Multidisciplinary management of idiopathic intracranial hypertension in pregnancy: case series and narrative review

Abstract Idiopathic intracranial hypertension (IIH) is a neurological condition characterized by raised intracranial pressure of unknown etiology with normal cerebrospinal fluid (CSF) composition and no brain lesions. It occurs in pregnant patients at approximately the same frequency as in general population, but obstetric and anesthetic management of the pregnancy and labor remains controversial. In this article we provide a multidisciplinary review of the main aspects of IIH in pregnancy including treatment options, mode of delivery and anesthetic techniques. Additionally, we report three cases of pregnant women diagnosed with IIH between 2012 and 2019 in our institution.

Clinical and imaging features of idiopathic intracranial hypertension / 中南大学学报(医学版)

OBJECTIVES@#Idiopathic intracranial hypertension (IIH) is a syndrome that excludes secondary causes such as intracranial space-occupying lesion, hydrocephalus, cerebrovascular disease, and hypoxic ischemic encephalopathy. If not be treated promptly and effectively, IIH can cause severe, permanent vision disability and intractable, disabling headache. This study aims to explore the clinical and image features for IIH, to help clinicians to understand this disease, increase the diagnose rate, and improve the outcomes of patients.@*METHODS@#We retrospectively analyzed 15 cases of IIH that were admitted to Xiangya Hospital, Central South University, during January 2015 to September 2020. The diagnosis of IIH was based on the updated modified Dandy criteria. We analyzed clinical data of patients and did statistical analysis, including age, gender, height, weight, medical history, physical examination, auxiliary examination, treatment and outcome.@*RESULTS@#There were 10 females and 5 males. Female patients were 22 to 42 years old with median age of 39.5. Male patients were 27 to 52 years old with the median age of 44.0. The BMI was 24.14-34.17 (28.71±2.97) kg/m@*CONCLUSIONS@#IIH primarily affects women of childbearing age who are overweight. The major hazard of IIH is the severe and permanent visual loss. Typical image signs have high specificity in IIH diagnosis. Prompt diagnosis and effective treatment are significantly important to improve the outcomes of patients.

Research Advances in Idiopathic Intracranial Hypertention / 中国医学科学院学报

Idiopathic intracranial hypertension,also known as pseudotumor cerebri,is a syndrome characterized by raised intracranial pressure of unknown cause.These patients present normal neuroimaging and cerebrospinal fluid analysis while increased intracranial pressure and associated symptoms and signs.Delay of treatment can cause severe visual impairment.There are some new understandings of this disease,and we will review the pathogenesis,diagnosis,and treatment of idiopathic intracranial hypertension.

Hipertensión intracraneal idiopática asociada a nitrofurantoína. Caso clínico / Idiopathic intracranial hypertension associated with nitrofurantoin. Case report

Resumen Introducción: La Hipertensión Intracraneal Idiopática (HICI) es un síndrome neurológico caracterizado por un aumento de la presión intracraneal en ausencia de lesión estructural o hidrocefalia. Los síntomas incluyen cefalea, tinnitus pulsátil, oscurecimientos visuales transitorios y pérdida visual. Dentro de los signos destacan diplopía por parálisis del VI par, edema de papila y disminución de la agudeza visual. Los pacientes no tienen compromiso de conciencia ni signos neurológicos focales. La principal complicación es la pérdida visual que puede ser irreversible. La asociación entre HICI y nitrofurantoína (NTF) se reportó en 1974. Caso clínico: Mujer de 42 años, con sobrepeso, que desarrolló una HICI aproximadamente 18 meses posterior al inicio de nitrofurantoína profiláctica. Consultó por cefalea frontal, opresiva que aumentaba con la maniobra de Valsalva, asociada a disminución fluctuante de la agudeza visual y episodios de oscurecimiento. Al examen destacó edema de papila bilateral, sin déficit neurológico focal. La presión del líquido cefalorraquídeo (LCR) fue de 25,5 cm de agua. La resonancia magnética mostró signos de aumento de la presión del LCR, sin lesiones estructurales ni hidrocefalia. El cuadro se recuperó concomitantemente a la suspensión de la NTF y el uso de topiramato. No se constató daño visual permanente. Conclusiones: Se debe sospechar la HICI en mujeres en edad fértil con sobrepeso. Dentro de los gatillantes del síndrome destacan varios fármacos, entre ellos la NTF. El principal objetivo del tratamiento de la HICI es preservar la función visual.

