Spontaneous retroclival hematoma presenting as a thunderclap headache. Case report

An excruciating headache of instantaneous onset, or thunderclap headache, may be caused by a variety of serious disorders, including aneurysmal subarachnoid hemorrhage, cerebral venous sinus thrombosis, pituitary apoplexy, and carotid artery or vertebral artery dissection. The authors describe a patient with this type of headache who was found to have a spontaneous retroclival hematoma. A 49-year-old woman experienced an instantaneous excruciating headache. Results of computerized tomography (CT) scans of the head were normal, but on examination of the cerebrospinal fluid xanthochromia was found. Magnetic resonance (MR) imaging of the cervical spine revealed a retroclival hematoma. Three cerebral angiographic studies did not reveal the source of the hemorrhage and a repeated MR image demonstrated resolution of the hematoma. The patient made an uneventful recovery. Spontaneous retroclival hematoma is an exceedingly rare type of intracranial hemorrhage and may be associated with normal findings on CT scans. Spontaneous retroclival hematoma should be included in the differential diagnosis of thunderclap headache.     

Recurrent spontaneous spinal cerebrospinal fluid leak associated with "nude nerve root" syndrome: case report

OBJECTIVE AND IMPORTANCE: Spontaneous spinal cerebrospinal fluid (CSF) leaks have been noted occasionally at multiple sites in the same patient, but recurrent spontaneous spinal CSF leaks have not been documented. We describe a patient with a recurrent CSF leak who was found at surgery to have an absence of the entire nerve root sleeve at multiple thoracic levels. CLINICAL PRESENTATION: A 29-year-old woman bodybuilder noted an excruciating orthostatic headache associated with nausea. The neurological examination was unremarkable, and a magnetic resonance imaging examination showed the typical changes of intracranial hypotension. Computed tomographic myelography showed an extensive bilateral lower cervical CSF leak. INTERVENTION: The patient underwent bilateral lower cervical nerve root explorations, and several small dural holes were found. The CSF leaks were repaired, but 3 months later, computed tomographic myelography showed a new CSF leak in the midthoracic area. A thoracic laminectomy was performed, and several nerve roots were found to be completely devoid of dura. After the CSF leaks were repaired, there was significant improvement in her headaches. CONCLUSION: A recurrent spontaneous spinal CSF leak may occur in patients with intracranial hypotension at a site previously documented not to be associated with a CSF leak. Absent nerve root sleeves may be found in patients with spontaneous spinal CSF leaks ("nude nerve root" syndrome), and these patients may be at increased risk of developing a recurrent CSF leak.     

Spectrum of subdural fluid collections in spontaneous intracranial hypotension

OBJECT: Spontaneous intracranial hypotension is a noteworthy but commonly misdiagnosed cause of new daily persistent headaches. Subdural fluid collections are frequent radiographic findings, but they can be interpreted as primary rather than secondary pathological entities, and uncertainties exist regarding their optimal management. The authors therefore reviewed their experience with subdural fluid collections in 40 consecutive patients with spontaneous spinal cerebrospinal fluid (CSF) leaks and intracranial hypotension. METHODS: The mean age of the 26 female and 14 male patients was 43 years (range 13-72 years). Subdural fluid collections were present in 20 patients (50%); 12 of these patients (60%) had subdural hygromas alone, and eight (40%) had subacute to chronic subdural hematomas (SDHs) associated with significant mass effect. The subdural hygromas resolved within several days to weeks following treatment of the underlying CSF leak. Three patients with SDHs underwent evacuation of the hematoma prior to the establishment of the diagnosis of spontaneous intracranial hypotension, but the SDHs did not resolve until the underlying spinal CSF leak was treated. In the remaining five patients, the CSF leak was treated primarily and the SDHs resolved over a 1- to 3-month period without the need for evacuation. CONCLUSIONS: Subdural fluid collections are common in spontaneous intracranial hypotension, varying in appearance from thin subdural hygromas to large SDHs associated with significant mass effect. These collections can be safely managed by directing treatment at the underlying CSF leak without the need for hematoma evacuation.     

