Early epidural blood patch in spontaneous intracranial hypotension

Thirty patients with a typical orthostatic headache were treated by early lumbar epidural blood patch (EBP) without previously performing lumbar puncture or identifying a CSF leak and with or without typical MRI changes. A complete cure was obtained in 77% of patients after one (57%) or two (20%) EBPs. Spontaneous intracranial hypotension with typical orthostatic headache can be diagnosed without lumbar puncture and can be cured by early EBP in a majority of patients.     

Spontaneous cerebrospinal fluid hypovolaemia: a therapeutic dilemma?

Spontaneous intracranial hypotension is characterized by orthostatic headaches in conjunction with reduced cerebrospinal fluid volume(CSF) and characteristic imaging findings. We report the clinical course of six consecutive patients with spontaneous intracranial hypotension who were followed between 4 months and 2.5 years. The characteristic orthostatic headaches were present in five patients. Diffuse pachymeningeal enhancement on brain magnetic resonance imaging (MRI) was evident in all cases. CSF detected elevated protein content in three of six patients. In only two of our six patients a first epidural blood patch resulted in complete symptom resolution lasting 4 months and 1 year. Four patients received a second epidural blood patch and one patient also received a third. In four patients, follow-up brain MRI revealed re-occurrence of the typical MRI features and all of them suffered from orthostatic symptoms at this time. Only four patients are free of complaints after an average follow-up period of 10 months. Symptom relief within 7 days from an epidural blood patch is accepted to be diagnostic for spontaneous intracranial hypotension. However, our data illustrate that the clinical course of the syndrome is very unstable and the epidural blood patch is less effective than widely accepted.     

Orthostatic headaches without CSF leak in postural tachycardia syndrome

Four women age 17 to 28 years presented with orthostatic headaches as the most prominent feature of their symptom complex. None had CSF leak or intracranial hypotension. Autonomic studies showed evidence of orthostatic intolerance with tachycardia in all cases. Treatment of orthostatic intolerance, mainly with volume expansion, was only partially effective. Orthostatic headaches are not always caused by CSF leak or supine intracranial hypotension. Occasionally they may be the major clinical manifestation of postural tachycardia syndrome or orthostatic intolerance.     

Severe orthostatic headache: a retrospective study on clinical presentation, radiological characteristics, and outcome in 12 patients]

To determine clinical characteristics and therapeutic outcome of severe orthostatic headache, we retrospectively studied 12 consecutive patients with severe orthostatic headache and unremarkable medical history. Headache is defined as severe when it interferes with patient's daily activities. Baseline characteristics of our patients are essentially similar to those reported in other published series of spontaneous intracranial hypotension: female preponderance; mean age of approximately 40 years; frequently associated nausea, hearing disturbances, or vertigo. Diffuse pachymeningeal gadolinium enhancement was demonstrated on MRI in 64% of the patients. Low CSF pressure was present in 78%. Of 8 patients underwent radionuclide cisternography, CSF leak site was identified in 4 patients. Severe orthostatic headache resolved in 13.6 days in 5 patients who received supportive measures only. Conservative treatment was ineffective in 7 patients. Of 6 patients treated with epidural blood patch (EBP), 5 patients (83%) responded well to the first EBP. Patients with severe orthostatic headache which persisted 2 weeks and longer in spite of conservative measures are candidate for EBP. Our study and a literature review suggest that at least 2 EBPs should be performed for each leak site. Studies with more patients are needed for establishing a better treatment protocol for this syndrome.     

Pseudo Chiari type I malformation secondary to cerebrospinal fluid leakage

Cerebrospinal fluid (CSF) leakage may occur spontaneously, iatrogenically or from spinal trauma. Postural headache is the cardinal symptom; dizziness, diminished hearing, nausea and vomiting are additional symptoms. In neurological examinations cranial nerve palsies may be found. Due to low CSF pressure neuroimaging studies may reveal dural enhancement and vertical displacement of the brain. We describe a patient with the history of an uncomplicated lumbar discectomy at the level L4-5 and the typical clinical symptoms of intracranial hypotension. MRI of the craniocervical junction schowed typical features of a Chiari type-I malformation. After neurosurgical ligation of a CSF leak at L4-5 caused by lumbar disc surgery, the patient was free of orthostatic headache. A repeated MRI showed a striking reduction of the previous downward displacement of the cerebellar tonsils and pons. Received: 24 March 1998 Received in revised form: 19 May 1998 Accepted: 14 June 1998     

Spontanes Liquorunterdrucksyndrom Klinische, neuroradiologische, nuklearmedizinische und Liquor-Befunde

