Interthalamic Adhesion: Can it be used to Diagnose Idiopathic Intracranial Hypertension?

OBJECTIVE: This retrospective study aimed at determining the dimension of the interthalamic adhesion (ITA) in patients with the idiopathic intracranial hypertension (IIH) for assisting in preoperative radiologic diagnosis. METHODS: The study universe consisted of magnetic resonance images of 20 patients with IIH (age: 22.70 ± 4.04 years, sex: 14 females and 6 males) and 20 normal subjects (age 22.30± 2.94 years, sex: 14 females and 6 males). To determine the morphology of ITA, its height (vertical diameter) and width (horizontal diameter) were measured on the coronal and axial planes, respectively. RESULTS: The height and width of ITA in IIH were measured as 2.58 ± 0.71 mm (range: 1.40-4.20 mm) and 2.73 ± 0.77 mm (range: 1.70-4.40 mm), respectively. Its height and width in controls were measured as 4.99 ± 1.04 mm (range: 2.70-6.30 mm) and 4.92 ± 1.11 mm (range: 2.60-6.50 mm), respectively. ITA height and width in IIH was significantly smaller compared with controls (P < 0.001). For an arbitrary cutoff of 3.85 mm, the sensitivity of the height of ITA was 85% with 95% specificity. For an arbitrary cutoff of 4.45 mm, the sensitivity of the width of ITA was 75% with 100% specificity. CONCLUSIONS: The height and width of ITA are approximately 50% smaller in IIH than controls; therefore alterations in the dimension of ITA may be a valuable radiologic sign for the diagnosis of IIH.

Accuracy and reliability of magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension.

PURPOSE: To determine the diagnostic utility of brain magnetic resonance imaging (MRI) findings in patients with idiopathic intracranial hypertension (IIH) and to investigate the significance of evaluating radiological findings together with neurological and ophthalmological data in the diagnosis of IIH. MATERIALS AND METHODS: All consecutive patients diagnosed with IIH in our tertiary neuro-ophthalmology center between January 1, 2018 and March 15, 2020, were included in the study. The clinical, radiological, and ophthalmological findings of IIH patients were compared with the control group with similar demographic characteristics. RESULTS: A total of 98 patients, 49 cases and 49 controls, were included in the study. Lateral ventricular index had the highest area under the curve (AUC) value (0.945) for prediction of disease group followed by sella height category (AUC = 0.915) and optic nerve tortuosity (AUC = 0.855) According to the multivariate model we developed, caudate index (OR = 0.572, 95% CI 0.329-0.996), lateral ventricle index (OR = 3.969, 95% CI 1.851-8.509) and bilateral optic nerve tortuosity (OR = 22,784, 95% CI 2.432-213.450) were significant predictors for disease group. CONCLUSION: Tortuosity in the optic nerve, lateral ventricular index and caudate index can be used as MRI parameters supporting the diagnosis of IIH in clinically suspicious cases. A holistic approach to the clinical and radiological findings of the cases in the diagnosis of IIH can prevent overdiagnosis and enable early correct diagnosis.

Idiopathic intracranial hypertension presenting as iron deficiency anemia: a case report.

BACKGROUND: The presentation of idiopathic intracranial hypertension (IIH) in association with iron deficiency anemia (IDA) is rare. CASE PRESENTATION: This case report depicts the unusual case of a 31-year-old woman of mixed Jamaican and English heritage with IIH who presented initially as IDA in the context of menorrhagia. Subsequent ophthalmic review, lumbar puncture, cerebrospinal fluid analysis and neuroimaging studies revealed severe bilateral optic disc swelling and raised intracranial pressure in keeping with IIH. Prompt treatment of IDA with blood transfusion and orally administered iron supplements, in addition to medical treatment for IIH, contributed to significant improvement of symptoms and prevented long-term visual deficits. CONCLUSION: The possibility of IDA, albeit rare, should always be considered and investigated appropriately in all patients with IIH, as the treatment of the anemia alone may be sight-saving.