Cerebrospinal fluid leak demonstrated by three-dimensional computed tomographic myelography in patients with spontaneous intracranial hypotension

BACKGROUND: Precise determination of the spinal level of a cerebrospinal fluid leak is important in the diagnosis and treatment of spontaneous intracranial hypotension. The authors report two cases of SIH in which a cerebrospinal fluid leak was demonstrated by three-dimensional computed tomographic (3D-CT) myelography. CASE DESCRIPTION: By overlaying 3D-CT images of contrast-enhanced CSF and the spine, the point of leakage was clearly depicted in the three-dimensional spinal structure, which assisted targeted epidural blood patch under the guidance of fluoroscopy. Although associated chronic subdural hematoma had to be treated by burr hole drainage, the patients' postural headaches subsided after treatment. CONCLUSION: Clear CSF images in relation to vertebral bones are obtainable with 3D-CT myelography, rendering this modality very useful for the diagnosis and treatment of spontaneous intracranial hypotension.     

False localizing sign of C1-2 cerebrospinal fluid leak in spontaneous intracranial hypotension

OBJECT: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is an important cause of new daily persistent headaches. Spinal neuroimaging is important in the treatment of these patients, particularly when direct repair of the CSF leak is contemplated. Retrospinal C1-2 fluid collections may be noted on spinal imaging and these are generally believed to correspond to the site of the CSF leak. The authors undertook a study to determine the significance of these C1-2 fluid collections. METHODS: The patient population consisted of a consecutive group of 25 patients (18 female and seven male) who were evaluated for surgical repair of a spontaneous spinal CSF leak. The mean age of the 18 patients was 38 years (range 13-72 years). All patients underwent computerized tomography myelography. Three patients (12%) had extensive retrospinal C1-2 fluid collections; the mean age of this woman and these two men was 41 years (range 39-43 years). The actual site of the CSF leak was located at the lower cervical spine in these patients and did not correspond to the site of the retrospinal C1-2 fluid collection. CONCLUSIONS: A retrospinal fluid collection at the C1-2 level does not necessarily indicate the site of the CSF leak in patients with spontaneous intracranial hypotension. This is an important consideration in the treatment of these patients because therapy may be inadvertently directed at this site.     

Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. Report of four cases

Spontaneous intracranial hypotension due to a cerebrospinal fluid (CSF) leak in the spine is an important cause of new, daily persistent headaches. Most patients respond well to conservative treatments including epidural blood patching. Limited options for effective treatment are available for patients in whom these treatments fail. The authors treated four patients (mean age 38 years; range 26-43 years) with percutaneous placement of a fibrin sealant. All these patients presented with intractable positional headaches. The CSF leak was located in the lower cervical spine in three patients and in the lower thoracic spine in one patient. Four to 20 milliliters of fibrin sealant was injected at the site of the CSF leak. Two of the four patients became asymptomatic within days of the procedure and thus avoided surgery. There were no complications of this procedure. Percutaneous placement of a fibrin sealant is a safe, minimally invasive treatment for spontaneous spinal CSF leaks and should be considered in patients in whom conservative treatment has failed.     

Fatal cerebral and cerebellar hemorrhagic infarction after thoracoscopic microdiscectomy. Case report

The authors present a rare case of fatal cerebral and cerebellar hemorrhagic infarction following an uneventful thoracoscopic microdiscectomy. They hypothesize that this complication was associated with cortical venous thrombosis secondary to intracranial hypotension, which was caused by an unnoticed leak of cerebrospinal fluid (CSF) into the thoracic cavity. Cortical venous thrombosis and intrathoracic CSF were confirmed at autopsy. The former disorder is the most severe manifestation of the pathophysiological mechanism occurring to a lesser degree in patients affected by mild intracranial hypotension, and occurs more frequently in these patients. Intracranial hypotension (of an orthostatic nature or not) must be considered in the differential diagnosis of every patient who complains of headaches after thoracoscopic or open transthoracic microdiscectomy.     

Spontaneous intracranial hypotension: dilemmas in diagnosis

Intracranial hypotension is not an uncommon diagnosis after lumbar puncture or neurosurgery. However, spontaneous intracranial hypotension (SIH) is a poorly understood entity that can present with a wide variety of symptoms/signs ranging from headache to coma. SIH may result from an occult spinal cerebrospinal fluid (CSF) leak. Alternatively, because a CSF leak is not always found, some posit that SIH is caused by venous hypotension that results in increased CSF absorption. The true incidence of SIH is unknown and the diagnosis is frequently missed given the wide range of presenting symptoms and imaging findings that are mistaken for other diagnoses (ie, subdural hematomas, Chiari malformation). Here, based on a comprehensive literature review, we describe the epidemiology, presentation, diagnostic workup and treatment of SIH.     