Abstract Introduction: Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure without a space occupying lesion or hydrocephalus. The symptoms are headache, pulsatile tinnitus, transient visual obscurations, and visual loss. Signs are diplopia caused by sixth cranial nerve paresis and papilledema with its associated loss of sensory visual function. The patient maintains an alert and oriented mental state, but has no localizing neurologic findings. The only major morbidity with IIH is visual loss. The association between IIH and nitrofurantoin was reported in 1974. Case: A 42 years old female, overweighed, who developed IIH 18 months after the start of prophylactic nitrofurantoin. She had frontal oppressive headache that increased with the Valsalva maneuver, fluctuant visual loss and transient visual obscurations. She had bilateral papilledema without localizing neurologic findings. The cerebrospinal fluid (CSF) pressure was 25.5 cm H2O. Magnetic resonance imaging showed signs of increased CSF pressure without structural lesions or hydrocephalus. IIH recovered with the withdrawal of nitrofurantoin and the use of topiramate. There was not permanent visual loss. Conclusions: It is recommendable to suspect IIH in obese women in the childbearing years. There are several drugs associated with IIH including nitrofurantoin. The main objective of treatment is to prevent visual loss.

Coexistence of papilledema and pseudopapilledema after remission of idiopathic intracranial hypertension by bariatric surgery / Coexistência de papiledema e pseudopapiledema após remissão de hipertensão intracraniana idiopática por cirurgia bariátrica

ABSTRACT A 37-year-old woman complained of headaches following bilateral visual loss in the past two years. She was obese and had undergone bariatric surgery three months earlier, followed by a considerable weight loss. Neuro-ophthalmic examination revealed a bilateral swollen optic disk. After a computerized analysis of the visual fields and magnetic resonance imaging of the brain and orbits, a diagnosis of idiopathic intracranial hypertension was made. At six months after the bariatric surgery, the patient reported no further headaches and exhibited better findings on computerized analysis of visual fields. However, fundus examination revealed persistent mild papilledema in both eyes. Ocular B-scan ultrasonography showed bilateral optic disk drusen. This report highlights the coexistence of true papilledema and pseudopapilledema due to optic disk drusen, following remission of idiopathic intracranial hypertension after a bariatric surgery.

RESUMO Uma mulher de 37 anos queixou-se de cefaleia após perda visual bilateral nos últimos dois anos. Apresentava história de obesidade e havia sido submetida à cirurgia bariátrica três meses antes, seguida de considerável perda de peso. O exame neuro-oftálmico revelou um disco óptico inchado bilateral. Após uma análise computadorizada dos campos visuais e ressonância magnética do crânio e órbitas, foi feito um diagnóstico de hiper tensão intracraniana idiopática. Após seis meses da cirurgia bariátrica, a paciente não relatou mais cefaleia e foram descobertas melhoras na análise computadorizada dos campos visuais. No entanto, o exame de fundo de olho revelou papiledema leve persistente em ambos os olhos. A ultrassonografia ocular B-scan mostrou drusas do disco óptico bilateralmente. Este relato destaca a coexistência de papiledema verdadeiro e pseudopapiledema devido à drusa de disco óptico após remissão da hipertensão intracraniana idiopática após uma cirurgia bariátrica.

Clinical performance of fixed-pressure Sphera Duo® hydrocephalus shunt / Performance clínica da válvula de pressão fixa Sphera Duo®

Abstract Introduction: Cerebral hydrodynamics complications in shunted patients are due to the malfunction of the system. The objective of this retrospective, single-center, single-arm cohort study is to confirm the safety and performance of Sphera® Duo when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cysts. Methods: Data were generated by reviewing 112 adult patient's charts, who were submitted to a ventriculoperitoneal shunt surgery and followed for one year after surgery. Results: The results show us that 76% of patients had their neurological symptoms improved and that the reoperation rate was 15% in the first year following surgery. Discussion: Sphera Duo® shunt system is an applicable shunt option in routine neurosurgical management of hydrocephalus by several causes. It has presented good results while mitigating effects of overdrainage. Overdrainage is especially important in adults with non-hypertensive hydrocephalus and can cause functional shunt failure, which causes subnormal ICP (particularly in the upright position) and is associated with characteristic neurological symptoms, such as postural headache and nausea. Conclusion: Sphera Duo® shunt system is safe when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cyst.

