Spontaneous intracranial hypotension: a case report in the context of differential diagnosis of chronic headaches / Hipotensão intracraniana espontânea: relato de caso no contexto do diagnóstico diferencial de cefaleias crônicas

Spontaneous intracranial hypotension (SIH) is a rare and debilitating condition caused by decreased intracranial pressure, which occurs more frequently in females. SIH can have several causes, among them the spontaneous formation of cerebrospinal fluid venous fistula (CSF-venous fistula), which is primarily responsible for the appearance of postural headache. Orthostatic headache is diagnosed by CSF pressure < 6 mmHg associated with specific imaging findings. Other specific symptoms such as dizziness, reduced muscle strength, blurred vision and syncope and other more systemic symptoms such as fatigue, mental confusion and difficulty concentrating are commonly observed. Etiological investigation through imaging studies such as magnetic resonance imaging and dynamic tomography of myelography is necessary for diagnosis. Due to the debilitating condition, several therapeutic approaches have been developed, ranging from more conservative approaches, with observation and use of analgesics, to more invasive interventions such as surgical ligation, transvenous embolization and blood tamponade.

A hipotensão intracraniana espontânea (HIH) é uma condição rara e debilitante causada pela diminuição da pressão intracraniana, que ocorre com mais frequência em mulheres. A HIE pode ter diversas causas, entre elas a formação espontânea de fístula venosa do líquido cefalorraquidiano (fístula liquórica-venosa), principal responsável pelo aparecimento da cefaleia postural. A cefaleia ortostática é diagnosticada pela pressão liquórica < 6 mmHg associada a achados de imagem específicos. Outros sintomas específicos como tontura, redução da força muscular, visão turva e síncope e outros sintomas mais sistêmicos como fadiga, confusão mental e dificuldade de concentração são comumente observados. A investigação etiológica por meio de exames de imagem como ressonância magnética e tomografia dinâmica da mielografia é necessária para o diagnóstico. Devido ao quadro debilitante, diversas abordagens terapêuticas têm sido desenvolvidas, desde abordagens mais conservadoras, com observação e uso de analgésicos, até intervenções mais invasivas como ligadura cirúrgica, embolização transvenosa e tamponamento sanguíneo.

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.

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.

Low cerebrospinal fluid pressure may not be necessary in the diagnosis of spontaneous intracranial hypotension: A report of four cases

@#We presented four patients with sub-acute onset orthostatic headache in occipital and sub-occipital regions and neck pain. No loss of consciousness, neurologic deficit, trauma or cranial/spinal surgery history was noted. They had normal cerebrospinal fluid (CSF) opening pressure, normal laboratory studies, and diffuse pachymeningeal enhancement or sagging of brain on gadolinium-enhanced magnetic resonance imaging (MRI). Their symptoms resolved with intravenous large isotonic fluid or epidural blood patch. The diagnosis of spontaneous intracranial hypotension requires history of orthostatic headache, demonstration of lower CSF pressure, and abnormal findings on MRI. But these patients may have normal CSF opening pressure. CSF hypovolemia rather than CSF hypotension has been proposed as the underlying cause. Therefore, the CSF pressure may not be necessary for diagnosis in such patients with typical radiographic features. Thus, in the presence of convincing clinical symptoms and imaging abnormalities, a normal CSF pressure should not discourage the clinician from searching for a source of CSF leak.

Rebound intracranial hypertension after noninvasive treatment of intracranial hypotension: Case report and literature review

