Abstracts 2013

Starling A, Hernandez F, Hoxworth JM, Trentman T, Halker R, Vargas BB, Hastriter E, Dodick D
Neurology. 2013 Nov 12;81(20):1789-92.
doi: 10.1212/01.wnl.0000435555.13695.22.
Abstract
OBJECTIVE:
To investigate the sensitivity of MRI of the spine compared with CT myelography (CTM) in detecting CSF leaks.
METHODS:
Between July 1998 and October 2010, 12 patients with orthostatic headache and a CTM-confirmed spinal CSF leak underwent an MRI of the spine with and without contrast. Using CTM as the gold standard, we retrospectively investigated the sensitivity of spinal MRI in detecting a CSF leak.
RESULTS:
Eleven of 12 patients with a CSF leak documented by CTM also had extradural fluid collections on spinal MRI (sensitivity 91.7%). Six patients with extradural fluid collections on spinal MRI also had spinal dural enhancement.
CONCLUSION:
When compared with the gold standard of CTM, MRI of the spine appears to be a sensitive and less invasive imaging modality for detecting a spinal CSF leak, suggesting that MRI of the spine should be the imaging modality of first choice for the detection of spinal CSF leaks.
PMID: 24107860

Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L.
J Pediatr. 2013 Aug;163(2):504-10.
doi: 10.1016/j.jpeds.2013.01.055.
Abstract
OBJECTIVES:
To describe the clinical and radiographic manifestations of spontaneous intracranial hypotension, a rarely diagnosed cause of headache in children.
STUDY DESIGN:
This study included patients 19 years of age or younger evaluated between January 1, 2001, and June 30, 2012, for spontaneous intracranial hypotension.
RESULTS:
We evaluated 24 children (18 girls and 6 boys) with spontaneous intracranial hypotension (age at onset of symptoms: 2-19 years, mean 14.3 years). Twenty-three patients presented with orthostatic headaches and 1 presented with a nonpositional headache. A generalized connective tissue disorder was diagnosed in 54% of patients. Magnetic resonance imaging showed the typical changes of spontaneous intracranial hypotension in most patients (79%). Spinal imaging demonstrated a cerebrospinal fluid (CSF) leak with or without an associated meningeal diverticulum in 12 patients (50%) and with dural ectasia or meningeal diverticula in 10 patients (42%), and it was normal in 2 patients (8%). Twenty-three patients initially underwent epidural blood patching, but 8 patients also were treated with percutaneous injections of fibrin glue and 11 patients eventually required surgical correction of the underlying CSF leak. There was no morbidity or mortality associated with any of the treatments, but 5 patients required acetazolamide for rebound high intracranial pressure headache. Overall, outcome was good in 22 patients (92%) and poor in 2 patients (8%).
CONCLUSIONS:
Spontaneous intracranial hypotension in childhood is rare. Most patients can be treated effectively using a combination of epidural blood patching and percutaneous injections of fibrin glue or surgical CSF leak repair in refractory cases.
PMID: 23453548

Hashizume K, Watanabe K, Kawaguchi M, Fujiwara A, Furuya H.
Clin Neurol Neurosurg. 2013 Aug;115(8):1403-6.
doi: 10.1016/j.clineuro.2013.01.022.
Abstract
OBJECTIVE:
Subdural hematoma (SDH) is a frequent complication of spontaneous intracranial hypotension (SIH), in which epidural blood patch (EBP) may be applied as a treatment to stop cerebrospinal fluid (CSF) leak. However, a clinical course of SDH in SIH patients has not been sufficiently evaluated. We retrospectively evaluated the temporal relationships between EBP and SDH in the patients with SIH.
METHODS:
Twenty-nine consecutive patients, diagnosed as SIH, were studied. Clinical records and images were retrospectively evaluated. When orthostatic headache continued for 2 weeks regardless of conservative treatment, EBP was performed under fluoroscopy.
RESULTS:
We detected 13(45%) cases of SDH (mean age 44 years, 8 males and 5 females). In 6 patients, SDHs disappeared after effective EBP, i.e., after the disappearance of orthostatic headache. In 3 patients, SDHs were enlarged or recurred after effective EBP, and in 4 patients, SDHs were first detected after effective EBP.
CONCLUSION:
The knowledge of the presence of these types of SDH (enlarged or recurred or detected after EBP) may deserve clinical attention.
PMID: 23462186