We report 11 patients with orthostatic headache due to spontaneous intracranial hypotension. Nausea (3 patients) and abducens palsy (2 patients) were the main additional symptoms. Ten patients had CSF pleocytosis (6 to 43 white cells/microliter) and/or increased protein (581 to 1668 mg/l). CT and/or MRI documented bifrontal accentuated subdural hygromas and hematomas in 5 patients. MRI also documented diffuse meningeal gadolinium enhancement in all 4 patients examined, and descent of the brain in one. Cisternography was done in 9 patients and revealed a decreased or absent activity over the convexities and early detection of the tracer in the bladder in all, and a CSF leak at the cervicothoracal junction in 2 patients. Most patients improved with bed rest, increased fluid intake (oral or intravenous), steroids, and/or epidural blood patch. Subdural hematomas increased in 2 patients and have to be drained. Spontaneous intracranial hypotension is due to a CSF leak followed by decreased CSF volume and hydrostatic CSF pressure changes. The locations of the leaks are mainly cervical or at the cervicothoracal junction. MRI always documents diffuse meningeal gadolinium enhancement. Treatment of choice is an epidural blood patch. Surgical treatment may be needed in patients with subdural hematomas or meningeal diverticula. Prognosis is typically good, but subdural hematomas may occasionally lead to an increased intracranial pressure.     

CSF hypotension: A review of its manifestations,investigation and management

Intracranial cerebrospinal fluid (CSF) hypotension usually arises in the context of known or suspected leak of CSF. This can be spontaneous, or due to central nervous system trauma or dural defects created during lumbar puncture or epidural anaesthesia. Spontaneous intracranial hypotension (SIH) is increasingly being recognised as a cause for orthostatic headache or spontaneous subdural haematoma where no other obvious cause is found. We review CSF physiology, the mechanism of symptom generation in CSF hypotension and the investigation and management of the syndrome. Whilst commonly mild and self-limiting, CSF hypotension may result in life threatening complications and is most often treatable. When the syndrome is severe, prolonged or complicated, epidural blood patching (EBP) is the mainstay of treatment.     

自发性低颅压症的影像学特征及硬膜外注液治疗的效果(附1例报告)

目的研究自发性低颅压症(SIH)的影像学特征及硬膜外注液治疗的效果。方法回顾性分析1例SIH患者的临床资料。结果本例患者临床表现体位性头痛伴恶心、呕吐,腰穿脑脊液(CSF)压力测不出。头颅MRI显示有典型的硬膜下积液、硬脑膜的弥漫性增强、静脉窦充血扩张、垂体充血增大、脑组织下移。高分辨率CT脊髓造影精确显示CSF漏出点。于硬膜外腔注入生理盐水15ml治疗,头痛症状缓解。结论SIH的影像学特征为静脉窦扩张、硬脑脊膜强化、硬膜下腔积液;硬膜外注液治疗的效果好。     

Normal pressure form of the spontaneous intracranial hypotension: a case report with pituitary enlargement and asymptomatic pituitary haemorrhage

Spontaneous intracranial hypotension is a rare condition caused by spontaneous cerebrospinal fluid leak and volume depletion. It is initially misdiagnosed as a cause of an orthostatic headache, which is the most important symptom of the syndrome. It can be presented as one of four types: classic form, normal pressure form, normal pachymeninges form and acephalgic form. The diagnosis is made based on the clinical presentation, physical examination, typical cerebrospinal fluid and magnetic resonance imaging findings. We present a case of a 29-year-old woman with uncommon normal pressure form of the spontaneous intracranial hypotension, characterized by normal cerebrospinal fluid opening pressure, and typical clinical and magnetic resonance imaging findings, including the finding of pituitary gland enlargement with asymptomatic pituitary haemorrhage as an unusual complication.     

Blood patch for spontaneous intracranial hypotension caused by cerebrospinal fluid leak at C1-2

A case of spontaneous intracranial hypotension (SIH) caused by a cerebrospinal fluid (CSF) leak at C1-2 is described. The patient, a 46-year-old gentleman, presented to the emergency department with a severe, orthostatic neck pain and occipital headache of sudden onset. He was diagnosed with SIH and admitted, but failed to respond to conservative management. Imaging studies suggested that C1-2 was the spinal level responsible for the CSF leak, and he underwent a blood patch therapy delivered via an epidural catheter inserted from C6-7. His neck pain disappeared a day after the procedure, and he remains free of symptom for more than a year. SIH with a CSF leak at the upper cervical spine may be least amenable to conventional epidural blood patch delivered from the lumbar spine. Delivery of autologous blood patch via an epidural catheter inserted from the lower cervical spine can be a safe and effective method for such patients.     

