0
Original Contribution |

Misdiagnosis of Spontaneous Intracranial Hypotension FREE

Wouter I. Schievink, MD
[+] Author Affiliations

From the Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, Los Angeles, Calif.


Arch Neurol. 2003;60(12):1713-1718. doi:10.1001/archneur.60.12.1713.
Text Size: A A A
Published online

Background  Spontaneous intracranial hypotension is an important cause of "new daily persistent headaches" but is not a well-recognized entity. The misdiagnosis of spontaneous intracranial hypotension can have serious consequences.

Methods  The clinical course in 18 consecutive patients with spontaneous intracranial hypotension who were evaluated for definitive surgical treatment of the underlying spontaneous spinal cerebrospinal fluid leak from January 1, 2001, through June 30, 2002, was investigated by correspondence with the patients and physicians.

Results  Seventeen patients (94%) initially received an incorrect diagnosis, and the diagnostic delay ranged from 4 days to 13 years (median, 5 weeks; mean, 13 months). Migraine, meningitis, and psychogenic disorder were the most commonly entertained diagnoses. Diagnostic or therapeutic procedures for disorders that mimicked spontaneous intracranial hypotension included cerebral arteriography in 2 patients, craniotomies for Chiari malformation in 2 patients, craniotomy for evacuation of subdural hematomas in 1 patient, and brain biopsy in 1 patient.

Conclusions  Patients with spontaneous intracranial hypotension are commonly misdiagnosed, causing a significant delay in the initiation of effective treatments and exposing patients to the risks associated with treatment for disorders that mimic intracranial hypotension. Increasing the awareness of this spontaneous type of intracranial hypotension is required to decrease the high rate of misdiagnosis.

Figures in this Article

A HEADACHE that worsens on assuming an upright position and is relieved by recumbency is a well-recognized complication of lumbar puncture.1 When the onset of such a positional headache is spontaneous, a diagnostic challenge is created even though spontaneous intracranial hypotension has been recognized for more than 6 decades, and magnetic resonance imaging has greatly facilitated the diagnosis.212 A spontaneous spinal cerebrospinal fluid (CSF) leak is the typical cause of spontaneous intracranial hypotension.612 Such spontaneous CSF leaks may result from simple dural rents or fragile arachnoid cysts, often in the presence of an underlying but previously unrecognized generalized connective tissue disorder.7,1316 The symptoms of spontaneous intracranial hypotension may become debilitating. Effective treatment is available, and the risks of treatment for the variety of conditions that may mimic spontaneous intracranial hypotension are significant. Therefore, the importance of an expeditious correct diagnosis in these patients cannot be overemphasized. I investigated the diagnostic delay in a cohort of patients with spontaneous intracranial hypotension.

The patient population consisted of a consecutive group of patients who were referred to the Maxine Dunitz Neurosurgical Institute (Los Angeles, Calif) for consideration of surgical repair of a spontaneous spinal CSF leak between January 1, 2001, and June 30, 2002. All patients were evaluated in an outpatient setting, a treatment plan was initiated, and clinical follow-up was obtained at regular intervals. Information regarding the initial clinical course and details of the various physician encounters was gathered by interviews with the patients and physicians by telephone or written correspondence and by review of medical records.

During the 18-month study period, 18 patients (15 women and 3 men; mean age, 38 years [range, 22-55 years]) were identified as having a spontaneous spinal CSF leak (Table 1). The CSF leak was documented by computed tomographic myelography in all patients. All patients had health insurance, and their level of educational attainment was high. Treatment consisted of surgical repair of the spontaneous spinal CSF leak in 13 patients and nonsurgical management, including epidural blood patching, in 5 patients.

Table Graphic Jump LocationTable 1. Characteristics of 18 Patients With Spontaneous Intracranial Hypotension*

All patients had a positional headache at initial examination. The headache was unlike any other they had ever experienced, and a variety of associated symptoms was present (Table 2). All but 2 patients remembered the exact time, date, and circumstances of the onset of the headache. Ten patients sought prompt medical attention (ie, within 24 hours from the onset of headache), and the remaining 8 patients waited 2 days to 2 months to seek medical attention (median, 3 days; mean, 22 days).

