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]

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

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

Occasionally, presentation is more sinister, with reported cases of decreased level of consciousness and coma 3. [radiopaedia.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]

  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]

  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]

  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]

• Thrombosis

  […] in patients with risk factors for thrombosis. [nnjournal.net]

  ◘ Dural sinus thrombosis. ◘ Coma or encephalopathy. 5. ◘ Headache in temporal relation to low CSF pressure or CSF leakage. ◘ Either or both of the following: 1. CSF pressure < 60 mm H2O. 2. [slideshare.net]

  Isolated intracranial hypertension as the only sign of cerebral venous thrombosis. Neurology 1999;53:1537-42. [ PUBMED ] [ FULLTEXT ] 57. Leker RR, Steiner I. Features of dural sinus thrombosis simulating pseudotumor cerebri. [neurologyindia.com]

  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. [annalsofian.org]

  Cerebral venous thrombosis in spontaneous intracranial hypotension. Headache 2008 ; 48(10): 1511 – 1519. Google Scholar 57. Shih, P, Yang, BP, Batjer, HH. Surgical management of superficial siderosis. Spine J 2009 ; 9(8): e16 – e19. [journals.sagepub.com]

• Blurred Vision

  vision, diplopia and was relieved by lying down. [annalsofian.org]

  In some cases, the headache can become severe enough to cause nausea, vomiting, vertigo, photophobia, blurred vision, phonophobia, and/or tinnitus. It may also be associated with neck stiffness and pain with neck flexion. [practicalneurology.com]

  For example, altered hearing, dizziness, and vertigo are common, caused by tension on the vestibulocochlear nerve, or by transmission of abnormally low CSF pressure to the perilymph. 16 There can be blurred vision, field defects, diplopia, 17 facial pain [anesthesiology.pubs.asahq.org]

  Other cardinal symptoms reported are nausea and vomiting occurred in about half of patients [ 8, 47 ], always accompanied by neck pain stiffness [ 26 ], blurred vision [ 12 ], “visual” filed defects and diplopia [ 48 ], cough headache [ 30 ], facial pain [thejournalofheadacheandpain.biomedcentral.com]

• Visual Impairment

  Visual impairment was probably due to stretching of the cranial nerves due to downward displacement of the brain [ 12 ]. 7. Radiological manifestations of SIH CNS imaging is key in the differential diagnosis. [intechopen.com]

• Neck Pain

  A young male presented with severe orthostatic headache associated with dizziness, neck pain and diplopia. Brain imaging revealed characteristic pachymeningeal enhancement and bilateral subdural hemorrhage. [annalsofian.org]

  […] the site of the leak Symptoms of Intracranial Hypotension Symptoms of intracranial hypotension include: Positional headache, which worsens when you sit up and improves when you lie down Sensitivity to light or sound Nausea, with or without vomiting Neck [barrowneuro.org]

  Besides headache, other common symptoms of SIH include tinnitus, abnormal hearing as if underwater, neck pain, imbalance, pain between the shoulder blades, and blurred or double vision. [mdedge.com]

  It is often accompanied by tinnitus, neck pain, and intrascapular pain. [neurologyadvisor.com]

  Venous hyperemia Iter (mm) Chamb (mm) Epidural fluid collections Venous plexuses engorgement Search for point of CSF leakage 1/60F 57 OH, dizziness, hearing changes, neck pain Weakness, difficulty in swallowing liquids Frontal signs, ataxia, ideomotor [academic.oup.com]

• Osteophyte

  Computed tomography myelogram showing a dural tear due to an osteophytic spur at C7–T1. ( A ) Sagittal reformation demonstrating an osteophytic spur mildly indenting the spinal cord at C7–T1. [anesthesiology.pubs.asahq.org]

  Spontaneous intracranial hypotension due to intradural thoracic osteophyte with superimposed disc herniation: report of two cases. Eur Spine J 2012 ;21(Suppl 4):S383–S386. Crossref, Medline, Google Scholar 4. Schievink WI, Moser FG, Maya MM. [pubs.rsna.org]