Coma resulting from spontaneous intracranial hypotension treated with the epidural blood patch in the Trendelenburg position pre-medicated with acetazolamide

A 62-year-old man had a new onset of severe, orthostatic headache which eventually progressed to a stupor and a coma 3 weeks later. A computed tomography (CT) scan showed bilateral chronic subdural haematoma and magnetic resonance imaging (MRI) of the brain showed the typical findings of spontaneous intracranial hypotension (SIH). After pre-medication with acetazolamide, he was treated with three lumbar autologous epidural blood patches (EBPs) and kept in the Trendelenburg position, with full recovery. The first lumbar autologous EBP was ineffective and the second was only partially effective because of incorrect execution of the procedure as shown by spinal neuroimaging examination post-EBP. A spinal neuroimaging examination post-EBP is therefore to be recommended in order to confirm the correct execution of procedure. Pre-medication with acetazolamide and keeping the patient in the Trendelenburg position could reduce the flow of spinal cerebrospinal (CSF) leak favouring sealing of the hole.     

Case of intracranial hypotension responsive to oral prednisolone]

A 31-year-old female patient with headache and nausea was admitted to our hospital, although there were no apparent neurological abnormalities except headache. Cerebrospinal fluid (CSF) pressure was 40 mmH2O on the first lumbar puncture and CT showed some fluid in the left maxillary sinus. She gradually developed orthostatic headache despite antibiotics, hydration and analgesics. MRI showed diffuse meningeal thickening and enhancement. CSF pressure was 0 mmH2O on the second lumbar puncture and RI cisternography demonstrated early excretion to the kidneys. She was diagnosed with intracranial hypotension due to CSF leakage. An autologous 10ml blood patch on the lumbar epidural space did not relieve the orthostatic headache. However, headache disappeared one day after oral intake of 40mg prednisolone. During the next three months, oral prednisolone was tapered off. Three months after the onset of the illness, MRI did not show either meningeal thickening or enhancement. We concluded that oral prednisolone was effective in a case of intracranial hypotension.     

原发性低颅压综合征的临床及影像学特点

目的探讨原发性低颅压综合征的临床及影像学特点。方法回顾性分析24例原发性低颅压综合征患者的临床资料。结果本组男8例,女16例;多以急性或亚急性起病。所有患者均表现为体位相关性头痛,即座位或站立位时头痛,平卧时减轻或明显缓解;头痛一般位于前额,也可位于后枕部及颞部;头痛性质均为胀痛。24例患者腰穿脑脊液检查示压力均≤60 mmH2O(1 mmH2O=0.0098 kPa)。本组24例患者行头颅CT检查发现,21例正常,2例脑肿胀,1例脑肿胀并蛛网膜下腔出血。14例患者行头颅MRI检查发现,10例脑下坠,8例静脉窦扩张(以上矢状窦扩张为主),6例脑室、脑池变小,9例硬膜下积液;4例行增强扫描检查发现,3例弥漫性硬脑膜强化。结论原发性低颅压综合征临床表现为急性或亚急性起病的体位性头痛,常伴有恶心、呕吐,脑脊液压力常≤60 mmH2O。头颅CT、MRI检查可有助诊断。     

自发性低颅压综合征31例临床特征和影像学分析

目的 探讨自发性低颅压综合征（SIH）的临床特点和影像学改变.方法 回顾性分析31例SIH患者的临床及影像资料.结果 所有患者均有体位性头痛,可伴有恶心、呕吐、头晕、耳鸣、听力丧失、行走不稳、一过性视物模糊、复视、颈肩部疼痛、颈强直 CSF压力均＜70 mmH2O,蛋白升高12例,WBC增多11例,RBC增多7例 全部患者行头颅CT检查,8例见脑肿胀,脑沟变浅,脑室变窄,临床症状消失后复查均恢复正常 15例行头颅MRI检查,8例行增强检查示硬脑膜弥漫性强化,头颅CT及MRI检查见4例硬膜下积液、2例硬膜下血肿 全部患者采用内科综合疗法,1例行硬膜下血肿清除术,均痊愈.结论 SIH的临床表现多样,体位性头痛是特征性症状,腰穿CSF及头颅MRI检查具有诊断价值 本病预后良好,但少数并发硬膜下血肿.     

