Pituitary pseudo-adenoma from cerebrospinal fluid leak in patient with Marfan syndrome.

Intracranial hypotension may result in pituitary gland enlargement due to compensatory hyperaemia and venous engorgement. Spontaneous intracranial hypotension (SIH) is frequently associated with connective tissue disorders predisposing patients to dural weakening including dural ectasia and meningeal diverticula. Symptoms of SIH typically include postural headache, dizziness and tinnitus. We present a case of a female in her 20s with Marfan syndrome and a history of pituitary adenoma, who reported intractable postural headaches. Hormonal workup revealed no abnormalities, whereas brain MRI showed sequelae of intracranial hypotension. Further MRI studies revealed thoracic and lumbar meningeal diverticula with significant dural sac ectasia at the L4-S2 level. Myelogram confirmed numerous lumbar spine diverticula with cerebrospinal fluid leak at the L5 and S1 right nerve roots. The patient underwent blood patch administrations at the level of the leak with improvement of symptoms.

Spontaneous intracranial hypotension in a patient without corpus callosum: A case report.

RATIONALE: Spontaneous intracranial hypotension (SIH) is a well-established condition typically presenting with disabling orthostatic headache. Corpus callosum agenesis (CCA) is one of the most common human brain malformations with a wide spectrum of associated malformations, chromosomal abnormalities, and clinical syndromes. PATIENT CONCERNS: A 53-year-old woman presented with recurrent orthostatic headache for about 1 month. The head computed tomography examination of the patient showed CCA and the initial pressure of subsequent lumbar puncture was only 5 centimeters cerebrospinal fluid. Magnetic resonance imaging examination confirmed CCA with gray matter heterotopia. DIAGNOSIS: The final diagnose was SIH related headache with CCA. INTERVENTION: The patient's symptom improved after oral hydration, intravenous fluids, and bed rest. OUTCOME: Favorable outcome was observed. LESSONS: Although this co-occurrence of SIH and CCA is probably coincidental, this finding suggests that the developmental malformations of the brain may lead to structural changes in brain tissue or disturbances in cerebrospinal fluid production and reflux, resulting in pathological changes over time.

Diagnostic and therapeutic insights in individuals with persistent post-dural puncture headache: A cross-sectional study.

BACKGROUND: Post-dural puncture headache (PDPH) is a frequent complication following lumbar puncture, epidural analgesia, or neuraxial anesthesia. The International Classification of Headache Disorders, third edition categorizes PDPH as a self-limiting condition; however, emerging evidence, including our findings, suggests that PDPH can have a prolonged course, challenging this traditional view. OBJECTIVES: To elucidate the diagnostic characteristics and treatment outcomes of persistent PDPH (pPDPH), offering insights into its demographic profiles and diagnostic features. METHODS: We executed an anonymous, web-based survey targeting individuals aged ≥18 years diagnosed with or suspected of having pPDPH. Recruitment occurred through self-help groups on Facebook. The survey comprised questions regarding diagnostic procedures, treatment regimens, outcomes, and healthcare consultation. RESULTS: The survey achieved a response rate of 179/347 (51.6%) individuals completing the questionnaire. Cerebrospinal fluid (CSF) leaks were confirmed in nine of 179 (5.0%) cases. Signs of intracranial hypotension without a CSF leak were observed in 70/179 (39.1%) individuals. All participants underwent magnetic resonance imaging scans of the brain and spine, with computed tomography myelography performed in 113/179 (63.1%) cases. Medications, including analgesics, theophylline, and gabapentin, provided minimal short-term relief. Epidural blood patch treatments resulted in slight-to-moderate short-term improvement in 136/179 (76.0%), significant improvement in 22/179 (12.3%), and complete effectiveness in eight of 179 (4.5%) individuals. For long-term outcomes, slight-to-moderate improvement was reported by 118/179 (66.0%) individuals. Surgical interventions were carried out in 42/179 (23.5%) patients, revealing pseudomeningoceles intraoperatively in 20/42 (47.6%) individuals. After surgery, 21/42 (50.0%) of the participants experienced slight-to-moderate improvement, 12/42 (28.6%) showed more pronounced improvement, and five of the 42 (11.9%) achieved complete effectiveness. CONCLUSION: This study underscores the complexities of managing pPDPH. The delay in diagnosis can impact the effectiveness of treatments, including epidural blood patch and surgical interventions, resulting in ongoing symptoms. This underscores the importance of tailored and adaptable treatment strategies. The findings advocate for additional research to deepen the understanding of pPDPH and improve long-term patient outcomes.

Cerebrospinal Fluid-Venous Fistulas.

