Differentiation of Chiari malformation type 1 and spontaneous intracranial hypotension using objective measurements of midbrain sagging

OBJECTIVE Chiari malformation type 1 (CM-1) and spontaneous intracranial hypotension (SIH) are causes of headache in which cerebellar tonsillar ectopia (TE) may be present. An accurate method for differentiating these conditions on imaging is needed to avoid diagnostic confusion. Here, the authors sought to determine whether objective measurements of midbrain morphology could distinguish CM-1 from SIH on brain MRI. METHODS This is a retrospective case-control series comparing neuroimaging in consecutive adult subjects with CM-1 and SIH. Measurements obtained from brain MRI included previously reported measures of brain sagging: TE, slope of the third ventricular floor (3VF), pontomesencephalic angle (PMA), mamillopontine distance, lateral ventricular angle, internal cerebral vein–vein of Galen angle, and displacement of iter (DOI). Clivus length (CL), an indicator of posterior fossa size, was also measured. Measurements for the CM-1 group were compared to those for the entire SIH population (SIHall) as well as a subgroup of SIH patients with > 5 mm of TE (SIHTE subgroup). RESULTS Highly significant differences were observed between SIHall and CM-1 groups in the following measures: TE (mean ± standard deviation, 3.1 ± 5.7 vs 9.3 ± 3.5 mm), 3VF (−16.8° ± 11.2° vs −2.1° ± 4.6°), PMA (44.8° ± 13.1° vs 62.7° ± 9.8°), DOI (0.2 ± 4.1 vs 3.8 ± 1.6 mm), and CL (38.3 ± 4.5 vs 44.0 ± 3.3 mm; all p < 0.0001). Eight (16%) of 50 SIH subjects had TE > 5 mm; in this subgroup (SIHTE), a cutoff value of < −15° for 3VF and < 45° for PMA perfectly discriminated SIH from CM-1 (sensitivity and specificity = 1.0). DOI showed perfect specificity (1.0) in detecting SIH among both groups. No subjects with SIH had isolated TE without other concurrent findings of midbrain sagging. CONCLUSIONS Measures of midbrain sagging, including cutoff values for 3VF and PMA, discriminate CM-1 from SIH and may help to prevent misdiagnosis and unnecessary surgery.

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Procedural predictors of epidural blood patch efficacy in spontaneous intracranial hypotension

Regional Anesthesia and Pain Medicine ◽

10.1136/rapm-2018-000021 ◽

2019 ◽

Vol 44 (2) ◽

pp. 212-220 ◽

Cited By ~ 3

Author(s):

Gabriel L Pagani-Estévez ◽

Jeremy K Cutsforth-Gregory ◽

Jonathan M Morris ◽

Bahram Mokri ◽

David G Piepgras ◽

...

Keyword(s):

Intracranial Hypotension ◽

Epidural Blood Patch ◽

Brain Mri ◽

Spontaneous Intracranial Hypotension ◽

Case Series ◽

Mri Findings ◽

Retrospective Case ◽

Volume Number ◽

Institutional Review ◽

Blood Patch

Background and objectiveEpidural blood patch (EBP) is a safe and effective treatment for spontaneous intracranial hypotension (SIH), but clinical and procedural variables that predict EBP efficacy remain nebulous.MethodsThis study is an institutional review board-approved retrospective case series with dichotomized EBP efficacy defined at 3 months. The study included 202 patients receiving 604 EBPs; iatrogenic cerebrospinal fluid leaks were excluded.ResultsOf the EBPs, 473 (78%) were single-level, 349 (58%) lumbar, 75 (12%) bilevel, and 56 (9%) multilevel (≥3 levels). Higher volume (OR 1.64; p<0.0001), bilevel (3.17, 1.91–5.27; p<0.0001), and multilevel (117.3, 28.04–490.67; p<0.0001) EBP strategies predicted greater efficacy. Only volume (1.64, 1.47–1.87; p<0.0001) remained significant in multivariate analysis. Site-directed patches were more effective than non-targeted patches (8.35, 0.97–72.1; p=0.033). Lower thoracic plus lumbar was the most successful bilevel strategy, lasting for a median of 74 (3–187) days.ConclusionsIn this large cohort of EBP in SIH, volume, number of spinal levels injected, and site-directed strategies significantly correlated with greater likelihood of first EBP efficacy. Volume and leak site coverage likely explain the increased efficacy with bilevel and multilevel patches. In patients with cryptogenic leak site, and either moderate disability, negative prognostic brain MRI findings for successful EBP, or failed previous lumbar EBP, a low thoracic plus lumbar bilevel EBP strategy is recommended. Multilevel EBP incorporating transforaminal administration and fibrin glue should be considered in patients refractory to bilevel EBP. An algorithmic approach to treating SIH is proposed.

