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.     

Atypical spontaneous intracranial hypotension (SIH) with nonorthostatic headache

BACKGROUND: Some patients with spontaneous intracranial hypotension (SIH) often do not demonstrate typical orthostatic headache, which is contrary to the typical SIH syndrome. They usually have an obscure and intermittent headache, regardless of their positional change. OBJECT: The objective of this study is to investigate the clinical course of atypical SIH that manifests with diffuse pachymeningeal enhancement, but no orthostatic headaches. METHODS: Between January 1997 and December 2005, we observed a total of 6 patients who revealed atypical presentations including nonpostural headaches and normal cerebrospinal fluid (CSF) pressure, despite the diffuse pachymeningeal enhancement seen on their MR images. For a comparison of the clinical features and the disease course, 13 other SIH patients with typical clinical manifestations were selected as a control group. RESULTS: Cerebrospinal fluid leakage sites were confirmed in only one patient through a variety of diagnostic tools; in contrast, definite focal CSF leakage sites were found in 12 of 13 patients with typical SIH. The 6 atypical SIH patients were treated with conservative treatment, including strict bed rest and intravenous hydration for 2 to 3 weeks. After a mean follow-up of 12 months, their headaches were gradually relieved after 2 to 3 weeks of conservative treatment only. CONCLUSIONS: All SIHs do not necessarily show the typical clinical manifestations. The atypical finding of SIH such as nonorthostatic headache or normal CSF pressure may be the result of a normal physiologic response to the typical SIH as a compensatory reaction. Therefore, when faced with patients showing findings compatible with SIH on the brain MR images, regardless of nonpostural headache, atypical SIH should be suspected.     

Spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is an increasingly recognized syndrome. Postural headache with typical findings on magnetic resonance imaging (MRI) are the key to diagnosis. Delay in diagnosing this condition may subject patients to unnecessary procedures and prolong morbidity. We describe a patient with SIH and outline the important clinical and radiographic features of this syndrome. Headache due to SIH is similar to headache occurring after lumbar puncture. Patients with postural headaches should have brain MRI before lumbar puncture. Meningeal abnormalities with typical clinical features are helpful in establishing the diagnosis. When correctly diagnosed, SIH management, in most cases, is easy and highly effective.     

Treatment Outcomes in Spontaneous Intracranial Hypotension: Do Epidural Blood Patches Stop the Leaks?

Abstract:   Spontaneous intracranial hypotension (SIH) is a postural headache syndrome unrelated to dural puncture. Because of the increasing failure of epidural blood patch (EBP) to relieve headache in SIH, we retrospectively investigated the epidemiological features and treatment outcomes in 55 cases of SIH. The study population was stratified by age and sex; continuous variables were compared for differences by t -tests; categorical variables were compared by Chi-squared analysis or Fisher exact tests. Significant differences were identified by P values of 0.05 or less. The mean age of the study population was 44 ± 12 years with a female to male ratio of 1.3:1.0. Men presented with subdural hematomas ( P  = 0.001) more often than women. Meningeal enhancement on contrast magnetic resonance imaging (MRI) was the most consistent radiographic finding. Radionuclide cisternography (RC) demonstrated thoracolumbar dural leaks in 16 of 22 patients. EBP failures were more common in patients aged 40 and younger than in older patients ( P  = 0.003). Postural headache from SIH was not uniformly responsive to EBP, and had significant comorbidities, especially in men. The management of postural headache in SIH by other techniques to restore brain position and cerebrospinal fluid dynamics should be investigated.     

A case of spontaneous intracranial hypotension with typical magnetic resonance images

Spontaneous intracranial hypotension (SIH) is a rare syndrome defined by postural headache, associated with a low cerebrospinal fluid pressure, without history of previous dural trauma or invasive treatment on rachis. We reported a case of a patient with postural headache caused by SIH identified by magnetic resonance images and treated with saline solution infusion with complete remission of symptoms.     

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.     

Headache Due to Spontaneous Intracranial Hypotension and Subsequent Cerebral Vein Thrombosis

Cerebral vein thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). When to suspect a thrombotic disorder during the course of intracranial hypotension is not fully elucidated. A 48‐year‐old woman was admitted because of SIH with no signs of CVT on neuroimaging. The occurrence of diplopia and blurred vision 12 days later led to the performance of further investigations, which revealed thrombosis of the left lateral sinus, in the absence of variations in the headache characteristics. Among the other 4 cases of SIH clearly preceding the occurrence of CVT reported so far, only one had a change in the headache pattern related to CVT development. Although a change in the characteristics of headache is considered a marker of CVT in patients with SIH, this is not invariably part of the clinical scenario. Any new neurologic finding on exam in the disease course should raise a suspicion of venous thrombosis, thus prompting further specific investigations.     

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.     

Subdural haematoma in patients with spontaneous intracranial hypotension

The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.     

