Spontaneous Intracranial Hypotension Syndrome. An Update.

Carlos Silva R., Natalia Pozo C., Heather Angus L., Alonso Quijada R., Gabriel Abudinen A., Nelson Barrientos U.

ABSTRACT


Introducction: Spontaneous intracranial hypotension is a syndrome caused by decreased CSF volume secondary to its leakage into the extradural space Although ICHD-3 provides a high level of diagnostic specificity, manifestations may be atypical, making diagnosis challenging.

The site of leakage may be undetermined in point Up to 30% of cases, although with recent refinement of imaging, this percentage has been reduced to 15-20%.

Currently, management is not standardized and recommendations are based on inconclusive evidence, with variability of protocols between centres.

Development. In this review, we update diagnostic and therapeutic procedures. We analyse the role of whole brain and spinal cord MRI as a first investigation and review tests aimed at determining cerebrospinal fluid leakage, such as MRI myelography, conventional CT myelography, dynamic CT myelography, and digital subtraction CT myelography, as well as 111-Indium-DPTA cisternography. Determining optimal use of these investigations remains a matter of debate. The same is true for treatment: rest, blind epidural blood patch, fluoroscopy or CT-guided epidural blood patch, fibrin patch and surgery are discussed.

Conclusión: Further research, especially multicentre controlled studies, is required to improve understanding of pathophysiology, diagnostic imaging, therapeutic approaches and to objectively assess clinical outcomes. Only then will diagnostic and treatment guidelines be evidence-based.

Keywords CSF-venous fistula; Epidural blood patch; Low CSF pressure; Orthostatic headache; Spinal CSF leak; Spontaneous intracranial hypotension.

INTRODUCTION


German neurologist George Schaltenbrand first described spontaneous intracranial hypotension (SIH) in 1938 (1,2). SIH was subsequently defined as a syndrome caused by decreased cerebrospinal fluid (CSF) volume secondary to its leakage into the extradural space. It is one of the causes of secondary headache included in the III edition of the International classification of headaches (ICHD-III) (3).

Among the established causes are tears of the dura mater, the presence of meningeal diverticula and CSF-venous fistulaes (4-6). In 10-30% of patients, the site and cause of the presumptive leak is unclear (5,6).

The clinical picture has heterogeneous manifestations, but the most frequent is orthostatic headache with a relationship between intensity and position (5,6). Imaging examinations are essential for establishing etiological, as well as the therapeutic strategies to be followed. There are still uncertainties and erroneous conceptions of the syndrome (6). There are several study and treatment protocols, with variability among specialized centres around the world (4,6,7-10). This is conditioned by the paucity of high-quality prospective studies, and more research is needed in this area (5).

The aim of this article is an update on SIH considering the most recent developments in the world literature.

Epidemiology

SIH is a rare cause of secondary headache, with incidence estimated at 5 per 100,000. By comparison, aneurysmal subarachnoid haemorrhage, occurs at a rate of 10 per 100,000 (6). The true incidence of SIH is expected to be higher, as it is frequently misdiagnosed initially (5). Most presentations occur between the ages of 20 and 65 years, with a peak age of 40 years, and it affects more women than men in a 2:1 ratio (7).

Clinical features

The typical presentation is an orthostatic headache, although it is not uncommon to have a sudden onset or thunderclap headache(11,12). It usually occurs within fifteen minutes of assuming an upright position, but in some cases this period may be much longer. The improvement of headache after lying down is less variable and occurs within fifteen to thirty minutes (7). Headache may be bilateral presentation, often with an occipital/suboccipital onset that radiates to the frontal area, or from onset the headache can be unilateral (5).

Pain intensity varies from mild to severe and may have an oppressive or throbbing character, worsening with Valsalva. Headaches that gradually worsen over the course of the day resulting in increasing pain in the evening hours, so-called ““End of the day” or “Second half of the day”(5,6,13). The orthostatic nature of the headache may become less evident with time and some patients may progress to chronic daily headache (5,13). It is accompanied by nausea, vomiting, tinnitus, sensation of pressure in the ear, dizziness, vertigo, hearing loss, neck stiffness and photophobia. Less typical presentations include non-orthostatic headaches, cough headache, paradoxical headache (worsens in decubitus) or even absence of headache or acephalgic symptomatology. In the latter case, auditory symptoms are the most recurrent (5).

