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 .     

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.     

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.     

Reversible cerebral vasoconstriction in spontaneous intracranial hypotension

Myelography showed an opening pressure of 0 cm H2O and multiple thoracic meningeal diverticula in a 52-year-old woman suffering from orthostatic headaches of instantaneous onset. MR-angiography showed severe segmental arterial stenosis of the anterior and posterior circulation, which resolved over a 4-day period following an epidural blood patch. Spontaneous intracranial hypotension should be considered in the differential diagnosis of reversible cerebral vasoconstriction.     

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.     

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 in intracranial hypotension

Abstract We describe the headache characteristics of two patients with intracranial hypotension (IH) and correlate the magnetic resonance imaging (MRI) pattern to the clinical aspects of this type of headache. The first case was that of a patient with spontaneous IH, whereas the second patient had IH following rhinorrhoea. Cerebral computed tomography (CT) performed at presentation of symptoms was normal in both patients. Cerebral MRI in the first case showed meningeal contrast enhancement and an MRI pattern consistent with a subdural hygroma, while in the second case there was only a mild meningeal thickening. The symptoms improved spontaneously in both cases in about 2–3 months, confirming that invasive manoeuvres are not mandatory in these patients.     

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.     

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。     

Upright MRI in spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

Orthostatic headaches are the hallmark of spontaneous intracranial hypotension, but MRIs are traditionally obtained in the supine position. We investigated the utility of upright MRI of the brain in 6 patients with spontaneous intracranial hypotension. No discernable differences were noted between the supine and upright images.     

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.     

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.     

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.     

Congenital predisposition to spontaneous intracranial hypotension: a case report

Dural rupture, cerebrospinal fluid leakage, and spontaneous intracranial hypotension may complicate significant or minimal spinal trauma and cause chronic headache with a positional component. While such cases typically reflect no pre-existing predilection, we encountered a patient whose cervicothoracic anatomy appeared to predispose him to this complication.     

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.     

Tracer clearance in radionuclide cisternography in patients with spontaneous intracranial hypotension

We semiquantitatively analysed radionuclide cisternography in three patients with spontaneous cerebrospinal fluid (CSF) leakage diagnosed by typical symptoms and magnetic resonance imaging findings before and several months after treatment with epidural blood patch. Radioactivity in the whole CSF space was measured immediately after and at 1, 5, 7 and 24 h after intrathecal injection of (111)In-diethylenetriaminepentaacetic acid (DTPA). Initial findings included the vague appearance of leakage in the thoracic spine in two patients, early bladder filling at 1 h in one and a lack of tracer filling into the high cranial convexity in all three. The radioactivity count rapidly decreased within several hours after injection and reached 20% of the initial value at 24 h. In contrast, no rapid decrease was observed after treatment and more than 50% of tracer remained at 24 h after injection. Semiquantitative analysis of tracer clearance may provide additional information in the diagnosis of CSF leakage, especially with no obvious qualitative findings.