Research Paper: Spontaneous Intracranial Hypotension -Not Always Benign (original) (raw)
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JAMA Neurology
IMPORTANCE Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain. OBJECTIVE To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment outcomes for SIH. DATA SOURCES Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline-compliant systematic review and meta-analysis of the literature on SIH. Three databases were searched from inception to April 30, 2020: PubMed/MEDLINE, Embase, and Cochrane. The following search terms were used in each database: spontaneous intracranial hypotension, low CSF syndrome, low CSF pressure syndrome, low CSF volume syndrome, intracranial hypotension, low CSF pressure, low CSF volume, CSF hypovolemia, CSF hypovolaemia, spontaneous spinal CSF leak, spinal CSF leak, and CSF leak syndrome. STUDY SELECTION Original studies in English language reporting 10 or more patients with SIH were selected by consensus. DATA EXTRACTION AND SYNTHESIS Data on clinical presentation, investigations findings, and treatment outcomes were collected and summarized by multiple observers. Random-effect meta-analyses were used to calculate pooled estimates of means and proportions. MAIN OUTCOMES AND MEASURES The predetermined main outcomes were the pooled estimate proportions of symptoms of SIH, imaging findings (brain and spinal imaging), and treatment outcomes (conservative, epidural blood patches, and surgical). RESULTS Of 6878 articles, 144 met the selection criteria and reported on average 53 patients with SIH each (range, 10-568 patients). The most common symptoms were orthostatic headache (92% [95% CI, 87%-96%]), nausea (54% [95% CI, 46%-62%]), and neck pain/stiffness (43% [95% CI, 32%-53%]). Brain magnetic resonance imaging was the most sensitive investigation, with diffuse pachymeningeal enhancement identified in 73% (95% CI, 67%-80%) of patients. Brain magnetic resonance imaging findings were normal in 19% (95% CI, 13%-24%) of patients. Spinal neuroimaging identified extradural cerebrospinal fluid in 48% to 76% of patients. Digital subtraction myelography and magnetic resonance myelography with intrathecal gadolinium had high sensitivity in identifying the exact leak site. Lumbar puncture opening pressures were low, normal (60-200 mm H 2 O), and high in 67% (95% CI, 54%-80%), 32% (95% CI, 20%-44%), and 3% (95% CI, 1%-6%), respectively. Conservative treatment was effective in 28% (95% CI, 18%-37%) of patients and a single epidural blood patch was successful in 64% (95% CI, 56%-72%). Large epidural blood patches (>20 mL) had better success rates than small epidural blood patches (77% [95% CI, 63%-91%] and 66% [95% CI, 55%-77%], respectively). CONCLUSIONS AND RELEVANCE Spontaneous intracranial hypotension should not be excluded on the basis of a nonorthostatic headache, normal neuroimaging findings, or normal lumbar puncture opening pressure. Despite the heterogeneous nature of the studies available in the literature and the lack of controlled interventional studies, this systematic review offers a comprehensive and objective summary of the evidence on SIH that could be useful in guiding clinical practice and future research.
Annals of Indian Academy of Neurology, 2013
A 35 year old Chinese man experienced severe generalized headache over a couple of days. The headache was throbbing in nature and maximum in the posterior parietal regions. In addition, he also complained of double vision, neck pain and dizziness; however, denied having nausea or vomiting. The headaches were aggravated when he assumed an upright posture (sitting or standing) and alleviated when lying down. He did not have preceding history of strenuous physical activities such as heavy weight lifting, trauma or a lumbar puncture procedure. Neurological examination revealed bilateral mild restriction of lateral gaze (likely due to VI nerve involvement), which resulted in binocular diplopia on extreme horizontal gaze. His brain computed tomography with contrast was normal.MRI brain showed prominence of cortical veins and significant distension of dural venous sinuses raising the possibility of pachymeningitis without venous sinus thrombosis [Figure 1 a,b]. In addition, cervical spine MRI revealed engorged anterior epidural venous plexus at cranio cervical junction and upper cervical spine, with mild effacement of the subarachnoid space, raising the possibility of spontaneous intracranial hypotension secondary to cerebrospinal fluid (CSF) leak; however, no nerve root diverticula was noted. Repeated lumbar puncture (LP) failed to extract any CSF. Subsequently, LP under fluoroscopic guidance confirmed a low CSF pressure (5 cm of water). The routine CSF investigations for infective and inflammatory markers were all negative.