Uncommon presentations described include subdural hematoma (14,15), oculomotor nerve palsy(16-19), endolymphatic hydrops (20), thrombosis of venous sinuses and cortical veins (21-23), reduced level of consciousness and coma(8), reversible vasoconstriction syndrome(24), cerebellar haemorrhage(25), involuntary movements and extrapyramidal symptoms (26-28), non-convulsive status epilepticus (29) and symptoms with the characteristics of reversible frontotemporal dementia (30).

The diagnosis is made more complex by the existence of other conditions that may present with orthostatic headache. Such is the case of orthostatic postural tachycardia syndrome (PoTS(31), idiopathic intracranial hypertension syndrome(32), post-traumatic headaches(5,13), cervicogenic (32,33), craniocervical instability (33), and meningitis (5).

Pathophysiology

According to the Monro-Kellie theory, intracranial pressure (ICP) depends on the balance of fluids and tissues: CSF, blood, and brain. Thus, if any of them decreases or increases its volume, it causes a compensation by the other two elements. In SIH there is loss of CSF and subsequent hypovolemia, which leads to a displacement of the brain towards caudal or sagging (34). It is evident that when the individual stands up, ICP decreases even more and the consequent headache appears. On the other hand, when lying down there is a decrease in symptoms. The increase in blood volume is directed towards the cerebral veins, in addition to a slight vasogenic oedema because of a greater resistance to venous drainage (34).

SIH is usually caused by CSF leakage from the dural space. Although CSF leakage can also arise from the skull base, these cases are most often associated with elevated CSF pressure and without the imaging findings of SIH (35).

Spinal CSF leaks are recognized to occur through three main mechanisms: meningeal diverticula, ventral dura tears, and CSF-venous fistulae (6). In one study diverticula were the most common (42%), ventral dura tears (27%) and CSF-venous fistulaes (3%). However, in 28% the cause was undetermined (36).

Meningeal diverticula were the first recognized cause of spontaneous CSF leakage into the spinal column. They correspond to leptomeningeal protrusions in relation to the spinal root sleeve. They are more frequent in patients with connective tissue diseases such as Marfan and Ehlers-Danlos syndrome, or polycystic kidney disease (36). Diverticula are located especially in the thoracic spine. Either spontaneously or in relation to Valsalva, there is a shredding of the dura mater and consequent leakage of CSF. The speed of CSF leakage is variable, since it is conditioned by the anatomical characteristics of the diverticulum(37,38). Usually, Tarlov cysts are not associated with SIH(38).

Ventral dural fistulas are caused by calcified disc protrusions or osteophytes that contact the dura mater and tear it. They are usually located in the lower cervical, thoracic, or lumbar spine. CSF leaks are high-flow and produce extensive extradural collections (37,38).

Venous-CSF fistulae were recently recognized(39). Here there is a direct communication between the spinal subarachnoid space and a paraspinal vein, allowing a rapid loss of CSF into the venous circulation. Under physiological conditions the flow of CSF is unidirectional towards the vein, but because of the low CSF volume the flow is reversed and rapidly increases the rate of leakage, with the consequent loss of CSF reabsorption capacity by the nerve roots and arachnoid villi. The dorsal spine is the most common location of CSF-venous fistulae. They are in the medial zone of the nerve root and in 80% are in close relation to a perineural diverticulum (40,41).

Diagnosis

The diagnostic criteria for SIH are well defined in the ICHD-3 (3) (Table I). Objective evidence includes abnormal brain imaging and demonstration of a cerebrospinal fluid (CSF) leak on spinal imaging, in addition to a CSF pressure of less than 6 cm H20 (3). However, ICHD-3 specifies that lumbar puncture (LP) for CSF pressure measurement is not a diagnostic criterion if magnetic resonance imaging (MRI) findings are suggestive of SIH. On the other hand, it stresses that the diagnosis should not be made if the patient underwent a LP within the previous month. Thus, ICHD-3 provides a good level of diagnostic accuracy. ICP measurement is reserved for cases with atypical clinical or imaging findings. The literature reveals that most patients with SIH have CSF pressures in the normal range (7-20 cm H20) Pressures can even be higher than 20 cm H20 (34). Even so, the concept that a low CSF opening pressure is a sine qua non diagnostic criterion for SIH often prevails(3,5,6).

Neuroimaging

First-line diagnostics of SIH should include brain and spinal MRI.