Spontaneous intracranial hypotension: The syndrome and its complications

Opinion statement Spontaneous intracranial hypotension (SIH) is a syndrome that was largely unknown until the advent of MRI. The incidence of SIH is estimated at 5 per 100,000, which is half the incidence of subarachnoid hemorrhage. The major feature is a postural headache of acute or subacute onset. This headache is absent or minimal when the patient is lying down and rapidly worsens to great intensity when the patient sits or stands. Other features may include nausea, vomiting, vertigo, tinnitus, and marked exacerbation by Valsalva maneuver. SIH is due to a leak of cerebrospinal fluid from a tear in the dural membrane, which occurs most often at the exit zones where the cervical spinal roots leave the subarachnoid space. Other leak sites may be the vestibular system, the cribriform plate, or the pituitary fossa. If the leak continues, the brain loses buoyancy within the cranial space and sags toward the foramen magnum. This, in turn, may produce subdural hygroma or hematoma, brainstem compression, focal cranial nerve palsies, or cerebellar tonsillar herniation. The initial therapy is generally strict bed rest. If this fails, an epidural blood patch is usually successful in sealing the leak and restoring brain buoyancy. A significant minority of patients require a repeat epidural blood patch. If the blood patch fails, a surgical approach may be needed. Repair of the leak and restoration of brain buoyancy will stop the postural headache and, in most cases, will reverse the complications.     

Antenatal blood patch in a pregnant woman with spontaneous intracranial hypotension

Spontaneous intracranial hypotension is a condition that presents with postural headaches similar to those caused by accidental dural puncture. The diagnosis is based on clinical presentation, cerebrospinal fluid evaluation and magnetic resonance imaging scanning. We present a case of spontaneous intracranial hypotension with typical clinical and magnetic resonance imaging findings in a pregnant patient who was treated with an epidural blood patch. The blood patch, performed at 32 weeks of gestation, produced transient improvement in symptoms but failed to completely cure the headache, which worsened over the next few days. Symptoms resolved over the subsequent three weeks with conservative therapy.     

Intracranial hypotension without meningeal enhancement on magnetic resonance imaging. Case report

Meningeal enhancement on magnetic resonance (MR) imaging is considered the hallmark radiological feature of intracranial hypotension. The authors report on a patient who exhibited progressively symptomatic intracranial hypotension due to a lumbar cerebrospinal fluid (CSF) leak, but in whom MR imaging demonstrated no pachymeningeal enhancement. This 24-year-old man presented with a 6-week history of progressive orthostatic headaches that were associated with photo- and phonophobia. Four weeks before the onset of the headaches, the patient had undergone a lumbar laminectomy. Brain MR images revealed subdural fluid collections and brain sagging; however, meningeal enhancement was not present. Myelography demonstrated a CSF leak at the site of the laminectomy. At surgery, a large dural tear was repaired. The patient recovered well from the surgery, with complete resolution of his headaches. The absence of meningeal enhancement on MR imaging does not exclude a diagnosis of symptomatic intracranial hypotension.     

Cerebellar hemorrhage after spinal anesthesia

Cerebellar hemorrhage is a rare complication of spinal anesthesia. We report a case in a 51-year-old woman with a history of hypertension who underwent uterine dilatation and curettage with spinal anesthesia. During recovery she vomited and complained of headache. Postdural puncture headache was diagnosed. When there was no response to conventional treatment, computed tomography and magnetic resonance scans of the head were performed. The scans confirmed cerebellar hemorrhage due to rupture of a cavernous angioma. The patient recovered fully without surgical decompression. We review the pathogenesis of headache and cerebral hemorrhage after spinal anesthesia and propose differential diagnosis between spontaneous rupture related to hypertension and cerebrospinal fluid hypotension syndrome caused by trauma from lumbar spinal puncture. Patients with prolonged severe headache after spinal anesthesia require neurologic and radiologic monitoring to rule out the possibility of intracranial complications.     

Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study

OBJECT: Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak. METHODS: The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22-55 years). The mean duration of follow up was 36 months (range 6-132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. CONCLUSIONS: Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.     

Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report

OBJECTIVE AND IMPORTANCE: Spontaneous intracranial hypotension is an increasingly recognized cause of postural headache. However, appropriate management of obtundation caused by intracranial hypotension is not well defined. CLINICAL PRESENTATION: A 43-year-old man presented with postural headache followed by rapid decline in mental status. Imaging findings were consistent with the diagnosis of spontaneous intracranial hypotension, with bilateral subdural hematomas, pachymeningeal enhancement, and caudal displacement of posterior fossa structures and optic chiasm. INTERVENTION: Despite treatment with lumbar epidural blood patch, worsening stupor necessitated intubation and mechanical ventilation. Contrast-enhanced magnetic resonance imaging and computed tomographic myelography of the spine failed to demonstrate the site of cerebrospinal fluid fistula. The enlarging subdural fluid collections were drained, and a ventriculostomy was performed. Postoperatively, the patient remained semicomatose. To restore intraspinal and intracranial pressures, intrathecal infusion of saline was initiated. After several hours of lumbar saline infusion, lumbar and intracranial pressures normalized, and the patient's stupor resolved rapidly. Repeat computed tomographic myelography accomplished via C1-C2 puncture demonstrated a large ventrolateral T1-T3 leak, which was treated successfully with a thoracic epidural blood patch. Follow-up magnetic resonance imaging demonstrated resolution of intracranial hypotension, and the patient was discharged in excellent condition. CONCLUSION: Spontaneous intracranial hypotension may cause a decline of mental status and require lumbar intrathecal saline infusion to arrest or reverse impending central (transtentorial) herniation. This case demonstrates the use of simultaneous monitoring of lumbar and intracranial pressures to appropriately titrate the infusion and document resolution of intracranial hypotension. Maneuvers aimed at sealing the cerebrospinal fluid fistula then can be performed in a less emergent fashion after the patient's mental status has stabilized.     

Epidemiology and outcome of postural headache management in spontaneous intracranial hypotension

BACKGROUND AND OBJECTIVES: Spontaneous intracranial hypotension is a postural headache syndrome unrelated to dural puncture. Due to the apparent failure of epidural blood patch to relieve headache in spontaneous intracranial hypotension, we investigated the epidemiologic features and treatment outcomes of this condition. METHODS: The clinical findings and management of 22 cases (21 published + 1 reported) of spontaneous intracranial hypotension were analyzed retrospectively. The study population was stratified by age and sex; continuous variables were compared for differences by t-tests; categorical variables were compared by Fisher exact tests. Significant differences were identified by P values of.05 or less. RESULTS: The mean age of the study population was 43 +/- 16 years, with a female:male ratio of 3.4:1.0. Females with spontaneous intracranial hypotension were younger (P =.050) than males. Men presented with tinnitus (P =.021) and visual field defects (P =.009) more often than women. Meningeal enhancement on contrast magnetic resonance imaging was the most consistent radiographic finding. Radionuclide cisternography showed thoracolumbar dural leaks in 7 of 9 patients. Cerebrospinal fluid opening pressure was low in all patients (33.13 +/- 31.02 mm H(2)O). Epidural blood patch was performed in 8 patients, repeated in 3 patients, failed in 3 patients, and offered only transient improvement in 5 patients. CONCLUSIONS: Spontaneous intracranial hypotension was more common in women than men, was not uniformly responsive to epidural blood patch, and had significant comorbidities. The management of postural headache in spontaneous intracranial hypotension by other techniques to restore cerebrospinal fluid dynamics and prevent its leakage should be investigated.     

Céphalées post-brèches méningées et blood-patch : aspects théoriques et pratiques

Objective

To review the current research and formulate a rational approach to the physiopathology, cause and treatment of post-dural puncture headache (PDPH).

Data sources

Articles published to December 2011 were obtained through a search of Medline for the MeSh terms “epidural blood-patch” and “post-dural puncture headache”.

Study selection

Six hundred and eighty-two pertinent studies were included and 200 were analysed.

Data synthesis

Resulting of a dural tap after spinal anaesthesia or diagnostic lumbar puncture or as a complication of epidural anaesthesia, PDPH occurs when an excessive leak of cerebrospinal fluid leads to intracranial hypotension associated to a resultant cerebral vasodilatation. Reduction in cerebrospinal fluid volume in upright position may cause traction of the intracranial structure and stretching of vessels. Typically postural, headache may be associated to nausea, photophobia, tinnitus or arm pain and changes in hearing acuity. In severe cases, there may be cranial nerve dysfunction and nerve palsies secondary to traction on those nerves. The Epidural Blood-Patch (EBP) is considered as the “gold standard” in the treatment of PDHP because it induces a prolonged elevation of subarachnoid and epidural pressures, whereas such elevation is transient with saline or dextran. EBP should be performed within 24–48 hours of onset of headache; the optimum volume of epidural blood appears to be 15–20 mL. Severe complications following EBP are exceptional. The use of echography may be safety puncture. The optimum timing of epidural blood-patch, the resort of repeating procedure if the symptomatology does not disappear, the alternative to the conventional medical treatment need to be determined by future clinical trial.     