Resumo Introdução: As complicações da hidrodinâmica cerebral em pacientes com derivação ventriculoperitoneal são frequentemente relacionadas ao malfuncionamento do sistema. O objetivo deste estudo retrospectivo de coorte de centro único é avaliar a segurança e performance clínica do Sistema Sphera® Duo quando utilizado em adultos com hidrocefalia, pseudotumor cerebral ou cistos aracnoides. Métodos: Avaliamos os prontuários de 112 pacientes adultos submetidos a cirurgia de derivação ventriculoperitoneal e acompanhados por 1 ano após a cirurgia. Resultados: O resultado mostra que 76% dos pacientes melhoraram dos sintomas neurológicos e a taxa de reoperação foi de 15% no primeiro ano após a cirurgia. Discussão: O sistema de derivação Sphera Duo® é uma opção de shunt adequada a ser usada no tratamento neurocirúrgico da hidrocefalia por causas diversas. Ele demonstrou bons resultados clínicos enquanto reduziu riscos de hiperdrenagem. A hiperdrenagem é especialmente preocupante e mórbida em pacientes adultos com hidrocefalia não hipertensiva e pode levar a prejuízo clínico e disfunção da válvula, com sintomas de hipotensão craniana, como cefaléia ortostática e náuseas. Conclusão: O sistema de derivação Sphera Duo® é seguro para tratamento da hidrocefalia, pseudotumor cerebri ou cistos aracnóides em adultos.

Bariatric surgery as a treatment for idiopathic intracranial hypertension in a male adolescent: case report / Cirurgia bariátrica como tratamento de pseudotumor cerebral primário em adolescente do sexo masculino: relato de caso

ABSTRACT Objective: To describe a case of a male adolescent with symptomatic idiopathic intracranial hypertension (IIH) associated with obesity treated with bariatric surgery. Case description: A 16-year-and-6-month-old severely obese boy [weight: 133.6 kg; height: 1.74 m (Z score: +0.14); BMI: 44.1 kg/m2 (Z score: +4.4)], Tanner pubertal stage 5, presented biparietal, high-intensity, and pulsatile headaches, about five times per week, associated with nocturnal awakenings, and partial improvement with common analgesics, for three months. Ophthalmologic evaluation evidenced bilateral papilledema. Cranial computed tomography revealed no mass or anatomic abnormalities. Lumbar puncture showed increased intracranial pressure of 40 cmH2O (reference value: <28 cmH2O) with a normal content. After being diagnosed with IIH, the patient was started on acetazolamide. However, after three months, he was still symptomatic. He was diagnosed with obesity due to excess energy intake and, as he had failed to lose weight after a conventional clinical treatment, bariatric surgery was indicated. The patient (at 16 years and nine months) underwent an uncomplicated laparoscopic sleeve gastrectomy. Ophthalmologic evaluation, performed five months after surgery, revealed normal visual acuity in both eyes and improvement of bilateral papilledema. Follow-up at 18 months showed a 67.5% loss of excess weight (weight: 94.5 kg and BMI: 31.2 kg/m2) and complete resolution of IIH symptoms. Comments: IIH is characterized by increased intracranial pressure with no evidence of deformity or obstruction of the ventricular system on neuroimaging. It has been associated with obesity. Bariatric surgery may be a valid alternative approach for morbidly obese adolescent patients with refractory symptoms.

RESUMO Objetivo: Descrever um caso de cirurgia bariátrica como tratamento de pseudotumor cerebral primário (PTCP) em adolescente do sexo masculino com obesidade. Descrição do caso: Adolescente, sexo masculino, 16 anos e 6 meses, com obesidade exógena [peso:133,6 kg; estatura:1,74 m (escore z: +0,14); IMC: 44,1 kg/m2 (escore z: +4,4)], estadiamento puberal de Tanner 5, apresentando cefaleia bi-parietal, pulsátil e de alta-intensidade, cerca de cinco vezes por semana, associada a despertares noturnos, e com melhora parcial com analgésicos comuns, há três meses. A avaliação oftalmológica evidenciou papiledema bilateral e a tomografia computadorizada de crânio não revelou massas ou alterações anatômicas. A punção lombar mostrou pressão intracraniana elevada de 40 cmH2O (Referência: <28 cmH2O) com conteúdo normal. Feito o diagnóstico, o paciente foi iniciou uso de acetazolamida. No entanto, após 3 meses, o paciente mantinha-se sintomático. Ele foi diagnosticado com obesidade devido ao consumo calórico excessivo e, como não havia obtido sucesso na perda de peso com tratamento clínico convencional, a cirurgia bariátrica foi indicada. Aos 16 anos e 9 meses, o paciente foi submetido a gastrectomia vertical laparoscópica sem complicações. A avaliação oftalmológica, cinco meses após a cirurgia, revelou melhora do papiledema bilateral com acuidade visual normal em ambos os olhos. Apresentou perda de excesso de peso de 67,5% (peso: 94,5 kg e IMC:31,2 kg/m2) e resolução completa dos sintomas de PPTC 18 meses após a cirurgia. Comentários: O PTCP é caracterizado pelo aumento da pressão intracraniana, sem evidência de deformidade ou obstrução do sistema ventricular na neuroimagem. Está associado à obesidade. A cirurgia bariátrica pode ser uma alternativa terapêutica válida para pacientes adolescentes obesos graves com sintomas refratários.

