Atypical Presentations of Intracranial Hypotension: Comparison with Classic Spontaneous Intracranial Hypotension

Capizzano, Arístides A.; Lai, Liangzhen; Kim, J.; Rizzo, M.; Gray, Linda; Smoot, M. Kyle; Moritani, Toshio

doi:10.3174/ajnr.a4706

Cited by 45 publications

(49 citation statements)

References 19 publications

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“…Atypical presentations reported in literature include short-term memory problems and a not-otherwise specified subcortical type of cognitive impairment, obtundation, stupor, drowsiness and even coma [130]. Although the compressions of the midbrain tegmentum and brainstem impression have been implicated in some of the clinical signs [128], we suggest that disturbed consciousness and memory could be attributed to splenial swelling and/or herniation (Figure 18).…”

Section: C1e Splenium In Intracranial Hypotension Syndromementioning

confidence: 82%

“…Intracranial hypotension is thought to result from CSF hypovolemia due to a spinal CSF leak. To compensate, several mechanisms come into play and can be seen on MRI: downward displacement of the brain with sagging brainstem, pituitary volume increase, dilatation of veins and dural sinuses, thickening and enhancement of the dura and sometimes subdural hematomas [128,129]. Another sign which has been described is a particular shape and position of the splenium, which appears stumpy and displaced downwards [129].…”

Section: C1e Splenium In Intracranial Hypotension Syndromementioning

confidence: 99%

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The splenium of the corpus callosum: embryology, anatomy, function and imaging with pathophysiological hypothesis

Blaauw

Meiners

2020

Neuroradiology

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Background and purpose The splenium of the corpus callosum is the most posterior part of the corpus callosum. Its embryological development, anatomy, vascularization, function, imaging of pathology, possible pathophysiological mechanisms by which pathology may develop and the clinical consequences are discussed. Methods A literature-based description is provided on development, anatomy and function. MR and CT images are used to demonstrate pathology. The majority of pathology, known to affect the splenium, and the clinical effects are described in three subsections: (A) limited to the splenium, with elaboration on pathophysiology of reversible splenial lesions, (B) pathology in the cerebral white matter extending into or deriving from the splenium, with special emphasis on tumors, and (C) splenial involvement in generalized conditions affecting the entire brain, with a hypothesis for pathophysiological mechanisms for the different diseases.Results The development of the splenium is preceded by the formation of the hippocampal commissure. It is bordered by the falx and the tentorium and is perfused by the anterior and posterior circulation. It contains different caliber axonal fibers and the most compact area of callosal glial cells. These findings may explain the affinity of specific forms of pathology for this region. The fibers interconnect the temporal and occipital regions of both hemispheres reciprocally and are important in language, visuospatial information transfer and behavior. Acquired pathology may lead to changes in consciousness. Conclusion The development, location, fiber composition and vascularization of the splenium make it vulnerable to specific pathological processes. It appears to play an important role in consciousness. AbbreviationAC anterior commissure ACC agenesis of the corpus callosum ADEM acute disseminated encephalomyelitis ADH anti-diuretic hormone AED anti-epileptic drugs AVP arginine-vasopressin CC corpus callosum CLOCC cytotoxic lesions of the corpus callosum DLBCL diffuse large B cell lymphoma GBM glioblastoma multiforme HC hippocampal commissure HIE hypoxic ischemic encephalopathy MERS mild encephalitis/encephalopathy with a reversible splenial lesion PCNSL primary central nervous system lymphoma RESLES reversible splenial lesions syndrome

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Section: C1e Splenium In Intracranial Hypotension Syndromementioning

confidence: 82%

Section: C1e Splenium In Intracranial Hypotension Syndromementioning

confidence: 99%

The splenium of the corpus callosum: embryology, anatomy, function and imaging with pathophysiological hypothesis

Blaauw

Meiners

2020

Neuroradiology

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“…But there can be many more debilitating symptoms to this condition, and the response of the body to a leak can vary Patient Experience Journal, Volume 6, Issue 3 -2019 significantly among individuals. [3][4][5][6][7][8] I experienced nearly all the classic symptoms of a CSF leak, 3,4 especially head pain that worsened when upright. Not a typical headache, it was as if a flat iron were pressing down on my brain.…”