  Idiopathic or spontaneous etiologies include preexisting weakness of the dural sac secondary to connective tissue disorders or meningeal diverticula, calcified disc herniations or spiculated osteophytes, and CSF-venous fistulas. 3-6 The headache associated [appliedradiology.com]

  Spontaneous dural damage to the meningeal diverticula triggering herniation are a primary cause of CSF leaks, along with ventral dural tears caused by calcified osteophytes and CSF-venous fistula. You can skip this ad in 3 seconds. [neurologyadvisor.com]

  Ultrafast dynamic computed tomography myelography for the precise identification of high-flow cerebrospinal fluid leaks caused by spiculated spinal osteophytes. J Neurosurg Spine. 2015 Mar;22(3):324–331. Castori M, Voermans NC. [rarediseases.org]

• Back Pain

  However, her headache became constant regardless of her posture, and within a month after the initial back pain, she became stuporous and was brought to a local emergency department. [karger.com]

• Facial Numbness

  Other symptoms can include nausea, vomiting, vertigo, tinnitus (ringing in the ears), change in hearing, blurred or double vision, facial numbness, and tingling of the arms. [columbiaspine.org]

  Patients may also experience visual changes, dizziness or vertigo, facial numbness or pain, or changes in taste. Pain between the shoulder blades and into upper arms is commonly reported. [cedars-sinai.org]

  ataxia, facial weakness, facial spasm (each 1/11). [intechopen.com]

  Thus, traction on the cranial nerves results in hyperacusis and dizziness (CN VIII), horizontal diplopia (CN VI) and facial numbness (CN V) or facial weakness (CN VII). [jpma.org.pk]

  numbness or pain (trigeminal nerve), facial weakness or spasm (facial nerve), and dysgeusia (chorda tympani). 7, 8 Transient visual obscurations, visual blurring, or visual field defects also have been reported and may be attributed to the stretching [jamanetwork.com]

• Meningism

  Postmyelography CTs showing ( A ) bilateral cervicothoracic CSF leaks without an associated meningeal diverticulum; B, Left thoracic meningeal diverticulum with an associated CSF leak; and C, right thoracic meningeal diverticulum without an associated [ajnr.org]

  meningitis [ 5 ]. [intechopen.com]

  Another noticeable finding on MR imaging is meningeal fibrosis and these findings provide a basis for the hypothesis that meningeal enhancement may be attributable to meningeal inflammation. 26 Evidence suggests that about 20% of patients never exhibit [jpma.org.pk]

  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]

  Meningeal diverticula have been known for some time to be associated with spinal CSF leaks. [journals.sagepub.com]

• Ataxia

  Venous hyperemia Iter (mm) Chamb (mm) Epidural fluid collections Venous plexuses engorgement Search for point of CSF leakage 1/60F 57 OH, dizziness, hearing changes, neck pain Weakness, difficulty in swallowing liquids Frontal signs, ataxia, ideomotor [academic.oup.com]

  Rarer symptoms include those typical of dementia or Parkinson's disease, very unsteady gait (ataxia) or quadriplegia. Stupor, coma, stroke and death have also been reported. [cedars-sinai.org]

  Patients may experience cranial nerve damage, ataxia, increased muscle tone, personality changes, and even disturbance of consciousness. [springerplus.springeropen.com]

  Intern Med. (1993). 32 228–31 [Article] [PubMed] Pakiam, AS, Lee, C, Lang, AE Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness.. [anesthesiology.pubs.asahq.org]

  Typically, the affected patients present with sensorineural hearing deficits and cerebellar ataxia. [journals.sagepub.com]

• Confusion

  This dearth of practical experience, and lack of awareness of recent investigations into SIH, produces heterogeneity in diagnostic and treatment pathways, driven in part by outdated, confusing, or unsubstantiated conceptions of the condition. [headachejournal.onlinelibrary.wiley.com]