Table Graphic Jump LocationTable 2. Symptoms Among 18 Patients With Spontaneous Intracranial Hypotension

Only 1 patient was diagnosed as having spontaneous intracranial hypotension at the first physician encounter, and 17 patients (94%) initially received an incorrect diagnosis (Table 3). These 17 patients sought attention from 1 to 6 physicians before the correct diagnosis was made, and a total of 33 working diagnoses were established (Table 4 and Table 5). The diagnostic delay ranged from 4 days to 13 years (median, 5 weeks; mean, 13 months).

Table Graphic Jump LocationTable 3. Initial Misdiagnosis in 17 Patients With Spontaneous Intracranial Hypotension
Table Graphic Jump LocationTable 4. Erroneous Working Diagnoses Among 17 Patients With Spontaneous Intracranial Hypotension
Table Graphic Jump LocationTable 5. Specialty of Physician Misdiagnosing Spontaneous Intracranial Hypotension

Diagnostic procedures associated with risk included cerebral arteriography in 2 patients, craniotomy with meningeal biopsy in 1 patient (Figure 1), and transesophageal echocardiography in 1 patient. Erroneous diagnoses resulted in craniotomies for decompression of cerebellar tonsillar descent (mimicking Chiari type I malformation) in 2 patients (Figure 2) and evacuation of subdural fluid collection in 1 patient (Figure 3).

Place holder to copy figure label and caption
Figure 1.

A, Axial gadolinium-enchanced T1-weighted magnetic resonance imaging demonstrates diffuse pachymeningeal enhancement (arrows) in a 44-year-old woman. B, She underwent a brain biopsy but was subsequently found to have a spontaneous cerebrospinal fluid leak (arrow) at the cervicothoracic junction on a radionuclide cisternogram.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

A, sagittal T1-weighted magnetic resonance imaging demonstrates brain sagging. Note the inferior displacement of the optic chiasm (arrowheads), flattening of the pons (straight arrows), and cerebellar tonsillar herniation (curved arrow) mimicking a Chiari malformation, for which this 40-year-old woman underwent a suboccipital decompressive craniotomy. B, Subsequent computed tomogaphic myelography demonstrates bilateral arachnoid cysts (arrows) and spontaneous cerebrospinal fluid leaks originating from the C7 nerve roots.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.

A, Axial fluid-attenuated inversion recovery magnetic resonance imaging demonstrates bilateral subdural hematomas of different ages (arrows) in a 37-year-old man. B, He underwent bilateral craniotomies for evacuation of the subdural hematomas, but the subsequent computed tomogaphic myelography demonstrates a spontaneous cerebrospinal fluid leak along the right T9nerve root (asterisk) and a contralateral arachnoid cyst (arrow).

Graphic Jump Location

In this study, the diagnosis of spontaneous intracranial hypotension was rarely made at the initial physician visit and typically patients endured weeks, months, or even years of treatment for a variety of neurologic disorders. Most likely, this was due to the unfamiliarity of physicians with the syndrome of spontaneous intracranial hypotension. Also, the findings from CSF examinations as well as the imaging features of spontaneous intracranial hypotension may mimic other, more serious neurologic conditions.

SYMPTOMS
Headache

The typical patient with spontaneous intracranial hypotension has a headache that occurs or worsens soon after assuming an upright position and disappears or improves after resuming a recumbent position.216 The onset of the headache is usually gradual, reaching maximal intensity in several minutes to hours. However, some patients report an acute onset of headaches (thunderclap headache), and it is not surprising that such patients are suspected of having a subarachnoid hemorrhage and may undergo not only computed tomography but cerebral angiography as well.17 The severity of the headache varies, and many mild cases undoubtedly are never diagnosed. In some patients, however, the headaches become incapacitating, preventing them from engaging in any useful activity while in an upright position. The headache most likely is the direct result of the decrease in CSF volume and the downward displacement of the brain, causing traction on pain-sensitive structures. Although a postural headache is the clinical hallmark of intracranial hypotension, it is well known that the posture-related component often becomes less prominent or even disappears over time when the underlying CSF leak is left untreated and becomes chronic. It is an exceptional patient who has no posture-related component to the headache from the onset of symptoms.18,19

