recent years modern neuroradiological procedures have also been applied (e.g. venous stenting in cases of sinus obstruction) in some centers.

Topics: Acetazolamide; Cerebrospinal Fluid Shunts; Combined Modality Therapy; Diagnosis, Differential; Fructose; Furosemide; Guideline Adherence; Headache Disorders; Neurologic Examination; Pseudotumor Cerebri; Spinal Puncture; Topiramate; Vision Disorders; Visual Acuity; Weight Loss

Pseudotumor cerebri syndrome (PTCS) refers to the primary and secondary disorders that cause elevated intracranial pressure without an intracranial mass lesion, ventriculomegaly, or central nervous system infection or malignancy. Headache is the most frequent symptom of PTCS, but there is considerable overlap between the headache features of raised intracranial pressure and the headache features of primary headache disorders. We review headache subtypes that occur in PTCS, non-headache features that help distinguish PTCS from other headache types, changes to the diagnostic criteria for PTCS with and without papilledema, and headache treatment strategies as they apply to PTCS.

Topics: Acetazolamide; Back Pain; Diagnosis, Differential; Diuretics; Fructose; Furosemide; Headache Disorders; Humans; Intracranial Hypertension; Migraine Disorders; Neuroprotective Agents; Papilledema; Pseudotumor Cerebri; Tinnitus; Topiramate; Vision Disorders; Weight Reduction Programs

To review the current standard of care in the diagnosis and treatment of idiopathic intracranial hypertension (IIH). We also discuss recent advances in the understanding of the pathogenesis of IIH with emerging trends in management of this syndrome.. There has been increasing literature suggesting a role of vitamin A, adipokines, sleep disorders, and venous sinus stenosis in the pathogenesis of IIH. Newer medical treatment options like topiramate and surgical treatment options like stereotactic ventriculoperitoneal shunting have been found to be attractive alternatives. There has also been an emerging interest in cerebral venous sinus stenting, though its role and utility remain debatable.. This article seeks to present a systematic approach to the management of IIH. Many newer treatment modalities are being explored for IIH refractory to standard medical therapy, but their efficacy and safety must be demonstrated in large studies before they can be adopted as part of standard treatment.

Topics: Cerebrospinal Fluid; Fructose; Humans; Intracranial Hypertension; Papilledema; Pseudotumor Cerebri; Risk Factors; Topiramate; Ventriculoperitoneal Shunt

OBJECTIVES - To assess the efficacy of topiramate in the treatment of idiopathic intracranial hypertension (IIH) and to compare it with acetazolamide. METHODS - Fourty patients diagnosed as IIH and randomly assigned to treatment with either acetazolamide or topiramate were assessed prospectively. Improvement in the visual fields at the end of third, sixth and twelfth months were taken into consideration. RESULTS - The demographic, clinical features and the cerebrospinal fluid (CSF) pressure of the two treatment groups were similar at the beginning of the study. When the follow-up visual field grades were compared with the visual field grades at the beginning of the study in each group a statistically significant improvement was detected with both drugs. When the results of the two treatment groups were compared with each other no statistically significant difference was present. Prominent weight loss was recorded in the topiramate group. CONCLUSIONS - Topiramate seems to be effective in the treatment of IIH. Weight reduction as well as the reduction of the CSF formation is the possible mechanism of action.

Topics: Acetazolamide; Adolescent; Adult; Anticonvulsants; Body Weight; Carbonic Anhydrase Inhibitors; Cerebrospinal Fluid Pressure; Female; Fructose; Humans; Male; Middle Aged; Prospective Studies; Pseudotumor Cerebri; Topiramate; Treatment Outcome; Vision Disorders; Visual Fields; Weight Loss

