A review of five patients presenting recently to our institution with classical imaging findings together with a review of the literature is presented. [ncbi.nlm.nih.gov]

(February 16, 2017) Spontaneous Intracranial Hypotension Presenting as a "Pseudo-Chiari 1". [cureus.com]

If both are present, then other pathogens need not be investigated. [neurologyadvisor.com]

Case Presentations 12 Noon Q&A 12:15 P.M. [radiology.duke.edu]

However even with the judicious use of these criteria, diagnosis of SIH is still thwarted by an atypical presentation without the classic postural headache. [practicalneurology.com]

• Hunting

  Akbar JJ, Luetmer PH, Schwartz KM, Hunt CH, Diehn FE, Eckel LJ. The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. [rarediseases.org]

  Akbar JJ, Luetmer PH, Schwartz KM, Hunt CH, Diehn FE, Eckel LJ (2012) The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. [thejournalofheadacheandpain.biomedcentral.com]

• Marfanoid Habitus

  habitus).[ 10 ] In spite of the different mechanisms each supports, all of these studies link structurally weak connective tissue to DE and dural structural abnormalities in general and, consequently, elevated risk of SIH. [surgicalneurologyint.com]

• Arachnodactyly

  Physical exam findings consistent with Marfan syndrome included dolichostenomelia and arachnodactyly, and her cardiovascular examination confirmed a mechanical aortic valve. The remainder of her exam was noncontributory. [surgicalneurologyint.com]

• Chills

  She experienced nausea with attempted sitting, but she denied photophobia, neck stiffness, fever, or chills. [anesthesiology.pubs.asahq.org]

• Hypotension

  Thoracic Epidural Blood Patches in the Treatment of Spontaneous Intracranial Hypotension: A Retrospective Case Series. Pain Physician. 2015 Aug;18(4):343–348. Mokri B. Spontaneous Intracranial Hypotension. [rarediseases.org]

  Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;295:2286–96. doi: 10.1001/jama.295.19.2286 Yuh EL, Dillon WP. Intracranial hypotension and intracranial hypertension. [ajnrdigest.org]

  Enlargement of epidural veins has been described previously in association with both spontaneous intracranial hypotension [ 20 – 23 ] and hypotension after lumbar puncture [ 24 ]. [ajronline.org]

  Early epidural blood patch in spontaneous intracranial hypotension. Neurology 2004 ; 63 : 1950 –51 Schievink WI, Meyer FB, Atkinson JL, et al. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. [ajnr.org]

  Spontaneous intracranial hypotension. Continuum (Minneap Minn) 2015 ;21(4 Headache):1086–1108. Medline, Google Scholar 2. Schievink WI, Gordon OK, Tourje J. [pubs.rsna.org]

• Focal Neurological Deficit

  No signs of meningeal irritation nor focal neurological deficits were found. [nnjournal.net]

• Cranial Neuropathy

  However, numerous other symptoms also were seen, including tinnitus, back and neck pain, nausea and vomiting, cranial neuropathy, and myelopathy. Nine of the patients responded to bed rest and blood patching. [ajronline.org]

Myth 4: Dural Enhancement Suggests a Multipathogen Workup Dural enhancement is highly specific to SIH; however, those unfamiliar with SIH imaging may consider ordering other tests to rule out infectious, granulomatous, and neoplastic disorders. [neurologyadvisor.com]

Triaging based on MRI findings instead could make sense "because brain MRI is the first imaging of choice in the workup of patients with clinical suspicion for SIH, to rule out an underlying intracranial pathology." [medscape.com]

This diagnostic confusion may consequently lead to invasive and inappropriate workup, and the brain imaging findings are not always present [ 9, 10 ]. [ajronline.org]

With this rigorous surgical workup, only a few cases (approximately 4%) showed abnormalities along the exiting nerve root, most often nude nerve roots or thin dural covering. [journals.sagepub.com]

The CT scan is the frontline imaging workup for headache, excepted the presence of the subdural hematoma, this exam can be easily interpreted as being unremarkable. [omicsonline.org]

In this study, the diagnosis and treatment results of a case of intracranial hypotension headache due to spinal cerebrospinal fluid leakage were reported. Up to now, there is not absolutely effective treatment for intracranial hypotension headache. [springerplus.springeropen.com]

Myth 10: One Treatment for SIH Is All It Takes The authors reported a high perception among clinicians that a single epidural blood patch treatment is all that is needed to completely resolve SIH. [neurologyadvisor.com]

Garcia-Carreira et al. [ 14 ] supported aggressive treatment of the underlying cerebrospinal fluid leak. [nnjournal.net]

Treatment No clear algorithm exists for the treatment of SIH. Many retrospective studies exist, but high quality evidence is lacking. Treatment recommendations are largely empiric. Conservative measures should always be used as an initial treatment. [appliedradiology.com]

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. [mayoclinic.pure.elsevier.com]

