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.

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.

Factors affecting visual loss and visual recovery in patients with pseudotumor cerebri syndrome / Fatores que influenciam na perda e na recuperação visual de pacientes com a síndrome do pseudotumor cerebral

ABSTRACT Purpose: To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome. Methods: We reviewed 50 PTC patients (43 females, seven males) who underwent neuro-ophthalmic examination at the time of diagnosis and after treatment. Demographic data, body mass index (BMI), time from symptom onset to diagnosis (TD), maximum intracranial pressure (MIP), occurrence of cerebral venous thrombosis (CVT), and treatment modalities were reviewed. VL was graded as mild, moderate, or severe on the basis of visual acuity and fields. Predictive factors for VL and improvement were assessed by regression analysis. Results: The mean ± SD age, BMI, and MIP were 35.2 ± 12.7 years, 32.0 ± 7.5 kg/cm2, and 41.9 ± 14.5 cmH2O, respectively. Visual symptoms and CVT were present in 46 and eight patients, respectively. TD (in months) was <1 in 21, 1-6 in 15, and >6 in 14 patients. Patients received medical treatment with (n=20) or without (n=30) surgery. At presentation, VL was mild in 16, moderate in 12, and severe in 22 patients. Twenty-eight patients improved and five worsened. MIP, TD, and hypertension showed a significant correlation with severe VL. The best predictive factor for severe VL was TD >6 months (p=0.04; odds ratio, 5.18). TD between 1 and 6 months was the only factor significantly associated with visual improvement (p=0.042). Conclusions: VL is common in PTC, and when severe, it is associated with a delay in diagnosis. It is frequently permanent; however, improvement may occur, particularly when diagnosed within 6 months of symptom onset. .

RESUMO Objetivo: Investigar a frequência de perda visual (PV) e os possíveis fatores preditivos para perda e para melhora visual em pacientes com a síndrome do pseudotumor cerebral (SPC). Métodos: Foram revisados 50 pacientes com SPC submetidos a exame neuroftalmológico no momento do diagnóstico e após o tratamento. Dados demográficos, índice de massa corpórea (IMC), tempo decorrido entre o início dos sintomas e o diagnóstico (TD), pressão intracraniana máxima (PIM), ocorrência de trombose venosa cerebral (TVC), e as modalidades de tratamento foram revisadas. PV foi graduada em discreta, moderada e grave, baseada na acuidade e no campo visual. Fatores preditivos para perda e melhora visual foram avaliados por análise de regressão linear. Resultados: Quarenta e três pacientes eram do sexo feminino. A média de idade, o IMC e a PIM (± desvio padrão) foram: 35,2 ± 12,7 anos, 32,0 ± 7,5 kg/cm2 e 41,9 ± 14,5 cmH2O, respectivamente. Sintomas visuais estavam presentes em 46 e TVC em 8 pacientes. TD (em meses) foi <1 em 21, 1-6 em 15 e >6 em 14 pacientes. Pacientes receberam tratamento clinico apenas (n=30) ou associado a tratamento cirúrgico (n=20). Na apresentação a PV era discreta em 16, moderada em 12 e grave em 22 pacientes. Vinte e oito pacientes melhoraram e 5 pioraram. PIM, TD e hipertensão arterial correlacionaram significativamente com PV grave. O melhor fator preditivo para PV grave foi o TD>6 meses (p=0,04; razão de chances 5,18). TD entre 1 e 6 meses foi o único fator significativamente associado com melhora visual após tratamento (p=0,042). Conclusões: Perda visual é comum na SPC e quando grave se mostra relacionado a atraso no diagnóstico. É usualmente permanente mas pode haver melhora visual especialmente quando a doença é diagnosticada nos primeiros 6 após o início dos sintomas. .