This retrospective single-center study evaluated the change in required dosage of acetazolamide and topiramate before and after dural venous sinus stent placement (VSSP) for idiopathic intracranial hypertension (IIH). Adults diagnosed with IIH who failed optimized medical management and were treated with VSSP were included. This study comprised 55 patients who underwent VSSP for the diagnosis of IIH. The median preprocedural dosage of acetazolamide and topiramate was 1,000 mg (range, 500-4,000 mg) and 100 mg (range, 0-200 mg), respectively, among patients able to tolerate the medications. The median postprocedural dosage of acetazolamide and topiramate was 375 mg (range, 0-4,000 mg), with a mean reduction of 52.9% (P = .001), and 0 mg (range, 0-200 mg), with a mean reduction of 45.9% (P = .005), respectively. Dural VSSP significantly reduced dosage requirements for acetazolamide and/or topiramate, potentially reducing the morbidity secondary to medication side effects.

Topics: Acetazolamide; Adult; Carbonic Anhydrase Inhibitors; Humans; Pseudotumor Cerebri; Retrospective Studies; Stents; Topiramate

Dural venous sinus stenting (VSS) is an effective intervention for patients with idiopathic intracranial hypertension (IIH) refractory to medical treatment. Our goal was to evaluate the efficacy by utilizing a large multi-institutional sample.. Five hundred forty-one patients >18 years old who underwent VSS within 3 years of IIH diagnosis were queried using Current Procedural Terminology and International Classification of Diseases, Tenth Revision codes from the TriNetX Analytics Network. Patient demographics, baseline symptoms, procedures, and clinical outcomes were evaluated within 1 year postoperatively. Outcomes examined were headache, tinnitus, blindness/low vision, optic nerve sheath fenestration (ONSF), cerebrospinal fluid (CSF) shunt, and use of medications (acetazolamide, methazolamide, furosemide, topiramate, tricyclic antidepressants, and valproate) for IIH. Prestent and poststent data were compared using Fisher exact test, and the odds ratios were computed using the Baptista-Pike method.. The mean age at VSS was 36.7 ± 10.6; 92% were female, 65% of patients were Caucasian, 25% were Black/African American, 1% were Asian, and 9% were of other/unknown race. Within the 1-year follow-up, acetazolamide and topiramate use were significantly reduced post-VSS (P < 0.0001∗; odds ratio, 0.45; confidence interval, 0.35-0.57 and P = 0.03∗; odds ratio, 0.71; confidence interval, 0.52-0.95, respectively). Also, headaches, visual disturbance, dizziness/giddiness, and tinnitus significantly improved post-VSS (P < 0.005∗). Finally, the number of CSF shunt procedures and ONSF procedures demonstrated no significant change post-VSS (P > 0.05).. VSS is an effective and safe procedure resulting in significant improvement of headaches, visual impairment, dizziness, and tinnitus, acetazolamide and topiramate usage were lower after VSS in patients with IIH. The paucity of pre-VSS and post-VSS CSF shunt and ONSF procedure data does not provide enough evidence to establish significance.

Topics: Acetazolamide; Cranial Sinuses; Dizziness; Female; Headache; Humans; Intracranial Hypertension; Male; Pseudotumor Cerebri; Stents; Tinnitus; Topiramate

This study aimed to identify the reversibility of MRI findings indicative of increased intracranial hypertension in idiopathic intracranial hypertension (IIH) patients after treatment.. This retrospective, observational study included demographic and clinical data from 10 patients with IIH and 10 controls. Brain MRI findings in IIH patients were recorded twice: once when patients had papilledema and again after resolution of papilledema. Neuroradiologists graded MRI findings in both groups based on an imaging grading scale.. After resolution of papilledema, all patients showed improvement in 2 or more of the MRI characteristics of IIH. This was especially the case for the height of the midsagittal pituitary gland and optic nerve sheath thickness (ONST), which were significantly different in all pairwise group comparisons. Sellar configuration, globe configuration, and horizontal orbital optic nerve tortuosity were different between the IIH pre-treatment group and controls, but not between controls and the IIH post-treatment group. We found no difference in optic nerve head hyperintensity or optic nerve thickness among the 3 groups.. We demonstrated that several morphometric MRI characteristics in IIH are reversible to a certain extent after treatment. Enlarged subarachnoid spaces filled with cerebrospinal fluid seem to remain reduced, and the ONST and height of the pituitary gland are not fully normalized after treatment.