@#Intracranial hypotension is a clinical syndrome characterized by orthostatic headache and low cerebrospinal fluid pressure. Noninvasive management is the usual first line treatment. Epidural blood patch is the treatment of choice if noninvasive treatments are ineffective. Cases with rebound intracranial hypertension after epidural blood patch treatment have been reported in the medical literature previously. We report here three patients with rebound intracranial hypertension who were treated noninvasively for intracranial hypotension. This phenomenon has not been reported previously. The underlying cause of intracranial hypotension was epidural anesthesia in the first, lumbar disc surgery in the second patient, and idiopathic in the third patient. They had been treated either with bed rest or with medical treatment not requiring epidural blood patch. After a short remission the patients were seen with a different headache pattern. They all had papilledema on examination. Automated perimetry revealed bilateral blind spot enlargement in Patient 1 and peripheral constriction in Patient 2. Cranial MRI and MRV in all three patients were normal. All the patients recovered very quickly with acetazolamide 1.5 or 2gm/day. In conclusion, rebound intracranial hypertension should be kept in mind in patients with intracranial hypotension who developed changes in the headache pattern, had new symptoms of nausea, vomiting, blurred or double vision during follow-up. Rebound intracranial hypertension can develop after conservative treatment of intracranial hypotension.

Analysis of clinical characteristics of cavernous sinus dural arteriovenous fistula / 中国神经精神疾病杂志

Objective To investigate the clinical characteristics and mechanism of cavernous sinus dural arteriovenous fistula (CSDAVF). Methods The clinical data of 17 CSDAVF patients, including clinical manifestations, characteristics of cerebrospinal fluid (CSF)and neuroimaging,were analyzed.The interventional catheter embolization of CSDAVF and postoperative follow-up were performed. Results Of the 17 subjects with CSDAVF, 5 (29.4%) were men and 12 (70.6%)were women (sex ratio=1:2.4).The average age of onset was (58.12±14.61)years old. Older CSDAVF patients were prone to be complicated with hypertension than general population at the same age. Onset symptoms included headache in 6 cases,eye symptoms in 5 cases,intracranial murmur in 2 cases,diplopia in 2 cases, dizziness in 1 case and slurred speech in 1 case. Digital subtraction angiography (DSA) was performed in all 17 patients. Further embolization was applied in 14 patients and had satisfactory outcome. In CSDAVF patients with headache, 2 patients showed orthostatic headache as initial symptom. One of them with intracranial hypotension reported exophthalmos 3 months after the onset. Those two patients remained asymptomatic during 3-year and 2-year follow-up after receiving interventional catheter embolization for CSDAVF. Conclusions CSDAVF usually occurs in middle-aged and older women, especially in those with hypertension. CSDAVF varies in clinical manifestations. A special attention should be given to orthostatic headache which can be presented as the initial symptom in few patients with CSDAVF. CTA or DSA should be considered in patients with clinically suspected CSDAVF to rule out the possibility of CSDAVF. The interventional catheter embolization is the primary treatment of CSDAVF.

Spontaneous Low Cerebrospinal Fluid Pressure Headache Initially Misdiagnosed as a Tension-type Headache / General Medicine

We report the case of a 34-year-old man who was initially diagnosed with a tension-type headache after complaining of a headache and nausea. His headache worsened in severity and it was exacerbated on standing in the upright position. The patient was admitted to the hospital on suspicion of spontaneous low cerebrospinal fluid (CSF) pressure headache. Gadolinium-enhanced brain magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement, brain sagging, cerebellar tonsillar herniation, brainstem descent and a subdural hematoma. Successful emergency surgery was undertaken.<br>Spontaneous low CSF pressure headache syndrome is characterized by orthostatic headache, and if such a headache worsens, clinicians should consider a subdural hematoma, a life-threatening complication of this unusual disorder.

Spontaneous Intracranial Hypotension Secondary to Lumbar Disc Herniation

Spontaneous intracranial hypotension is often idiopathic. We report on a patient presenting with symptomatic intracranial hypotension and pain radiating to the right leg caused by a transdural lumbar disc herniation. Magnetic resonance (MR) imaging of the brain revealed classic signs of intracranial hypotension, and an additional spinal MR confirmed a lumbar transdural herniated disc as the cause. The patient was treated with a partial hemilaminectomy and discectomy. We were able to find the source of cerebrospinal fluid leak, and packed it with epidural glue and gelfoam. Postoperatively, the patient's headache and log radiating pain resolved and there was no neurological deficit. Thus, in this case, lumbar disc herniation may have been a cause of spontaneous intracranial hypotension.

