Relationship of Bern Score, Spinal Elastance, and Opening Pressure in Patients With Spontaneous Intracranial Hypotension.

BACKGROUND AND OBJECTIVES: Existing tools to diagnose spontaneous intracranial hypotension (SIH), namely spinal opening pressure (OP) and brain MRI, have limited sensitivity. We investigated whether evaluation of brain MRI using the Bern score, combined with calculated craniospinal elastance, would aid in diagnosing SIH and provide insight into its pathophysiology. METHODS: A retrospective chart review was performed of patients who underwent brain MRI and pressure-augmented dynamic CT myelography (dCTM) for suspicion of SIH. Two blinded neuroradiologists assigned Bern scores for each brain MRI. OP and incremental pressure changes after intrathecal saline infusion were recorded to calculate craniospinal elastance. The relationship between Bern score, OP, and elastance and whether a leak was found were analyzed. RESULTS: Seventy-two consecutive dCTMs were performed in 53 patients. Twelve CSF-venous fistulae, 2 ruptured meningeal diverticula, 2 dural defects, and 1 dural bleb were found (17/53, 32%). Among patients with imaging-proven CSF leak/fistula, OP was normal in all but 1 patient and was not significantly different in those with a leak compared with those without (15.1 vs 13.6 cm H2O, p = 0.24, A = 0.40). The average Bern score in individuals with a leak was significantly higher than that in those without (5.35 vs 1.85, p < 0.001, A = 0.85), even when excluding pachymeningeal enhancement from the score (3.77 vs 1.57, p = 0.001, A = 0.78). The average elastance in those with a leak was higher than that in those without, but this difference was not statistically significant (2.05 vs 1.20 mL/cm H2O, p = 0.19, A = 0.40). Increased elastance was significantly associated with an increased Bern score (95% CI -0.55 to 0.12, p < 0.01) and was significantly associated with venous distention, pachymeningeal enhancement, prepontine narrowing, and subdural collections, but not a narrowed mamillopontine or suprasellar distance. DISCUSSION: OP is not an effective predictor for diagnosing CSF leak and if used in isolation would result in misdiagnosis of 94% of patients in our cohort. The Bern score was associated with a higher diagnostic yield of dCTM. Elastance was significantly associated with certain components of the Bern score.

Spontaneous intracranial hypotension: from cerebral venous thrombosis to neurosurgical treatment: a case series experience from a Brazilian tertiary health care center.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a secondary cause of headache and an underdiagnosed disease. The clinical presentation can be highly variable. It typically presents with isolated classic orthostatic headache complaints, but patients can develop significant complications such as cerebral venous thrombosis (CVT). OBJECTIVE: To report 3 cases of SIH diagnosis admitted and treated in a tertiary-level neurology ward. METHODS: Review of the medical files of three patients and description of clinical and surgical outcomes. RESULTS: Three female patients with SIH with a mean age of 25.6 ± 10.0 years old. The patients had orthostatic headache, and one of them presented with somnolence and diplopia because of a CVT. Brain magnetic resonance imaging (MRI) ranges from normal findings to classic findings of SIH as pachymeningeal enhancement and downward displacement of the cerebellar tonsils. Spine MRI showed abnormal epidural fluid collections in all cases, and computed tomography (CT) myelography could determine an identifiable cerebrospinal fluid (CSF) leak in only one patient. One patient received a conservative approach, and the other two were submitted to open surgery with laminoplasty. Both of them had uneventful recovery and remission in surgery follow-up. CONCLUSION: The diagnosis and management of SIH are still a challenge in neurology practice. We highlight in the present study severe cases of incapacitating SIH, complication with CVT, and good outcomes with neurosurgical treatment.