Brain MRI should be obtained with contrast. It is the most sensitive test and should be the first performed. In addition, MR spinal imaging, fat-saturated axial slices of cervical and thoracic levels, may find tears and epidural collections that may lead to targeted therapy (42).

In the first-line diagnostic stage, brain and spinal MRI findings have a sensitivity and specificity of 83% and 94%, respectively (42).

Brain neuroimaging

Pachymeningeal gadolinium enhancement

It is homogeneous and diffuse (Fig.1), both supra and infratentorial(7). It is differentiated from a meningeal or granulomatous carcinomatosis, the latter showing irregular or nodular uptake of contrast medium. (43).

Subdural fluid collections

They are usually bilateral hygromas in the cerebral convexity (Figure 2a), usually without mass effect(7). Chronic subdural hematoma may develop as a complication of SIH index of suspicion for SIH in young patients with no history of trauma and with hygromas or subdural hematomas is of fundamental importance (44,45).

Sagittal sagging signs

There is a caudal displacement of the cerebral basal and brainstem structures or sagging. It can be objectified through measurement of the mamillopontine distance (? 6.5 mm), the suprasellar cistern (? 4 mm)) and the prepontine cistern (? 5 mm) (46) (Figure 2b). In addition, there is a flattening of the ventral pons, a lowering of the midbrain, and cerebellar tonsillar descent(5,7).

Venous engorgement

Congestion is evident in sagittal, transverse, and cavernous sinuses, as well as cortical veins of the cerebral convexity (Fig. 3) (6,34). The rounded appearance of the medial portion of the dominant transverse sinus, visible in the sagittal section, is characteristic (Figure. 3). The normal image is characterized by straight borders, with a triangular appearance (47).

Pituitary gland hyperaemia

There is an increase in volume of the pituitary gland (Figure 3) and a greater enhancement with contrast medium due to vascular dilatation (48).

Optic nerve sheath diameter and thickness

Significantly reduced optic nerve sheath diameter and thickness have been shown in SIH (7).

Objective MRI parameters

Despite attempts to provide objective parameters for MRI evaluation, there is no validated system for interpretation and integration of all MRI diagnostic criteria for SIH. Dobrocky et al (46) reported a series of 56 SIH patients with confirmed CSF leaks. They developed a probability scoring scale for this syndrome using six imaging findings. Three are weighted as major: pachymeningeal enhancement, engorgement of venous sinus, and effacement of the suprasellar cisterna (4 mm or less). The minors are the presence of subdural fluid collection, effacement of prepontine cistern of 5 mm or less, mamillopontine distance of 6.5 mm or less. On a scale of 9 points, where major criteria confer 2 points per finding and minor criteria 1 point per finding. Depending on the likelihood of finding a spinal CSF leak patients are classified into three main groups: high probability (? 5 points), intermediate (3-4 points), low (? 2 points). The authors recommend the score system to be used for further decision on invasive procedures this system for the subsequent decision of invasive procedures; patients with ? 5 points should be treated promptly, and further work-up for scores ? 4 points (46).

Spine neuroimaging

Spinal MRI

Pachymeningeal enhancement

As in the MRI of the brain, it is homogeneous and diffuse (4).

Epidural fluid collections

Leaks due to major dural defects cause a rapid outflow of CSF into the epidural space, resulting in a large collection that may extend longitudinally through several vertebral segments. These are the high-flow or rapid leaks (4,6).

Smaller dural defects result in an epidural collection that spans no more than one vertebral body. These are the low-flow or slow leaks (4,6).

CSF-venous fistulaes usually present without epidural fluid accumulation (4,6).

Others: Distention of the epidural veins, abnormal visualization of a nerve root sleeve and meningeal diverticula along the nerve root sleeves (4,6).

The conjunction of clinical and MRI findings of the brain and spinal cord allows the diagnosis of SIH to be established. The next step is to determine the point of spinal CSF leakage, where spinal myelography imaging are essential.

MRI myelography

MRI myelography has a high sensitivity for the detection of low-flow leaks and CSF-venous fistulae (43). However, its main disadvantage is the inability to visualize subtle bone alterations. In addition, gadolinium has been associated with neurological injury and there is evidence of its long-term negative effects (49-51). In fact, its intrathecal use is not approved by the U.S. Food and Drug Administration (FDA). Without a contrast medium, this procedure has low sensitivity, even with specific sequences (43).

