Das spontane Liquorunterdrucksyndrom

The spontaneous intracranial hypotension syndrome is a rare event but with increasing tendency. The clinical characteristics are comparable to those occurring after dural puncture and the most important clinical finding is the postural headache. The syndrome results from cerebrospinal fluid leakage but its etiology is still nearly unknown. The leaks are mainly located cervically or at the cervicothoracic junction. The syndrome may be associated with cranial subdural fluid build-up. Magnetic resonance imaging of the brain typically reveals diffuse pachymeningeal enhancement, frequently in association with displacement of the brain. Knowledge of this can be helpful to facilitate the diagnosis. Although conservative measures are often initially undertaken, placement of an epidural blood patch is the treatment of choice. Because of its similarity to postdural puncture headache, anaesthesiologists and pain therapists are increasingly involved in diagnosis and therapy. We report 2 patients with spontaneous intracranial hypotension. In addition to the cardinal feature of a postural headache, the patients suffered from subdural fluid build-up demonstrated by cranial magnetic resonance imaging.     

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.     

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.     

Chronic hydrocephalus and suprasellar arachnoid cyst presenting with rhinorrhea.

Spontaneous CSF leak with rhinorrhea may be secondary to many intracranial congenital and acquired conditions. However, no cases of chronic hydrocephalus and suprasellar arachnoid cyst presenting with rhinorrhea as the unique clinical manifestation are reported in the literature. A 29-year-old-man with four-month history of episodic rhinorrhea had a large suprasellar arachnoid cyst with chronic hydrocephalus on magnetic resonance. Endoscopic ventricular fenestration of the cyst failed to obtain remission of the CSF leak, because it was not possible to fenestrate the cyst with the almost completely obliterated suprasellar cistern. Clinical remission occurred after restoration of the CSF flow from the cyst to the cisternal spaces by a direct approach. The CSF leak in this case was secondary to the chronic compression over the dural and bone structures of the sellar region by the cyst or chronic hydrocephalus.     

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.     

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.     

Nontraumatic intradiploic arachnoid cysts--report of five cases.

Five cases of nontraumatic intradiploic arachnoid cysts in elderly patients are reported. All cysts were located in the occipital bone and appeared as well-demarcated radiolucent lesions. The cysts were multiple in three cases. Presenting symptoms included headache or dizziness, but most lesions were asymptomatic and found incidentally. In the most recent three cases, magnetic resonance (MR) imaging revealed intradiploic cysts containing cerebrospinal fluid (CSF) with cerebellar herniation. Operation found the cysts filled with CSF and dural defects through which cerebellar tissue was herniating. In two patients, CSF leakage from the outer table occurred. Intradiploic arachnoid cyst seems to be congenital in origin but commonly found in the elderly. MR imaging is the most useful diagnostic method for differential diagnosis from other osteolytic skull lesions.     

Cranial subdural hematoma with intracranial hypotension related to epidural anesthesia and Trendelenburg position: a case report

We report a case of cranial subdural hematoma with intracranial hypotension. A 34-year-old woman had laparoscopic ovarial cysterectomy under general anesthesia combined with epidural anesthesia. Two days later, she developed a severe headache and nausea. She underwent cranial magnetic resonance imaging (MRI) scanning, and was diagnosed with cranial subdural hematoma with intracranial hypotension. The patient had had no anticoagulant therapy before the surgery. She was managed conservatively with bed rest and additional intravenous infusion. Her symptoms gradually improved except a slight headache, and she was discharged on the 38th postoperative day. Intracranial hypotension is a syndrome characterized by orthostatic headaches and hypovolemia of cerebrospinal fluid (CSF). There were typical findings on MRI, which include linear enhancement of the pachymeninges, pituitary hyperemia and subdural hemorrhage. We thought that these were due to epidural anesthesia first, but there was no evidence of dural puncture. It was also considered that it is influenced by change in CSF pressure, and intracranial venous engorgement may be due to Trendelenburg position for several hours. Because cranial subdural hematoma is a life-threatening complication, it is necessary to reconsider application of epidural anesthesia for laparoscopic surgery with Trendelenburg position.     