Short-term Optic Disc Cupping Reversal in a Patient With Mild Juvenile Open-angle Glaucoma Due to Early Idiopathic Intracranial Hypertension.

PURPOSE: The purpose of this study was to report a case of optic disc cupping reversal in an adult without significant intraocular pressure-lowering treatment. PATIENT: A 20-year-old female with a history of mild juvenile open-angle glaucoma who developed subjective blurred vision and a decrease in cupping of her optic discs. RESULTS: Dilated examination demonstrated decreased cup-to-disc ratios in both eyes with a slight blurring of the disc margin in the right eye. The appearance of both optic discs returned to baseline after weight loss therapy. CONCLUSIONS: An unexplained reduction of optic nerve cup-to-disc ratio should prompt a workup for other etiologies, such as increased intracranial pressure. Baseline photographs not subjected to computerized scan obsolescence are extremely useful in monitoring the long-term appearance of asymmetric optic discs as an adjunct to the clinical examination.

Hipertensión endocraneana idiopática: respuesta al tratamiento Topiramato vs Acetazolamida / Endocranial idiopathic hypertension: response to treatment Topiramate vs. Acetazolamide

INTRODUCCIÓN: El término pseudotumorcerebri se reserva para denominar aquellas hipertensiones endocraneanas (HE) que clínicamente asemejan la existencia de un tumor cerebral, debido a la alteración de la circulación del líquido cefalorraquídeo (LCR). Para su diagnóstico se describen los criterios de Dandy-Smith. OBJETIVOS: Objetivo Primario: Determinar el beneficio del uso de Azetazolamida (ACZ) o Topiramato (TPM) en el tratamiento de la hipertensión endocraneana idiopática. Objetivos Secundarios: Uso de Presión de apertura como parámetro indicador para uso de ACZ o TPM. MATERIALES Y MÉTODOS: Tipo de estudio: Descriptivo Retrospectivo Observacional. RESULTADOS: La media de seguimiento fue de 11 meses, con un rango entre 6-12 meses Se estudiaron 5 pacientes con diagnóstico de Hipertensión Endocraneana Idiopática. Del total de los pacientes 5 (100%) tenían F.O patológico y como síntoma cardinal cefalea, 2 (40%) además vómitos. 3 (60%) fue tratado con Topiramato (TPM) mientras que 2 (40%) recibió acetazolamida (ACZ), ambos sin complicaciones (p= 0,07) Del total de los pacientes 3(60%) presento presión de apertura menor de 40 mmHg mientras que en los restantes 2 (40%) fue mayor a 40 mmHg. De estos últimos el 1 paciente recibió TPM y 1 paciente ACZ. Dos pacientes (40%) presentaron en el seguimiento una recaída sintomática, al intentar descender la medicación. No se pudo definir como parámetro de decisión la presión de apertura en del uso de uno u otro medicamento ya que al evaluar el uso de TPM y ACZ en pacientes con presión de apertura mayor a 41 mmHg solo se detallaron 2 pacientes cada uno tratado con un medicamento de los anteriormente descriptos. (Chi cuadrado p= 0.44). Ninguno de los pacientes tratados requirió otro tratamiento complementario como PL seriadas o válvula de derivación ventrículo peritoneal. CONCLUSION: No se logró determinar beneficio en el uso de un medicamento sobre otro en el tratamiento de la hipertensión endocraneana idiopática (p=0,07), pese al tamaño muestral, el cual podría ser un limitante. Coincidentemente con la literatura sigue sin haber evidencia suficiente. No existe un algoritmo de consenso en cuanto al correcto manejo terapéutico y farmacológico de esta entidad. El uso de TPM o ACZ no condiciona la posterior aparición de complicaciones (p= 0.45) El estudio oftalmológico es esencial para diagnóstico y seguimiento. No se pudo establecer correlación entre el valor obtenido en la medición de la presión de apertura y el tratamiento instaurado. (AU)