Conventional CT myelography

Conventional computerized tomography (CT) myelography was the gold standard for CSF leak detection (34,52) until the advent of technical improvements of this procedure. It allows the identification of high-flow leaks and some low-flow leaks (53,54). Among its advantages are its high spatial resolution, CSF-surrounding structures differentiation and good visualization of diverticula, as well as its ability to observe osteophytes (5,7). Moreover, it is available in most centres in the world.

It is estimated that conventional CT myelography is positive in 55% to 70% of cases (34,55).

Dynamic CT myelography

Dynamic CT myelography reduces image acquisition time. The contrast material is injected with the patient in the CT scanner. This allows for immediate CT acquisition and localization of fast CSF leaks (34,55). However, it involves high doses of radiation.

Digital subtraction CT myelography

Even dynamic CT acquisition may be too long to accurately detect the leakage site. A solution to this problem is digital subtraction CT myelography. It decreases the time between contrast administration and image acquisition (5,55). By suppressing the surrounding images, it facilitates the detection of subtle leaks (7,55). Through this examination, in approximately one fifth of patients with a negative conventional spinal study it is possible to identify CSF-venous fistulaes (56).

Radionuclide cisternography

This test is currently considered to have a low spatial resolution, lack of axial images, with limited sensitivity and specificity (5,7). However, there are authors who give value to 111-Indium-DPTA cisternography (34).

Therefore, with traditional studies in one third of the cases it is not possible to reach the aetiology of SIH. However, with the refinement of imaging examinations the group of undetermined cause has been reduced to approximately 15% to 20% (6,34,57).

Treatment

Given the limited evidence available, treatment of SIH is varied, mostly based on uncontrolled case series and expert opinion (5,6). There are no validated clinical guidelines, although consensus has been sought (5,6,34,57).

Conservative treatment

This is the first-line treatment. It consists of bed rest, hydration, and oral caffeine administration (4-7,34). The optimum period of rest has not been reliably established. Although it has traditionally been considered that most cases respond to this therapy, there are few series that validate this (5). One study involving 73 patients found that only 7% of participants showed a favourable response without requiring further intervention (4).

Non-directed epidural blood patch

Non-directed or blind way epidural blood patch is the most frequently therapeutic intervention (5,6) and it is to perform before CT myelography. The blind procedure is performed through the lumbar route and using autologous blood volumes between 10 and 50 ml. It is recommended that the patient remain in Trendelenburg for eight hours following the procedure (13). In addition to tamponade of the leak point, a key mechanism would be the decrease in the distensibility of the thecal sac and a reversal of CSF pressure towards the cranial (5,6). Some authors perform this procedure three to five times weekly or until symptomatologic resolution (13). It is estimated that the ascent of blood can reach six or more spinal segments (58,59). Regarding the efficacy of this therapeutic modality, very disparate values are given, between 36% and 90% (60-63). The current recommended approach is not to delay the next step, it is to perform CT myelography (57).

Targeted patches

Targeted blood/ fibrin sealant patches should be performed in patients who remain symptomatic following appropriate conservative management and/or non-directed epidural blood patch, and causative lesion has been identified on conventional CT myelography or digital subtraction CT myelography (5-7,34,43,57). Targeted patching must be performed by an experienced neuroradiologist (57).

Surgical intervention

Conservative measures and epidural patching are estimated to be successful in approximately 75% of cases (6,58,60). Surgery is reserved for patients with refractory leaks, very high flow fistulas and CSF fistulae (5-7,34,57,63). It consists of ligation of meningeal diverticula, suturing of dural tears, filling of the epidural space with fibrin or an absorbable gelatine sponge (purified porcine skin) and repair of CSF-venous fistulae (6,7,13,34,57). Detailed surgical treatment is beyond the scope of this review.

Several algorithms have been proposed for standardization in decision making. We show a SIH diagnostic and therapeutic SIH scheme that could serve as a guideline for the clinician (Figure 4).

The prognosis of SIH is generally good. However, it is a condition with mortality, with invasive diagnostic and therapeutic procedures not free of risks, requiring a multidisciplinary management with subspecialist neurologists in headaches, anaesthesiologists highly trained in cervical and thoracic epidural space punctures, neuroradiologists and experienced neurosurgeons, all supported by an increasingly sophisticated diagnostic technology (4,6,13,34, 57).