Evaluation of spontaneous intracranial hypotension: assessment on ICP monitoring and radiological imaging

We describe two recent cases of spontaneous intracranial hypotension. A 38-year-old woman developed a severe postural headache. Magnetic resonance imaging (MRI) showed marked dural enhancement. Histopathological findings of dural biopsy showed numerous dilated vessels in the dura, rather than hypertrophic change. Lumber CSF pressure was 5 cmH2O and RI cisternography suggested CSF leakage. A 58-year-old woman with postural headache and vertigo had bilateral subdural haematoma associated with diffuse dural enhancement on MRI. Lumber CSF monitoring confirmed persistent low pressure ranging from 0-5 cm H2O. MRI myelography revealed multiple CSF pouches along the whole spinal axis. CSF leakage was demonstrated on Radioisotope (RI) cisternography. Both cases described in this report were diagnosed as spontaneous intracranial hypotension caused by CSF leakage from spinal meningeal diverticula and were successfully treated by intravenous Factor XIII administration.     

Spontaneous intracranial hypotension associated with transdural thoracic osteophyte reversed by primary. dural repair. Case report

Spontaneous intracranial hypotension (SIH) is an increasingly recognized syndrome associated with a specific set of clinical and imaging findings; however, determining the site of spinal cerebrospinal fluid (CSF) leakage in these patients is often difficult, and indications for surgical intervention need to be better defined. The authors report on a 55-year-old woman who presented with posture-related headache, disorientation, and memory impairment. Imaging features were consistent with SIH. Computerized tomography myelography demonstrated a large T2-3 anterior transdural osteophyte associated with a CSF fistula. After an unsuccessful trial of conservative therapy, the patient underwent median sternotomy, T2-3 discectomy and removal of osteophyte, which allowed adequate exposure for primary dural repair. Postoperatively, there was immediate and prolonged resolution of all of her symptoms. This case of SIH was caused by transdural penetration by an anterior osteophyte and CSF leakage in the upper thoracic spine, which was treated effectively by anterior exposure and primary dural repair. Aggressive surgical intervention may be required to treat upper thoracic CSF leaks refractory to other measures.     

Spinal cord herniation associated with an intradural spinal arachnoid cyst diagnosed by magnetic resonance imaging

Two rare cases of spinal cord herniation associated with intradural spinal arachnoid cyst are reported. A preoperative magnetic resonance imaging scan demonstrated the presence of spinal cord herniation, identified as a protrusion continuous with the spinal cord. Surgery upon the intradural spinal arachnoid cyst improved progressive neurological dysfunction. The authors postulate that spinal cord herniation occurred for the following reason: The pressure of the intradural arachnoid cyst on the dorsal aspect of the spinal cord caused thinning of the dura, leading to a tear and, thus, the development of an extradural arachnoid cyst. Along with the enlargement of intradural arachnoid cyst, the spinal cord herniated through the tear in the dura into the extradural arachnoid cyst.     

Magnetic resonance in dural post-puncture headache in patient with cerebrospinal fluid hypotension

Magnetic resonance imaging (MRI) has allowed us to establish a set of radiologic signs associated with intracranial hypotension syndrome. Findings are partly influenced by cerebral displacement. Intracranial hypotension syndrome is characterized by a decrease in cerebrospinal fluid (CSF) pressure to less than 60 mm H2O associated with occipital headache radiating to the frontal and temporal zones. For diagnostic purposes, the most common cause is anesthetic or therapeutic dural puncture, although spontaneous CSF leakage can occur. CSF protein and lymphocyte counts may be high, while the cranial meninges biopsy is normal. MRI images may show a descended brain, taking the start of the sylvian aqueduct and the location of the cerebellar amygdalae as points of reference; diminished size of the subarachnoidal cisterns and occasionally of the cerebral ventricles; meningeal enhancement from increased uptake of the contrast solution; subdural hygromas and hematomas; and pituitary enlargement. Paraspinal fluid and dilated epidural veins may be observed. Radiologic images and clinical signs are related. When CSF pressure is very low, there is greater meningeal enhancement, subdural collection and cerebral displacement. Findings gradually disappear as symptoms diminish. The signs and symptoms that might develop during intracranial hypotension syndrome vary according to the brain structure that might be affected during descent, repositioning and the traction of anchoring structures. MRI allows the degree of cerebral and spinal involvement to be ascertained, to predict whether resolution of the clinical picture will be early or late and to visualize the effect of approaches to reducing CSF leakage.     

