Spontaneous intracranial hypotension: case report

Spontaneous intracranial hypotension is a well defined entity, however it is characterised by a relevant clinical heterogeneity. The main feature is orthostatic headache, often accompanied by other symptoms. The diagnosis is easily made in the presence of orthostatic headache, excluding an obvious aetiology of low cerebrospinal fluid (CSF) pressure, and often there is no evidence of direct or indirect CSF leak.     

Atypical Spontaneous Intracranial Hypotension With a Head‐Shaking Headache

Spontaneous intracranial hypotension (SIH) is typically characterized by orthostatic headache; however, various atypical manifestations of SIH have been reported recently. We report here the case of a 46‐year‐old man with headache secondary to SIH, which was nonorthostatic, triggered only when the patient shook his head. We suggest that SIH should be suspected in patients with headache induced by head‐shaking, even without orthostatic features, especially when the headache is accompanied by other symptoms commonly associated with SIH.     

Pathological contrast enhancement of the oculomotor and trigeminal nerves caused by intracranial hypotension syndrome

The typical symptom of intracranial hypotension syndrome is orthostatic headache. The headache may also be accompanied by neck pain and stiffness, low backache, radicular symptoms, quadriplegia, interscapular pain, nausea/vomiting, and cranial nerve involvement symptoms (hearing and visual problems, face pain and numbness, hypogeusia). Radiologically, on cranial magnetic resonance imaging, intracranial hypotension syndrome is characterized by dural thickening and contrast enhancement, subdural effusion, engorgement of the venous structures, sagging or downward displacement of the brain, and pituitary hyperemia. Although clinical findings related to cranial nerves 3 and 5 have been described in intracranial hypotension, pathological contrast enhancement of these nerves has not. We present a 32-year-old patient whose cranial magnetic resonance imaging shows bilateral pathological contrast enhancement of cranial nerves 3 and 5 and describe a new imaging finding in intracranial hypotension syndrome.     

Spontaneous intracranial hypotension syndrome: report of twelve cases

PURPOSE: To investigate clinical, MRI, and radioisotope findings and therapeutic outcome of the syndrome of spontaneous intracranial hypotension (SIH). BACKGROUND: Spontaneous intracranial hypotension is characterized by orthostatic headache, low CSF pressure, and MRI findings of diffuse pachymeningeal gadolinium enhancement without previous history of head trauma or lumbar puncture. Spontaneous CSF leakage from a spinal dural tear has been suggested as the underlying pathogenic mechanism of SIH. Most patients recover without sequelae, but subdural collections have been described in a few. METHODS: Twelve consecutive patients (10 females, 2 males, mean age 39 years) with headache related to the syndrome of spontaneous intracranial hypotension were investigated. RESULTS: Eleven patients presented orthostatic headache, one patient had continuous nonpostural headache. Additional clinical symptoms included nausea, vomiting, tinnitus, diplopia, and back pain. All the patients had low CSF opening pressure, seven had increased CSF albumin, and four had pleocytosis. Brain MRI showed diffuse pachymeningeal gadolinium enhancement. Other features included subdural fluid collections (hematoma/hygroma) in four patients, downward displacement of the brain in four patients, and enlargement of the pituitary gland in one patient. Radioisotope cisternography results indicated, in two patients, a CSF leakage site in the cervico-thoracic region, and in one patient showed limited ascent of the tracer to the cerebral convexity and early appearance of radioisotope in the bladder. All the patients had complete resolution of headache with conservative treatment. CONCLUSIONS: Patients with SIH have distinct MRI and sometimes radioisotope cisternographic abnormalities and generally respond favorably to conservative management.     

Filum ependymoma mimicking spontaneous intracranial hypotension

A 34-year-old man with a 2-week history of orthostatic headaches and a "dry tap" at lumbar puncture was found to have a lumbar intradural mass on magnetic resonance imaging (MRI) examination. A myxopapillary ependymoma was resected and the patient's headache completely resolved. The combination of spontaneous orthostatic headaches and a "dry tap" at the time of lumbar puncture does not always indicate the presence of a spontaneous cerebrospinal fluid (CSF) leak and intracranial hypotension.     