Topics: Acetazolamide; Adult; Carbonic Anhydrase Inhibitors; Female; Humans; Magnetic Resonance Imaging; Papilledema; Pseudotumor Cerebri; Retrospective Studies; Topiramate; Treatment Outcome; Young Adult

A robust predictor of visual outcome in idiopathic intracranial hypertension (IIH) would be useful in management, but there is limited information on this point. The purpose of this study was to ascertain whether visual field mean deviation on standard static perimetry performed at diagnosis in a large patient cohort is a reliable predictor of visual outcome.. We retrospectively reviewed the automated visual field mean deviations at diagnosis and at final encounter in 79 patients with IIH examined in the neuro-ophthalmology clinics at a single academic medical center from 1999 to 2015.. Of the 79 study patients, 66 (84%) entered with visual field mean deviations of -7 dB or better. Of those 66 patients, 59 (89%) had final mean deviations of -4 dB or better and 33 (56%) had final mean deviations of -2 dB or better. The single patient who had an initial mean deviation of -7 dB or better and a poor final mean deviation (-32 dB) was nonadherent to prescribed medication. Of the 13 (21%) patients who entered with mean deviations worse than -7 dB, 11 (85%) ended up with poor visual outcomes, their final mean deviations ranging from -5 dB to -32 dB. Over half of those 13 patients had required surgery for IIH, often within 3 weeks of diagnosis, owing to severe papilledema and visual dysfunction at the time of diagnosis.. Based on this retrospective study, patients with IIH who have relatively mild visual dysfunction at diagnosis are likely to have a favorable visual outcome, provided they are adherent to recommended treatment. Many of those with poor visual function at diagnosis will have unfavorable visual outcomes despite aggressive treatment.

Topics: Acetazolamide; Adolescent; Adult; Carbonic Anhydrase Inhibitors; Diuretics; Drug Therapy, Combination; Female; Furosemide; Humans; Hypoglycemic Agents; Male; Methazolamide; Middle Aged; Papilledema; Pseudotumor Cerebri; Retrospective Studies; Topiramate; Vision Disorders; Visual Acuity; Visual Field Tests; Visual Fields; Young Adult

Pseudotumor cerebri syndrome (PTCS) is a disorder defined by increased intracranial pressure in the absence of an intracranial space-occupying lesion. This retrospective study aimed to examine the outcomes in children with PTCS.. Data was collected retrospectively from the charts of consecutive pediatric patients treated for PTCS at our hospital between 2000 and 2007 (60 patients; 36 females, 24 males).. Forty-six patients (76.6%) responded well to acetazolamide therapy, with full resolution of symptoms, including papilledema (average treatment duration 1 year; range: 1 month-5 years). Of the 14 patients with no response to treatment, 9 (23.4%) required surgical intervention. Nonresponders tended to be younger at presentation (8.7 vs 11.5 years, P = 0.04). Twelve patients (26%) experienced relapse after acetazolamide was discontinued. The group that experienced relapse was significantly younger than the nonrelapsers (8.9 vs 12.1 years, P < 0.05).. Younger age at presentation with PTCS was found to be a risk factor for treatment failure or relapse.

Topics: Acetazolamide; Adolescent; Brain; Child; Child, Preschool; Diuretics; Drug Therapy, Combination; Female; Follow-Up Studies; Forecasting; Fructose; Furosemide; Glucocorticoids; Humans; Intracranial Pressure; Magnetic Resonance Imaging; Male; Neuroprotective Agents; Pseudotumor Cerebri; Retrospective Studies; Spinal Puncture; Topiramate; Treatment Outcome

Topics: Adolescent; Anticonvulsants; Antihypertensive Agents; Drug Therapy, Combination; Female; Fructose; Glaucoma, Angle-Closure; Humans; Intraocular Pressure; Migraine Disorders; Papilledema; Pseudotumor Cerebri; Topiramate