Cerebrospinal fluid-venous fistulas (CSFVFs) were first described in 2014 and have since become an increasingly diagnosed cause of spontaneous intracranial hypotension due to increased clinical recognition and advancements in diagnostic modalities. In this review, the authors discuss CSFVF epidemiology, the variety of clinical presentations, the authors' preferred diagnostic approach, recent advancements in diagnostic methods, treatment options, current challenges, and directions of future research.

Spontaneous Intracranial Hypotension: Clinical Presentation, Diagnosis, and Treatment Strategies.

Spontaneous intracranial hypotension (SIH) typically presents as an acute orthostatic headache during an upright position, secondary to spinal cerebrospinal fluid leaks. New evidence indicates that a lumbar puncture may not be essential for diagnosing every patient with SIH. Spinal neuroimaging protocols used for diagnosing and localizing spinal cerebrospinal fluid leaks include brain/spinal MRI, computed tomography myelography, digital subtraction myelography, and radionuclide cisternography. Complications of SIH include subdural hematoma, cerebral venous thrombosis, and superficial siderosis. Treatment options encompass conservative management, epidural blood patches, and surgical interventions. The early application of epidural blood patches in all patients with SIH is suggested.

Rebound Intracranial Hypertension.

PURPOSE OF REVIEW: Rebound intracranial hypertension (RIH) is a post-procedural treatment complication in patients with spontaneous intracranial hypotension (SIH) characterized by transient high-pressure headache symptoms. This article reviews the epidemiology, clinical features, risk factors, and treatment options for RIH. RECENT FINDINGS: This article discusses how changes in underlying venous pressure and craniospinal elastance can explain symptoms of RIH, idiopathic intracranial hypertension (IIH), and SIH. The pathophysiology of RIH provides a clue for how high and low intracranial pressure disorders, such as IIH and SIH, are connected on a shared spectrum.

The cervical blood patch: A therapeutic "miraculous" for cerebrospinal fluid leaks: A case report.

RATIONALE: Cerebrospinal fluid (CSF) leaks, arising from abnormal openings in the protective layers surrounding the spinal cord and brain, are a significant medical concern. These leaks, triggered by various factors including trauma, medical interventions, or spontaneous rupture, lead to the draining of CSF-an essential fluid safeguarding the nervous system. A classic symptom of CSF leaks is an incapacitating headache exacerbated by sitting or standing but relieved by lying down. Spontaneous intracranial hypotension (SIH) denotes the clinical condition marked by postural headaches due to spontaneous CSF leakage and hypotension, often misdiagnosed or underdiagnosed. While orthostatic headaches are the hallmark, SIH may manifest with an array of symptoms including nausea, tinnitus, hearing loss, visual disturbances, and dizziness. Treatment options encompass conservative measures, epidural blood patches (EBP), and surgery, with EBP being the primary intervention. PATIENT CONCERN: The patient did not express any specific concerns regarding their medical diagnosis. However, they did harbor apprehensions that their condition might necessitate surgical intervention in the future. DIAGNOSIS: The patient had been treated with antibiotics with a pre-diagnosis of sinusitis and was admitted to the neurology department of our hospital when his symptoms failed to improve. Cranial magnetic resonance imaging was interpreted as thickening of the dural surfaces and increased contrast uptake, thought to be due to intracranial hypotension. Cranial MR angiography was normal. Full-spine magnetic resonance imaging revealed a micro-spur at the C2 to 3 level and the T1 to 2 level in the posterior part of the corpus. INTERVENTIONS: The cervical EBP was performed in the prone position under fluoroscopic guidance. There were no complications. OUTCOMES: The patient was invited for follow-up 1 week after the procedure, and control examination was normal. LESSONS: SIH poses a diagnostic challenge due to its diverse clinical presentation and necessitates precise imaging for effective intervention. Cervical EBP emerges as a promising treatment modality, offering relief and improved quality of life for individuals grappling with this condition. However, clinicians must carefully assess patients and discuss potential risks and benefits before opting for cervical blood patches.

Headache associated with intracranial hypotension: diagnostic challenges and difficulties in everyday neurological practice.

Low pressure of cerebrospinal fluid (CSF) is a rare cause of headache, except when the patient undergoes a lumbar puncture. Headache associated with a low CSF pressure i.e. intracranial hypotension causes diagnostic difficulties. Headaches related to spontaneous intracranial hypotension (SIH) pose a significant diagnostic challenge in everyday neurological practice. Patients with headaches due to SIH are usually diagnosed only after a long delay. Diagnostic problems may result in unnecessary invasive diagnostic procedures, or even neurosurgical operations. Diagnosing headaches attributed to SIH requires the consideration of several clinical scenarios, and the disease's features causing primary or secondary disturbances. In this review, we discuss the differential diagnosis of SIH-related headaches with reference to accumulated knowledge, including meta-analyses, guidelines, casuistry, and the applicable criteria of the International Classification of Headache Disorders. In addition, we discuss head and spine magnetic resonance imaging abnormalities, which may indicate intracranial hypotension.