CONCLUSIONS


SIH corresponds to a cause of secondary headache that was considered rare, but with increased recognition in recent years. The clinical picture has heterogeneous manifestations. Most cases present as an orthostatic headache, bilateral, of mild to severe intensity, oppressive or throbbing character and worsening with Valsalva. It is usually accompanied by nausea, vomiting, tinnitus, sensation of pressure in the ear, dizziness, vertigo, hearing loss, neck stiffness and photophobia. It is not uncommon for the headache to begin as a thunderclap headache. It may take months or years for the disease to progress before it is suspected. The diagnosis is established with clinical and brain and spine MRI. Brain MRI may show homogeneous supra- and infratentorial pachymeningeal enhancement, signs of cerebral descent or sagging, venous congestion, and bilateral subdural effusions. Spinal MRI shows the same pattern of pachymeningeal contrast uptake and extradural CSF collections of variable extension. With these elements it is not a diagnostic necessity to perform a dural puncture with a recorded ICP less than 6 cm of H20, except in atypical cases, specified in the ICHD-3.

Once the diagnosis is made, conservative treatment with rest and caffeine or blind epidural patch should be performed. If there is no response, a search for the CSF leak site in the spine should be done: conventional CT myelography, dynamic CT myelography or digital subtraction CT myelography.

Causes of CSF leakage are meningeal diverticula (42%), ventral dural fistulas (27%), CSF-venous fistulae (3%) and undetermined (28%). refinement of diagnostic techniques has reduced the indeterminate causes to approximately 15% to 20%.

Treatment is not standardized and recommendations are based on evidence of limited methodological quality, in addition to the variability of protocols between different centres.

Algorithms of therapeutic measures have been proposed in stages, from less to more invasive: rest and use of caffeine, blind lumbar epidural blood patch, epidural blood/ fibrin sealant patch guided by fluoroscopy or cervical or dorsal CT, and surgery.

The prognosis of SIH is generally good. However, it is a potentially life-threatening disease, with invasive diagnostic and therapeutic procedures not without risk. Therefore, further research is needed, especially controlled multicentre studies, to offer the best alternative for the diagnosis and treatment of patients.

Key points

SIH is an increasingly recognized cause of secondary headache.

SIH has heterogeneous manifestations, most commonly presenting as an orthostatic headache, not infrequently with thunderclap onset.

The diagnosis is established by clinical and MRI findings of the brain and spine. An ICP of less than 6 cm of H2O is not usually required as a diagnostic criterion, except in cases with atypical symptoms and/or images, as specified in the ICHD-3.

Once the diagnosis has been made, conservative treatment with rest-caffeine or a blind epidural patch should be undertaken. If there is no therapeutic response, a search for the CSF leak point in the spinal column should be performed with conventional, dynamic, or digital subtraction CT myelography.

Although treatment is not standardised, therapeutic algorithms ranging from less to more invasive have been proposed, from rest and caffeine to surgery.

Prospective randomised studies are crucial to guide and systematise recommendations for the management of this condition.

