Spontaneous Intracranial Hypotension Presenting Research Articles

Hiccups, Headaches, Hygromas: Severe Presentation of Spontaneous Intracranial Hypotension (P5-4.012)

Hiccups, Headaches, Hygromas: Severe Presentation of Spontaneous Intracranial Hypotension (P5-4.012)

A hidden pathophysiology of endolymphatic hydrops: case report of a patient with spontaneous intracranial hypotension presenting with sudden sensorineural hearing loss with vertigo.

Spontaneous intracranial hypotension (SIH) is characterized by decreased cerebrospinal fluid (CSF) volume due to leakage through the dural membrane. We present the case of a patient with SIH manifested by fluctuating low-frequency hearing loss, tinnitus, and vertigo. In this patient, endolymphatic hydrops in the cochlea and saccule were visualized by means of a special sequence of inner ear magnetic resonance imaging scans, with a gadolinium-based contrast agent administered intravenously. Endolymphatic hydrops is a potential underlying pathophysiology of SIH-associated hearing impairment. We hypothesize that SIH may be a rare cause of endolymphatic hydrops.

Spinal Presentation of Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH), which generally presents as orthostatic headache, is increasingly being identified due to improved imaging technologies and heightened awareness. Many prior studies have reported the characteristic brain MRI findings of SIH. However, recently, focus has shifted to spinal MRI, as SIH is believed to be caused by leakage of cerebrospinal fluid from the spinal dural sac. Advanced techniques such as ultrafast CT myelography and digital subtraction myelography have emerged as useful technique to identify the site of cerebrospinal fluid leakage. In this review, we discuss the diagnosis, spinal MRI findings, imaging techniques, and treatment of SIH.

Spontaneous Intracranial Hypotension Presenting as Progressive Gait Impairment

Orthostatic headache is a hallmark symptom of spontaneous intracranial hypotension (SIH). However, SIH can manifest without headache, which can lead to a significant delay in treatment initiation. We hereby report a 70-year-old male with progressive gait disturbance and superficial siderosis. Magnetic resonance myelography showed an extensive extradural cerebrospinal fluid collection in the cervicothoracic spine. After a series of targeted epidural blood patch, the patient’s symptoms significantly improved. SIH should be considered as a potential cause of otherwise unexplained superficial siderosis to prompt an early diagnosis and treatment.

201 Management of an unusual presentation of spontaneous intracranial hypotension (SIH)

A 49-year-old woman presented with 9 months of progressive cognitive decline and somnolence. In the last few weeks she developed right hand tremor, unsteadiness and urinary incontinence, which improved after lying flat. There was no headache. Examination findings included vertical gaze restriction, hypomimia, right-sided rest tremor, rigidity and bradykinesia, globally brisk reflexes and an unsteady gait. She scored 50/100 on the Addenbrooke’s Cognitive Examination.Brain MRI showed features consistent with intracranial hypotension with marked ‘brain sag’ but standard imaging did not identify the site of the leak. Two blind blood patches were followed by only transient symptomatic improvement. Initial Digital Subtraction Myelogram (DSM) was non-diagnostic, but repeat DSM identified a leak at C6–7 with an irregular diverticulum/CSF-venous fistula.A CT-guided fibrin patch injected at C6/7 resulted in transient improvement. Finally, a neurosurgical procedure was performed to coagulate the venous plexus adjacent to the C7 nerve root. This effected a dramatic and sustained improvement in her cognitive and motor symptoms.SIH is a treatable cause of cognitive decline and parkinsonism. DSM is a novel approach to localising CSF leaks. This is the first reported cognitive presentation of SIH where identification of a CSF leak with DSM led to successful surgical treatment.saamir.mohideen@nhs.net

Spontaneous Intracranial Hypotension Presenting with Chiari Malformation and Hydrocephalus Associated With Lumbar Pseudoarachnoiditis

Spontaneous intracranial hypotension (SIH) is a rare occurrence, especially in children. In the pediatric population, three cases of Chiari malformation in children and no cases of hydrocephalus due to SIH have been discussed in the literature. We present two patients under the age of five who presented with imaging concerning for the need for neurosurgical intervention later found to be due to SIH that resolved spontaneously.

Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension is a disorder caused by spinal CSF leakage. This article reviews the clinical presentation, diagnosis, and treatment of spontaneous intracranial hypotension. The hallmark symptom of spontaneous intracranial hypotension is acute orthostatic headache; however, clinical presentations can be heterogeneous. New evidence shows that lumbar puncture is not always necessary or sufficient to establish the diagnosis. Some patients may have normal opening pressure, which suggests that insufficiency of CSF volume (hypovolemia) rather than CSF pressure might be the underlying mechanism. Several neuroimaging modalities can aid in diagnosis and localization of the CSF leakage, including brain MRI, spinal MRI, CT myelography, digital subtraction myelography, and radionuclide cisternography. Complications, such as subdural hematoma, can lead to a change in the headache pattern and potentially life-threatening consequences. Conservative treatments, such as fluid supplementation, can provide temporary relief; however, epidural blood patches, especially targeted ones, are more effective and definitive. For patients with refractory spontaneous intracranial hypotension, surgical repair of spinal CSF leakages should be considered. Brain and spinal MRIs are important for the diagnosis and treatment of patients with spontaneous intracranial hypotension. Early treatment with epidural blood patches may be considered to shorten the disease duration and minimize the potential risk of complications.

Spontaneous Intracranial Hypotension Presenting as Postural Headache and Cranial VI Palsy: A Case Report (2915)

Spontaneous Intracranial Hypotension Presenting as Postural Headache and Cranial VI Palsy: A Case Report (2915)

Spontaneous Intracranial Hypotension: Atypical Radiologic Appearances, Imaging Mimickers, and Clinical Look-Alikes.

Spontaneous intracranial hypotension is a condition characterized by low CSF volume secondary to leakage through a dural defect with no identifiable cause. Patients classically present with orthostatic headaches, but this symptom is not specific to spontaneous intracranial hypotension, and initial misdiagnosis is common. The most prominent features of spontaneous intracranial hypotension on intracranial MR imaging include "brain sag" and diffuse pachymeningeal enhancement, but these characteristics can be seen in several other conditions. Understanding the clinical and imaging features of spontaneous intracranial hypotension and its mimickers will lead to more prompt and accurate diagnoses. Here we discuss conditions that mimic the radiologic and clinical presentation of spontaneous intracranial hypotension as well as other disorders that CSF leaks can imitate.

Spontaneous intracranial hypotension

Purpose of review Spontaneous intracranial hypotension is a disorder caused by spinal CSF leakage. This article reviews the clinical presentation, diagnosis, and treatment of spontaneous intracranial hypotension. Recent findings The hallmark symptom of spontaneous intracranial hypotension is acute orthostatic headache; however, clinical presentations can be heterogeneous. New evidence shows that lumbar puncture is not always necessary or sufficient to establish the diagnosis. Some patients may have normal opening pressure, which suggests that insufficiency of CSF volume (hypovolemia) rather than CSF pressure might be the underlying mechanism. Several neuroimaging modalities can aid in diagnosis and localization of the CSF leakage, including brain MRI, spinal MRI, CT myelography, digital subtraction myelography, and radionuclide cisternography. Complications, such as subdural hematoma, can lead to a change in the headache pattern and potentially life-threatening consequences. Conservative treatments, such as fluid supplementation, can provide temporary relief; however, epidural blood patches, especially targeted ones, are more effective and definitive. For patients with refractory spontaneous intracranial hypotension, surgical repair of spinal CSF leakages should be considered. Summary Brain and spinal MRIs are important for the diagnosis and treatment of patients with spontaneous intracranial hypotension. Early treatment with epidural blood patches may be considered to shorten the disease duration and minimize the potential risk of complications.

A Rare Presentation of Spontaneous Intracranial Hypotension

A Rare Presentation of Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension: 12 case reports and follow-up

Objective: To investigate the pathogenesis, clinical presentation, imaging features and prognosis of spontaneous intracranial hypotension. Methods: Clinical manifestation and imaging changes of 12 patients with spontaneous intracranial hypotension were reported. They were followed up regularly. The clinical and neuroimaging characteristics were summarized. Results: All the 12 cases were adults, presenting with orthostatic headache. They all recovered with conservative therapy. Head MRI demonstrated cerebral lobe hemorrhage, subdural hematoma, pituitary hyperemia, subarachnoid hemorrhage, enhancement of the pachymeninges, sagging of the brain, etc. Conclusions: Spontaneous intracranial hypotension is characterized by orthostatic headaches and is associated with compensatory changes following loss of CSF volume. The long-time prognosis is not bad.

