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.

Headache attributed to spontaneous intracranial hypotension and associated possible causes / Cefaleia atribuída à hipotensão intracraniana espontânea e possíveis causas associadas

Bem Junior and coworkers published a very interesting and opportunely case report on spontaneous intracranial hypotension, associated with cerebrospinal fluid (CSF) fistula in this issue. In recent decades, many publications have been addressing the subject. Spontaneous intracranial hypotension is little known among physicians, and the diagnosis is difficult even considering neurologists. Many patients progress without the correct diagnosis for weeks or even months. The clinical expression is classically similar to that found in post-dural puncture headache syndrome, an entity prevalent after spinal anesthesia. The most frequent symptom presentation is orthostatic headache, which worsens in the upright position and subsides after lying down.

Bem Junior e colaboradores publicaram nesta edição um relato de caso muito interessante e oportuno sobre hipotensão intracraniana espontânea, associada à fístula do líquido cefalorraquidiano (LCR). Nas últimas décadas, muitas publicações têm abordado o assunto. A hipotensão intracraniana espontânea é pouco conhecida entre os médicos e o diagnóstico é difícil mesmo entre neurologistas. Muitos pacientes evoluem sem o diagnóstico correto por semanas ou até meses. A expressão clínica é classicamente semelhante à encontrada na síndrome da cefaleia pós-punção dural, entidade prevalente após raquianestesia. O sintoma mais frequente é a cefaleia ortostática, que piora na posição ortostática e cede após deitar.

Spontaneous intracranial hypotension associated with posterior reversible encephalopathy syndrome

Spontaneous intracranial hypotension (SIH) is usually due to cerebrospinal fluid (CSF) leak, resulting in loss of CSF volume. Posterior reversible encephalopathy syndrome (PRES) is the inability of the posterior circulation of the brain to autoregulate in response to significant variation in blood pressure.Altered perfusion with associated blood-brain barrier disruption may lead to vasogenic edema, usually without infarction, commonly in the parieto-occipital regions. Magnetic resonance (MR) imaging of the brain and spine and cerebral MR venography are essential tools for diagnosing clinically suspected SIH and PRES. We present 18-year-young gentleman with clinical and radiological signs of SIH, which later evolved toward PRES.

Clinical efficacy of CT-guided epidural autogenous blood patch in treatment of spontaneous refractory intracranial hypotension headache / 中国介入影像与治疗学

Objective: To observe the efficacy and safety of CT-guided epidural blood patch (EBP) in treatment of spontaneous refractory intracranial hypotension headache. Methods: Clinical and imaging data of 12 patients with spontaneous intracranial hypotension headache treated with CT-guided EBP were retrospectively analyzed, and visual analogue scale (VAS) and complications were calculated before and after procedures. Results: A total of 12 patients received 19 CT-guided EBP therapy, among which 7 patients received secondary EBP therapy. Five patients had postoperatively localized neck pain unrelated to body position, and then healed spontaneously within 1 week. No serious complication occurred. VAS of all patients at each time point after operation was lower than that before operation (all P<0.01), and the clinical symptoms relieved or disappeared after operation. No recurrence was detected during 3 months' follow-up. Conclusion: CT-guided EBP is effective and safe in treatment of spontaneous refractory intracranial hypotension headache.

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.

Spontaneous Intracranial Hypotension Complicated by Cerebral Venous Thrombosis Relieved by Epidural Blood Patch

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse dural thickening, and enhancement in magnetic resonance imaging. Cerebral venous thrombosis (CVT) has been reported to be a rare complication of SIH. There is no consensus in anticoagulation treatment of CVT secondarily caused by SIH. We report a female patient with SIH complicated by CVT and spontaneously regressed CVT not by anticoagulation but by epidural blood patch.

Susceptibility-Weighted MR Imaging for the Detection of Isolated Cortical Vein Thrombosis in a Patient with Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) can be a rare risk factor of cerebral venous thrombosis. We describe a case of isolated cortical vein thrombosis (CVT) secondary to SIH and discuss the value of susceptibility-weighted imaging for the detection of isolated CVT.

