Diagnosis and treatment of disorders of intracranial pressure: consensus statement of the Spanish Society of Neurology's Headache Study Group.

Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology's Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.

Hipotensión intracraneal espontánea. Una puesta al día

Introducción: La hipotensión intracraneal espontánea es un síndrome causado por la disminución del volumen de líquido cefalorraquídeo consecuencia de su fuga al espacio extradural. Aunque la ICHD-3 proporciona un alto nivel de especificidad diagnóstica, esta enfermedad puede manifestarse de forma atípica. Hasta en un 30% no es posible establecer el punto de escape, pero con el refinamiento de los exámenes de imágenes este porcentaje se ha reducido a un 15%-20%. Actualmente, su manejo no se encuentra estandarizado y las recomendaciones se basan en evidencia de limitada calidad metodológica, además de la variabilidad de protocolos entre distintos centros. Desarrollo En esta revisión actualizamos los procedimientos diagnósticos y terapéuticos. Por un lado, analizamos el rol de la resonancia nuclear magnética de encéfalo y médula espinal completa como primer paso diagnóstico y, por otro lado, señalamos los exámenes destinados a determinar la fuga de líquido cefalorraquídeo. Tal es el caso de la mielo-resonancia, la mielo-tomografía computarizada, tanto estándar, dinámica y por sustracción digital, además de la cisternografía con 111-Indium-DPTA. Sin embargo, determinar cuál de estos exámenes es el óptimo es objeto de debate. Lo mismo ocurre con el tratamiento: reposo; parche sanguíneo epidural a ciegas, parche guiado por fluoroscopia o tomografía computarizada, parche de fibrina; o cirugía. Conclusiones Se requiere de una mayor investigación, especialmente con trabajos multicéntricos controlados, para una mejor comprensión de la fisiopatología, el diagnóstico por imágenes, los enfoques terapéuticos y evaluación objetiva de los resultados clínicos. Solo así se establecerán pautas diagnósticas y de tratamiento validadas.

Introducction: Spontaneous intracranial hypotension is a syndrome caused by decreased CSF volume secondary to its leakage into the extradural space Although ICHD-3 provides a high level of diagnostic specificity, manifestations may be atypical, making diagnosis challenging. The site of leakage may be undetermined in point Up to 30% of cases, although with recent refinement of imaging, this percentage has been reduced to 15-20%. Currently, management is not standardized and recommendations are based on inconclusive evidence, with variability of protocols between centres. Development. In this review, we update diagnostic and therapeutic procedures. We analyse the role of whole brain and spinal cord MRI as a first investigation and review tests aimed at determining cerebrospinal fluid leakage, such as MRI myelography, conventional CT myelography, dynamic CT myelography, and digital subtraction CT myelography, as well as 111-Indium-DPTA cisternography. Determining optimal use of these investigations remains a matter of debate. The same is true for treatment: rest, blind epidural blood patch, fluoroscopy or CT-guided epidural blood patch, fibrin patch and surgery are discussed. Conclusión: Further research, especially multicentre controlled studies, is required to improve understanding of pathophysiology, diagnostic imaging, therapeutic approaches and to objectively assess clinical outcomes. Only then will diagnostic and treatment guidelines be evidence-based.

Síndrome de hipotensión intracraneal en paciente pediátrico con síndrome de Marfan / Intracranial hypotension syndrome in a pediatric patient with Marfan syndrome

El síndrome de hipotensión intracraneal (SHI) es consecuencia del descenso de la presión de líquido cefalorraquídeo (LCR), espontáneo o secundario a antecedente traumático o enfermedad sistémica.Presentamos el caso de un niño de 11 años con cefalea ortostática y vómitos de 12 horas de evolución tras una caída sobre región sacrocoxígea; antecedente de síndrome de Marfan. La resonancia craneomedular mostró colecciones líquidas extradurales a nivel dorsal y lumbosacro compatibles con fístula de LCR. El cuadro se resolvió tras tratamiento, pero hubo dos nuevos episodios durante el seguimiento, por lo que se aplicó un parche hemático epidural a los dos años del inicio.Aunque el SHI es infrecuente en niños, debe sospecharse ante pacientes con cefalea ortostática, especialmente si existe patología favorecedora como enfermedades del tejido conectivo. Existe poca evidencia sobre el manejo en edad pediátrica, por lo que se presenta este caso y se realiza una revisión de la literatura.(AU)