Associated Symptoms

A wide variety of symptoms may be associated with spontaneous intracranial hypotension, sometimes becoming more prominent or more worrisome than the headache, but the presence of the unique positional features of the headache should direct the physician to the correct diagnosis. Nausea, vomiting, photophobia, and posterior neck pain or stiffness are commonly seen and suggest meningeal irritation, mimicking subarachnoid hemorrhage or infectious meningitis. Many of the other associated symptoms are believed to be directly related to the downward displacement of the brain due to loss of CSF buoyancy. Diplopia, changes in hearing, and vertigo are common and may be caused by stretching of the ocular motor, cochlear, and vestibular nerves, respectively.218,2023 Similar mechanisms could explain less commonly reported manifestations of intracranial hypotension, such as facial 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 of the optic apparatus over the pituitary fossa.20 Stretching of cervical nerve roots could be implicated as a cause of the radicular arm pain or arm numbness occasionally found in patients with spontaneous intracranial hypotension. An alternative hypothesis to explain the disturbances of hearing or balance is that the abnormal change in CSF pressure is directly transmitted to that in the cochlea or labyrinth.24

Rarely, severe sagging of the brain may result in coma from diencephalic or hind brain herniation.2527 Other rare manifestations may include parkinsonism and dementia.28,29 More subtle cognitive deficits, however, are probably underrecognized and may not be appreciated until cognition improves following successful treatment of the spinal CSF leak.

INITIAL DIAGNOSTIC PROCEDURES
Lumbar Puncture

Typically, patients with spontaneous intracranial hypotension have an opening pressure at the time of lumbar puncture of less than 60 mm of water (normal, 65-195 mm of water).6 Occasionally, a dry tap is initially encountered, and CSF can only be obtained with aspiration using a syringe, with the patient in an upright position, or with a Valsalva maneuver. 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. Examination of CSF often demonstrates abnormal findings, such as an elevated protein content and pleocytosis.610 The pleocytosis is primarily lymphocytic, and the white blood cell count may exceed 200 white blood cells per milliliter, a finding that may be confused with infectious meningitis.610

Magnetic Resonance Imaging

Magnetic resonance imaging of the head has now supplanted lumbar puncture as the diagnostic study of choice in the initial evaluation of patients suspected of having spontaneous intracranial hypotension. The 3 most characteristic imaging features of intracranial hypotension are enhancement of the pachymeninges, downward displacement of the brain (brain sagging), and subdural fluid collections.613,30

Most, but not all, patients with spontaneous intracranial hypotension demonstrate pachymeningeal enhancement on magnetic resonance imaging.613,30,31 The enhancement is diffuse, only involves the pachymeninges (dura mater), and spares the leptomeninges.613,30,31 There may be diagnostic confusion with neoplastic, inflammatory, or granulomatous disease. It is not uncommon for patients with spontaneous intracranial hypotension to have undergone a meningeal biopsy. Reactive hyperemia of the pituitary gland may also be seen, mimicking a pituitary tumor,32,33 and may occasionally be associated with a concurrent increase in the level of circulating prolactin.34

Brain sagging is a very specific finding in spontaneous intracranial hypotension.710,30 It can be identified by several features, such as effacement of the suprasellar cistern, bowing of the optic chiasm over the pituitary fossa, flattening of the pons against the clivus, and downward displacement of the cerebellar tonsils. The displacement of the cerebellar tonsils may be mistaken for a Chiari I malformation, and some patients have undergone decompressive posterior fossa surgery, with no relief of their symptoms.7

Subdural fluid collections are common in patients with spontaneous intracranial hypotension.712,18,19,35 Most of these fluid collections are bilateral, thin, and do not cause any appreciable mass effect. Occasionally, larger symptomatic, subdural hematomas require surgical evacuation, but if the underlying spinal CSF leak is left untreated, the recurrence rate of the subdural hematoma is high.35 Loss of CSF volume may also result in compensatory enlargement of the spinal epidural venous plexus, which may become symptomatic and mimic neoplastic, inflammatory, or degenerative disc disease.3639