Schievink WI.
Curr Neurol Neurosci Rep. 2013 Jul;13(7):358.
doi: 10.1007/s11910-013-0358-z.
Abstract
Although for the vast majority of patients with spontaneous intracranial hypotension knowledge of the exact site of the underlying spinal CSF leak is not necessary, it is for patients with recalcitrant symptoms. Such patients may require directed treatments such as percutaneous fibrin glue injections or surgery. A variety of MRI techniques have been shown to be able to detect CSF leaks as well and sometimes better than the “gold standard” – CT-myelography. For unusually rapid CSF leaks – particularly those ventral to the spinal cord – digital subtraction myelography or dynamic CT-myelography are indicated. Some patients with spontaneous intracranial hypotension verified by intracranial MRI are never found to have a spinal CSF leak using current techniques.
PMID: 23703239

Mokri B.
Headache. 2013 Jul-Aug;53(7):1034-53.
doi: 10.1111/head.12149.
Abstract
Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short-term recurrences are not uncommon, and long-term recurrences are not rare.
PMID: 23808630
Full text

Kranz PG, Stinnett SS, Huang KT, Gray L.
Am J Neuroradiol. 2013 Jun-Jul;34(6):1284-9.
doi: 10.3174/ajnr.A3359.
Abstract
BACKGROUND AND PURPOSE:
Spinal meningeal diverticula have been implicated in the pathogenesis of spontaneous intracranial hypotension and have been proposed as both diagnostic features of and therapeutic targets for the condition. We compared the prevalence and myelographic appearance of spinal diverticula in patients with SIH and healthy controls.
MATERIALS AND METHODS:
Patients satisfying the ICHD-2 criteria for SIH were retrospectively identified. CT myelograms of 19 patients with SIH were compared with CT myelograms of 18 control patients. Images were reviewed by 2 blinded neuroradiologists. The prevalence, morphology (round versus multilobulated), size, and location (cervical, upper thoracic, lower thoracic, or lumbar) of spinal meningeal diverticula were analyzed.
RESULTS:
There was no difference in the proportion of patients with diverticula in the SIH group compared with the control group (68% versus 44%, P = .14) or in the mean number of diverticula per patient (6.3 versus 2.2, P = .099). No difference was seen in the morphology (P = .95) or size (P = .71) of diverticula between groups. There was a difference between groups that just reached statistical significance (P = .050) in the location of the diverticula along the spinal axis, but substantial overlap was seen between groups for all spinal locations.
CONCLUSIONS:
Despite the well-established association between spinal meningeal diverticula and SIH, we found no difference in the prevalence or myelographic appearance of diverticula in patients with SIH compared with controls. Further investigation into the role of diverticula in the diagnosis and treatment of SIH is necessary.
PMID: 23221945
full text: ajnr.org/content/34/6/1284.long

Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI.
Eur J Hum Genet. 2013 Apr;21(4):386-90.
doi: 10.1038/ejhg.2012.191.
Abstract
We aimed to assess the frequency of connective tissue abnormalities among patients with cerebrospinal fluid (CSF) leaks in a prospective study using a large cohort of patients. We enrolled a consecutive group of 50 patients, referred for consultation because of CSF leak. All patients have been carefully examined for the presence of connective tissue abnormalities, and based on findings, patients underwent genetic testing. Ancillary diagnostic studies included echocardiography, eye exam, and histopathological examinations of skin and dura biopsies in selected patients. We identified nine patients with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome and other unclassified forms. In seven patients, spontaneous CSF leak was the first noted manifestation of the genetic disorder. We conclude that spontaneous CSF leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. We propose that there is a clinical basis for considering spontaneous CSF leak as a clinical manifestation of heritable connective tissue disorders, and we suggest that patients with CSF leaks should be screened for connective tissue and vascular abnormalities.
PMID: 22929030
PMC: PMC3598315