Topics: Adult; Chemotherapy, Adjuvant; Female; Fructose; Humans; Male; Pseudotumor Cerebri; Retreatment; Topiramate

The association of pseudotumor cerebri, visual impairment, hypothyroidism, polycystic ovary syndrome (PCOS), and a hypercoagulable state due to a factor V and a prothrombin mutation has not been reported previously.. A 20-year-old obese woman developed menstrual cycle irregularities since age 14 years, initially bitemporal and latter diffuse headache since age 14 years, and bilateral visual impairment, described as sparkling black points. Ophthalmologically there was a recurrent papilledema. Clinical neurologic investigations revealed sore neck muscles and hirsutism. Magnetic resonance imaging of the brain, orbita and cervical spine, and investigations of cerebrospinal fluid were non-informative. Visually evoked potentials revealed demyelination of the optic nerves. Gynecologic investigations revealed PCOS and endocrinologic investigations hypothyroidism and hyperandrogenism. Tests for thrombophilia disclosed a heterozygote state for the G1.697A factor V Leiden and the G20.210A prothrombin mutation. A possible relationship between pseudotumor cerebri and the ophthalmologic, gynecologic, endocrinologic and coagulation abnormalities is discussed.. For the first time we describe the association of pseudotumor cerebri, optic nerve demyelination, PCOS, other endocrinologic abnormalities, and thrombophilia due to a factor V and prothrombin mutation. A causal relationship between these abnormalities remains elusive.

Topics: Adult; Demyelinating Diseases; Factor V; Female; Fructose; Humans; Hyperandrogenism; Hypothyroidism; Neuroprotective Agents; Optic Nerve Diseases; Papilledema; Polycystic Ovary Syndrome; Prothrombin; Pseudotumor Cerebri; Thrombophilia; Topiramate

Topics: Adult; Female; Fructose; Headache; Humans; Neuroprotective Agents; Pseudotumor Cerebri; Topiramate

A 14-year-old adolescent was seen with an 8-month history of almost daily incapacitating headaches due to idiopathic intracranial hypertension in Behçet syndrome. All his clinical signs and symptoms, including headache, resolved 2 to 4 weeks after topiramate was initiated. An effect on carbonic anhydrase isoenzymes II and IV, reducing cerebrospinal fluid production, could potentially explain the beneficial effect of topiramate in intracranial hypertension. Further studies are necessary, however, to confirm the significance of topiramate in this indication.

Topics: Adolescent; Anticonvulsants; Behcet Syndrome; Fructose; Headache; Humans; Male; Pseudotumor Cerebri; Topiramate

Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure without hydrocephalus or mass lesion with elevated cerebrospinal fluid (CSF) pressure but otherwise normal CSF composition. It has been found that pregnancy occurs in IIH patients at about the same rate as in the general population, that IIH can occur in any trimester of pregnancy, that patients have the same spontaneous abortion rate as the general population, and that the visual outcome is the same as for nonpregnant patients with IIH. Although it is also stated that pregnant patients with IIH should be managed and treated the same way as any other patient with IIH, the use of imaging and drug contraindications do make a difference between the two groups. The treatment has two major goals, which are to preserve vision and to improve symptoms. The medical therapy includes weight control, nonketotic diet, serial lumbar punctures, diuretics, steroids, and certain analgesics. When medical therapy fails, surgical procedures should be considered. The two main procedures are optic nerve sheath fenestration and lumboperitoneal shunt. Anesthetic considerations in the pregnant patient are an additional factor when surgeries are contemplated. It is also noted that therapeutic abortion to limit progression of disease is not indicated and that subsequent pregnancies do not increase the risk of recurrence.

Topics: Adolescent; Adult; Anticonvulsants; Antihypertensive Agents; Diagnosis, Differential; Diuretics; Female; Fructose; Humans; Pregnancy; Pregnancy Complications, Cardiovascular; Prognosis; Propranolol; Pseudotumor Cerebri; Spinal Puncture; Steroids; Topiramate; Vision Disorders