  (From Semin Neurol 1996 Mar;16(1):5-10; Adams et al., Principles of Neurology, 6th ed, pp637-8) Note générale sur le champ d'application : do not confuse with INTRACRANIAL HYPERTENSION; coord IM with disease /physiopathol (IM) causing or caused by intracranial [idref.fr]

  Patients may appear confused, agitated, or obtunded. Results of imaging in patients with encephalitis may or may not demonstrate abnormal radiographic findings on CT or MRI modalities. [intechopen.com]

  Because of the difficulties in obtaining a CSF sample, a traumatic tap is not infrequent, contributing to the confusion with regard to a diagnosis of subarachnoid hemorrhage. [jamanetwork.com]

  Some of these brain imaging findings, however, such as diffuse pachymeningeal enhancement, are nonspecific and can cause diagnostic confusion [ 8 ]. [ajronline.org]

• Quadriplegia

  Quadriplegia and cerebellar hemorrhage in spontaneous intracranial hypotension. Neurology 2006 ; 13 : 1777 –78 ↵ Hong S, Shah GV, Adams KM, et al. Spontaneous intracranial hypotension causing reversible frontotemporal dementia. [ajnr.org]

  Rarer symptoms include those typical of dementia or Parkinson's disease, very unsteady gait (ataxia) or quadriplegia. Stupor, coma, stroke and death have also been reported. [cedars-sinai.org]

  Rare complications of SIH include reversible frontotemporal dementia, Parkinsonian-like symptoms, quadriplegia, coma, and even death ( 12 – 15 ). [frontiersin.org]

  "Quadriplegia and cerebellar hemorrhage in spontaneous intracranial hypotension". Neurology. 66 (11): 1777–8. doi : 10.1212/01.wnl.0000218210.83855.40. [en.wikipedia.org]

• Neurologic Manifestation

  Neurological manifestations of Ehlers-Danlos syndrome(s): A review. Iranian journal of neurology. 2014 Oct 6;13(4):190–208. Mokri B. Spontaneous CSF leaks: low CSF volume syndromes. Neurol Clin. 2014 May;32(2):397–422. [rarediseases.org]

  Neurologic manifestations of SIH Hearing changes including disturbed sense of balance, tinnitus and echoing; visual changes including visual blurring and diplopia; various neurological symptoms including photophobia, phonophobia, amnesia and facial numbness [intechopen.com]

  The most common clinical manifestation of intracranial hypotension is orthostatic headache. [jkna.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]

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]

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]

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]

Diagnosis and Treatment of Postpatch Headaches – Michael Malinzak, MD, PhD 9:45 A.M. Research Trials – Timothy Amrhein, MD 10:15 A.M. Q&A Break 10:30 A.M. Surgical Treatment of Disc Induced CSF Leaks – Christopher Brown, MD 11:00 A.M. [radiology.duke.edu]

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 [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]

Although at one point the etiology of spontaneous CSF leaks was thought to be secondary to loss of CSF volume ( Mokri 1999 ), leading to terms such as “CSF hypovolemia” or “CSF volume depletion” appearing in the literature and being used interchangeably [medlink.com]

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]

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]

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]

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]

Nanotechnology, Neonatology, Nursing Care, Nutrition and Metabolism, Nephrology, Neurology, Neurophysiology and Brain Research, Orthopedics, Obstetrics and Gynecology, Occupational Medicine, Oncology, Ophthalmology, Oral medicine, Otorhinolaryngology, Pathophysiology [dergipark.org.tr]

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]

Neuere Anschauungen zur Pathophysiologie der Liquorzirkulation (Recent views on the pathophysiology of CSF circulation.). Zentralbl Neurochir 1938;3:290-300. 3. Mokri B, Piepgras DG, Miller GM. [neurologyindia.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]

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

Internal Medicine, Infectious Diseases, Infertility, Laboratory Medicine, Medical Microbiology, Medical Statistics, Medico-Social Problems of the Youth and the Erderly, Mental Disorders, Mental Health, Metabolic Disorders, Methods of Epidemiology and Other Preventive [dergipark.org.tr]

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]