MANAGEMENT

Most patients with spontaneous intracranial hypotension respond well to bed rest, a generous intake of oral fluids, and the administration of caffeine, glucocorticoid medication, or mineralocorticoid agents. The mainstay of treatment of persistent spontaneous intracranial hypotension is the placement of 1 or more lumbar epidural blood patches, using approximately 10 to 15 mL of blood. If the patient fails to respond to this therapy, computed tomographic myelography is performed to confirm and localize the CSF leak. In most patients, a CSF leak is found in the cervical or thoracic spine. Therefore, consideration should be given to the placement of a high-volume (20-40 mL) thoracolumbar epidural blood patch, after which the patient is placed supine in the Trendelenburg position for 20 to 30 minutes, followed by the prone position for a similar time period. This allows blood to travel over many spinal segments toward the site of the CSF leak. If this fails to alleviate the symptoms, a directed epidural blood patch or percutaneous placement of fibrin glue is recommended. At the Maxine Dunitz Neurosurgical Institute, surgical treatment is reserved for those patients in whom these nonsurgical measures have failed. Computed tomographic myelography is the diagnostic test of choice to precisely localize the CSF leak,7 but a retrospinal fluid collection at the C1-C2 level should not be mistaken for the actual site of CSF leakage.36 Rather, these C1-C2 CSF collections are the result of CSF leaking into the epidural space at another site, extending rostrally within the spinal canal and eventually escaping into the soft tissues at the C1-C2 level.37

These data clearly show that for most patients with spontaneous intracranial hypotension, the diagnosis is missed initially and the diagnostic delay is significant. This exposes patients to risks associated with treatment for the disorders that can mimic intracranial hypotension. It is likely that for other patients with spontaneous intracranial hypotension, the diagnostic delay is even more pronounced, or the correct diagnosis is never established. It should be noted that the patients in this report were well educated, had medical insurance with ready access to medical care, and had symptoms severe enough to warrant consideration of surgical correction of the underlying spinal CSF leak and thus were more likely to be diagnosed. Headache is one of the most common reasons for a consultation with a primary care physician, a visit to the emergency department, or referral to a neurologist.40 Clearly, spontaneous intracranial hypotension is rare among all patients with headache complaints, but it is an important diagnostic consideration for patients with "new daily persistent headaches."41,42 Increasing the awareness of this spontaneous form of positional headaches will likely result in fewer patients in whom this diagnosis is missed.

Corresponding author and reprints: Wouter I. Schievink, MD, Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, 8631 W Third St, Suite 800 E, Los Angeles, CA 90048 (e-mail: schievinkw@cshs.org).

Accepted for publication July 28, 2003.