A Case of Pediatric Idiopathic Intracranial Hypertension Presenting with Divergence Insufficiency

An 11-year-old female presenting diplopia only at distance was found to have comitant esotropia of 20 prism diopters (PD) at distance and normal alignment at nearer proximity. Other ocular movement, including abduction, was normal and a thorough neurologic examination was also normal. The deviation angle of esotropia was increased to 35 PD in 6 months, and a brain magnetic resonance imaging with venogram at that time demonstrated no intracranial lesion. A lumbar puncture showed increased opening pressure but the cerebrospinal fluid composition was normal. The patient was diagnosed as having idiopathic intracranial hypertension and treated with oral acetazolamide. Three months after treatment, the deviation angle decreased to 10 PD. This is a case report of divergence insufficiency in pediatric idiopathic intracranial hypertension, with an increasing deviation angle of esotropia. Although sixth cranial nerve palsy is a common neurologic manifestation in intracranial hypertension, clinicians should be aware of the possibility of divergence insufficiency. Also, ophthalmoparesis may not be apparent and typical at first presentation, as seen in this case, and therefore ophthalmologists should be aware of this fact, while conducting careful and proper evaluation, follow-up, and intervention.

Clinical profile of cerebral venous sinus thrombosis and the role of imaging in its diagnosis in patients with presumed idiopathic intracranial hypertension.

Retrospective descriptive study reporting the rate of occurrence of cerebral venous sinus thrombosis (CVST), highlighting the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in patients with presumed idiopathic intracranial hypertension (IIH). Study was conducted in the department of neuro-ophthalmology at a tertiary eye care center in South India. Data from 331 patients diagnosed with IIH from June 2005 to September 2007 was included. Inclusion criteria were: Elevated opening cerebrospinal fluid (CSF) pressure of more than 200 mm of water on lumbar puncture, normal CSF biochemistry and microbiology, and normal neuroimaging as depicted by computed tomography(CT) scan. Exclusion criteria were: Space-occupying lesions, hydrocephalus, meningitis, intracranial pressure within normal range, abnormal CSF biochemistry and microbiology. The remaining patients were evaluated with MRI and MRV. CVST was present in 11.4% of patients who were presumed to have IIH (35/308). MRI alone identified 24 cases (68%) of CVST, while MRI used in combination with MRV revealed an additional 11 cases (32%). Risk factors associated with CVST were identified in nine out of 35 patients (26%). CVST may be misdiagnosed as IIH if prompt neuroimaging by MRI and MRV is not undertaken. Risk factors of CVST may not be apparent in all the cases and these patients are liable to be missed if CT scan alone is used for neuroimaging, hence MRI, combined with MRV should be undertaken to rule out CVST.

Hypophosphatasia associated with pseudotumor cerebri and respiratory insufficiency.

We report a 3-month-old male with infantile hypophosphatasia who later developed Pseudotumor cerebri. At the age of 3 months, he was referred to our hospital because of pneumonia and respiratory insufficiency. He had short extremities, and radiographs of the bones were consistent with lack of metaphyseal mineralization and bowed lower extremities. Vomiting and bulging fontanelle developed 3 months after admission, and CSF opening pressure was notably high at 430 mm/H2O. Hypophosphatasia is a very rare cause of pseudotumor cerebri. This report is the first case where PTC is associated with hypophosphatasia and responded well to corticosteroid therapy.

Pseudotumor cerebri with transient oculomotor palsy.

Pseudotumor cerebri is a clinical syndrome characterized by raised intracranial pressure with normal ventricular size, anatomy and position. Headache, vomiting and diplopia are the most common symptoms. Signs include those of raised intracranial pressure including papilledema and absence of focal neurological signs. A secondary cause is identifiable in 50% of children; the most common predisposing conditions are otitis media, viral infection and medications. Management is mainly directed towards identifying and treating the cause and measures to reduce the raised intracranial pressure. Though it is mostly a self limited condition, optic atrophy and blindness can occur. Oculomotor nerve palsy is very rarely associated with pseudotumor cerebri. We report a unique case of pseudotumor cerebri who had left Oculomotor palsy with sparing of the pupillary fibres, which resolved following treatment with oral acetazolamide.

Traumatismo craneoencefálico grave: complicaciones y manejo / Severe head injury: complications and treatment

Se revisaron 100 expedientes de niños con traumatismo craneoencefálico grave. Se analizó el sitio donde se presentó el accidente, la causa del accidente y las complicaciones observadas. La mayoría de los casos (77 por ciento) recibió tratamiento médico, el resto de ellos fueron intervenidos quirúrgicamente. Se comentan los hallazgos tomográficos. Treinta y seis pacientes quedaron con secuelas y 19 fallecieron

Vigilancia de la presión intracraneana en niños gravemente enfermos con riesgo de hipertensión intracraneana / Invasive intracranial pressure monitoring in critically ill children