Cerebrospinal Fluid Refill Test as a Novel Diagnostic Tool for Cerebrospinal Fluid Hypovolemia: Preliminary Result.

OBJECTIVE: Cerebrospinal fluid (CSF) hypovolemia presents with orthostatic headaches due to CSF leakage. However, a direct association between the lack of CSF and clinical symptoms has not been found. CSF hypovolemia can be improved by refilling CSF. Therefore, we assessed the validity of a CSF refill test. METHODS: From November 2019 to August 2021, we included 10 patients (≥18 years old) with potential CSF hypovolemia, clear orthostatic headaches, and a CSF opening pressure <10 cmH2O. In the CSF refill test, 10 mL of artificial CSF was injected intrathecally. The primary outcome was improvement in orthostatic headache assessed using a visual analog scale (VAS), while the secondary outcomes were the 10-m walk time and adverse events. When the symptoms temporarily improved after intrathecal injection, the patients underwent radiologic imaging to identify the CSF leak, and an epidural blood patch was proposed accordingly. RESULTS: All patients showed post-test improvements in the VAS score (median [interquartile range], pretest 63.0 [50.3-74.3] vs. post-test 1.5 [0.0-26.0]). The 10-m walk time also significantly improved (9.5 [8.5-10.2] s vs. 8.2 [7.9-8.7] s). One patient experienced temporary right leg numbness associated with a lumbar puncture. After radiologic investigation, 9 patients underwent epidural blood patches, of which 6 were completely cured, and 3 revealed partial improvement. CONCLUSIONS: The cerebrospinal fluid (CSF) refill test was safe and effective in demonstrating the direct association between the lack of CSF and clinical symptoms and may help predict the outcome of an epidural blood patch.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. METHODS: A retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. RESULTS: Among the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. CONCLUSIONS: This procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.

CSF-Venous Fistula of the Clival Skull Base: A Unique Case Study and Literature Review.

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024.

Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

BACKGROUND AND PURPOSE: Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. METHODS: This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. RESULTS: Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8-12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. CONCLUSIONS: Long-standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time-dependent manner.

Patient-reported symptomatology and its course in spontaneous intracranial hypotension - Beware of a chameleon.

OBJECTIVE: Although orthostatic headache is the hallmark symptom of spontaneous intracranial hypotension (SIH), patients can present with a wide range of different complaints and thereby pose a diagnostic challenge for clinicians. Our aim was to describe and group the different symptoms associated with SIH and their course over time. METHODS: We retrospectively surveyed consecutive patients diagnosed and treated for SIH at our institution from January 2013 to May 2020 with a specifically designed questionnaire to find out about their symptomatology and its course. RESULTS: Of 112 eligible patients, 79 (70.5%) returned the questionnaire and were included in the analysis. Of those, 67 (84.8%) reported initial orthostatic headaches, whereas 12 (15.2%) denied having this initial symptom. All except one (98.7%) patients reported additional symptoms: most frequently cephalic pressure (69.6%), neck pain (68.4%), auditory disturbances (59.5%), nausea (57%), visual disturbances (40.5%), gait disturbance (20.3%), confusion (10.1%) or sensorimotor deficits (21.5%). Fifty-seven (72.2%) patients reported a development of the initial symptoms predominantly in the first three months after symptom onset. Age and sex were not associated with the symptomatology or its course (p > 0.1). CONCLUSION: Although characteristic of SIH, a relevant amount of patients present without orthostatic headaches. In addition, SIH can manifest with non-orthostatic headaches at disease onset or during the course of the disease. Most patients report a wide range of associated complaints. A high degree of suspicion is crucial for an early diagnosis and targeted treatment.

Spontaneous intracranial hypotension.

Spontaneous intracranial hypotension (SIH) is a highly disabling but treatable secondary cause of headache. Recent progress in neuroradiological techniques has catalysed understanding of its pathophysiological basis and clinical diagnosis, and facilitated the development of more effective investigation and treatment methods. A UK-based specialist interest group recently produced the first multidisciplinary consensus guideline for the diagnosis and treatment of SIH. Here, we summarise a practical approach to its clinical and radiological diagnosis, symptomatic and non-targeted interventional treatment, radiological identification of leak site and targeted treatment of the leak once it has been localised.

Temporal Characteristics of CSF-Venous Fistulas on Dynamic Decubitus CT Myelography: A Retrospective Multi-Institution Cohort Study.