Evans  RW Complications of lumbar puncture. Neurol Clin.1998;16:83-105.
PubMed
Schaltenbrand  G Neuere anschauungen zur pathophysiologie der liquorzirkulation. Zentralbl Neurochir.1938;3:290-300.
Lindquist  TMoberg  E Spontaneous hypoliquorrea. Acta Med Scand.1949;132:556-561.
Bell  WEJoynt  RJSahs  AL Low spinal fluid pressure syndromes. Neurology.1960;10:512-521.
Teng  PPapatheodorou  C Primary cerebrospinal fluid hypotension. Bull Los Angeles Neurol Soc.1968;33:121-128.
PubMed
Rando  TAFishman  RA Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology.1992;42:481-487.
PubMed
Schievink  WIMeyer  FBAtkinson  JLDMokri  B Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg.1996;84:598-605.
PubMed
Mokri  BPiepgras  DGMiller  GM Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc.1997;72:400-413.
PubMed
Dillon  WPFishman  RA Some lessons learned about the diagnosis and treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol.1998;19:1001-1002.
PubMed
Schievink  WI Spontaneous spinal cerebrospinal fluid leaks: a review. Neurosurg Focus.2000;9(article 8):1-9.
Chung  SJKim  JSLee  MC Syndrome of cerebral spinal fluid hypovolemia: clinical and imaging features and outcome. Neurology.2000;55:1321-1327.
PubMed
Miyazawa  KShiga  YHasegawa  T  et al CSF hypovolemia vs intracranial hypotension in "spontaneous intracranial hypotension syndrome." Neurology.2003;60:941-947.
PubMed
Schievink  WIReimer  RFolger  WN Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg.1994;80:736-739.
PubMed
Schievink  WITorres  VE Spinal meningeal diverticula in autosomal dominant polycystic kidney disease. Lancet.1997;349:1223-1224.
PubMed
Schievink  WIMorreale  VMAtkinson  JLMeyer  FBPiepgras  DGEbersold  MJ Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg.1998;88:243-246.
PubMed
Schrijver  ISchievink  WIGodfrey  MMeyer  FBFrancke  U Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg.2002;96:483-489.
PubMed
Schievink  WIWijdicks  EFMeyer  FBSonntag  VK Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery.2001;48:513-517.
PubMed
Not Available Case records of the Massachusetts General Hospital: weekly clinicopathological exercises: case 2-1998: a 50-year-old woman with increasing headache and a left abducent-nerve palsy N Engl J Med..1998;338:180-188.
PubMed
Schievink  WISmith  KA Nonpositional headache caused by spontaneous intracranial hypotension. Neurology.1998;51:1768-1769.
PubMed
Horton  JCFishman  RA Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology.1994;101:244-251.
PubMed
Weitz  SRDrasner  K Spontaneous intracranial hypotension: a series. Anesthesiology.1996;85:923-925.
PubMed
Ferrante  ESavino  ABrioschi  AMarazzi  RDonato  MFRiva  M Transient oculomotor cranial nerves palsy in spontaneous intracranial hypotension. J Neurosurg Sci.1998;42:177-179.
PubMed
Warner  GT Spontaneous intracranial hypotension causing a partial third cranial nerve palsy: a novel observation. Cephalalgia.2002;22:822-823.
PubMed
Portier  Fde Minteguiaga  CRacy  EHuy  PTHerman  T Spontaneous intracranial hypotension: a rare cause of labyrynthine hydrops. Ann Otol Rhinol Laryngol.2002;111:817-820.
PubMed
Beck  CERizk  NWKiger  LTSpencer  DHill  LAdler  JR Intracranial hypotension presenting with severe encephalopathy. J Neurosurg.1998;89:470-473.
PubMed
Pleasure  SJAbosch  AFriedman  J  et al Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology.1998;50:1854-1857.
PubMed
Binder  DKDillon  WPFishman  RASchmidt  MH Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery.2002;51:830-837.
PubMed
Pakiam  ASLee  CLang  AE Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol.1999;56:869-872.
PubMed
Hong  MShah  GVAdams  KMTurner  RSFoster  NL Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology.2002;58:1285-1287.
PubMed
Fishman  RADillon  WP Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology.1993;43:609-611.
PubMed
Moayeri  NNHenson  JWSchaefer  PWZervas  NT Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension: report of three cases and review of the literature. J Neurosurg.1998;88:912-918.
PubMed
Shimazu  NOba  HAoki  SMonzawa  SMakita  KAraki  T Pituitary enlargement in spontaneous intracranial hypotension on MRI [in Japanese]. Nippon Igaku Hoshasen Gakkai Zasshi.1998;58:349-352.
PubMed
Alvarez-Linera  JEscribano  JBenito-Leon  JPorta-Etessam  JRovira  A Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology.2000;55:1895-1897.
PubMed
Yamamoto  MSuehiro  TNakata  H Primary low cerebrospinal fluid pressure syndrome associated with galactorrhea. Intern Med.1993;32:228-231.
PubMed
Sato  YHonda  YMaruoka  HFujimatsu  YOizumi  K Recurrence of spontaneous intracranial hypotension with subdural hematomas. Cephalalgia.1997;17:42-45.
PubMed
Yousry  IForderreuther  SMoriggl  B  et al Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol.2001;22:1239-1250.
PubMed
Dillon  WP Spinal manifestations of intracranial hypotension. AJNR Am J Neuroradiol.2001;22:1233-1234.
PubMed
Chiapparini  LFarina  LD'Incerti  L  et al Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology.2002;44:143-152.
PubMed
Chun  JYDillon  WPBerger  MS Symptomatic enlarged cervical anterior epidural venous plexus in a patient with Marfan syndrome. AJNR Am J Neuroradiol.2002;23:622-624.
PubMed
Evans  RWRozen  TD Etiology and treatment of new daily persistent headache. Headache.2001;41:830-832.
PubMed
Goadsby  PJBoes  C New daily persistent headache. J Neurol Neurosurg Psychiatry.2002;72(suppl 2):116-119.
PubMed
Evans  RW Diagnostic testing for headache. Med Clin North Am.2001;85:865-885.
PubMed

Figures

Place holder to copy figure label and caption
Figure 1.