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Comparisons of clinical characteristics, brain MRI findings, and responses to epidural blood patch between spontaneous intracranial hypotension and post-dural puncture headache: retrospective study

BMC Neurology ◽

10.1186/s12883-021-02279-5 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Gha-Hyun Lee ◽

Jiyoung Kim ◽

Hyun-Woo Kim ◽

Jae Wook Cho

Keyword(s):

Intracranial Hypotension ◽

Dural Puncture ◽

Epidural Blood Patch ◽

Brain Mri ◽

Spontaneous Intracranial Hypotension ◽

Vein Of Galen ◽

Mri Findings ◽

Post Dural Puncture Headache ◽

Straight Sinus ◽

Blood Patch

Abstract Background Spontaneous intracranial hypotension and post-dural puncture headache are both caused by a loss of cerebrospinal fluid but present with different pathogeneses. We compared these two conditions concerning their clinical characteristics, brain imaging findings, and responses to epidural blood patch treatment. Methods We retrospectively reviewed the records of patients with intracranial hypotension admitted to the Neurology ward of the Pusan National University Hospital between January 1, 2011, and December 31, 2019, and collected information regarding age, sex, disease duration, hospital course, headache intensity, time to the appearance of a headache after sitting, associated phenomena (nausea, vomiting, auditory symptoms, dizziness), number of epidural blood patch treatments, and prognosis. The brain MRI signs of intracranial hypotension were recorded, including three qualitative signs (diffuse pachymeningeal enhancement, venous distention of the lateral sinus, subdural fluid collection), and six quantitative signs (pituitary height, suprasellar cistern, prepontine cistern, mamillopontine distance, the midbrain-pons angle, and the angle between the vein of Galen and the straight sinus). Results A total of 105 patients (61 spontaneous intracranial hypotension patients and 44 post-dural puncture headache patients) who met the inclusion criteria were reviewed. More patients with spontaneous intracranial hypotension required epidural blood patch treatment than those with post-dural puncture headache (70.5% (43/61) vs. 45.5% (20/44); p = 0.01) and the spontaneous intracranial hypotension group included a higher proportion of patients who underwent epidural blood patch treatment more than once (37.7% (23/61) vs. 13.6% (6/44); p = 0.007). Brain MRI showed signs of intracranial hypotension in both groups, although the angle between the vein of Galen and the straight sinus was greater in the post-dural puncture headache group (median [95% Confidence Interval]: 85° [68°-79°] vs. 74° [76°-96°], p = 0.02). Conclusions Patients with spontaneous intracranial hypotension received more epidural blood patch treatments and more often needed multiple epidural blood patch treatments. Although both groups showed similar brain MRI findings, the angle between the vein of Galen and the straight sinus differed significantly between the groups.

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Postural Headache In A Patient With Marfan's Syndrome

Cephalalgia ◽

10.1046/j.1468-2982.2003.00587.x ◽

2003 ◽

Vol 23 (7) ◽

pp. 552-555 ◽

Cited By ~ 24

Author(s):

E Ferrante ◽

A Citterio ◽

A Savino ◽

P Santalucia

Keyword(s):

Intracranial Hypotension ◽

Brain Mri ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Marfan’S Syndrome ◽

Marfan's Syndrome ◽

Postural Headache ◽

Intracranial Hypotension Syndrome ◽

Increased Risk ◽

Diffuse Pachymeningeal Enhancement

A 26-year-old man with Marfan's syndrome had postural headache. Brain MRI with gadolinium showed diffuse pachymeningeal enhancement. MRI myelography revealed bilateral multiple large meningeal diverticula at sacral nerve roots level. He was suspected to have spontaneous intracranial hypotension syndrome. Eight days later headache improved with bed rest and hydration. One month after the onset he was asymptomatic and 3 months later brain MRI showed no evidence of diffuse pachymeningeal enhancement. The 1-year follow-up revealed no neurological abnormalities. The intracranial hypotension syndrome likely resulted from a CSF leak from one of the meningeal diverticula. In conclusion patients with spinal meningeal diverticula (frequently seen in Marfan's syndrome) might be at increased risk of developing CSF leaks, possibly secondary to Valsalva maneuver or minor unrecognizedtrauma.

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