Bilateral Transverse Sinus Angioplasty for the Treatment of Idiopathic Intracranial Hypertension ­ Case Report and Literature Review

Idiopathic intracranial hypertension (IIH) is a disease characterized by an increase in intracranial pressure, without presence of parenchymal lesions or hydrocephalus that justify it. Over 90% of cases there is association with stenosis of the dural venous sinuses. It is characterized by headache, tinidus, nausea, vomiting and visual disturbances. Initial treatment is clinical and when it fails there is indication of invasive procedures, among them shunts and fenestration of the optic nerve sheath. Angioplasty of dural venous sinuses, when indicated, has shown an alternative with better results and less complications. We report a case of a female patient, with 27 years old, diagnosed with IIH and bilateral transverse sinus stenosis, which was treated by bilateral stenting and total resolution of symptoms. Besides describing the case we review the literature about the subject.

A case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome / Caso de paralisia bilateral de quarto nervo associada com síndrome do pseudotumor cerebral

ABSTRACT Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.

RESUMO A sindrome do pseudotumor cerebral é uma síndrome enigmática caracterizada por aumento da pressão intracraniana sem causa definida. Na síndrome do pseudotumor cerebral, as paralisias de nervo oculomotor além da paralisia unilateral ou bilateral do sexto nervo craniano são raramente vistas. Nós reportamos o caso de uma paciente feminina de 25 anos com paralisia bilateral de quarto nervo craniano como resultado da síndrome do pseudotumor cerebral. Após tratamento médico para síndrome do pseudotumor cerebral, os sinais e sintomas oculares e sistêmicos da paralisia nervosa foram resolvidos. Em conclusão, a paralisia de quarto nervo craniano pode estar associada com síndrome de pseudotumor cerebral, portanto médicos devem considerar paralisias raras de nervo oculomotor, mesmo bilateralmente, em pacientes com síndrome do pseudotumor cerebral.

Ultrasonographic Evaluation of the Optic Nerve Sheath in the Diagnosis of Idiopathic Intracranial Hypertension

Intracranial hypertension (ICH) is a life-threatening condition that can be observed in several diseases. Its clinical presentation is variable, with headache, nausea, vomiting, visual disturbances, papilledema, and alterations in the level of consciousness. The gold standard for the diagnosis of ICHis still the intracranial implantation of invasive devices. Non-invasive techniques, such as ultrasonography of the optic nerve sheath (USONS), have emerged in recent years with promising clinical results. The authors report the case of a patient with progressive headache associated with visual impairment and papilledema, and the eventual diagnosis of idiopathic intracranial hypertension using USONS.

Pseudotumor cerebri in childhood and adolescence: data from a specialized service / Pseudotumor cerebral na infância e adolescência: dados de um serviço especializado

ABSTRACT Objective: To report cases of children and adolescents diagnosed with pseudotumor cerebri associated or not with rheumatic disease. Methods: This was a retrospective study based on medical reports of 29 patients, up to 18 years of age and diagnosed with pseudotumor cerebri, followed up in the Pediatric Rheumatology and Neurology outpatient clinics of a tertiary hospital, until December 2016. Results: Among the 29 patients diagnosed with pseudotumor cerebri, 51.7% were girls and the mean age at the disease onset was 12.3 years. In 18 patients (62%) where an etiology was found, four were associated with a rheumatic disease. The most common symptom was headache (69%) and acetazolamide was the most used medication (69%). Two patients developed blindness and 10 are still being followed up. Conclusion: Although rare, pseudotumor cerebri should be considered in children with headaches, especially in patients with rheumatic disease.