REFERENCES


  1. 1. Schaltenbrand G. New observations on the pathological physiology of the cerebrospinal fluid circulation (in German). Zentralbl Neurochir 1938; 3: 290-300.
  2. 2. Schaltenbrand G. Normal and pathological physiology of the cerebrospinal fluid circulation. Lancet 1953; 261(6765): 805-8.
  3. 3. Headache Classification Committee of the International Headache Society (IHS). (2013). The International Classification of Headache Disorders, 3rd edition (beta version). International Headache Society. https://doi.org/10.1177/0333102413485658.
  4. 4. Perthen J, Dorman P, Morland D, Redfern N, Butteriss D. Treatment of spontaneous intracranial hypotension: experiences in a UK regional neurosciences centre. Clinical Medicine 2021; 21: 247-51.
  5. 5. Kranz P, Gray L, Malinzak M, Amrhein T. Spontaneous intracranial hypotension. Neuroimag Clin N Am 2019; 29: 581-94.
  6. 6. Dobrocky T, Nicholson P, Häni L, Mordasini P, Krings T, Brinjikji W, et al. Spontaneous intracranial hypotension: searching for the CSF leak. Lancet Neurol 2022; 21(4): 369-80.
  7. 7. Urbach H. Intracranial hypotension: clinical presentation, imaging findings, and imaging guided therapy. Curr Opin Neurol 2014; 27: 414-24.
  8. 8. He FF, Li L, Liu M-J, Zhong T-D, Zhang Q-W, Fang X-M. Targeted epidural blood patch treatment for refractory spontaneous intracranial hypotension in China. J Neurol Surg B Skull Base 2017; 79(03): 217-23.
  9. 9. Amoozegar F, Guglielmin D, Hu W, Chan D, Becker WJ. Spontaneous intracranial hypotension: Recommendations for management. Can J Neurol Sci 2013; 40:144-57.
  10. 10. Mokri B. Spontaneous cerebrospinal fluid leaks: From intracranial hypotension to cerebrospinal fluid hypovolemia–evolution of a concept. Mayo Clin Proc 1999; 74: 1113-23.
  11. 11. Ferrante E, Savino A. Thunderclap headache caused by spontaneous intracranial hypotension. Neurol Sci 2005; 26 (SUPPL. 2): 155-57.
  12. 12. Idrissi AL, Lacour JC, Klein O, Schmitt E, Ducrocq X, Richard S. Spontaneous intracranial hypotension: characteristics of the serious form in a series of 24 patients. World Neurosurg 2015; 84(6): 1613-20.
  13. 13. Ferrante E, Trimboli M, Rubino F. Spontaneous intracranial hypotension: review and expert opinion. Acta Neurol Belg. 2020 Feb; 120(1): 9-18.
  14. 14. De Noronha RJ, Sharrack B, Hadjivassilou M, Romanowski CAJ. Subdural haematoma: a potentially serious consequence of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry 2003; 74: 752-5.
  15. 15. Redondo-Carazo MV, Vázquez-Sáez V, Miñano-Soliva V, Puerta-Sales A, Torregrosa-Sala B, Flores-Ruiz JJ, et al. Hematoma subdural precoz como forma de inicio del síndrome de hipotensión intracraneal: hallazgos en resonancia magnética Rev Neurol 2006; 42(4): 220-2.
  16. 16. Berlit P, Berg-Dammer E, Kuehne D. Abducens nerve palsy in spontaneous intracranial hypotension. Neurology 1994; 44:1552.
  17. 17. Ferrante E, Savino A, Brioschi A. Transient oculomotor cranial nerves palsy in spontaneous intracranial hypotension. J Neurosurg Sci 1998; 42:177-79.
  18. 18. Warner GT. Spontaneous intracranial hypotension causing a partial third cranial nerve palsy: a novel observation. Cephalalgia 2002; 22: 822-23.
  19. 19. González-Sánchez M, Llorente-Ayuso L, López-Blanco R, de Fuenmayor-Fernández de la Hoz CP, Díaz-Guzmán J. Ptosis fluctuante como presentación del síndrome de hipotensión licuoral espontánea. Rev Neurol 2014; 58 (09): 429-30.
  20. 20. Portier F, de Minteguiaga C, Racy E. Spontaneous intracranial hypotension: a rare cause of labyrinthine hydrops. Ann Otol Rhinol Laryngol 2002; 111: 817-20.
  21. 21. Berroir S, Grabli D, Heran F. Cerebral sinus venous thrombosis in two patients with spontaneous intracranial hypotension. Cerebrovasc Dis 2004; 17: 9-12.
  22. 22. Schievink WI, Maya MM. Cerebral venous thrombosis in spontaneous intracranial hypotension. Headache 2008; 48:1511-19.
  23. 23. Yoon KW, Cho MK, Kim YJ, Lee SK. Sinus thrombosis in a patient with intracranial hypotension: a suggested hypothesis of venous stasis. a case reports. Interv Neuroradiol 2011; 17: 248-51.