Introducción. La experiencia reportada de la vigilancia invasiva de la presión intracraneana (PIC) en niños es muy escasa. Material y métodos. Se efectuó un estudio de monitorización de la PIC en la Unidadad de Terapia Intensiva Pediátrica del Centro Hospitalario 20 de Noviembre, del ISSSTE México, D.F., entre julio de 1988 a octubre de 1989. Las indicaciones para monitorizar la PIC fueron una valoración de Glasgow ó 8 sospecha clínica de herniación inminente o posoperados de resección tumoral con gran edema cerebral. Resultados. fueron 20 pacientes con edades entre uno a 12 años (x=7.3 años) con trauma craneoencefálico severo (n=9), neuroinfección (n=6), síndrome de Reye (n=2), postoperados de tumoración en sistema nervioso central (n=2) y herida en cráneo por proyectil de arma de fuego (n=1). El dispositivo utilizado fue cánula subaracnoidea (n=10), tornillo subaracnoideo modificado (n=6) y cánula intraventricular (n=4). El tiempo de monitorización varió de 18 a 200 horas (x=72.3 horas). La única complicación fue la infección localizada al sitio de la colocación del dispositivo en dos pacientes sin influir en la mortalidad. la infección se asoció con un tiempo de monitoreo mayor de 180 horas (X²=15,P<0.005). La mortalidad global fue de 65 por ciento (n=12); hubo asociación con el retraso del inicio de monitorización de mas de 12 horas (X²=8.2,P<0.005), una PIC mayor de 10 mmHg durante las primeras 24 horas de monitoreo (X²=7.9 P<0.05) y con una pobre respuesta a la hipocapnia (PaCO2=24ñ1 mmHg con PIC de 16ñ2.6 mmHg)(t=2.85,P<0.01).La presión de perfusión cerebral (PPC) tendió a ser más baja en el grupo de fallecimientos, sobre todo en las primeras 24 horas sin llegar a tener asociación significativa. Conclusiones. El estudio parece indicar que la vigilancia invasiva de la PIC es un procedimiento útil y seguro en niños con deterioro neurológico grave asociado a hipertensión intracraneana con un tiempo menor de 180 horas en el mismo sitio

Facial nerve involvement in pseudotumor cerebri.

A woman with history of bifrontal headache, vomiting and loss of vision was diagnosed as a case of pseudotumor cerebri based on clinical and MRI findings. Bilateral abducens and facial nerve palsies were detected. Pseudotumor cerebri in this patient was not associated with any other illness or related to drug therapy. Treatment was given to lower the raised intracranial pressure to which the patient responded.

Complicaciones y secuelas de la hipertensión endocraneana benigna (seudotumor cerebral) / Benign endocraneal hypertension: complications and sequelas (cerebral pseudotumor)

A pesar de que la hipertensión endocraneana benigna (HEB) fue descrita desde 1893, aún en los momentos actuales permanecen sin aclarar las principales incógnitas sobre su etiología y fisiopatología. Una muestra de ello son las numerosas denominaciones de la enfermedad señaladas por los distintos autores, de acuerdo con su interpretación de la fisiopatología y de la causa del síndrome. Una denominación más utilizada es la de hipertensión endocraneana benigna al presuponerse la ausencia de complicaciones y secuelas en esta entidad. A pesar de que es el término que más nos complace, en este trabajo revisamos las principales complicaciones y secuelas de la HEB, lo que motiva dudas sobre la conveniencia de este término. La complicación más temible de la HEB es la visual, cuya frecuencia de presentación es variable y depende de distintas condiciones.

Cranial nerve palsies in benign intracranial hypertension.

Benign intracranial hypertension may occasionally be associated with various cranial nerve palsies. A case with multiple cranial nerve involvement is presented.

Papiledema unilateral na síndrome do pseudotumor cerebral / Unilateral papilledema in pseudotumor cerebri

Säo apresentadas as fotografias de fundo de olho de 6 mulheres com papiledema inilateral e a síndrome do pseudotumor cerebral. Achados associados foram: obesidade em 5 e a síndrome de sela vazia parcial em 2. Säo discutidas as possíveis explicaçöes para o desenvolvimento de papiledema unilateral nesses pacientes e que favorecem um mecanismo presente na porçäo distal dos nervos ópticos