Intracranial hypotension syndrome (IHS) is attributed to reduced cerebrospinal fluid (CSF) pressure. It may be spontaneous or secondary to a history of trauma or systemic disease. We present the case of an 11-year-old boy, with medical history of Marfan syndrome, with orthostatic headache and persistent vomiting (12 hours) following a fall on the sacrococcygeal region. Magnetic resonance showed extradural fluid collections at dorsal and lumbosacral levels, compatible with CSF leak. The condition was resolved with treatment, but the patient had two new episodes during the follow-up period. Thus, an epidural blood patch was performed two years after the first episode.Although HIS is uncommon in children, it should be suspected in patients with orthostatic headache, particularly if the patient presents a connectivopathy. Few studies have assessed the management of HIS in paediatric age. The case presented here and the reviewed available literature provides further data for these type of cases.(AU)

Spontaneous intracranial hypotension due to sacral diverticula: Two-case history and a pocket-sized review.

Spontaneous intracranial hypotension is an increasingly recognized cause of atypical, secondary headaches. Nevertheless, its clinical and imaging spectrum is far from an exhaustive definition, ranging from straightforward cases with unambiguous findings and prompt response to treatment to more challenging ones, requiring advanced, more complex imaging and targeted therapies. We describe two unusual cases as a cue to draw a literature-based, practical approach to the management of the syndrome.

Spontaneous intracranial hypotension syndrome: contribution of radioisotope cisternography. / Síndrome de hipotensión intracraneal espontánea: aportación de la cisternografía radioisotópica.

Spontaneous intracranial hypotension is a clinical syndrome caused by a loss of cerebrospinal fluid volume, usually secondary to leaking through structural defects of the spinal dura mater. Radioisotope cisternography (RC) can confirm the diagnosis of spontaneous intracranial hypotension, especially in doubtful or atypical case presentations. A retrospective study was conducted on 8 patients who underwent RC because spontaneous intracranial hypotension was suspected, and they presented with atypical clinical manifestations and/or inconclusive findings in other imaging techniques. RC detected paradural extravasation of cerebrospinal fluid in 7 patients. Moreover, there was indirect evidence of cerebrospinal fluid leaks in all 8 patients (early appearance of radioactivity in the bladder, soft tissue uptake of radioisotope and/or reduction in the amount of radiotracer in the brain at 24hours). RC had a significant impact on the diagnosis of 6 patients, and on the therapeutic management of 4 patients.

[Management of transient radicular pain after receiving an epidural blood patch for headaches due to spontaneous intracranial hypotension]. / Radiculalgia transitoria tras administración de parche hemático epidural en la cefalea por hipotensión intracraneal espontánea, conducta a seguir.

Spontaneous intracranial hypotension headache is an uncommon disease that resolves spontaneously in most of the cases and in a short period of time. The initial treatment should be symptomatic. In some patients the symptomatology is extremely disabling, and in these cases both the diagnosis and treatment may be performed by an epidural blood patch. A 49-year-old Caucasian woman, with no previous record of epidural or intrathecal puncture, consulted in the Emergency Department complaining of a 9-day history of frontal headache and diplopia, along with nausea and vomiting. The patient was diagnosed with spontaneous intracranial hypotension headache. Considering the symptomatology and the uncontrolled pain, the Pain Unit of our hospital performed an epidural blood patch. In the first 24h the patient reported a remarkable relief of both headache and diplopia but developed a left lumbar radiculopathy that was treated successfully with supportive measures. Transient lumbar radiculopathy is a common and acceptable event secondary to the use of epidural blood patch as a treatment for spontaneous intracranial hypotension headache.

[Paradoxical transtentorial herniation, extreme trephined syndrome sign: A case report]. / Herniación paradójica transtentorial, manifestación extrema del síndrome del craniectomizado. Caso clínico.

The current increasing use of decompressive craniectomy carries the implicit appearance of complications due to alterations in both intracranial pressure and in the hydrostatic-hemodynamic equilibrium. Paradoxical transtentorial herniation represents a rare manifestation, included in "trephine syndrome", extremely critical but with relatively simple treatment. We present the case of a 56-year-old woman with no interesting medical history, who, after an olfactory groove meningioma surgery, presented a haemorrhage located in the surgical area with an important oedema. The patient required a second emergency surgery without any chance of conserving the cranial vault. During the post-operational period, great neurological deterioration in orthostatic position was noticed, which resolved spontaneously in decubitus. This deficit was resolved with bone replacement afterwards. We discuss possible predisposing factors and aetiologies of this pathology.