Spontaneous intracranial hypotension associated with bilateral chronic subdural hematomas--case report

A 34-year-old female presented with spontaneous intracranial hypotension (SIH) manifesting as severe postural headache and meningism. Magnetic resonance (MR) imaging with gadolinium showed diffuse pachymeningeal enhancement. She developed bilateral chronic subdural hematomas 4 weeks after the onset of the symptoms. MR imaging showed descent of the midline structures of the brain. The bilateral chronic subdural hematomas were surgically drained, with no remarkable pressure. Postoperative MR imaging showed complete resolution of the pachymeningeal enhancement and relevation of the midline structures of the brain. SIH is an uncommon and probably unrecognized condition because of the usually benign course. However, this case emphasizes that SIH is not entirely benign. SIH should be considered if there is no identifiable risk for intracranial hemorrhage, particularly in young patients. Neurosurgical intervention for the treatment of the underlying cerebrospinal fluid leak may be required if SIH persists.     

Headache caused by endocranial hypotension secondary to sacrococcygeal trauma in a patient with a congenital arachnoid cyst

Headache caused by intracranial hypotension after sacrococcygeal trauma, is a rare syndrome with very similar symptoms and physiopathology to post dural puncture headache. In both situations, cerebrospinal fluid (CSF) leaks through a dural tear, leading to a decrease in its pressure. A 13 year old patient presented a frontal and occipital positional headache, after undergoing sacrococcygeal trauma. In magnetic resonance images, the presence of a spinal arachnoid diverticulum (cyst) and CSF leak were confirmed. After the establishment of conservative medical treatment, complete clinical remission was observed. The diagnosis of intracranial hypotension headache syndrome is mainly clinical, once other possible headache etiologies have been discarded. Magnetic resonance imaging can be used to detect small CSF leaks, and in this case, magnetic resonance imaging clearly showed the existence of an arachnoid cyst. The presence of some meningeal diverticulums such as arachnoid cyst, constitute a risk to undergo this syndrome, due to the possibility of its rupture by minor traumatisms. The initial treatment should be conservative, as the dural tear seals spontaneously and the liquid is reabsorbed.     

Posterior reversible encephalopathy syndrome complicating intracranial hemorrhage after phenylpropanolamine exposure

A 57-year-old woman suffered sudden onset of thunderclap headache after exposure to phenylpropanolamine (PPA), and subsequently developed posterior reversible encephalopathy syndrome (PRES) complicated by occipital intracranial hemorrhage (ICH) with cerebral vasoconstriction. PPA is well known to be associated with ICH and vasoconstriction, but this case illustrates the association with PRES. The danger of exposure to PPA and subsequent adverse events is quite low at present, but we must consider the possibility of exposure to medical agents in patients with repeated severe headache who have no organic disorder.     

Diagnostic and surgical strategies for intractable spontaneous intracranial hypotension. Case report

The authors present the case of a 55-year-old man suffering from intractable spontaneous intracranial hypotension, in whom conservative treatment with 19 weeks of bed rest was not effective. In this period the patient twice underwent surgery for bilateral chronic subdural hematoma, a complication of spontaneous intracranial hypotension. Conventional radionuclide cisternography, magnetic resonance imaging, and computerized tomography myelography did not demonstrate cerebrospinal fluid (CSF) leakage. Repeated radionuclide cisternography with the patient in an upright position revealed leakage of the tracer at upper cervical levels. Computerized tomography myelography with breath holding also showed CSF leakage of the contrast medium bilaterally at upper cervical levels. The patient underwent surgery, and bilateral C-2 and C-3 spinal nerve root pouches were sealed off from the subarachnoid space with oxidized cellulose cotton and fibrin glue. Epiarachnoid spaces around the root sleeves were also sealed to ensure complete resolution of the CSF leakage. After the surgery, the patient was completely free of the disease. In the case of intractable persistent spontaneous intracranial hypotension, surgical treatment is preferable to long-term conservative management. To identify CSF leakage, radionuclide cisternography with the patient in the upright position is useful. When obvious leakage is encountered, surgical sealing of the lesion should be performed via a subarachnoid approach.