Spontaneous intracranial hypotension syndrome in a patient with marfan syndrome and autosomal dominant polycystic kidney disease

Intracranial hypotension is typically manifested by orthostatic headache. The most frequent underlying factor is cerebrospinal fluid leakage. It has been suggested that dural structural weakness in some connective tissue diseases may be responsible for dural tears and diverticula and consequently leakage. We present a case of spontaneous intracranial hypotension associated with Marfan syndrome and autosomal dominant polycystic kidney disease. The patient was treated successfully with epidural autologous blood patch. Dural involvements of these hereditary connective tissue diseases are also discussed.     

原发性低颅压综合征的临床表现与MRI诊断

目的：探讨原发性低颅压综合征的临床与MRI表现。方法：分析15例原发性低颅压综合征患者的临床资料、脑脊液生化及MRI表现。结果：15例患者均有体位性头痛,伴有或不伴有其他神经系统症状。脑脊液检查,脑脊液压力均低于70mmH20（1mmHzO=0．0098kPa）,生化检查9例正常。MRI检查硬脑膜均匀性增厚强化。经临床积极治疗,预后良好。结论：体位性头痛是低颅压综合征特征性临床表现,腰椎侧卧位穿刺脑脊液压力测量及头颅MRI检查具有诊断价值,临床积极治疗预后良好。     

Patulous Eustachian tube in spontaneous intracranial hypotension syndrome

A 48-year-old slender woman presented with a rare case of spontaneous intracranial hypotension syndrome manifesting as patulous Eustachian tube. The patient complained of sudden onset of ear fullness and nasal voice as well as typical orthostatic headache. Patulous Eustachian tube was identified by observation of synchronous movement of the tympanic membrane with respiration and swallowing. The diagnosis of spontaneous intracranial hypotension was confirmed by negative cerebrospinal fluid pressure, and typical magnetic resonance imaging and radioisotope cisternography findings. All symptoms completely resolved within a few days after epidural blood patch treatment. Changes in the venous blood distribution led by collapse of the dural sac of the cervical spine in the standing position presumably caused decreased size of the pterygoid venous plexus around the Eustachian tube.     

My headache does not get better when I lie down: spontaneous intracranial hypotension complicated by venous thrombosis

Two patients with spontaneous intracranial hypotension (SIH) subsequently developed cerebral venous thrombosis (CVT) when their headache lost its distinctive orthostatic characteristic. In addition to typical MRI features of tonsillar descent, subdural fluid collections, and diffuse pachymeningeal enhancement, hyperintense thrombi were detected in the dural sinuses, and filling defects on magnetic resonance venography. Knowledge of the typical neuroimaging features of SIH and CVT and change in headache characteristics are important for prompt diagnosis and treatment.     

Orthostatic headache and bilateral abducens palsy secondary to spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is a well-documented syndrome characterized typically by a benign, self-limited course. Patients typically present with postural or exertional headaches that can be temporarily relieved by lying in a supine or recumbent position. A 35-year-old Caucasian male suffered orthostatic headache that developed to a bilateral abducens palsy. We ordered relative rest and the patient improved and completely recovered after 3 months. Although SIH is considered as a benign and self limited process it could also be associated with disabling complications. We should be aware of the possible complications and inform our patients. SIH can present with headache and bilateral abducens palsy even when the headache is improving.     

Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective

The clinical and radiographic manifestations of spontaneous intracranial hypotension are highly variable and many patients do not satisfy the 2004 International Classification of Headache Disorders criteria. We developed new diagnostic criteria for spontaneous intracranial hypotension based on cases we have seen reflecting the variable manifestations of the disorder. These criteria provide a basis for change when the classification criteria are next revised. The diagnostic criteria consist of A, orthostatic headache; B, the presence of at least one of the following: low opening pressure (≤ 60 mm H(2) O), sustained improvement of symptoms after epidural blood patching, demonstration of an active spinal cerebrospinal fluid leak, cranial magnetic resonance imaging changes of intracranial hypotension (eg, brain sagging or pachymeningeal enhancement); C, no recent history of dural puncture; and D, not attributable to another disorder.     

Spontaneous intracranial hypotension--a case report

Positional or orthostatic headache symptoms secondary to intracranial hypotension are usually seen as complications in patients after medical procedures, such as lumbar puncture. We describe a case of spontaneous intracranial hypotension (SIH) in a 43-year-old man without a previous history of headaches. He presented to the Emergency Department (ED) with a new-onset orthostatic headache without any history of recent medical procedure, such as lumbar puncture (LP). The ED evaluation included a normal neurologic examination and normal computed tomography (CT) scan of the brain. An LP showed low opening pressure, elevated protein, 46 RBCs and one lymphocyte. Subsequent evaluation with radionuclide cisternography confirmed a cerebrospinal fluid (CSF) leak in the area of the upper thoracic spine, and treatment with an epidural blood patch dramatically improved his symptoms. SIH is due to CSF leakage, usually in the area of the cervical or upper thoracic spine, often without a clear etiology. Conservative medical management including bed rest, oral hydration and caffeine intake is the usual first line treatment. An epidural blood patch is often a dramatically effective treatment that can be done once the presence and location of the leak is identified. Although mostly benign, this condition occasionally can be associated with the formation of clinically significant subdural fluid collections or hematomas. SIH is an increasingly recognized cause of headache.     

Low-Pressure/Spinal Fluid Leak Headache

Low cerebrospinal fluid (CSF) pressure results in neurologic deficits, of which the most common manifestation is headache. Typically, the headache is postural – and specifically, orthostatic – in presentation. There are three hypotheses to explain the occurrence of headache associated with low CSF fluid. The first is traction on pain-sensitive intracranial and meningeal structures; the second is CSF hypovolemia; and the third is spinal loss of CSF resulting in increased compliance at the caudal end of the CSF space. Spontaneous intracranial hypotension (SIH), once believed to be rare, is now more commonly recognized. It is typically associated with orthostatic headache (although initially it may not be) and one or more other symptoms such as alterations in hearing, nausea, vomiting, neck stiffness, diplopia, and visual field cuts. Magnetic resonance imaging (MRI) of the brain with gadolinium is the first study of choice, which typically reveals diffuse pachymeningeal enhancement and, frequently, cerebellar tonsillar descent and posterior fossa crowding. Epidural blood patch (EBP) is the treatment of choice. Surgery and epidural fibrin glue injection are options for those who fail conservative therapy and/or EBP.     

原发性低颅压综合征13例临床分析

目的 探讨原发性低颅压综合征的临床表现、脑脊液和影像学特点.方法 回顾分析13例临床诊断明确的原发性低颅压综合征患者的临床表现、脑脊液及影像资料.结果 所有患者均有体位性头痛,并可伴有恶心、呕吐、头晕、复视及颈强直;CSF压力均<60 mm H2O,蛋白升高5例、白细胞增多8例、红细胞增多9例;均行头CT检查,4例分别出现硬膜下积液和脑室变小;2例头颅MRI增强检查示弥漫性硬脑膜强化;均采用保守治疗,均痊愈.结论 体位性头痛是原发性低颅压综合征最典型的临床症状,硬脑膜弥漫性强化是最常见的影像学表现,CSF容量减少是原发性低颅压综合征临床及影像学表现的病理生理基础.     