Broadening the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia. Case report

The syndrome of spontaneous intracranial hypotension is characterized by orthostatic headaches in conjunction with reduced cerebrospinal fluid (CSF) pressure or CSF volume, and characteristic magnetic resonance (MR) imaging findings. A 50-year-old man presented with a 1-year history of paroxysmal ataxia of gait and short attacks of blurred vision when he stood up from a recumbent position and began to walk. Orthostatic headache was not a feature of his clinical presentation. Magnetic resonance images of the brain revealed diffuse enhancement of the dura mater and hygromas over both cerebral convexities. Magnetic resonance images of the spine demonstrated dilated cervical epidural veins and dilation of the perimedullary veins. Radionuclide cisternography identified a CSF leakage that was localized to the T12-L1 level on subsequent myelograms and on computerized tomography scans obtained after the myelograms. An epidural blood patch was administered and visualized with tungsten powder. The patient's clinical symptoms and sites of disease on imaging completely resolved. The unusual clinical presentation in this case--paroxysmal ataxia of gait, lack of orthostatic headaches, and dilated epidural and perimedullary venous plexus--supports a recently noted broadening of both the clinical and imaging characteristics of spontaneous intracranial hypovolemia.     

Regional Anesthesia and Pain Targeted thoracic epidural blood patch placed under electrical stimulation guidance (Tsui test)

PURPOSE: This case report describes the use of electrical epidural stimulation (Tsui test) to confirm accurate placement of a thoracic epidural catheter when administering an epidural blood patch for headache management in a patient suffering from spontaneous intracranial hypotension. CLINICAL FEATURES: A 41-yr-old female presented to the Chronic Pain Clinic with a history of postural headache symptoms worsening in severity over several years. Two previous blood patches performed at T11-12 and T10-11 respectively provided short-term relief only. The presumed diagnosis of a spontaneous dural tear was confirmed by a nuclear flow test to be at T2-T4. The epidural site was accessed at T6 with a Tuohy needle. To accurately place the epidural blood patch at the level of the dural tear, the Arrow catheter with electrode adapter was advanced under nerve stimulation guidance to T4. Ten millilitres of autologous blood injected through the catheter was confirmed on magnetic resonance imaging, one hour postprocedure, to lie between T3 and T9. Sustained headache relief was achieved. CONCLUSION: The use of electrical stimulation guidance may be useful when precise epidural blood patch placement is required.     

Intracranial hypotension syndrome treated with an epidural blood patch

A 41-year-old woman was admitted to the internal medicine department to assess incapacitating postural headache. Clinical findings suggested the need for computed tomography and nuclear magnetic resonance scanning of the head, which led to a diagnosis of spontaneous intracranial hypotension syndrome. Later, isotopic cysternography and nuclear magnetic resonance imaging of the spine were used unsuccessfully to try to locate the cerebrospinal fluid leak that caused the syndrome. When conservative treatment proved ineffective, the pain clinic was called in to perform an epidural blood patch procedure. The patch led to an improvement in symptoms and the syndrome resolved completely after a second lumbar blood patch was used.     

Treatment of spontaneous intracranial hypotension secondary to C-2 meningeal cyst by surgical packing--case report

A 41-year-old man presented with progressive worsening of postural headache. Computed tomography (CT) showed bilateral subdural hematomas without prior history of trauma. The diagnosis was spontaneous intracranial hypotension (SIH). Conservative treatment with oral steroids failed to prevent gradual deterioration of the patient's consciousness. CT myelography revealed massive cerebrospinal fluid (CSF) leakage between the C-1 and C-2 levels. The leak was repaired surgically via a laminectomy. A cyst, thought to be a meningeal cyst, was discovered adjacent to the right C-2 nerve root, and CSF was seen seeping out from around the cyst after a Valsalva maneuver. The presumed dural defect of the cyst was sealed by packing with muscle fragments and fibrin glue. The symptoms disappeared soon after surgery. He was discharged 1 month after surgery without deficits. Most SIH cases are benign and can be managed conservatively, or by the epidural blood patch method. Surgery is more invasive than the epidural blood patch method, but should be performed in patients with a high cervical lesion and massive CSF leakage.     

Dural tear and resultant cerebrospinal fluid leaks after cervical spinal trauma

Purpose

Traumatic cervical spinal cord injuries (SCIs) frequently develop dural tears and resultant cerebrospinal fluid (CSF) leaks. They are not usually identified with advanced imaging, and there are no reports on managing CSF leaks after cervical trauma. Hence, the authors evaluated the incidence of CSF leaks after cervical SCIs and described how to predict and manage CSF leaks.

Methods

An observational retrospective study was done confirming intraoperative CSF leaks among 53 patients with anterior cervical surgery after cervical spine trauma between 2004 and 2011.