We are hoping that the SIH registry will improve our understanding of the epidemiology, diagnosis, clinical characteristics, useful imaging modalities, treatments & management, prognosis and natural history of SIH. [ucalgary.ca]

Coma due to SIH is generally reversible and the prognosis is good. But timely recognition is essential, and the index of suspicion needs to be high. Dr. Schievink told Neurology Today View Full Article [wfneurology.org]

DIAGNOSIS AND PROGNOSIS The diagnosis is suspected from the patient’s history and is then confirmed if the meninges are enhanced in an MRI of the brain. [headaches.org]

The prognosis is variable, many patients may have only mild symptoms, some patients may deteriorate with cranial nerve palsies, sinus thrombosis, large vessel strokes, or even encephalopathy and coma. 12. (1) Conservative measures for small dural defects [slideshare.net]

Although its prognosis is usually benign, cases with a rapid neurologic deterioration resulting in an altered mental status have been reported. [karger.com]

Akbar et al reported no intrathecal complications of GdM in 24 cases. 25 Recent reports are defining CSF to venous fistulas as another etiology of SIH. 3 These fistulas are suggested on cisternography by early renal activity. [appliedradiology.com]

Spontaneous intracranial hypotension remains an underdiagnosed etiology of new-onset headache. [practicalneurology.com]

Jeffrey Ross The Agony of Aliquorrhea Intracranial hypotension can occur in a variety of clinical settings and can be grouped by primary or secondary etiologies. [ajnrdigest.org]

Cases with encephalitis have special MRI findings varying due to the etiology, being the temporal lobe involvement of HSV 1 encephalitis the most common. [intechopen.com]

In order to improve clinical diagnosis and provide effective treatment, the precise etiology of spontaneous intracranial hypotension should be investigated in each patient. [springerplus.springeropen.com]

We are hoping that the SIH registry will improve our understanding of the epidemiology, diagnosis, clinical characteristics, useful imaging modalities, treatments & management, prognosis and natural history of SIH. [ucalgary.ca]

Epidemiology of cervical artery dissection. In: Baumgartner RW, Bogousslavsky J, Caso V, et al eds. Handbook of cerebral artery dissection. Basel, Germany: Karger, 200512–15. 7. Mokri B. Spontaneous low cerebrospinal pressure/volume headaches. [ncbi.nlm.nih.gov]

Critical Care and Emergency Medicine, Complementary and Alternative Medicine, Congenital Disorders, Cytology, Dentistry, Dermatology, Developmental Biology, Dietetics, Diseases of Bones and Joints, Embryology, Endocrinology, Environmental Toxicology, Epidemiology [dergipark.org.tr]

Epidemiology of secondary intracranial hypotension is variable and matches that of the underlying cause. The condition often presents as a positional headache which is relieved by lying in a recumbent position, usually within 15-30 minutes 12. [radiopaedia.org]

Schievink WI, Roiter V (2005) Epidemiology of cervical artery dissection. Front Neurol Neurosci 20:12–5 8. Schievink WI (2006) Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 295:2286–96 9. [thejournalofheadacheandpain.biomedcentral.com]

While it is clear from the literature that coma can occur in SIH, little is known about the frequency of the occurrence, correlation with other features of SIH, treatment options, outcomes, and the pathophysiological mechanism that may underlie the development [wfneurology.org]

Conclusions The understanding of SIH has evolved over the recent decades, leading to improvements in knowledge about the pathophysiology of the condition, diagnostic strategies, and expanded treatments. [headachejournal.onlinelibrary.wiley.com]

Pathophysiology in these cases is unclear. It may be related to a dilated epidural venous plexus and/or persistent/redeveloped arachnoid granulations. Treatment No clear algorithm exists for the treatment of SIH. [appliedradiology.com]

In this issue of the AJNR News Digest, we focus on the work of authors who refined the pathophysiology, imaging patterns, use of advanced imaging techniques, and treatment of intracranial hypotension and CSF leaks. [ajnrdigest.org]

Investigation of the risk factors for SDH may help to better understand its pathophysiology. Despite numerous publications [ 6, 13 – 16 ], studies on the risk factors for SDH in SIH patients are largely lacking. [journals.plos.org]

Prevention of iatrogenic risk factors Risk factors for intracranial hypotension are variable and some are avoidable. [thejournalofheadacheandpain.biomedcentral.com]

Therefore, improved therapeutic interventions are required in order to resolve such symptoms and prevent further complications. [springerplus.springeropen.com]

The presence of subdural hematoma may prevent orthostatic headaches from developing by correcting the abnormally low intracranial pressure or volume. [karger.com]

CONCLUSION: Being aware of orthostatic character of a headache and imaging findings of SIH could prevent diagnostic delay and serious consequences. [tjn.org.tr]

Phal Journal of Clinical Neuroscience. 2015; 22(12): 1973 4 Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: An update Merve Gulbiz Kartal,Oktay Algin Insights into Imaging. 2014; 5 Pulsed Estrogen Therapy Prevents Post-OVX [neurologyindia.com]