A, Axial gadolinium-enchanced T1-weighted magnetic resonance imaging demonstrates diffuse pachymeningeal enhancement (arrows) in a 44-year-old woman. B, She underwent a brain biopsy but was subsequently found to have a spontaneous cerebrospinal fluid leak (arrow) at the cervicothoracic junction on a radionuclide cisternogram.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

A, sagittal T1-weighted magnetic resonance imaging demonstrates brain sagging. Note the inferior displacement of the optic chiasm (arrowheads), flattening of the pons (straight arrows), and cerebellar tonsillar herniation (curved arrow) mimicking a Chiari malformation, for which this 40-year-old woman underwent a suboccipital decompressive craniotomy. B, Subsequent computed tomogaphic myelography demonstrates bilateral arachnoid cysts (arrows) and spontaneous cerebrospinal fluid leaks originating from the C7 nerve roots.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.

A, Axial fluid-attenuated inversion recovery magnetic resonance imaging demonstrates bilateral subdural hematomas of different ages (arrows) in a 37-year-old man. B, He underwent bilateral craniotomies for evacuation of the subdural hematomas, but the subsequent computed tomogaphic myelography demonstrates a spontaneous cerebrospinal fluid leak along the right T9nerve root (asterisk) and a contralateral arachnoid cyst (arrow).

Graphic Jump Location

Tables

Table Graphic Jump LocationTable 1. Characteristics of 18 Patients With Spontaneous Intracranial Hypotension*
Table Graphic Jump LocationTable 2. Symptoms Among 18 Patients With Spontaneous Intracranial Hypotension
Table Graphic Jump LocationTable 3. Initial Misdiagnosis in 17 Patients With Spontaneous Intracranial Hypotension
Table Graphic Jump LocationTable 4. Erroneous Working Diagnoses Among 17 Patients With Spontaneous Intracranial Hypotension
Table Graphic Jump LocationTable 5. Specialty of Physician Misdiagnosing Spontaneous Intracranial Hypotension