INTRODUCTION: The term Pseudotumor cerebri is reserved for those endocranial hypertensio (EH) that resemble clinically the existence of a brain tumor, due to alteration of the circulation of the cerebrospinal fluid (CSF). Classically, the Dandy-Smith criteria for diagnosis are described. TYPE OF STUDY: Descriptive observational. OBJECTIVE: Primary Objective: To determine the benefit of the use of Azetazolamide (ACZ) or Topiramate (TPM) in the treatment of idiopathic endocranial hypertension Secondary Objectives: Use of Opening Pressure as indicator parameter for use of ACZ or TPM. MATERIALS AND METHODS: Observed patients(N:5) per clinic with diagnosis of EIH by criteria of Dandy-Smith in the period 2013-2017. I was performed in all patients: RMNC s/contrast Fundus oculi Lumbar puncture + opening pressure. RESULTS: Of the total of patients (5) 100% had F.O pathological and as cardinal symptom headache, and 40% also vomiting. The mean follow-up was 11 months 60% of the patients was treated with topiramate (TPM) while 40% received acetazolamide (ACZ), both without complications. Of the total of patients 60% presented less than 40 opening pressure mmHg, while that in the remaining 40% was greater than 40 mmHg, of which 50% received TPM and 50% ACZ. 40% presented in tracking a symptomatic relapse, trying to get off the medication. None of the treated patients required other adjunctive therapy such as serial PL or ventricleperitoneal shunt. CONCLUSIONS: It was not possible to determine benefit in the use of one drug over another in the treatment of idiopathic intracranial hypertension (p = 0.07), despite the sample size, which could be a limitation. Coincidentally with the literature there is still not enough evidence. There is no consensus algorithm regarding the correct therapeutic and pharmacological management of this entity. The use of TPM or ACZ does not condition the subsequent appearance of complications (p = 0.45) The ophthalmological study is essential for diagnosis and follow-up. No correlation could be established between the value obtained in the measurement of the opening ression and the treatment established. (AU)

Mujer de 25 años con lesión seudotumoral talámica posvacunal / A 25 years-old woman with a postvaccine thalamic pseudotumoral lesion

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Pediatric lumbar puncture and cerebrospinal fluid analysis.

BACKGROUND: Lumbar puncture (LP) is a commonly performed procedure in pediatrics. Accurate analysis of cerebrospinal fluid (CSF) profile is essential in diagnosing and managing a variety of infectious and inflammatory conditions involving the brain, meninges, and spinal cord. It can also provide useful diagnostic information in the evaluation of possible subarachnoid hemorrhage and demyelinating syndromes, and aid in the diagnosis and management of pseudotumor cerebri. OBJECTIVES: To review anatomic, physiologic, and pathologic aspects of performing pediatric lumbar puncture and CSF analysis. DISCUSSION: Although still a commonly performed procedure in the outpatient setting, effective vaccines to prevent invasive infection due to Streptococcus pneumoniae and Haemophilus influenzae type b have greatly reduced pediatric bacterial meningitis rates due to these pathogens, resulting in decreased opportunity for physician-trainees to perfect this important skill (among nonneonates) during the 3 years of supervised residency training. Success in performing pediatric LP is augmented by a thorough understanding of medical aspects related to this procedure. This article discusses technical aspects involved in successfully performing a lumbar puncture to obtain CSF, and interpreting a CSF profile in children. CONCLUSION: A thorough understanding of anatomic, physiologic, and pathologic considerations regarding performing lumbar puncture and CSF analysis can augment success in diagnosing a variety of potentially serious pediatric conditions.

Elevated cerebrospinal fluid (CSF) leptin in idiopathic intracranial hypertension (IIH): evidence for hypothalamic leptin resistance?