RESUMO Relatar os casos de crianças e adolescentes com diagnóstico de pseudotumor cerebral com ou sem doença reumática. Métodos: Estudo retrospectivo através de revisão de prontuários, 29 pacientes com idade até 18 anos e diagnóstico de pseudotumor, atendidos nos ambulatórios de Reumatologia Pediátrica e Neurologia de um hospital terciário, registrados até dezembro de 2016. Resultados: Dentre os 29 pacientes com diagnóstico de pseudotumor cerebral, 51,7% eram meninas. A média de idade de aparecimento dos sintomas foi de 12,3 anos. Em relação à etiologia do pseudotumor cerebral, em 18 pacientes (62%) foi possível identificar uma causa, sendo o diagnóstico de doença reumática associada em quatro desses casos. Cefaléia foi o sintoma mais frequente (69%), e a medicação mais utilizada foi a acetazolamida (69%). Dois pacientes evoluíram para cegueira e 10 ainda se encontram em seguimento ambulatorial. Conclusão: Concluímos que, apesar de raro, o diagnóstico de pseudotumor cerebral deve ser considerado em crianças com cefaleia, principalmente nos pacientes com doença reumática.

Pseudotumor cerebral asociado a hipovitaminosis A, B6 y D: A propósito de dos casos / Pseudotumor cerebri associated with hypovitaminosis A, B6 and D: About two cases

La hipertensión endocraneana idiopàtica se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mm^O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografia axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mm^O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mm^O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

Síndrome de seudotumor cerebral en una mujer embarazada y con lupus eritematoso sistémico / Pseudotumor cerebri syndrome in a pregnant woman with systemic lupus erythematous

Resumen El lupus eritematoso sistémico es una enfermedad autoinmunitaria crónica que afecta múltiples sistemas orgánicos, incluido el sistema nervioso central. El seudotumor cerebral es un síndrome clínico que se caracteriza por aumento de la presión intracraneal en ausencia de lesiones que ocupen espacio u otra causa detectable, que afecta con frecuencia a mujeres jóvenes y obesas. Se presenta el caso de una mujer con diagnóstico de seudotumor cerebral y lupus eritematoso sistémico diagnosticado de novo durante el embarazo.

Abstract Systemic lupus erythematous is a chronic multi-systemic autoimmune disease that affects multiple organ systems, including the central nervous system. Pseudotumor cerebri is a disorder associated with increased intracranial pressure in the absence of a space-occupying lesion or other identifiable cause that affects young and obese women. We present the case of a pregnant woman with both pseudotumor cerebri and a new diagnosis of active systemic lupus erythematous.

Pseudotumor Cerebri Presenting Unilateral Papilledema Associated with Iron-deficiency Anemia

Pseudotumor cerebri is a relatively common pathology that is characterized by intracranial hypertension in the absence of mass lesions. It commonly affects young and obese women, and its presentation with visual loss and bilateral papilledema is well-described in the literature. We present a case of a 44-year-old, non-obese, female patient presenting with unilateral papilledema and iron-deficiency anemia. This case emphasizes this unusual presentation and the rare association with iron deficiency.

O pseudotumor cerebral é uma patologia relativamente comum, caracterizada pela hipertensão intracraniana na ausência de lesões causando efeito de massa. Frequentemente afeta mulheres jovens e obesas, e sua apresentação, com perda do campo visual e papiledema bilateral, é bem descrita na literatura. Nós apresentamos um caso de uma paciente de 44 anos, não obesa, com papiledema unilateral e anemia ferropriva. Esse caso destaca essa apresentação incomum e a rara associação com a deficiência de ferro.