ANTECEDENTES: Hipotensão intracraniana espontânea (HIE) é uma causa secundária de cefaleia e uma doença subdiagnosticada. A apresentação clínica pode ser muito variável. Tipicamente, se apresenta com queixas isoladas de cefaleia ortostática clássica, porém pode evoluir com complicações significativas como trombose venosa cerebral (TVC). OBJETIVO: Relatar 3 casos de diagnóstico de hipotensão intracraniana espontânea manejados em uma enfermaria de neurologia de nível terciário. MéTODOS: Revisão dos prontuários de três pacientes e descrição dos resultados clínicos e cirúrgicos. RESULTADOS: Três pacientes do sexo feminino com média de idade de 25.6 ± 10.0 anos. As pacientes apresentavam cefaleia ortostática e uma delas apresentou sonolência e diplopia devido a TVC. A ressonância magnética (RM) do encéfalo varia de achados normais até achados clássicos de HIE como realce paquimeníngeo e deslocamento inferior das tonsilas cerebelares. A RM da coluna mostrou coleções anormais de líquido epidural em todos os casos e a mielografia por tomografia computadorizada (TC) foi capaz de determinar fístula liquórica identificável em apenas uma paciente. Uma paciente recebeu abordagem conservadora e as outras duas foram submetidas a cirurgia aberta com laminoplastia. Ambas tiveram recuperação e remissão sem intercorrências no seguimento cirúrgico. CONCLUSãO: O diagnóstico e manejo da hipotensão intracraniana ainda são desafios na prática neurológica. Destacamos no presente estudo casos graves, complicação com TVC e bons resultados com tratamento neurocirúrgico.

Structured Reporting for Findings of Spontaneous Intracranial Hypotension on Brain MRI.

A voluntary structured reporting template (based on the Bern score) for brain MRI examinations performed for suspected spontaneous intracranial hypotension (SIH) was associated with an increase in reporting of intracranial MRI findings of SIH and a reduction in discordant assessments with respect to a reference reader.

Síndrome de hipotensión endocraneana espontánea: reporte de un caso clínico / Spontaneous intracranial hypotension syndrome: clinic case report

La hipotensión intracraneal espontánea (SIH) es una patología con una incidencia anual aproximada de 5 por cada 100.000 personas al año, caracterizada clásicamente por cefalea ortostática comúnmente secundaria a una fuga espontánea de líquido cefalorraquídeo (LCR), pudiendo existir a su vez una amplia gama de síntomas asociados. El diagnóstico se centra en la clínica y en hallazgos típicos en la resonancia nuclear magnética (RM), sin embargo, según diversos estudios, puede corresponder a una patología subdiagnosticada por la dificultad que ha existido en definir criterios diagnósticos universales y un manejo terapéutico estandarizado, el cual varía inicialmente entre manejo médico conservador y/o parches de sangre epidural (PHE). Reportamos el caso de una paciente femenina de 51 años quien fue atendida en el Hospital Herminda Martin de Chillán donde se realizó el diagnóstico y tratamiento sintomático con PHE.

Spontaneous intracranial hypotension (SIH) is a pathology with an annual incidence of approximately 5 per 100,000 people per year, classically characterized by orthostatic headache commonly secondary to a spontaneous cerebrospinal fluid (CSF) leak, and there may also be a wide range of of associated symptoms. The diagnosis is centered on the clinic and on typical findings in magnetic resonance imaging (MRI), however, according to various studies, it may correspond to an underdiagnosed pathology due to the difficulty that has existed in defining universal diagnostic criteria and standardized therapeutic management. which initially varies between conservative medical management and/or epidural blood patches (PHE). We report the case of a 51-year-old female patient who was treated at the Herminda Martin de Chillán Hospital where the diagnosis and symptomatic treatment with PHE were made.

Prenatal intracranial hypotension syndrome in congenital cephalocele: Insights into pathophysiologic mechanisms and importance of defect coverage.

OBJECTIVE: To compare specific fetal cranial and cerebral findings indicative of prenatal intracranial hypotension (PICH) syndrome between open and closed cephaloceles. METHODS: Two groups of fetuses with cephalocele who underwent magnetic resonance imaging scan were selected based on the defect coverage: covered ("closed cephalocele" group, n = 4) and uncovered by skin ("open cephalocele" group, n = 8). The presence of signs associated with PICH syndrome was evaluated and findings compared between groups. RESULTS: Cases from the open cephalocele group showed statistically significant differences in comparison with the closed cephalocele group regarding reduction of the interpeduncular angle (p = 0.006), reduced amount of cerebrospinal fluid in the subarachnoid space (p = 0.01), collapsed/severely reduced fourth ventricle (p = 0.03), cephalocele content (p = 0.03), and identification of a vector traction (p = 0.03). There were no differences in the presence of cerebellar tonsillar descent, obstructive ventriculomegaly, and presence of the lemon sign. In comparison with gestational age-matched controls, only the biparietal diameter but not the head circumference was significantly smaller in both the open and closed cephalocele groups (p < 0.05). CONCLUSION: Congenital cephaloceles present distinct imaging behavior depending on the defect coverage. Therefore, the classification of cephalocele should include, in addition to location and content, the presence or absence of skin fully covering the defect.

Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder.

Spontaneous intracranial hypotension (SIH) is a rare neurological condition caused by low cerebrospinal fluid (CSF) volume, most commonly due to a CSF leak. The most common presenting symptom is an orthostatic headache, but some patients may present with atypical neurological manifestations such as cranial nerve palsies, an altered mental status, and movement disorders, which complicate the clinical diagnosis. Therefore, the diagnosis is based on the combination of clinical signs and symptoms, neuroimaging, and/or a low cerebrospinal fluid pressure. In this review, we describe the wide variety of neurological manifestations and complications seen in patients with SIH as well as the most common features described on imaging studies, including both subjective and objective measurements, in order to lead the clinician to a correct diagnosis. The prompt and correct management of patients with SIH will help prevent the development of life-threatening complications, such as subdural hematomas, cerebral venous thrombosis, and coma, and avoid unnecessary invasive procedures.

Dural ectasia and intracranial hypotension in Marfan syndrome. / Ectasia dural e hipotensión endocraneal en síndrome de Marfán.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Spontaneous intracranial hypotension and its complications.

Spontaneous intracranial hypotension (SIH) is a syndrome that was unknown until the advent of magnetic resonance imaging (MRI). It is a cause of orthostatic headache, which remains underdiagnosed and, rarely, can result in several complications including dural venous sinus thrombosis, subdural hematoma and subarachnoid hemorrhage. Some of these complications are potentially life-threatening and should be recognized promptly, mainly by imaging studies. We reviewed the MRI of nine patients with SIH and describe the complications observed in three of these patients. Two of them had subdural hematoma and one had a dural venous sinus thrombosis detected by computed tomography and MRI. We concluded that MRI findings are of great importance in the diagnosis of SIH and its complications, which often influence the clinical-surgical treatment of the patient.

Spontaneous intracranial hypotension and its complications / Hipotensão intracraniana espontânea e complicações

ABSTRACT Spontaneous intracranial hypotension (SIH) is a syndrome that was unknown until the advent of magnetic resonance imaging (MRI). It is a cause of orthostatic headache, which remains underdiagnosed and, rarely, can result in several complications including dural venous sinus thrombosis, subdural hematoma and subarachnoid hemorrhage. Some of these complications are potentially life-threatening and should be recognized promptly, mainly by imaging studies. We reviewed the MRI of nine patients with SIH and describe the complications observed in three of these patients. Two of them had subdural hematoma and one had a dural venous sinus thrombosis detected by computed tomography and MRI. We concluded that MRI findings are of great importance in the diagnosis of SIH and its complications, which often influence the clinical-surgical treatment of the patient.

RESUMO Hipotensão Intracraniana Espontânea (HIE) é uma síndrome desconhecida até o advento das imagens de Ressonância Magnética (RM). É uma causa de cefaleia ortostática que permanece subdiagnosticada e raramente resulta em complicações, como trombose de seios venosos durais, hematoma subdural e hemorragia subaracnoidea. Algumas dessas complicações são potencialmente ameaçadoras à vida e devem ser prontamente reconhecidas pelos estudos de imagem. Nós revisamos as RM de 9 pacientes com HIE e descrevemos as complicações observadas em 3 casos. Dois deles tiveram hematoma subdural e um teve trombose de seio venoso dural detectados por tomografia computadorizada e RM. Concluímos que achados de RM são de grande importância no diagnóstico de HIE e suas complicações, frequentemente influenciando o tratamento clínico-cirúrgico do paciente.