Ataxia Without Headache: Atypical Presentation of Spontaneous Intracranial Hypotension. (P2.107)

Ataxia Without Headache: Atypical Presentation of Spontaneous Intracranial Hypotension. (P2.107)

Spontaneous intracranial hypotension presenting as thunderclap headache: a case report

BackgroundSpontaneous intracranial hypotension is a rare but treatable cause of a disabling headache syndrome. It is characterized by positional orthostatic headache, pachymeningeal enhancement and low cerebrospinal fluid pressure. However, the spectrum of clinical and radiographic manifestations is varied and misdiagnosis is common even in the modern era of magnetic resonance imaging. Spontaneous intracranial hypotension presenting as thunderclap headache is recognized but rare.Case presentationA 41-year-old Sri Lankan female presented with thunderclap headache associated with nausea and vomiting, but the headache was characterized by positional variation with aggravation in the upright posture and relief in the supine posture. Gadolinium-enhanced cranial magnetic resonance imaging demonstrated generalized meningeal enhancement and normal magnetic resonance angiography while lumbar puncture revealed a cerebrospinal fluid opening pressure of less than 30 millimetres of water. Magnetic resonance myelography failed to identify the site of cerebrospinal fluid leak. The patient was managed conservatively with bed-rest, intravenous hydration, analgesics and an increased intake of oral coffee which led to a gradual relief of headaches in the upright posture.ConclusionsSpontaneous intracranial hypotension can rarely present as thunderclap headache. Awareness of its varied spectrum of presentations would avoid inappropriate investigations, misinterpretation of imaging results and ineffective treatment.

Successful surgical treatment of intractable spontaneous intracranial hypotension due to a calcified thoracic disc prolapse

We present a patient with spontaneous intracranial hypotension due to a calcified thoracic disc prolapse. Although most cases of spontaneous intracranial hypotension resolve with conservative measures, a few reported patients require surgical intervention. We discuss an uncommon presentation of spontaneous intracranial hypotension, requiring surgical repair of a thoracic dural defect to achieve symptom resolution.

Intracranial subdural hematoma coexisting with improvement in spontaneous intracranial hypotension after an epidural blood patch

A 36-year-old male had spontaneous intracranial hypotension (SIH) presenting with refractory headache for 4 months. Multiple epidural blood patches (EBPs) yielded relief of symptoms, but the course was complicated, with asymptomatic intracranial subdural hematoma (SDH). Except for SDH, other radiological diagnostic signs of SIH were resolved and the patient’s headaches improved after EBP. Owing to a mass effect and persistent cerebrospinal fluid (CSF) leakage, surgical repair of the spinal leakage was performed, but no cranial procedures were carried out. Postoperatively, the SDH completely resolved, but there was still CSF leakage at the level where surgery was performed. The patient has remained free of headache or other events for 3 years. It was reduction rather than elimination of the spinal CSF leak that yielded remission of SIH. In summary, intracranial SDH can be a complication of inadequately treated SIH (i.e. persistent minor CSF leakage). Management of SDH should focus on correction of the underlying SIH rather than craniotomy for hematoma evacuation.

Spontaneous Intracranial Hypotension Presenting With Severe Sensorineural Hearing Loss and Headache

Spontaneous Intracranial Hypotension Presenting With Severe Sensorineural Hearing Loss and Headache

Intracranial hypotension headache after uncomplicated caudal epidural injection

A caudal epidural injection was performed on a middle-aged woman for pain in her right foot. Although the procedure was uncomplicated and a good epidurogram was obtained, the patient went on to develop an orthostatic headache with generalised weakness and syncopal episodes that were treated successfully by epidural blood patching. We describe the aetiology, presentation and treatment of spontaneous intracranial hypotension and review the similarities with our patient's clinical presentation. We hypothesise as to how our intervention may have resulted in a dural tear.

Spontaneous intracranial hypotension presenting as pseudo-subarachnoid hemorrhage on CT scan

We report a 20-year-old man who suffered from severe headache. A brain CT scan revealed that he had diffuse brain swelling and increased attenuation of the basal cisterns and Sylvian fissure. Subarachnoid hemorrhage (SAH) was diagnosed but the results of CT angiography and conventional arterioangiography were negative. His headache relapsed and remitted several times during the following months. Brain MRI demonstrated brain sagging with a crowded foramen magnum. Spontaneous intracranial hypotension (SIH) was finally diagnosed and his headache subsided after application of an epidural blood patch. Pseudo-SAH on CT scan is an unusual manifestation of SIH that often results in delay of diagnosis and adequate treatment.

Spontaneous Intracranial Hypotension Presenting Without Orthostatic Headache Complicated by Acute Subdural Hematoma After Drainage for Chronic Subdural Hematoma-Case Report-

A 28-year-old man presented with a case of spontaneous intracranial hypotension (SIH) manifesting as a bilateral chronic subdural hematoma (CSDH) without orthostatic headache. He developed life-threatening acute SDH as a complication of CSDH drainage. Neurosurgeons should be aware that SIH patients do not always present with orthostatic headache. Brain magnetic resonance imaging with gadolinium may be recommended for young adults with non-traumatic CSDH before drainage to exclude SIH, even if they do not present with orthostatic headache.