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]

• Pain

  Headache: The Journal of Head and Face Pain. doi:10.1111/head.13328 [americanheadachesociety.org]

  […] scp=33846519933&partnerID=8YFLogxK U2 - 10.1007/s11916-007-0023-9 DO - 10.1007/s11916-007-0023-9 M3 - Article VL - 11 SP - 56 EP - 61 JO - Current Pain and Headache Reports JF - Current Pain and Headache Reports SN - 1531-3433 IS - 1 ER - [mayoclinic.pure.elsevier.com]

  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]

  Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain. Am J Med Genet C Semin Med Genet. 2015 Mar;169C(1):84–96. Feltracco P, Galligioni H, Barbieri S, Ori C. [rarediseases.org]

  Pain Med 2015;16:1897-904. DOI PubMed 16. Wang E, Wang D. Successful treatment of spontaneous intracranial hypotension due to prominent cervical cerebrospinal fluid leak with cervical epidural blood patch. Pain Med 2015;16:1013-8. DOI PubMed 17. [nnjournal.net]

• Falling

  Common reported events or mechanical factors associated with the onset of symptoms include lifting small or large items, straining, stretching, positional changes, sporting activities, roller coaster rides and falls. [spinalcsfleak.org]

  In two-thirds of cases, patients report no history of a triggering event, such as lifting, straining, stretching, sporting activities, roller coaster rides and falls. [cedars-sinai.org]

  The fall in CSF pressure is secondary to a CSF leak, likely due to a recent lumbar puncture or epidural anesthesia [3-4]. CSF leakage can result in venous engorgement resulting in pachymeningeal enhancement on MRI. [cureus.com]

  A few weeks after the onset of headaches, the patient started experiencing sleep attacks, wherein she would fall asleep during a conversation and even while standing up. [frontiersin.org]

  A similar mechanism of CSF leakage may occur with lumbar nerve sleeve tears, which have been identified in patients presenting with postural headaches following a fall on the buttocks. [practicalneurology.com]

• Severe Pain

  The remaining weight is supported by suspensions formed by several pain sensitive structures. [jpma.org.pk]

• Short Stature

  Additionally, other relevant factors include malnutrition [ 26 ], short stature [ 27 ] and etc. [thejournalofheadacheandpain.biomedcentral.com]

• Rhinorrhea

  In cases of traumatic or iatrogenic intracranial hypotension, a history of abundant, clear rhinorrhea or otorrhea can sometimes be elicited. [radiopaedia.org]

  At initial presentation, it may be difficult to differentiate nasal discharge attributed to CSF rhinorrhea from that of respiratory secretions, tears, or blood. [practicalneurology.com]

  None of the patients had a history of CSF rhinorrhea or CSF otorrhea. A total of 37 patients resided within Los Angeles County, 20 patients resided outside of Los Angeles County but within California, and 50 resided outside of California. [ajnr.org]

  Beckhardt RN, Setzen M, Carras R (1991) Primary spontaneous cerebrospinal fluid rhinorrhea. Otolaryngol Head Neck Surg 104:425–32 21. Schievink WI, Torres VE (1997) Spinal meningeal diverticula in autosomal dominant polycystic kidney disease. [thejournalofheadacheandpain.biomedcentral.com]

• 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]

• 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]

• 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]

• 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 Stiffness

  Symptoms The symptoms may include headache, neck stiffness and arm pain. Causes and Risk Factors The condition is usually caused by the leakage of cerebrospinal fluid. [cedars-sinai.edu]

  Some patients also experience nausea and neck stiffness. SIH is rare in the general population; however patients with this disorder are frequently seen in headache clinics. [ucalgary.ca]

  It is usually accompanied by neck stiffness and subjective hearing symptoms. It remits after normalization of CSF pressure. [ichd-3.org]

  stiffness, and other symptoms. [idref.fr]

  Key points • Spontaneous intracranial hypotension is characterized by an orthostatic headache accompanied by neck stiffness, tinnitus, hypacusia, photophobia, or nausea. • Spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal [medlink.com]

• Dizziness

  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]

  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]

  Other symptoms that may occur include visual blurring, dizziness, memory problems and problems with balance. Sometimes patients may have a collagen disease that causes the leak. [headaches.org]

  […] which worsens when you sit up and improves when you lie down Sensitivity to light or sound Nausea, with or without vomiting Neck pain or stiffness Hearing changes, such as muffled hearing or ringing in the ears Difficulty concentrating Double vision Dizziness [barrowneuro.org]

  Also auditory or vestibular symptoms including hypo or hyperacousis, tinnitus, nausea, vomiting and dizziness were observed in about 20% of patients and resulting probably from a low intralabyrinthine pressure [ 2, 10 ]. [omicsonline.org]

• 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]

• Agitation

  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]

• Aura

  The patient did not have aura, visual change, or other neurological symptoms. Clinical examination was normal, and did not found any hyperflexibility of skin or joints. The first cerebral computed tomography scan was normal. [dovepress.com]

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]