Pneumocephalus in Patients With Orthostatic Headache

Cerebrospinal fluid (CSF) leak or shunt overdrainage is a well-known cause of orthostatic headaches and low CSF pressures. We report two cases of orthostatic headache with pneumocephalus on brain imaging. The orthostatic headache developed after drainage of spinal operation site and epidural block. Brain MRI revealed characteristic findings of CSF hypovolemia including pachymeningeal enhancement and mild subdural fluid collections. Air was also observed in the ventricular or subarachnoid space in both patients, which might enter the subarachnoid or ventricular space during a procedure via the pressure gradient or an injection.

Treatment with Repeated Lumbar Epidural Blood Patches for a Patient with Orthostatic Headache after Suboccpital Craniectomy

Orthostatic headache after suboccipital craniectomy has been rarely reported. A 32-year-old man underwent emergent suboccipital craniectomy due to acute bilateral cerebellar infarctions with massive brain edema. Although his neurological deterioration halted after the craniectomy, he still suffered from a severe disabling orthostatic headache. We performed two epidural blood patches at the levels of the lumbar and thoracic spine. He improved considerably a few days after the repetitive epidural blood patches. Epidural blood patches may be a useful treatment modality for post-craniectomy postural headaches.

The Effectiveness of Epidural Blood Patch in Patients with Syndrome of Cerebrospinal Fluid Hypovolemia

BACKGROUND: Therapy using a targeted epidural blood patch (EBP) has been linked to cerebrospinal fluid (CSF) hypovolemia syndrome empirically, when conservative therapies failed to relieve headaches. The aim of this study is to investigate the effectiveness of EBP, for those who are refractory to conservative therapy, as a treatment for syndrome of CSF hypovolemia. METHODS: Sixteen patients were studied, where six patients were treated with conservative therapy and 10 patients were treated with EBP. After the treatments, we evaluated the difference in the duration of headaches between the two groups. With the patients treated with EBP, we also evaluated the differences in the duration of headaches between those with or without pachymeningeal enhancement on a brain MRI, and those with or without CSF leakage site on RI cisternography. RESULTS: Mean duration (1.30 +/- 0.48 days) of headaches in those treated with EBP was significantly shorter than in those without EBP (p=0.022). There was no significant difference in duration of headaches between those with and without pachymeningeal enhancement in a brain MRI. There was also no significant difference in duration of headache between those with and without CSF leakage in RI cisternography. CONCLUSIONS: The EBP is an effective therapy for syndrome of CSF hypovolemia regardless of pachymeningeal enhancement in a brain MRI and CSF leakage in RI cisternography when conservative therapies fail to relieve headaches.

Leaked CSF on Spine MRI in a Patient with Syndrome of CSF Hypovolemia

A 34-year-old woman was admitted because of severe orthostatic headache. On CSF examination, the opening pressure was below 30 mmH2O. A spine MRI showed a large collection of extradural fluid indicating that CSF leaked into the extradural space. The headache was resolved by an epidural blood patch. We present a patient with a syndrome of CSF hypovolemia, whose spine MRI showed a large amount of leaked CSF in the epidural space of the spine.

Orthostatic Headache Caused by Cerebrospinal Fluid Leakage with Normal Cerebrospinal Pressure

Cerebrospinal fluid(CSF) leakage is known to cause orthostatic headaches. It shows diffuse pachymeningeal gadolinium enhancement on magnetic resonance(MR) imaging, typically associated with nonmeasurable or very low CSF pressures. The authors report a patient with orthostatic headache and a documented CSF leakage with normal CSF pressure. A previously healthy 37-year-old woman complained of sudden severe occipitonuchal pain with a five-day history. Brain MR images showed a hernia of the cerebellar tonsil, absence of subdural fluid collection, and no enhancement of pachymeninges after gadolinium injection. Lumbar puncture in lateral decubitus revealed an opening pressure of 140mm H2O. Radioisotopic cisternography revealed accumulation of the tracer in the lumbar region, suggestive of CSF leakage. Complete relief of the headache was obtained after applying two epidural blood patches. In the presence of convincing clinical features and imaging abnormalities, a normal CSF pressure should not discourage the clinician from searching for a source of CSF leakage.