Epidural blood patch treatment of diplopia that developed after headache resolution in a patient with spontaneous intracranial hypotension

Sudden headache onset may rarely be caused by spontaneous intracranial hypotension (SIH). Other associated symptoms in patients with SIH are nausea, vomiting, vertigo, hearing alteration, and visual disturbance. This case report describes a 43-year-old female diagnosed with SIH who developed diplopia after resolution of an abrupt-onset headache, which was managed with conservative treatments, including bed rest and hydration. She was also diagnosed with secondary right sixth cranial nerve palsy. Although conservative management relieved her headache, the diplopia was not fully relieved. Application of an autologous epidural blood patch successfully relieved her diplopia, even after 14 days from the onset of visual impairment.

Prognostic factors in 26 patients with spontaneous intracranial hypotension / 第二军医大学学报

Objective To explore the clinical characteristics and prognostic factors of spontaneous intracranial hypotension (SIH) patients. Methods The clinical data of patients with SIH, who admitted to Changhai Hospital of Navy Medical University (Second Military Medical University) from 2010 to 2017 and met the SIH diagnostic criteria of international classification of headache disorders, 3rd edition (ICHD-3), were collected. The effects of the general clinical features, such as gender, age and course of disease, and cerebrospinal fluid pressure and imaging features on prognosis of the SIH patients were analyzed. Results Of 26 SIH patients, 19 patients (73.08%) were cured and 7 patients (26.92%) were relieved. The gender, age, course of disease, severity of headache, headache types and cerebrospinal fluid pressure had no significant effect on the prognosis of the SIH patients. Among the 13 SIH patients with abnormal cranial magnetic resonance imaging findings, such as strenthening signals in cerebral dura, subdural fluid accumulation and brain sagging, 12 patients (63.16%) were cured and 1 (14.28%) was relieved, and the difference was significant (P=0.027). Conclusion SIH patients with headache have a good prognosis; imaging examination contributes to the diagnosis of SIH and may indicate the prognosis.

Clinical analysis of 36 cases with spontaneous intracranial hypotension headache / 中国医师进修杂志

Objective To explore the clinical features of spontaneous intracranial hypotension (SIH) headache. Methods Thirty-six cases with SIH headache who were admitted between August 2009 and September 2014 were retrospectively analyzed. Results Twenty-five (69.4%) of 36 cases were female, and 11 cases (30.6%) were male. The age of onset was (39.6 ± 11.9) years. All the cases had headache, among whom 91.7% (33/36) had typical postural headache. The common concomitant symptoms were nausea, vomiting and neck stiffness. The average cerebrospinal fluid (CSF) pressure was (45.3 ± 28.6) mmH2O (1 mmH2O=0.009 8 kPa) which in male was (67.4 ± 15.4) mmH2O , and in female was (37.3 ± 28.2) mmH2O, and there was significant difference (P<0.05). The content of CSF protein was (545.6 ± 377.1) mg/L and the level of IgG was (57.4 ± 41.1) mg/L. Seventeen cases showed abnormality on MRI scan, with diffuse pachymeningeal enhancement, subdural hematoma, hyperemia and swelling pituitary. Eleven cases (52.4%, 11/21) showed abnormal on EEG examination, with theta waves and epiletiform waves in bilateral cerebral hemisphere. All cases were cured after conservative treatment, 3 cases relapsed within 1 month and was cured again. Conclusions Typical postural headache, cerebrospinal fluid pressure less than 60 mmH2O, higher levels of CSF protein and IgG, diffuse pachymeningeal enhancement and hyperemia and swelling pituitary on MRI scan are the main features in SIH headache, and this disease has favorable prognosis.