  24. 24. Schievink WI, Maya MM, Chow W, Louy C. Reversible cerebral vasoconstriction in spontaneous intracranial hypotension. Headache 2007; 47: 284-87.
  25. 25. Schievink WI, Maya MM. Quadriplegia and cerebellar hemorrhage in spontaneous intracranial hypotension. Neurology 2006; 66: 1777-78.
  26. 26. Pakiam AS, Lee C, Lang AE. Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol 1999; 56: 869-72.
  27. 27. Turgut N, Unlu E, Hamamcioglu M, Güldiken B, Albayram S. Postural tremor as a manifestation of spontaneous intracranial hypotension. J Clin Neurosci 2010; 17: 255-57.
  28. 28. Mokri B, Ahlskog JE, Luetmer PH. Chorea as a manifestation of spontaneous CSF leak. Neurology 2006; 67: 1490-91.
  29. 29. Hedna VS, Kumar A, Miller B, Bidari S, Salardini A, Waters MF, et al. Intracranial hypotension masquerading as nonconvulsive status epilepticus: report of 3 cases. J Neurosurg. 2014 Mar; 120(3): 624-7.
  30. 30. Hagemann C, Christ M, Maurer C, Wegerer H, Naumann, et al. Spontane intrakranielle Hypotension mit “brain sagging“ und reversibler frontotemporaler Demenz: Fallbericht und Übersicht über die Literatur (Spontaneous intracranial hypotension with brain sagging and reversible frontotemporal dementia : Case report and review of the literature). Nervenarzt 2022 Oct; 93(10) :1049-52.
  31. 31. Mokri B, Low A. Orthostatic headaches without CSF leak in postural tachycardia syndrome. Neurology 2003; 61: 980-2.
  32. 32. Schievink WI, Deline CR. Headache secondary to intracranial hypotension. Curr Pain Headache Rep 2014; 18: 457.
  33. 33. Bogduk N. The neck and headaches. Neurol Clin. 2014; 32: 471-87.
  34. 34. Perez-Vega C, Robles-Lomelin P, Robles-Lomelin I, Garcia Navarro V. Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder. Neurol Sci 2020 Sep ;41(9): 2433-41.
  35. 35. Schievink WI, Schwartz MS, Maya MM, Moser FG, Rozen TD. Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks. J Neurosurg 2012 Apr; 116(4): 749-54.
  36. 36. Schievink WI, Maya MM, Jean-Pierre S, Nuño M, Prasad RS, et al. A classification system of spontaneous spinal CSF leaks. Neurology 2016 Aug 16; 87(7): 673-9.
  37. 37. Cohen-Gadol AA, Mokri B, Piepgras DG, Meyer FB, Atkinson JL. Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Neurosurgery 2006 Apr; 58(4 Suppl 2): ONS-238-45.
  38. 38. Kranz PG, Stinnett SS, Huang KT, Gray L. Spinal meningeal diverticula in spontaneous intracranial hypotension: analysis of prevalence and myelographic appearance. AJNR Am J Neuroradiol 2013 Jun-Jul; 34(6): 1284-9.
  39. 39. Edsbagge M, Tisell M, Jacobsson L, Wikkelso C. Spinal CSF absorption in healthy individuals. Am J Physiol Regul Integr Comp Physiol 2004 Dec; 287(6): R1450-5.
  40. 40. Kranz PG, Amrhein TJ, Gray L. CSF Venous Fistulas in Spontaneous Intracranial Hypotension: Imaging Characteristics on Dynamic and CT Myelography. AJR Am J Roentgenol 2017 Dec; 209(6): 1360-66.
  41. 41. Farb RI, Nicholson PJ, Peng PW, Massicotte EM, Lay C, et al. Spontaneous intracranial hypotension: a systematic imaging approach for CSF leak localization and management based on MRI and digital subtraction myelography. Am J Neuroradiol 2019; 40(4): 745-53.
  42. 42. Watanabe A, Horikoshi T, Uchida M, Koizumi H, Yagishita T, et al. Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome. Am J Neuroradiol 2009: 30(1): 147-51.
  43. 43. Kranz P, Gray L, Amrhein T. Spontaneous intracranial hypotension: 10 myths and misperceptions. Headache 2018; 58: 948-59.
  44. 44. Takahashi K, Mima T, Akiba Y. Chronic subdural hematoma associated with spontaneous intracranial hypotension: therapeutic strategies and outcomes of 55 cases. Neurol Med Chir (Tokyo) 2016; 56: 69-76.
  45. 45. Wan Y, Xie J, Xie D, Xue Z, Wang Y, et al. Clinical characteristics of 15 cases of chronic subdural hematomas due to spontaneous intracranial hypotension with spinal cerebrospinal fluid leak. Acta Neurol Belg 2016; 116: 509-12.