Bariatric surgery as a treatment for pseudotumor cerebri: case study and narrative review of the literature / Cirurgia bariátrica como tratamento para pseudotumor cerebri: estudo de caso e revisão narrativa de literatura

ABSTRACT CONTEXT: Pseudotumor cerebri occurs when there is an increase in intracranial pressure without an underlying cause, usually leading to loss of vision. It is most commonly observed in obese women of child-bearing age. CASE REPORT: A 46-year-old woman presented at our service with idiopathic intracranial hypertension that had been diagnosed two years earlier, which had led to chronic refractory headache and an estimated 30% loss of visual acuity, associated with bilateral papilledema. She presented partial improvement of the headache with acetazolamide, but the visual loss persisted. Her intracranial pressure was 34 cmH2O. She presented a body mass index of 39.5 kg/m2, also associated with high blood pressure. Computed tomography of the cranium with endovenous contrast did not show any abnormalities. She underwent Roux-en-Y gastric bypass with uneventful postoperative evolution. One month following surgery, she presented a 24% excess weight loss. An ophthalmological examination revealed absence of visual loss and remission of the papilledema. There were no new episodes of headache following the surgery. There was also complete resolution of high blood pressure. The intracranial pressure decreased to 24 cmH2O, six months after the surgery. CONCLUSION: Although the condition is usually associated with obesity, there are few reports of bariatric surgery among individuals with pseudotumor cerebri. In cases studied previously, there was high prevalence of resolution or improvement of the disease following bariatric surgery. There is no consensus regarding which technique is preferable. Thus, further research is necessary in order to establish a specific algorithm.

RESUMO CONTEXTO: O pseudotumor cerebri ocorre quando há aumento na pressão intracraniana sem causa subjacente, comumente levando a perda visual. É mais comum em mulheres obesas em idade fértil. RELATO DE CASO: Mulher de 46 anos, foi admitida com hipertensão intracraniana idiopática diagnosticada há dois anos, que levou a cefaleia refratária crônica e perda estimada de 30% da acuidade visual, associada a papiledema bilateral. Apresentou melhora parcial da cefaleia com acetazolamida, mas a perda visual persistiu. A pressão intracraniana era de 34 cmH2O. Apresentava índice de massa corpórea de 39,5 kg/m2, associado a hipertensão arterial. Tomografia computadorizada com contraste endovenoso de crânio não apresentou anormalidades. Foi submetida ao bypass gástrico em Y de Roux, com evolução pósoperatória sem intercorrências. Um mês após a cirurgia, apresentou perda de peso em excesso de 24%. Um exame oftalmológico demonstrou ausência de perda visual e remissão do papiledema; não houve novos episódios de cefaleia após a cirurgia. Houve também resolução completa da hipertensão arterial. A pressão intracraniana caiu para 24 cmH2O após seis meses da cirurgia. CONCLUSÃO: Embora a condição seja usualmente associada à obesidade, há escassos relatos de cirurgia bariátrica em indivíduos com pseudotumor cerebri. Nos casos previamente estudados, há alta prevalência de resolução ou de melhora da doença após a cirurgia bariátrica. Não há consenso sobre qual é a técnica cirúrgica de escolha. Portanto, mais estudos são necessários para estabelecer um algoritmo específico.

Weight reduction for a better visual outcome in idiopathic intracranial hypertension / Redução de peso para a melhora do resultado visual na hipertensão intracraniana idiopática

ABSTRACT Purpose: To evaluate the correlation between weight reduction and visual outcome in overweight patients with idiopathic intracranial hypertension. Methods: Thirty-nine newly diagnosed, overweight (body mass index >25 kg/m2) patients with idiopathic intracranial hypertension were studied retrospectively. All patients underwent medical treatment with acetazolamide, and a weight reduction program was also offered. Patients were grouped according to their compliance with this weight reduction program into the diet-success (Group 1) and diet-failure groups (Group 2). Body mass index, papilledema, visual acuity, and perimetric mean deviation were compared at the end of the 6-month study period. Results: Groups 1 and 2 did not differ regarding the baseline mean body mass index (32.63 ± 5.61, 32.35 ± 5.06 kg/m2), visual acuity (0.080 ± 0.13, 0.130 ± 0.24 logMAR), perimetric mean deviation (-9.978 ± 0.68, -12.86 ± 8.91), or papilledema grade (2.94 ± 0.22, 2.90 ± 0.30), respectively (p>0.05). During the 6 months' follow-up, Group 1 patients, who complied with both medical and diet therapy, improved significantly in all parameters, including body mass index (p<0.001), visual acuity (p=0.001), perimetric mean deviation (p=0.016), and papilledema grade (p<0.001). Conversely, Group 2 patients, who only underwent medical therapy, improved only in papilledema grade (p<0.001). However, coincident development of optic disc pallor was observed in three patients. Further, they also had significant loss in visual acuity (p=0.047) during the study period. Conclusion: Weight reduction combined with medical treatment is associated with significantly better improvement in visual acuity, visual field, and papilledema in idiopathic intracranial hypertension patients. Compliance with an efficient diet program should be encouraged in overweight patients with idiopathic intracranial hypertension.