Spontaneous Intracranial Hypotension‐Hypovolemia Associated With Tacrolimus

There is little precedent for a medication‐induced spontaneous intracranial hypotension/cerebrospinal fluid (CSF) hypovolemia (SIH). This case history of a woman with low CSF pressure, orthostatic headache, and radiographic findings consistent with SIH but without a detectable leak was notable for its association, both onset and resolution, with the use of the calcineurin inhibitor tacrolimus (FK506). A literature review for potential causes of a tacrolimus‐induced CSF hypotension suggests many potential mechanisms of action, including effects on blood brain barrier and dural compliance, and supports further vigilance for this condition in the medically complex setting of tacrolimus use.     

特发性低颅压头痛的临床及影像学特点

目的:探讨特发性低颅压头痛的临床及影像学特点.方法:回顾性分析29例特发性低颅压头痛患者的临床表现、腰椎穿刺结果、头颅MRI特征以及治疗方法.结果:28例患者存在体位性头痛,卧位时缓解,1例患者出现体位性头晕;28例腰穿压力低,其中压力为0 mm H2O者9例,蛋白增高是较常见的脑脊液改变;26例行头颅MRI检查,其中16例可见异常,9例为硬膜下出血或积液,9例为硬脑膜强化;除1例患者因硬膜下出血转至外科手术治疗外,其余均经保守治疗后痊愈.结论:熟悉特发性低颅压头痛的临床特点、脑脊液改变和头颅MRI特征,有利于早期诊断和治疗.     

Epidural Blood Patch for the Treatment of Abducens Nerve Palsy due to Spontaneous Intracranial Hypotension -A Case Report-

Intracranial hypotension is characterized by a postural headache which is relieved in a supine position and worsened in a sitting or standing position. Although less commonly reported than postural headache, sixth nerve palsy has also been observed in intracranial hypotension. The epidural blood patch (EBP) has been performed for postdural puncture headache, but little is known about the proper timing of EBP in the treatment of sixth nerve palsy due to intracranial hypotension. This article reports a case of sixth nerve palsy due to spontaneous intracranial hypotension which was treated by EBP 10 days after the onset of palsy.     

The syndrome of spontaneous intracranial hypotension

The authors report four cases of headache and other symptomatology related to the syndrome of intracranial hypotension. They were seen in a routine clinical practice over the past 3 years. The clinical features, magnetic resonance imaging (MRI) findings, and follow-up of these patients are described. Review of the prior literature on the topic is also included. All four patients presented with orthostatic headache syndrome. Three of the four demonstrated diffuse leptomeningeal thickening and enhancement on MRI studies. One subsequently developed a subdural effusion. One patient demonstrated downward displacement of the posterior fossa initially, which resolved on follow-up MRI scanning. Possible pathophysiologies of the syndrome are discussed.     

Bilateral subdural hematomas following routine lumbar diskectomy

Intracranial hypotension is a rare, and possibly underrecognized, cause of headache in middle age. Occurring spontaneously in the vast majority of cases, it has been occasionally reported after certain neurosurgical procedures involving craniectomy. We report a unique situation in which a patient developed severe postural headache typical of intracranial hypotension, which was complicated by bilateral subdural hematomas, immediately following a routine lumbar diskectomy; the headache resolved spontaneously. We suggest that an intraoperative microscopic dural breach was the site of sustained, but self-limited, cerebrospinal fluid leakage that eventually led to intracranial hypotension.     

Cranial Magnetic Resonance Imaging in Spontaneous Intracranial Hypotension after Epidural Blood Patch

▪ Abstract:   Spontaneous intracranial hypotension (SIH) is a syndrome characterized by orthostatic headache, nausea, vomiting, photophobia, and diplopia. Subdural effusion, diffuse dural enhancement, dilatation of epidural veins, and increased height of hypophysis are cranial magnetic resonance (MR) imaging findings in SIH. Epidural blood patch is reportedly one of the effective treatment options. We present the follow-up MR imaging findings in a case of SIH after a successful epidural blood patch treatment. We propose that cranial MR imaging as an objective test to evaluate the success of epidural blood patch treatment. ▪