  46. 46. Dobrocky T, Grunder L, Breiding PS, Branca M, Limacher A, et al. Assessing spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension with a scoring system based on brain magnetic resonance imaging findings. JAMA Neurol 2019; 76(5): 580-87.
  47. 47. Farb R, Forghani R, Lee S, Mikulis D, Agid R. The venous distension sign: a diagnostic sign of intracranial hypotension at MR imaging of the brain. Am J Neuroradiol 2007; 28: 1489-93.
  48. 48. Albes G, Weng H, Horvath D, Musahl C, Bäzner H, et al. Detection and treatment of spinal CSF leaks in idiopathic intracranial hypotension. Neuroradiology 2012; 54: 1367-73.
  49. 49. Albayram S, Kilic F, Ozer H, Baghaki S, Kocer N, et al. Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. Am J Neuroradiol 2008; 29: 116-21.
  50. 50. Algin O, Turkbey B. Intrathecal gadolinium-enhanced MR cisternography: a comprehensive review. AJNR Am J Neuroradiol 2013; 34: 14-22.
  51. 51. Park K, Im S, Kim B, Hwang S, Park J, et al. Neurotoxic manifestations of an overdose intrathecal injection of gadopentetate dimeglumine. J Korean Med Sci 2010; 25: 505-8.
  52. 52. Wendl C, Schambach F, Zimmer C, Förschler A. CT myelography for the planning and guidance of targeted epidural blood patches in patients with persistent spinal CSF leakage. Am J Neuroradiol 2012; 33: 541-4.
  53. 53. Kranz P, Luetmer P, Diehn F, Amrhein T, Tanpitukpongse P, et al. Myelographic techniques for the detection of spinal CSF leaks in spontaneous intracranial hypotension. Am J Roentgenol 2016; 206: 8-19.
  54. 54. Luetmer PH, Mokri B. Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol 2003; 24: 1711-4.
  55. 55. Farb RI, Nicholson PJ, Peng PW, Massicotte EM, Lay C, et al. Spontaneous Intracranial Hypotension: A Systematic Imaging Approach for CSF Leak Localization and Management Based on MRI and Digital Subtraction Myelography. AJNR Am J Neuroradiol. 2019 Apr; 40(4): 745-53.
  56. 56. Schievink W, Moser F, Maya M, Prasad R. Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas. J Neurosurg Spine 2016; 24: 960-4.
  57. 57. Cheema S, Anderson J, Angus-Leppan H, Armstrong P, Butteriss D, et al. Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 2023 May 5: jnnp-2023-331166. doi: 10.1136/jnnp-2023-331166. Epub ahead of print. PMID: 37147116.
  58. 58. Berroir S, Loisel B, Ducros A, Boukobza M, Tzourio C, et al. Early epidural blood patch in spontaneous intracranial hypotension. Neurology 2004; 63: 1950-1.
  59. 59. Su C, Lan M, Chang Y, Lin W, Liu K. Clinical features, neuroimaging and treatment of spontaneous intracranial hypotension and magnetic resonance imaging evidence of blind epidural blood patch. Eur J Neurol 2009; 61: 301-7.
  60. 60. Cho K, Moon H, Jeon H, Park K, Kong D. Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology 2011; 76: 1139-44.
  61. 61. He F, Li L, Liu M, Zhong T, Zhang Q, et al. Targeted epidural blood patch treatment for refractory spontaneous intracranial hypotension in China. J Neurol Surg B Skull Base 2018; 79: 217-23.
  62. 62. Wu J, Hseu S, Fuh J, Lirng J, Wang Y, Chen W, et al. Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension. Brain 2017; 140: 344-52.
  63. 63. Franzini A, Messina G, Nazzi V, Mea E, Massimo L, et al. Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and novel patch method in a series of 28 consecutive patients. J Neurosurg 2010; 112: 300-6.


HOW TO QUOTE?


(2024). Spontaneous Intracranial Hypotension Syndrome. An Update..Journal of Neuroeuropsychiatry, 57(4).
Recovered from https://www.journalofneuropsychiatry.cl/articulo.php?id= 168
2024. « Spontaneous Intracranial Hypotension Syndrome. An Update.» Journal of Neuroeuropsychiatry, 57(4). https://www.journalofneuropsychiatry.cl/articulo.php?id= 168
(2024). « Spontaneous Intracranial Hypotension Syndrome. An Update. ». Journal of Neuroeuropsychiatry, 57(4). Available in: https://www.journalofneuropsychiatry.cl/articulo.php?id= 168 ( Accessed: 14junio2024 )
Journal Of Neuropsichiatry of Chile [Internet]. [cited 2024-06-14]; Available from: https://www.journalofneuropsychiatry.cl/articulo.php?id=168

 

DOWNLOAD PDF VERSION