Relationship Between Findings on Brain MRI and Prognosis in Patients With Spontaneous Intracranial Hypotension

BACKGROUND: The outcome of spontaneous intracranial hypotension (SIH) is unpredictable and some patients have persistent and often incapacitating symptoms. This study was aimed to investigate whether abnormalities on initial magnetic resonance imaging (MRI) can predict the outcome in patients with SIH. METHODS: We retrospectively included 44 patients with SIH. Brain MRI was available for all patients. Treatment consisted of conservative treatment and/or high-volume epidural blood patching. Patients were divided into two groups: favorable or non-favorable group. Favorable group was defined as clinical improvement by conservative therapy or one trial of autologous epidural blood patching; non-favorable group as more than two week of admission, two or more trials of autologous epidural blood patching, or relapse of orthostatic headache. RESULTS: Twenty-one (48%) of 44 patients were classified as the favorable group. The non-favorable group had several abnormal findings on brain MRI (16 cases vs. 5 cases in favorable group, p<0.003), including platybasia (1), skull base tumor (1), Chiari I malformation (1), diffuse mild thickening and enhancement of dural and epidural layer of thoracic spine (1), pituitary enlargement (3), sagging brain (3) and subdural hemorrhage (4). In the non-favorable group, 13 out of 23 patients (57%) showed pachymeningeal enhancement in brain MRI (2 patients in favorable group, p<0.001). CONCLUSIONS: Brain MRI abnormalities were more frequently related with non-favorable outcomes in SIH. Pachymeningeal enhancement in particular could suggest an unfortunate prognosis.

Epidural Blood Patch for the Treatment of Abducens Nerve Palsy due to Spontaneous Intracranial Hypotension: A Case Report

Intracranial hypotension is characterized by a postural headache which is relieved in a supine position and worsened in a sitting or standing position. Although less commonly reported than postural headache, sixth nerve palsy has also been observed in intracranial hypotension. The epidural blood patch (EBP) has been performed for postdural puncture headache, but little is known about the proper timing of EBP in the treatment of sixth nerve palsy due to intracranial hypotension. This article reports a case of sixth nerve palsy due to spontaneous intracranial hypotension which was treated by EBP 10 days after the onset of palsy.

Delayed-Onset Subdural Hematoma and Oculomotor Nerve Palsy After Improving Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is a well-known disorder improving with conservative treatment or epidural blood patch in the majority of cases. However, SIH may develop neurological complications such as cranial nerve palsy, subdural hematoma, and altered consciousness. Subdural hematoma in SIH is usually found during intracranial hypotension state and delayed subdural hematoma is rarely reported. We report a case of delayed subdural hematoma and oculomotor nerve palsy after improving spontaneous intracranial hypotension.

Analysis of Clinical Characteristics and Correct Diagnosis Rate Associated with Spontaneous Intracranial Hypotension in the Emergency Department

PURPOSE: Spontaneous Intracranial Hypotension (SIH) is rare condition and may accompany other clinical symptoms which inhibit accurate diagnosis in an emergency department (ER). Only a few studies have reported the clinical characteristics and root causes associated with SIH. This study evaluates the rate of accuracy of SIH diagnosis and compares clinical characteristics and diagnostic test results for correct versus incorrect diagnosis groups. METHODS: Medical records of SIH patients admitted to an emergency department (ER) over a twelve year period were retrospectively reviewed. Patients were grouped as having received correct or incorrect diagnosis, and their clinical characteristics and diagnostic test results were compared. In the incorrect diagnosis group, the number of times they were misdiagnosed, and the specialties of the clinic (s) they visited prior to arrival at this ER were reviewed. RESULTS: Adhering to the inclusion criteria of our study, 72 patients were enrolled with 54 patients in the correct diagnosis group and 18 patients in the incorrect diagnosis group. Of the twenty one cases in the incorrect diagnosis group, the majority 7 cases (33.3%) had been examined by an emergency physician. Among the clinical symptoms observed, there was significant variability in the location of the headache (p=0.020) and time interval between symptom onset and diagnosis (p=0.035). CONCLUSION: There were no differences in most of the clinical observations and diagnostic test results between the correct and incorrect diagnosis groups. To improve the correct diagnosis rate, it is suggested to have 'SIH' included as a differential diagnosis when encountering patients reporting headache in the emergency department. Emergency physicians should be required to recognize clinical SIH characteristics such as orthostatic headache.

