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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.
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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.
Sujet(s)
Humains , Adolescent , Adulte , Adulte d'âge moyen , Sujet âgéRÉSUMÉ
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.
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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.
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Objective:To explore the clinical features of patients with syndrome of intracranial hypotension (SIH) complicated by bilateral chronic subdural hematoma (CSDH).Methods:A case-control study was conducted; 16 patients with SIH complicated with bilateral CSDH (SIH group) and 32 patients with bilateral CSDH (non-SIH group) admitted to Department of Neurology and Neurosurgery, Shanghai Tenth People's Hospital Affiliated to Tongji University from January 2016 to October 2020 were selected. The differences of demographic characteristics, initial symptoms, medical history and CT image features between the two groups were compared.Results:(1) In 16 patients from the SIH group, 13 (81.3%) complained of typical postural headache symptoms, 3 (18.6%) showed fake subarachnoid hemorrhage on CT, 80.0% (12/15) showed dural diffuse enhancement on MRI, and 33.3% (5/15) showed signs of brain droop. Spinal MRI showed 27.3% patients (3/11) had signs of intracranial cerebrospinal fluid leakage. Of the 10 patients underwent bilateral trepanation and drainage, 6 experienced postoperative deterioration (4 received multiple additional surgeries including decompressive craniectomy, and 1 severe patient died in hospital after giving up treatment due to malignant tumor). (2) SIH group had significantly younger age, and significantly lower percentages of patients with limb weakness symptoms, hypertension, head trauma histories and increased hematoma pressure during trepanation and drainage, significantly lower age-adjusted comorbidities index, significantly decreased total and differential thickness of bilateral hematoma on CT, significantly shorter disease course, and statistically higher proportion of patients with postural headache and hematoma uniform density than non-SIH group ( P<0.05). Conclusion:According to age, initial symptoms and CT features, bilateral CSDH patients caused by SIH can be identified to a certain extent, and cranial and spinal MRI is recommended for definitive diagnosis of SIH.
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Objective:To investigate the clinical characteristics and therapeutic efficacy of spontaneous intracranial hypotension (SIH) complicated with cerebral venous thrombosis (CVT).Methods:The clinical data of 4 patients with SIH complicated with CVT admitted to our hospital from March 2014 to April 2020 were retrospectively analyzed. And the clinical data of 35 patients with SIH complicated with CVT were included for summary analysis through literature retrieval (the databases included PubMed, CNKI and Wanfang; retrieval period was from database construction to December 31, 2020).Results:These 4 patients were with onset of orthostatic headache; one was with recurred orthostatic headache after relief, and the other 3 developed persistent headache and epileptic seizure; case 1 was with superior sagittal sinus and cortical vein thrombosis, case 3 was with superior sagittal sinus thrombosis, and other 2 patients were with isolated cortical vein thrombosis. Twenty-six documented cases demonstrated headache changes: 12 patients (46.15%) developed persistent headache, 12 patients (46.15%) showed orthostatic headache persistently, and 2 patients (7.69%) had disappeared headache. The most common new symptoms were epilepsy in 17 patients (48.57%) and limb weakness in 10 patients (28.57%). Totally, these 31 patients (4 patients from our hospital+27 patients from literature retrieval) had hemorrhage after treatment; the percentage of patients having hemorrhage changes in the 17 patients accepted anticoagulant therapy was significantly increased as compared with that in 14 patients accepted other treatments (7/17 vs. 1/14, P<0.05); there were no bleeding changes in 5 patients accepted epidural blood patch and anticoagulant therapy. Conclusions:The clinical features of SIH complicated with CVT are various, and the change of headache is not a reliable marker. In the course of SIH, it is necessary to be alert to the occurrence of CVT if there are new symptoms such as epileptic attack or limb weakness. The etiological treatment of SIH is essential and the hemorrhage risk after anticoagulant therapy should be concerned in patients with SIH complicated with CVT.
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@#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.
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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.
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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.
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Humains , Hypotension intracrânienne , Imagerie par résonance magnétique , Facteurs de risque , Thrombose , Veines , Thrombose veineuseRÉSUMÉ
Objective To explore the diagnoses of spontaneous intracranial hypotension,and discuss the therapeutic efficacy of epidural blood patch therapy in spontaneous intracranial hypotension patients.Methods The clinical data of 12 patients with spontaneous intracranial hypotension,admitted to our hospital from January 2013 to December 2018,were retrospectively analyzed.The lumbar puncture results,MR imaging features of the skull and spine,and CT myelography (CTM) features of these patients were analyzed.The treatment efficacies of epidural blood patch,which included blind epidural blood patch and targeted epidural blood patch,were compared.Results The cerebrospinal fluid pressure of 12 patients was ≤ 60 mmH2O.Ten patients (83.3%) showed subdural fluid collections,enhancement of the pachymeninges,engorgement of venous structures,pituitary hyperemia,and sagging of the brain on brain MR imaging,and one of the patient showed pituitary hemorrhage.Seven patients (63.3%) showed spinal dural epithelial fluid accumulation and venous plexus expansion on spine MR imaging,and one of the patient showed dorsolateral dural discontinuous thickening of T6 and forward movement of the spinal cord caused by massive dorsal epidural effusion.Twelve patients in this group underwent CTM,and were found cerebrospinal fluid leakage.Twelve patients applied 14-times epidural blood tests;4 responded well to one-time targeted epidural blood patch therapy,with success rate of 100%;8 patients used blind epidural blood patch therapy,and 6 patients responded well to one-time therapy,with success rate of 75%,one patient improved with blind epidural blood stick twice,and one patient was ineffective twice.Conclusions Head MR imaging combined with spinal MR imaging is a non-invasive method to diagnose spontaneous intracranial hypotension.Myelogram can determine whether there is a leakage of spinal cerebrospinal fluid and accurately locate the leakage site.Epidural blood patch therapy is an effective method for treatment of patients with spontaneous intracranial hypotension.With the precise location of leak points by myelography,targeted epidural blood patch is more effective.
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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.
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Femelle , Humains , Colmatage sanguin épidural , Consensus , Céphalée , Hypotension intracrânienne , Imagerie par résonance magnétique , Thrombose veineuseRÉSUMÉ
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.
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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.
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Adulte , Femelle , Humains , Atteintes du nerf abducens , Alitement , Colmatage sanguin épidural , Diplopie , Céphalée , Ouïe , Hypotension intracrânienne , Nausée , Vertige , Troubles de la vision , VomissementRÉSUMÉ
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.
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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.
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Humains , Colmatage sanguin épidural , Encéphale , Céphalée , Hématome subdural , Hypotension intracrânienne , Imagerie par résonance magnétique , Platybasie , Pronostic , Récidive , Études rétrospectives , Base du crâne , RachisRÉSUMÉ
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.
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Colmatage sanguin épidural , Conscience , Atteintes des nerfs crâniens , Hématome subdural , Hypotension intracrânienne , Nerf oculomoteur , Atteintes du nerf moteur oculaire communRÉSUMÉ
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.
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Nerf abducens , Atteintes du nerf abducens , Colmatage sanguin épidural , Céphalée , Hypotension intracrânienne , Paralysie , Céphalée post-ponction durale , Décubitus dorsalRÉSUMÉ
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.
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Humains , Diagnostic différentiel , Erreurs de diagnostic , Tests diagnostiques courants , Urgences , Céphalée , Hypotension intracrânienne , Dossiers médicaux , Études rétrospectivesRÉSUMÉ
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.
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Humains , Colmatage sanguin épidural , Hématome subdural , Hypotension intracrânienne , Méningite , Manifestations neurologiques , RachisRÉSUMÉ
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.