BACKGROUND AND PURPOSE: CSF-venous fistula can be diagnosed with dynamic decubitus CT myelography. This study aimed to analyze the temporal characteristics of CSF-venous fistula visualization on multiphase decubitus CT myelography. MATERIALS AND METHODS: A retrospective, multisite study was conducted on patients diagnosed with CSF-venous fistula at 2 institutions between June 2017 and February 2023. Both institutions perform decubitus CT myelography with imaging immediately following injection and usually with at least 1 delayed scan. The conspicuity of CSF-venous fistula was assessed on each phase of imaging. RESULTS: Forty-eight patients with CSF-venous fistula were analyzed. CSF-venous fistulas were better visualized on the early pass in 25/48 cases (52.1%), the delayed pass in 6/48 cases (12.5%) and were seen equally on both passes in 15/48 cases (31.3%). Of 25 cases in which the CSF-venous fistula was better visualized on the early pass, 21/25 (84%) fistulas were still at least partially visible on a delayed pass. Of 6 cases in which the CSF-venous fistula was better visualized on a delayed pass, 4/6 (67%) were partially visible on the earlier pass. Six of 48 (12.5%) CSF-venous fistulas were visible only on a single pass. Of these, 4/6 (66.7%) were seen only on the first pass, and 2/6 (33.3%) were seen only on a delayed pass. One fistula was found with one pass only, and one fistula was discovered upon contralateral decubitus imaging without a dedicated second injection. CONCLUSIONS: A dynamic decubitus CT myelography imaging protocol that includes an early and delayed phase, likely increases the sensitivity for CSF-venous fistula detection. Further studies are needed to ascertain the optimal timing and technique for CSF-venous fistula visualization on dynamic decubitus CT myelography and its impact on patient outcomes.

Spontaneous intracranial hypotension: a case series and literature review.

Spontaneous intracranial hypotension (SIH) is a condition of negative intracranial pressure resulting from cerebrospinal fluid (CSF) leakage from the dural sac and is a well-known cause of orthostatic headache. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines. Low CSF pressure and diffuse meningeal enhancement on brain MRI are the major instrumental features of the classic syndrome. Neuroimaging plays a key role in diagnosing SIH, particularly in atypical clinical presentations, by recognizing the specific findings of brain sagging on MRI and detecting the level of CSF leak on spinal imaging, thus guiding therapy accordingly. Since SIH could present with such a heterogeneous clinical picture, careful history taking and increased awareness of atypical presentations are of utmost importance. We review the existing SIH literature, illustrate management, clinical and neuroimaging findings of four consecutive patients with atypical SIH, who were recently referred to our hospital for evaluation to simplify and streamline the management of SIH.

[Spontaneous intracranial hypotension]. / ABC om Spontan intrakraniell hypotension.

Spontaneous intracranial hypotension (SIH) is a disease presenting mostly with orthostatic head and neck pain due to a spontaneous cerebrospinal fluid (CSF) leak or a CSF-venous fistula in the spinal region. It demonstrates typical MRI findings with sagging of the brain causing tension of the meninges and sometimes the cranial nerves. It shares some clinical similarities with post puncture headache but differs in its pathophysiological cause, diagnosis, and treatment. Many patients remain misdiagnosed or wait too long for the correct diagnosis. The diagnostic work-up includes an MRI of the head and spine in search of typical SIH signs. Myelography and CT scans are performed to identify the location of the CSF leak or CSF-venous fistula. Treatment options may involve (1) initial conservative treatment with bed rest, caffein and fluids, (2) interventions such as epidural blood patch, fibrin patch, and embolization, or (3) surgical closure of the leak.

Spontaneous Intracranial Hypotension in Pregnancy.

PURPOSE OF REVIEW: Spontaneous intracranial hypotension (SIH) is a debilitating disease typically featuring orthostatic headache and caused by a spinal CSF leak. This review will describe the characteristics of SIH in pregnant patients and the associated unique management and treatment considerations. RECENT FINDINGS: Herein, a novel case is reported of a 41-year-old woman who presented with SIH pre-conception but saw marked improvement of symptoms after 5 weeks antepartum and symptom recurrence 2 months post-partum. A literature review of SIH in pregnancy revealed 14 reported patients across 10 studies since 2000. All the reported cases resulted in delivery of healthy infants and symptomatic improvement with conservative management or a variety of treatment modalities including non-targeted epidural blood patch (EBP). Clinical and imaging features of SIH in pregnancy are reviewed. We hypothesize an antenatal protective mechanism against SIH symptoms through cephalad redistribution of CSF volume from the spinal to intracranial compartments related to uterine growth and decreased CSF volume within the lumbar cistern. Treatment recommendations are discussed including duration of bed rest and decision for non-targeted multi-site EBPs. When required, non-invasive diagnostic spine MRI using fat-suppressed axial T2-weighted imaging may be helpful.