Chotai S, Kim JH, Kim JH, Kwon TH.
Asian J Neurosurg. 2013 Apr;8(2):112-5.
doi: 10.4103/1793-5482.116390.
Abstract
Spontaneous intracranial hypotension (SIH), typically presents with orthostatic headache, low pressure on lumbar tapping, and diffuse pachymeningeal enhancement on magnetic resonance imaging. SIH is often accompanied by subdural fluid collections, which in most cases responds to conservative treatment or spinal epidural blood patch. Several authors advocate that large subdural hematoma with acute deterioration merits surgical drainage; however, few have reported complications following craniotomy. We describe a complicated case of SIH, which was initially diagnosed as acute subarachnoid hemorrhage with bilateral chronic subdural hematoma (SDH), due to unusual presentation. Burr hole drainage of subdural hematoma was performed due to progressive decrease of consciousness, which then resulted in a huge postoperative epidural hematoma collection. Prompt hematoma evacuation did not restore the patient’s consciousness but aggravated downward brain herniation. Trendelenburg position and spinal epidural blood patch achieved a rapid improvement in patient’s consciousness. This case indicates that the surgical drainage for chronic SDH in SIH can lead to serious complications and it should be cautiously considered.
PMID: 24049555
PMC: PMC3775182

Schievink WI.
Neurocrit Care. 2013 Apr;18(2):248-51.
doi: 10.1007/s12028-012-9800-3.
Abstract
BACKGROUND:
Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension.
METHODS:
Medical records and radiologic imaging of the two patients were reviewed.
RESULTS:
Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.
Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia.
CONCLUSIONS:
Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.
PMID: 23196352

Amoozegar F, Guglielmin D, Hu W, Chan D, Becker WJ.
Can J Neurol Sci. 2013 Mar;40(2):144-57.
Abstract
A literature search found no clinical trials or guidelines addressing the management of spontaneous intracranial hypotension (SIH). Based on the available literature and expert opinion, we have developed recommendations for the diagnosis and management of SIH. For typical cases, we recommend brain magnetic resonance (MR) imaging with gadolinium to confirm the diagnosis, and conservative measures for up to two weeks. If the patient remains symptomatic, up to three non-directed lumbar epidural blood patches (EBPs) should be considered. If these are unsuccessful, non-invasive MR myelography, radionuclide cisternography, MR myelography with intrathecal gadolinium, or computed tomography with myelography should be used to localize the leak. If the leak is localized, directed EPBs should be considered, followed by fibrin sealant or neurosurgery if necessary. Clinically atypical cases with normal brain MR imaging should be investigated to localize the leak. Directed EBPs can be used if the leak is localized; non-directed EBPs should be used only if there are indirect signs of SIH.
PMID: 23419561
Full text: headachenetwork.ca/wp-content/uploads/Amoozegar-et-al.pdf

Hébert-Blouin MN, Mokri B, Shin AY, Bishop AT, Spinner RJ.
J Neurosurg. 2013 Jan;118(1):149-54.
doi: 10.3171/2012.9.JNS112368.
Abstract
OBJECT:
Patients with brachial plexus injury (BPI) present with a combination of motor weakness/paralysis, sensory deficits, and pain. Brachial plexus injury is generally not believed to be associated with headaches. However, CSF leaks may be associated with CSF volume-depletion (low-pressure) headaches and can occur in BPI secondary to nerve root avulsion. Only a few cases of headaches associated with BPI have been reported. It is unknown if headaches in patients with BPI occur so rarely, or if they are just unrecognized by physicians and/or patients in which the focus of attention is the affected limb. The aim of this study was to determine the prevalence of CSF volume-depletion headaches in patients with BPI.
METHODS:
All adult patients presenting at the Mayo brachial plexus clinic with traumatic BPI were asked to complete a questionnaire addressing the presence and quality of headaches following their injury. The patients’ clinical, injury, and imaging characteristics were subsequently reviewed.
RESULTS:
Between December 2008 and July 2010, 145 patients completed the questionnaire. Twenty-two patients reported new onset headaches occurring after their BPI. Eight of these patients experienced positional headaches, suggestive of CSF volume depletion. One of the patients with orthostatic headaches was excluded because the headaches immediately followed a lumbar puncture for a myelogram. Six of the other 7 patients with positional headaches had a clear preganglionic BPI. The available imaging studies in these 6 patients revealed evidence of CSF leaks: pseudomeningoceles (n = 5), CSF tracking into soft tissues (n = 3), CSF tracking into the intraspinal compartment (n = 3), CSF tracking into the pleural space (n = 2), and low-positioned cerebellar tonsils (n = 2).
CONCLUSIONS:
In this retrospective study, 15.2% of patients (22 of 145 patients) with traumatic BPI suffered from a new-onset headache. Seven of these patients (4.8%) experienced postural headaches clearly suggestive of CSF volume depletion likely secondary to a CSF leak associated with the BPI, whereas the other 15 patients (10.3%) suffered headaches that may have represented a variant of CSF depletion headaches without a postural characteristic or a headache from another cause. These data suggest that CSF volume-depletion headaches occur in a significant proportion of patients with BPI and have been underrecognized and underreported.
PMID: 23082886
Full text