Results

Seven patients (13.2 %) had dural tears and resultant CSF leaks intraoperatively (M:F ratio of 6:1; mean age, 44.7 years). An initial poor American Spinal Injury Association (ASIA) scale was significantly associated with CSF leaks (p = 0.009). From magnetic resonance imaging (MRI), disruption of the ligamentum flavum was correlated with CSF leaks (p = 0.02). Intraoperative application of fibrin glue on the operated site, postoperative management through the early removal of the wound drain within the first 24 h and early rehabilitation were performed in patients with CSF leaks without perioperative insertion of a lumbar drain. During the follow-up period, none of the patients developed CSF-leak-related complications.

Conclusion

The incidence of CSF leaks after traumatic cervical SCI is relatively higher than that of degenerative cervical spinal surgery. An initial poor neurological status and disruption of the ligamentum flavum on the MRI in patients were predictable factors of dural tears and CSF leaks.     

Spinal extradural arachnoid cyst

Background contextSpinal extradural arachnoid cysts are uncommon expanding lesions. Idiopathic arachnoid cysts are not associated with trauma or other inflammatory insults. If they enlarge, they usually present with progressive signs and symptoms of neural compression.PurposeTotal removal of the cyst and repair of the dural defect is the primary treatment for large thoracolumbar spinal extradural arachnoid cysts causing neurogenic claudication. Laminoplasty may prevent spinal deformities in long segmental involvement.Study designA clinical case was performed.Patient sampleWe report a case of 25-year-old man with 1-year history of progressive back pain radiating to both legs. His diagnosis was dorsal intraspinal extradural cystic lesion longing from the level of T11 to L2 on magnetic resonance imaging.Outcome MeasuresThe patient's pain levels were noted as he reported. Physiologic outcome was assessed on pre- and postoperative motor and sensory examination.MethodsThe patient underwent a T11–L2 laminotomy and radical cyst wall resection was performed. A small communication with the subarachnoid space was seen at the level of T12. It was sealed with tissue fibrinogen after repair with primary suture. Titanium miniplates were used for laminoplasty.ResultsFollow-up magnetic resonance imaging demonstrated cyst resolution, and neurologic examination revealed no sensory and motor deficit.ConclusionExtradural arachnoid cysts are primarily treated with total removal of the cyst wall and closure of the dural defect. Surgical treatment is curative for this rare lesion.     

Purely endoscopic repair of traumatic cerebrospinal fluid rhinorrhea from the anterior skull base: case report

A 38-year-old woman presented with a case of post-traumatic ethmoidal cerebrospinal fluid (CSF) leak that was repaired using a purely endoscopic endonasal approach. Six weeks after a mild domestic maxillofacial trauma, she started complaining of clear, watery discharge from the left nostril and headache. Neuroimaging investigations disclosed a linear fracture of the left anterior ethmoidal roof without evidence of large bony breaches or any brain tissue damage. After conservative medical treatment with carbonic anhydrase inhibitors failed, she was referred to our hospital for surgical repair of the osteodural defect. An endoscopic endonasal transethmoidal approach was successfully performed, and an overlay technique was used to reconstruct the defect. The patient was discharged without clinical evidence of CSF rhinorrhea and no leaks were apparent at the 3-month follow-up clinical and radiological examinations. Post-traumatic CSF rhinorrhea occurs in cases of dural tears associated with small bone breaks, most frequently involving the anterior skull base. Recovery is often spontaneous following only bed rest, or with medical treatments such as inhibitors of carbonic anhydrase diuretics, steroids, or eventually stool softeners to help reduce CSF pressure. Surgical repair is required when first-line conservative treatment proves ineffective. The present case shows that the endoscopic endonasal technique for the management of CSF leaks provides a less invasive surgical route to achieve valid dural repair.     

Endoscopic fenestration of posterior fossa arachnoid cyst for the treatment of presyrinx myelopathy--case report

A 32-year-old man presented with an arachnoid cyst of the posterior fossa manifesting as cervical syringomyelic myelopathy. Magnetic resonance (MR) imaging demonstrated edematous enlargement and T2 prolongation of the cervical spinal cord, indicating a "presyrinx" state. MR imaging showed the inferior wall of the cyst, which disturbed cerebrospinal fluid (CSF) pulsatile movement between the intraspinal and intracranial subarachnoid spaces. The cyst wall was fenestrated with a neuroendoscope. The presyrinx state and the CSF movement improved. Posterior fossa arachnoid cyst, as well as Chiari malformation, can cause CSF flow disturbance at the craniocervical junction and syringomyelia. Endoscopic fenestration is less invasive than foramen magnum decompression and should be the procedure of choice.