OBJECTIVE: The aetiology of idiopathic intracranial hypertension (IIH) is not known, but its association with obesity is well-recognized. Recent studies have linked obesity with abnormalities in circulating inflammatory and adiposity related cytokines. The aim of this study was to characterize adipokine and inflammatory cytokine profiles in IIH. DESIGN: Paired serum and cerebrospinal fluid (CSF) specimens were collected from 26 patients with IIH and compared to 62 control subjects. Samples were analysed for leptin, resistin, adiponectin, insulin, IL-1beta, IL-6, IL-8 (CXCL8), TNFalpha, MCP-1 (CCL2), hepatocyte growth factor, nerve growth factor and PAI-1 using multiplex bead immunoassays. RESULTS: CSF leptin was significantly higher in patients with IIH (P = 0.001) compared to controls after correction for age, gender and body mass index (BMI). In the control population, BMI correlated with serum leptin (r = 0.34; P = 0.007) and CSF leptin (r = 0.51; P < 0.0001), but this was not the case for the IIH population. Profiles of other inflammatory cytokines and adipokines did not differ between IIH patients and controls once anthropometric factors had been accounted for. CONCLUSIONS: IIH was characterized by significantly elevated CSF leptin levels which did not correlate with BMI. We suggest that CSF leptin may be important in the pathophysiology of IIH and that obesity in IIH may occur as a result of hypothalamic leptin resistance.

A novel splicing mutation in SLC12A3 associated with Gitelman syndrome and idiopathic intracranial hypertension.

We report a case of Gitelman syndrome (GS) in a dizygotic twin who presented at 12 years of age with growth delay, metabolic alkalosis, hypomagnesemia and hypokalemia with inappropriate kaliuresis, and idiopathic intracranial hypertension with bilateral papilledema (pseudotumor cerebri). The patient, her twin sister, and her mother also presented with cerebral cavernous malformations. Based on the early onset and normocalciuria, Bartter syndrome was diagnosed first. However, mutation analysis showed that the proband is a compound heterozygote for 2 mutations in SLC12A3: a substitution of serine by leucine at amino acid position 555 (p.Ser555Leu) and a novel guanine to cytosine transition at the 5' splice site of intron 22 (c.2633+1G>C), providing the molecular diagnosis of GS. These mutations were not detected in 200 normal chromosomes and cosegregated within the family. Analysis of complementary DNA showed that the heterozygous nucleotide change c.2633+1G>C caused the appearance of 2 RNA molecules, 1 normal transcript and 1 skipping the entire exon 22 (r.2521_2634del). Supplementation with potassium and magnesium improved clinical symptoms and resulted in catch-up growth, but vision remained impaired. Three similar associations of Bartter syndrome/GS with pseudotumor cerebri were found in the literature, suggesting that electrolyte abnormalities and secondary aldosteronism may have a role in idiopathic intracranial hypertension. This study provides further evidence for the phenotypical heterogeneity of GS and its association with severe manifestations in children. It also shows the independent segregation of familial cavernomatosis and GS.

Dronabinol reduces signs and symptoms of idiopathic intracranial hypertension: a case report.

A case is presented in which a woman diagnosed with a longstanding history of idiopathic intracranial hypertension reported improvement of frontal headaches, photophobia, transient blindness, enlarged blind spots, and tinnitus after smoking marijuana. All these symptoms and signs were associated with increased intracranial pressure (220-425 mm of water). Treatment with dronabinol at a dose of 10 mg twice a day, then reduced to 5 mg twice a day, relieved all of her symptoms. Previously noted papilledema and enlargement of blind spots also resolved, and this, in the absence of psychoactive effect or weight gain.

Evidence based or theoretical concern? Pseudotumor cerebri and depression as acitretin side effects.