Misdiagnosis of Spontaneous Intracranial Hypotension as a Risk Factor for Subdural Hematoma
Headache, 2017
This study aimed to evaluate the association between misdiagnosis of spontaneous intracranial hypotension (SIH) and subdural hematoma development. Although SIH is more prevalent than expected and causes potentially life-threatening complications including subdural hematoma (SDH), the association between misdiagnosis of SIH and SDH development is not yet evaluated. Retrospective observational study was conducted between January 1, 2005, and December 31, 2014. Adult patients with spontaneous intracranial hypotension (age ≥ 18 years) were enrolled. Of the 128 patients with SIH, 111 (86.7%) were in no SDH group and 17 (13.3%) were in SDH group. Their clinical presentation did not show significant different between the two groups, except age, the days from symptom onset to correct diagnosis, and the number of misdiagnoses. Age (odds ratio [OR], 1.15; 95% confidence interval [CI], 1.07-1.23) and the number of times SIH was misdiagnosed (OR, 1.82; 95% CI, 1.03-3.21) were independent risk f...
Journal of the Chinese Medical Association : JCMA, 2012
A 36-year-old male had spontaneous intracranial hypotension (SIH) presenting with refractory headache for 4 months. Multiple epidural blood patches (EBPs) yielded relief of symptoms, but the course was complicated, with asymptomatic intracranial subdural hematoma (SDH). Except for SDH, other radiological diagnostic signs of SIH were resolved and the patient's headaches improved after EBP. Owing to a mass effect and persistent cerebrospinal fluid (CSF) leakage, surgical repair of the spinal leakage was performed, but no cranial procedures were carried out. Postoperatively, the SDH completely resolved, but there was still CSF leakage at the level where surgery was performed. The patient has remained free of headache or other events for 3 years. It was reduction rather than elimination of the spinal CSF leak that yielded remission of SIH. In summary, intracranial SDH can be a complication of inadequately treated SIH (i.e. persistent minor CSF leakage). Management of SDH should focus on correction of the underlying SIH rather than craniotomy for hematoma evacuation.
2014
Alt›nc› sinir parezisi ile baflvuran, ortostatik bafl a¤r›s› tan›mlamayan ve spontan intrakraniyal hipotansiyon saptanan 2 hastada, alt›nc› sinir parezisi tedavisiz düzelirken, manyetik rezonans görüntülemelerinde subdural hematom saptanm›flt›r. Ortostatik bafl a¤r›s›n›n olmamas› ve alt›nc› sinir parezisi düzelirken, subdural hematom geliflmesi gibi özellikleri nedeniyle olgular bildirilmeye de¤er bulunmufltur.
Surgical neurology, 2008
Spontaneous intracranial hypotension is an increasingly recognized cause of new-onset, daily, persistent headaches. Although these headaches are similar to post-lumbar puncture headaches, characteristic differences include intracranial pachymeningeal enhancement, subdural fluid collections, and downward displacement of the brain. The identification of upper cervical epidural fluid collections as a false localizing sign in patients with SIH has provided significant insight into the selection of management options. We review a case of a 57-year-old woman who presented to our institution with progressive orthostatic headaches relieved by recumbency. The patient had no recent history of lumbar puncture, spinal, or intracranial procedure. The patient isolated the onset of symptoms to 3 weeks prior, when she was lifting heavy items in her home, and was diagnosed with SIH. Subsequently, she was found to have a C1-C2 epidural fluid collection. After much diagnostic consideration and review ...
The Journal of international medical research, 2016
Spinal leakage of cerebrospinal fluid (CSF) is considered to be the primary cause of spontaneous intracranial hypotension (SIH). Subdural haematoma (SDH) is a serious complication of SIH. This current report presents a case of bilateral SDH with SIH that was treated with epidural blood patching (EBP). A 43-year-old male complained of experiencing orthostatic headaches for 2 months without neurological signs. The patient worsened in a local hospital and was transferred to the Sir Run Run Hospital. Brain computed tomography showed bilateral SDH with a midline shift. The patient underwent emergency trephination in the left frontal temporal region. Postoperative magnetic resonance myelography showed a CSF leak originating at the T11-L2 level. As a consequence of clinical deterioration of the patient, EBP was subsequently performed at the T12-L1 level. The headache was rapidly relieved and later the SDH was completely absorbed. This case report and literature review aims to remind clinic...