Application of IHS criteria to headache attributed to spontaneous intracranial hypotension in a large population

We applied the recent International Headache Society (IHS) criteria for headache related to spontaneous intracranial hypotension (SIH) to 90 consecutive patients with a final diagnosis of SIH confirmed by cerebral magnetic resonance imaging with contrast. Orthostatic headache (developing within 2 h of standing or sitting up) was present in 67 patients (75%) but appeared within 15 min after standing or sitting—as required by point A of the criteria—in only 53 (59%). Forty-four (49%) patients did not satisfy point A, including 22 (24%) with non-orthostatic headache and 14 (16%) with headache developing ≥ 15 min after standing or sitting up; 80 (89%) did not satisfy point D. Only three (3%) patients had headache fully satisfying the IHS criteria. These findings indicate that the current IHS criteria do not capture most patients with SIH-associated headache. Excluding the requirement for response to epidural blood patch (criterion D) and considering headaches appearing within 2 h of sitting or standing up would capture more patients.     

Recurrence of spontaneous intracranial hypotension with subdural hematomas

A 59-year-old man developed postural headache associated with a low CSF pressure. A CT scan revealed no abnormal findings and the orthostatic symptoms resolved without treatment 6 weeks after onset. He was diagnosed as having spontaneous intracranial hypotension (SIH) and remained symptom-free until he experienced recurrence of postural headache 9 months later. A lumbar puncture demonstrated low CSF pressure, and a CT scan revealed slit-like ventricles with narrowing of the sulci, Sylvian fissures, and infratentorial cisterns, in addition to bilateral subdural masses. After draining the hematomas, his symptoms resolved completely, and a follow-up CT scan was normal. We hypothesize that recurrent SIH in this case was due to small recurrent tears of a root sleeve. This case emphasizes the importance of follow-up of SIH for at least 9 months after resolution of symptoms.     

Spontaneous intracranial hypotension: a cause of severe acute headache

Severe acute headache is a common presenting symptom to an accident and emergency department. Spontaneous intracranial hypotension (SIH) is an increasingly recognised cause of these symptoms and has characteristic clinical and imaging findings. SIH is characterised by headache worse on standing, low opening cerebrospinal fluid pressures at lumbar puncture and uniform pachymeningeal enhancement with gadolinium enhanced magnetic resonance imaging of the brain, all in the absence of dural trauma. Atypical presentations occur and severe neurological decline can rarely be associated with this condition. A review of five patients presenting recently to our institution with classical imaging findings together with a review of the literature is presented.     

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.     

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.     

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.     

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. ▪     

Spontaneous intracranial hypotension with pituitary adenoma

Spontaneous intracranial hypotension (SIH) is an unusual syndrome that is characterised by positional headache, neck rigidity, nausea and vomiting. The characteristic magnetic resonance imaging (MRI) findings are diffuse smooth pachymeningeal thickening and enhancement, downward displacement of posterior fossa structures and pituitary gland enlargement. An unusual case of SIH with pituitary macro–adenoma and subsequent subdural haemorrhage is presented, and its clinical picture, MRI findings and possible pathophysiological mechanism are discussed. An erratum to this article is available at .     

MR增强在自发性低颅压诊断和鉴别诊断中的应用价值

目的探讨MRI增强扫描在自发性低颅压诊断和鉴别诊断中的应用价值。方法回顾性分析经腰穿测压证实的9例自发性低颅压患者的临床和MRI表现。结果本组9例自发性低颅压患者中7例具有特征性的体位性头痛。MRI平扫表现为脑下垂6例次：垂体增大5例次;硬膜下积液4例次;硬膜下出血3例次,增强扫描9例均见弥漫性硬脑膜强化。结论自发性低颅压的MRI平扫表现较有特征,增强扫描显示硬脑膜弥漫强化对本病的诊断和鉴别诊断很有价值。     

Spontaneous intracranial hypotension: case report and relevant review of the literature

Spontaneous intracranial hypotension (SIH) is a well-defined clinical entity that is frequently misdiagnosed because little information regarding this type of headache exists in the Emergency Medicine literature. This report presents a case and relevant review of the literature on SIH. An understanding of the characteristic symptomatology, appropriate evaluation, treatment options, and prognosis is essential if the emergency physician is to provide the most appropriate care to these patients.     

Spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is caused by leakage of cerebrospinal fluid (CSF), with resultant CSF hypovolemia and intracranial hypotension. Although in some patients SIH may be preceded by minor trauma, it often occurs in the absence of any identifiable initiating event. Orthostatic headache is the primary clinical manifestation, usually accompanied by one or more other symptoms of SIH. Diagnosis can be reached by a combination of imaging studies, which may include MRI with gadolinium, nuclear cisternography, and myelography. Treatment ranges from conservative management, such as bed rest and hydration, to invasive procedures, such as lumbar puncture with autologous blood patch, CT-guided fibrin glue injection at the site of the leak, and open surgical intervention. Outcomes vary from complete resolution of CSF leak with alleviation of symptoms to continued and/or recurrent leaks with chronic unremitting symptomatology.