Oral retinoids are among the first line agents for treatment of pustular and erythrodermic psoriasis, and they are effective in combination with phototherapy and other topical and systemic agents for the treatment of plaque psoriasis. Acitretin is the leading oral retinoid used today for the treatment of psoriasis. Recently, possible side effects such as pseudotumor cerebri and depression have gained a warning and precaution respectively on the acitretin package insert. This paper presents a review of the scientific literature and attempts to clarify whether warnings of these side effects have arisen from a scientific evidence base or from theoretical concern/class labeling. A paucity of scientific evidence was found in this review for acitretin-associated pseudotumor cerebri and depression. The authors conclude that these 2 acitretin side effects must be further investigated to assess whether these associations are scientifically certain or if class labeling has led to inclusion in the package insert.

Pseudotumor cerebral maligno en niños por intoxicación plumbica / Lead poisoning presenting as malignant pseudotumor cerebri in children

Introducción: El pseudotumor cerebral (PTC), o hipertensión endocraneana benigna, se caracteriza por sintomatología compatible con aumento de la presión endocraneana en ausencia de lesión espacio-ocupante o dilatación ventricular por obstrucción en la circulación del LCR. Se presenta con cefalea, náuseas-vómitos, y variadas alteraciones visuales con edema de papila. Las causas subyacentes son múltiples: alteraciones endocrinas y metabólicas, obesidad, medicamentos, trombosis senos venosos, megadosis de vitamina A, sinusopatías, etc. como así también intoxicación plúmbica. Objetivo: Detectar etiología tóxica en pacientes con PTC en los cuales no se evidenció factor causal mediante interrogatorio dirigido a pesquisa de fuentes contaminantes de plomo. Material y Métodos: Análisis prospectivo de pacientes ambulatorios o internados con diagnóstico de PTC. Estudios de Rx de cráneo, oftalmológicos y neuroimágenes. Aplicación de protocolo de investigación de contacto con plomo. Determinaciones en sangre y orina del bioperfil plúmbico: ALA-D, ALA-U, plombemia, PPE, coproporfirinas III. Comparación con grupo control de los niños intoxicados. Aplicación del test de Fisher. Resultados: Fueron evaluados 66 niños entre 4 meses y 13 años, 49 varones. Las manifestaciones clínicas fueron: cefalea (85 por ciento), náuseas-vómitos (94 por ciento), alteraciones visuales: diplopia, disminución agudeza visual, estrabismo (91 por ciento), edema de papila (89 por ciento). Hipertensión radiológica (55 por ciento). Neuroimágenes: negativas para tumor (100 por ciento), compatible con edema cerebral (27 por ciento). Los factores etiológicos fueron identificados en 55 casos (83 por ciento) obesidad, endocrinopatía, corticoterapia, megadosis de vitamina A, sinusopatías, etc. En el 17 por ciento restante (11 casos) se sospechó etiología tóxica, comprobándose intoxicación plúmbica en 8 niños (12 por ciento). Conclusiones: Sugerimos aplicar interrogatorio dirigido hacia pesquisa de fuentes contaminantes de plomo en casos de PTC sin causa establecida, y confirmación con análisis de nivel de plomo en sangre y bioperfil plúmbico. El diagnóstico y tratamiento oportunos evitarán el pronóstico desfavorable de la afección, por eso consideramos como "maligno" a este tipo de pseudotumor.

Pseudotumor cerebral maligno en niños por intoxicación plumbica / Lead poisoning presenting as malignant pseudotumor cerebri in children