Schievink WI, Maya MM.
J Neurosurg Pediatr. 2013 Jan;11(1):48-51.
doi: 10.3171/2012.10.PEDS12353.
Abstract
Headache occurs after dural puncture in about 1%-25% of children who undergo the procedure-a rate similar to that seen in adults. Persistence of post-dural puncture headache in spite of bed rest, increased fluid intake, and epidural blood patch treatment, however, is rare. The authors reviewed the medical records and imaging studies of all patients 19 years of age or younger who they evaluated between 2001 and 2010 for intracranial hypotension, and they identified 8 children who had persistent post-dural puncture headache despite maximal medical treatment and placement of epidural blood patches. A CSF leak could be demonstrated radiologically and treated surgically in 3 of these patients, and the authors report these 3 cases. The patients were 2 girls (ages 14 and 16 years) who had undergone lumbar puncture for evaluation of headache and fever and 1 boy (age 13 years) who had undergone placement of a lumboperitoneal shunt using a Tuohy needle for treatment of pseudotumor cerebri. The boy also had undergone a laminectomy and exploration of the posterior dural sac, but no CSF leak could be identified. All 3 patients presented with new-onset orthostatic headaches, and in all 3 cases MRI demonstrated a large ventral lumbar or thoracolumbar CSF collection. Conventional myelography or digital subtraction myelography revealed a ventral dural defect at L2-3 requiring surgical repair. Through a posterior transdural approach, the dural defect was repaired using 6-0 Prolene sutures and a dural substitute. Postoperative recovery was uneventful, with complete resolution of orthostatic headache and of the ventral cerebrospinal fluid leak on MRI. The authors conclude that persistent postdural puncture headache requiring surgical repair is rare in children. They note that the CSF leak may be located ventrally and may require conventional or digital subtraction myelography for exact localization and that transdural repair is safe and effective in eliminating the headaches.
PMID: 23140214
Full text

Schievink WI, Chu RM, Maya MM, Johnson JP, Cohen HC.
J Neurosurg Spine. 2013 Jan;18(1):96-101.
doi: 10.3171/2012.10.SPINE12469.
Abstract
OBJECT:
The goal of the study was to elucidate the spinal manifestations of spontaneous intracranial hypotension.
METHODS:
The authors reviewed the medical records and imaging studies of 338 consecutive patients with spontaneous intracranial hypotension who were evaluated at their institution between 2001 and 2010.
RESULTS:
Twenty patients (6%; mean age 35.8 [range 16 to 60 years]; 5 males and 15 females) had convincing signs or symptoms referable to the spinal cord or spinal nerve roots. The spinal manifestations consisted of radiculopathy in 11 patients (unilateral in 8 and bilateral in 3), myelopathy in 8 patients, and bibrachial amyotrophy in 1 patient. The cervical spine was involved in 12 patients, the thoracic spine in 5, and the lumbosacral spine in 3. The spinal symptoms were positional in only 3 patients. The spinal manifestations occurred around the time of the headache onset in 16 patients, and months to years after the positional headache had resolved in 4 patients. A large extrathecal CSF collection causing compression of the spinal cord or nerve root was responsible for the spinal manifestations in the majority of patients. Treatment of the spinal CSF leak resulted in resolution of the spinal manifestations along with the headache, except for those in the patient with bibrachial amyotrophy.
CONCLUSIONS:
Spinal manifestations are uncommon in cases of spontaneous intracranial hypotension, occurring in about 6% of patients, but myelopathy and radiculopathy involving all spinal segments do occur. Unlike the headache, the spinal manifestations usually are not positional and are caused by mass effect from an extradural CSF collection.
PMID: 23121651