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.

A Case of Spontaneous Intracranial Hypotension with Subdural Hematoma Mimicking Meningitis / 대한뇌혈관외과학회지

Spontaneous intracranial hypotension (SIH) results from spontaneous cerebral spinal fluid leakage, of unknown etiology, at the level of the spine. Physicians' unfamiliarity with SIH and a its varied clinical and radiographic manifestations may contribute to a delayed or erroneous diagnosis. We report an SIH patient whose subdural hematoma (SDH) clinically mimicked meningitis, but who recovered 1 week later, without any neurologic deficit, after an epidural blood patch (EBP) treatment.

Clinical analysis of 13 cases of spontaneous intracranial hypotension syndrome / 中国综合临床

Objective To study the clinical and imaging characteristics as well as cerebrospinal fluid chan-gea(CSF) of spontaneous intracranial hypotensian syndrome (SIHS).Methods The clinical characteristics, CSF and imaging data of 13 patients diagnosed as SIHS were retrospectively analyzed.Results All the 13 patients had orthostatic headache accompaning one or more numerous symptoms including nausea, vomiting, dizziness, diplopia and neck stiffness.All the patients had low CSF pressure,which was below 60 mm H2O and high CSF protein was in 5 patients, 8 had increased white cell counts and 9 had increased red cells counts;CT was performed in all patients.On CT scan the subdural effusion or small ventricles were compressed in 4 patients.MRI typically revealed diffused pachymeningeal enhancement in 2 patients;All the patients experienced relief of symptoms through conventional treatment.Conclusion Orthostatic headache is the most typical symptom in spontaneous intracranial hypotension syndrome and diffused pachymeuingeal enhancement is the most common imaging manifestation, and CSF hypovol-emia is the basis of pathophysiology of spontaneous intracranial hypotension syndrome.

Spontaneous Intracranial Hypotension Complicated by Cortical Vein Thrombosis

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse dural thickening, and enhancement in MRI associated with low cerebrospinal fluid pressure. While SIH causes several complications such as subdural hematoma, cranial nerve palsies, and altered consciousness, cerebral venous thrombosis caused by SIH has been rarely described. We report a patient presenting with generalized tonic-clonic seizure that was probably caused by cortical vein thrombosis that had developed after SIH.

Treatment of spontaneous intracranial hypotension with multiple leakage sites of cerebrospinal fluid : A case report

Spontaneous intracranial hypotension (SIH) is an uncommon disease that's caused by cerebrospinal fluid (CSF) leakage and this disease is considered to be an important cause of persistent headache. It is characterized by a postural headache in patients who are without any history of dural puncture or trauma. Conservative management, including bed rest analgesics and intravenous fluid administration, are the first-line treatment of SIH, and an autologous epidural blood patch (EBP) is generally indicated for those who fail the conservative management. We report here on a case of SIH with confirmed CSF leakage at the cervical, thoracic and lumbar levels, and this was successfully managed with a single autologous EBP at the lumbar level. We believe that an EBP at the leakage site with the highest pressure level should be considered as a primary treatment for SIH with multiple sites of leakage.

Spontaneous Intracranial Hypotension Treated with CT-guided Cervical Epidural Blood Patch : A case report / 대한마취과학회지

Spontaneous intracranial hypotension due to spinal cerebrospinal fluid leakage is uncommon, relatively benign and usually self-limiting. However it is being increasingly recognized as a cause of postural headaches. The treatment options range from conservative supportive measures to an pidural blood patch. We report a 40-year-old woman who developed intracranial hypotention without any preceding events such as lumbar puncture, back trauma, surgical procedures or medical illnesses. The site of the cerebrospinal fluid leakage was identified at between the C1 to C2 level using computerized tomographic myelography. Consequently, the patient underwent a CT-guided autologous epidural blood patch at the C3-C4 level. Her symptoms were relieved immediately without recurrence.