Introducción: El pseudotumor cerebral (PTC), o hipertensión endocraneana benigna, se caracteriza por sintomatología compatible con aumento de la presión endocraneana en ausencia de lesión espacio-ocupante o dilatación ventricular por obstrucción en la circulación del LCR. Se presenta con cefalea, náuseas-vómitos, y variadas alteraciones visuales con edema de papila. Las causas subyacentes son múltiples: alteraciones endocrinas y metabólicas, obesidad, medicamentos, trombosis senos venosos, megadosis de vitamina A, sinusopatías, etc. como así también intoxicación plúmbica. Objetivo: Detectar etiología tóxica en pacientes con PTC en los cuales no se evidenció factor causal mediante interrogatorio dirigido a pesquisa de fuentes contaminantes de plomo. Material y Métodos: Análisis prospectivo de pacientes ambulatorios o internados con diagnóstico de PTC. Estudios de Rx de cráneo, oftalmológicos y neuroimágenes. Aplicación de protocolo de investigación de contacto con plomo. Determinaciones en sangre y orina del bioperfil plúmbico: ALA-D, ALA-U, plombemia, PPE, coproporfirinas III. Comparación con grupo control de los niños intoxicados. Aplicación del test de Fisher. Resultados: Fueron evaluados 66 niños entre 4 meses y 13 años, 49 varones. Las manifestaciones clínicas fueron: cefalea (85 por ciento), náuseas-vómitos (94 por ciento), alteraciones visuales: diplopia, disminución agudeza visual, estrabismo (91 por ciento), edema de papila (89 por ciento). Hipertensión radiológica (55 por ciento). Neuroimágenes: negativas para tumor (100 por ciento), compatible con edema cerebral (27 por ciento). Los factores etiológicos fueron identificados en 55 casos (83 por ciento) obesidad, endocrinopatía, corticoterapia, megadosis de vitamina A, sinusopatías, etc. En el 17 por ciento restante (11 casos) se sospechó etiología tóxica, comprobándose intoxicación plúmbica en 8 niños (12 por ciento). Conclusiones: Sugerimos aplicar interrogatorio dirigido hacia pesquisa de fuentes contaminantes de plomo en casos de PTC sin causa establecida, y confirmación con análisis de nivel de plomo en sangre y bioperfil plúmbico. El diagnóstico y tratamiento oportunos evitarán el pronóstico desfavorable de la afección, por eso consideramos como "maligno" a este tipo de pseudotumor. (AU)

Familial hypomagnesemia--hypercalciuria and pseudotumor cerebri.

Approximately 30 patients with familial hypomagnesemia-hypercalciuria have been reported. We describe an 8-year-old girl with cardinal findings of familial hypomagnesemia-hypercalciuria (hypomagnesemia, hypermagnesiuria, hypercalciuria, renal insufficiency, hyperuricemia, elevated serum parathormone, hyposthenuria and nephrocalcinosis), who received combination therapy consisting of magnesium salts, thiazide diuretic and potassium supplementation. At the 4-year follow-up investigation under this treatment, the patient was found to have cerebral pseudotumor (increased intracranial pressure with normal or small ventricles on neuroimaging, no evidence of an intracranial mass and normal cerebrospinal fluid composition) with papilledema and visual field defects. Thiazide therapy was terminated and the cerebral pseudotumor disappeared. The authors hypothesize that cerebral pseudotumor in this patient was related to severe hypocalcemia, as a consequence of profound hypomagnesemia induced by protracted thiazide treatment. To our knowledge, this is the first report of a child with familial hypomagnesemia-hypercalciuria who developed pseudotumor cerebri after thiazide therapy.

Safety issues in children and adolescents during growth hormone therapy--a review.

The action of growth hormone (GH) via its receptor involves many organ systems and metabolic pathways. These diverse actions are reviewed in this paper in the context that they may represent unwanted side-effects of GH therapy for growth promotion. The monitoring of GH therapy in large multicentre international databases has demonstrated a low frequency of adverse events. Tumour recurrence or new malignancy are not increased. Headaches, especially in the first few months of therapy, require close evaluation as benign intracranial hypertension is found infrequently, especially in children with GH deficiency and chronic renal failure (CRF). Children at risk for slipped capital femoral epiphysis and scoliosis require close monitoring during therapy. Decreased insulin sensitivity that is dose-dependent is observed during GH therapy. Glucose homeostasis, however, is not affected, but a recent report of increased incidence of Type 2 diabetes mellitus in children undergoing GH therapy requires prospective surveillance.