References

Evans  RW Complications of lumbar puncture. Neurol Clin.1998;16:83-105.
PubMed
Schaltenbrand  G Neuere anschauungen zur pathophysiologie der liquorzirkulation. Zentralbl Neurochir.1938;3:290-300.
Lindquist  TMoberg  E Spontaneous hypoliquorrea. Acta Med Scand.1949;132:556-561.
Bell  WEJoynt  RJSahs  AL Low spinal fluid pressure syndromes. Neurology.1960;10:512-521.
Teng  PPapatheodorou  C Primary cerebrospinal fluid hypotension. Bull Los Angeles Neurol Soc.1968;33:121-128.
PubMed
Rando  TAFishman  RA Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology.1992;42:481-487.
PubMed
Schievink  WIMeyer  FBAtkinson  JLDMokri  B Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg.1996;84:598-605.
PubMed
Mokri  BPiepgras  DGMiller  GM Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc.1997;72:400-413.
PubMed
Dillon  WPFishman  RA Some lessons learned about the diagnosis and treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol.1998;19:1001-1002.
PubMed
Schievink  WI Spontaneous spinal cerebrospinal fluid leaks: a review. Neurosurg Focus.2000;9(article 8):1-9.
Chung  SJKim  JSLee  MC Syndrome of cerebral spinal fluid hypovolemia: clinical and imaging features and outcome. Neurology.2000;55:1321-1327.
PubMed
Miyazawa  KShiga  YHasegawa  T  et al CSF hypovolemia vs intracranial hypotension in "spontaneous intracranial hypotension syndrome." Neurology.2003;60:941-947.
PubMed
Schievink  WIReimer  RFolger  WN Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg.1994;80:736-739.
PubMed
Schievink  WITorres  VE Spinal meningeal diverticula in autosomal dominant polycystic kidney disease. Lancet.1997;349:1223-1224.
PubMed
Schievink  WIMorreale  VMAtkinson  JLMeyer  FBPiepgras  DGEbersold  MJ Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg.1998;88:243-246.
PubMed
Schrijver  ISchievink  WIGodfrey  MMeyer  FBFrancke  U Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg.2002;96:483-489.
PubMed
Schievink  WIWijdicks  EFMeyer  FBSonntag  VK Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery.2001;48:513-517.
PubMed
Not Available Case records of the Massachusetts General Hospital: weekly clinicopathological exercises: case 2-1998: a 50-year-old woman with increasing headache and a left abducent-nerve palsy N Engl J Med..1998;338:180-188.
PubMed
Schievink  WISmith  KA Nonpositional headache caused by spontaneous intracranial hypotension. Neurology.1998;51:1768-1769.
PubMed
Horton  JCFishman  RA Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology.1994;101:244-251.
PubMed
Weitz  SRDrasner  K Spontaneous intracranial hypotension: a series. Anesthesiology.1996;85:923-925.
PubMed
Ferrante  ESavino  ABrioschi  AMarazzi  RDonato  MFRiva  M Transient oculomotor cranial nerves palsy in spontaneous intracranial hypotension. J Neurosurg Sci.1998;42:177-179.
PubMed
Warner  GT Spontaneous intracranial hypotension causing a partial third cranial nerve palsy: a novel observation. Cephalalgia.2002;22:822-823.
PubMed
Portier  Fde Minteguiaga  CRacy  EHuy  PTHerman  T Spontaneous intracranial hypotension: a rare cause of labyrynthine hydrops. Ann Otol Rhinol Laryngol.2002;111:817-820.
PubMed
Beck  CERizk  NWKiger  LTSpencer  DHill  LAdler  JR Intracranial hypotension presenting with severe encephalopathy. J Neurosurg.1998;89:470-473.
PubMed
Pleasure  SJAbosch  AFriedman  J  et al Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology.1998;50:1854-1857.
PubMed
Binder  DKDillon  WPFishman  RASchmidt  MH Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery.2002;51:830-837.
PubMed
Pakiam  ASLee  CLang  AE Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol.1999;56:869-872.
PubMed
Hong  MShah  GVAdams  KMTurner  RSFoster  NL Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology.2002;58:1285-1287.
PubMed
Fishman  RADillon  WP Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology.1993;43:609-611.
PubMed
Moayeri  NNHenson  JWSchaefer  PWZervas  NT Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension: report of three cases and review of the literature. J Neurosurg.1998;88:912-918.
PubMed
Shimazu  NOba  HAoki  SMonzawa  SMakita  KAraki  T Pituitary enlargement in spontaneous intracranial hypotension on MRI [in Japanese]. Nippon Igaku Hoshasen Gakkai Zasshi.1998;58:349-352.
PubMed
Alvarez-Linera  JEscribano  JBenito-Leon  JPorta-Etessam  JRovira  A Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology.2000;55:1895-1897.
PubMed
Yamamoto  MSuehiro  TNakata  H Primary low cerebrospinal fluid pressure syndrome associated with galactorrhea. Intern Med.1993;32:228-231.
PubMed
Sato  YHonda  YMaruoka  HFujimatsu  YOizumi  K Recurrence of spontaneous intracranial hypotension with subdural hematomas. Cephalalgia.1997;17:42-45.
PubMed
Yousry  IForderreuther  SMoriggl  B  et al Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol.2001;22:1239-1250.
PubMed
Dillon  WP Spinal manifestations of intracranial hypotension. AJNR Am J Neuroradiol.2001;22:1233-1234.
PubMed
Chiapparini  LFarina  LD'Incerti  L  et al Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology.2002;44:143-152.
PubMed
Chun  JYDillon  WPBerger  MS Symptomatic enlarged cervical anterior epidural venous plexus in a patient with Marfan syndrome. AJNR Am J Neuroradiol.2002;23:622-624.
PubMed
Evans  RWRozen  TD Etiology and treatment of new daily persistent headache. Headache.2001;41:830-832.
PubMed
Goadsby  PJBoes  C New daily persistent headache. J Neurol Neurosurg Psychiatry.2002;72(suppl 2):116-119.
PubMed
Evans  RW Diagnostic testing for headache. Med Clin North Am.2001;85:865-885.
PubMed

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 74

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
PubMed Articles