RESUMO Objetivo: Avaliar a correlação entre a redução de peso e o resultado visual em pacientes com hipertensão intracraniana idiopática e sobrepeso. Métodos: Trinta e nove pacientes, recém-diagnosticados com hipertensão intracraniana idiopática e sobrepeso (índice de massa corporal >25 kg/m2), foram estudados retrospectivamente. Todos os pacientes foram submetidos a tratamento médico com acetazolamida e receberam um programa para redução de peso. Os pacientes foram classificados de acordo com o cumprimento do programa de redução de peso em: sucesso da dieta (Grupo 1) e falha da dieta (Grupo 2). Os índices de massa corporal, a papiledema, a acuidade visual e desvios médios perimétricos foram comparados no final de 6 meses. Resultados: Os grupos não apresentaram diferenças em relação às médias da linha de base de índice de massa corporal (32,63 ± 5,61/32,35 ± 5,06 kg/m2), acuidade visual (0,080 ± 0,13/0,130 ± 0,24 logMAR), desvios médios perimétricos (-9,978 ± 0,68/-12,86 ± 8,91) e níveis de papiledema (2,94 ± 0,22/2,90 ± 0,30) (p>0,05). Durante o período de acompanhamento de 6 meses, os pacientes do grupo 1, que obedeceram as terapias médicas e dietéticas, melhoraram significativamente em todos os parâmetros, incluindo o índice de massa corporal (p<0,001), a acuidade visual (p=0,001), o desvio médio perimétrico (p=0,016) e o nível de papiledema (p<0,001). Por outro lado, os pacientes do grupo 2, que receberam apenas terapia médica, apresentaram melhoras somente no nível de papiledema (p<0,001). No entanto, observou-se o desenvolvimento coincidente de palidez de disco óptico em três pacientes. Além disso, esses pacientes também apresentaram perda significativa de acuidade visual (p=0,047) durante o período de estudo. Conclusão: A redução de peso combinada ao tratamento médico está associada à melhora significativa das acuidades visuais, dos campos visuais e de papiledema em pacientes com hipertensão intracraniana idiopática. O cumprimento de programas de dietas eficientes deve ser encorajado em pacientes obesos com hipertensão intracraniana idiopática.

Endovascular Treatment of Idiopathic Intracranial Hypertension with Stenting of the Transverse Sinus Stenosis / 신경중재치료의학

For many years, the pathophysiology of idiopathic intracranial hypertension (IIH) was interpreted as “secondary intracranial hypertension,” and IIH was considered to be caused by brain edema due to obstructive sleep apnea. Another theory proposed cerebrospinal fluid (CSF) absorption impairment due to excessive medication with vitamin A derivatives. Other reports pointed out the importance of obesity, which may cause an impairment of intracranial venous drainage due to elevated right atrial pressure. Patients with medically refractory IIH have traditionally undergone a CSF diversion. Venous outlet impairment on IIH has recently been reported as a causative or contributory cause, and thus focused venoplasty of the stenotic sinus with a stent has emerged as a new treatment strategy. We report the cases of two patients who presented with headache and papilledema with IIH. They successfully underwent stent placement at the stenosis of the transverse sinus and experienced complete resolution of symptoms.

Esclerose múltipla pseudotumoral: relato de caso e revisão de literatura / Pseudotumoral form of multiple sclerosis: case report and review of literature

A forma pseudotumoral de esclerose múltipla é extremamente rara e possui uma grande variedade de diagnósticos diferenciais, o que a torna um desafio na prática neurológica. Neste artigo, apresentamos um caso desta manifestação incomum de esclerose múltipla, que foi avaliado em uma clínica especializada no Rio de Janeiro. O caso é de um individuo do sexo masculino de 21 anos que apresentou apenas crises convulsivas. Apresentou curso benigno da doença.

Pseudotumoral forms of multiple sclerosis are very and have a great variety of differential diagnosis, which poses it as a great challenge in neurological practice. In this paper, we report one case of this uncommon manifestation of multiple sclerosis, which was assessed in a specialized clinic in Rio de Janeiro. The case is a 21 year old male patient that had only seizures. He presented a benign course of the disease.