Hipotensión intracraneal secundaria a fístula espinal espontánea de líquido cefalorraquídeo: Presentación de tres casos

Spontaneous intracranial hypotension syndrome (SIH) is a rare condition. The main symptom is orthostatic headache, although other symptoms such as vegetative symptoms, meningism, or focal neurological deficits may appear. The most common cause is a cerebrospinal fluid leak, usually traumatic. Spontaneous cerebrospinal fluid leaks are rare and associated with the presence of meningeal cysts / diverticula or in the setting of connective tissue diseases. The diagnosis is based on imaging tests, both to detect intracranial complications and bilateral subdural hematomas and to locate the leak point at the intracranial or spinal level. The treatment of SIH is usually conservative: bed rest, caffeine and analgesics. Epidural blood patch is a good option when symptoms persist. Surgery is indicated in refractory cases or when there is an evident and accessible anatomic defect. This article describes three clinical cases with intracranial hypotension syndrome secondary to a spontaneous spinal cerebrospinal fluid leak.     

Stroke and Death Due to Spontaneous Intracranial Hypotension

Background

Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension.

Methods

Medical records and radiologic imaging of the two patients were reviewed.

Results

Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.  Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia.

Conclusions

Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.     

Spontaneous intracranial hypotension after labor without spinal intervention

We present a 29-year-old woman admitted with severe postural headache after spontaneous term labor. Lactation ceased for the duration of headache. Magnetic resonance imaging (MRI) revealed dural thickening that is suggestive of spontaneous intracranial hypotension. CT-cisternography disclosed cervicodorsal dural leak. She was treated with a high-volume epidural blood patch (EBP) and her symptoms were relieved. Lactation returned to normal after EBP. She had normal findings on follow-up MRI examination at 6 months.     

Chronic Subdural Hematoma after Spontaneous Intracranial Hypotension : A Case Treated with Epidural Blood Patch on C1-2

Spontaneous cerebrospinal fluid (CSF) leak is a recognized cause of spontaneous intracranial hypotension (SIH). Subdural hematoma (SDH) is a serious but rare complication of SIH. An autologous epidural blood patch at the CSF-leak site can effectively relieve SIH. We report a case of bilateral SDH with SIH caused by a CSF leak originating at the C1-2 level. A 55-year-old male complained of orthostatic headache without neurological signs. His symptoms did not respond to conservative treatments including bed rest, hydration and analgesics. Magnetic resonance imaging showed a subdural hematoma in the bilateral fronto-parietal region, and computed tomography (CT) myelography showed a CSF leak originating at the C1-2 level. The patient underwent successful treatment with a CT-guided epidural blood patch at the CSF-leak site after trephination for bilateral SDH.     

Epidural blood patch in Trendelenburg position pre‐medicated with acetazolamide to treat spontaneous intracranial hypotension

Background: Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse pachymeningeal enhancement on brain magnetic resonance imaging (MRI) and low cerebrospinal fluid (CSF) pressure. Treatment ranges from conservative management, such as bed rest, overhydration and caffeine, to invasive procedures, such as the autologous epidural blood patch (EBP), computed tomography (CT)‐guided fibrin glue injection at the site of the leak and open surgical intervention. EBP has emerged as the treatment of choice for SIH when initial conservative measures fail to bring relief. Methods: Forty‐two patients with SIH were treated with lumbar autologous EBP in Trendelenburg position preceded by pre‐medication with acetazolamide. Results: A complete recovery was obtained in all patients after one (90%), two (5%) or three (5%) EBPs. After EBP, two patients (5%) also performed evacuation of bilateral chronic subdural hematoma with mass effect. Conclusions: Spontaneous intracranial hypotension can be effectively cured by lumbar autologous EBP in Trendelenburg position pre‐medicated with acetazolamide.     

Subdural effusions in the posterior fossa associated with spontaneous intracranial hypotension

BACKGROUND: Misdiagnosis of spontaneous intracranial hypotension remains a problem, despite increasing recognition. METHODS: Three patients with spontaneous intracranial hypotension presented with typical findings on lumbar puncture, magnetic resonance (MR) imaging, and radioisotope cisternography. All patients showed subdural effusions in the posterior fossa on axial T2-weighted MR imaging. Axial MR images of 112 patients with other conditions were also screened for this finding. RESULTS: One of three patients had typical orthostatic headache, and the other two had continuous headache. The finding of subdural effusions in the posterior fossa on axial T2-weighted MR imaging disappeared after treatment. Similar findings were found in 14 of 112 patients with other conditions. Most of the patients were over 60 years old or had dementia or previous radiation therapy. CONCLUSIONS: Subdural effusions in the posterior fossa can be identified by T2-weighted axial MR imaging, and are useful for the diagnosis of spontaneous intracranial hypotension and for verifying the effectiveness of treatment.