Section: A Deficit Of Csfmentioning

confidence: 99%

“…Although rare, all of these additional symptoms have been evidenced in the literature as related to CSF leaks. [3][4][5][6][7][8] As a result, I was unable to work, to go out regularly with friends, or to engage in all the activities I would have liked to with my family.…”

Section: A Deficit Of Csfmentioning

confidence: 99%

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Awakening from a medical mystery: one patient’s experience of being undiagnosed

UnRuh¹

2019

Patient Experience Journal

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This personal narrative pleads for a supportive and comprehensive system or sub-system similar to that which exists for cancer patients, to deal with undiagnosed illnesses. By describing the torment of living with a debilitating illness that medicine could not easily recognize, then by contrasting this experience with my wife's experience of the cancer care system, and by referring along the way to lessons learned many years ago from reading the works of the inciteful neurologist, Dr. Oliver Sacks, I hope to inspire the medical system to develop a separate, supportive and comprehensive system to deal with the undiagnosed. As it turns out, my mystery illness was a spontaneous cerebrospinal fluid (CSF) leak. This condition is all too often missed in diagnosis or dismissed, and treatment is often significantly delayed, such as it was in my case for nearly two years. At the same time, my wife experienced the cancer system, allowing us to draw contrasts between the two experiences and leading me to implore the system to draw the two approaches together by identifying some of the missing elements for those with undiagnosed illnesses. To conclude, I make reference to the relatively new Undiagnosed Diseases Network as one possible model to accomplish the above goal across the medical system or within local medical systems.

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Assessing Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension With a Scoring System Based on Brain Magnetic Resonance Imaging Findings

et al. 2019

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IMPORTANCE Various signs may be observed on brain magnetic resonance imaging (MRI) in patients with spontaneous intracranial hypotension (SIH). However, the lack of a classification system integrating these findings limits decision making in clinical practice. OBJECTIVE To develop a probability score based on the most relevant brain MRI findings to assess the likelihood of an underlying spinal cerebrospinal fluid (CSF) leak in patients with SIH. DESIGN, SETTING, AND PARTICIPANTS This case-control study in consecutive patients investigated for SIH was conducted at a single hospital department from February 2013 to October 2017. Patients with missing brain MRI data were excluded. Three blinded readers retrospectively reviewed the brain MRI scans of patients with SIH and a spinal CSF leak, patients with orthostatic headache without a CSF leak, and healthy control participants, evaluating 9 quantitative and 7 qualitative signs. A predictive diagnostic score based on multivariable backward logistic regression analysis was then derived. Its performance was validated internally in a prospective cohort of patients who had clinical suspicion for SIH. MAIN OUTCOMES AND MEASURES Likelihood of a spinal CSF leak based on the proposed diagnostic score. RESULTS A total of 152 participants (101 female [66.4%]; mean [SD] age, 46.1 [14.3] years) were studied. These included 56 with SIH and a spinal CSF leak, 16 with orthostatic headache without a CSF leak, 60 control participants, and 20 patients in the validation cohort. Six imaging findings were included in the final scoring system. Three were weighted as major (2 points each): pachymeningeal enhancement, engorgement of venous sinus, and effacement of the suprasellar cistern of 4.0 mm or less. Three were considered minor (1 point each): subdural fluid collection, effacement of the prepontine cistern of 5.0 mm or less, and mamillopontine distance of 6.5 mm or less. Patients were classified into groups at low, intermediate, or high probability of having a spinal CSF leak, with total scores of 2 points or fewer, 3 to 4 points, and 5 points or more, respectively, on a scale of 9 points. The discriminatory ability of the proposed score could be demonstrated in the validation cohort. CONCLUSIONS AND RELEVANCE This 3-tier predictive scoring system is based on the 6 most relevant brain MRI findings and allows assessment of the likelihood (low, intermediate, or high) of a positive spinal imaging result in patients with SIH. It may be useful in identifying patients with SIH who are leak positive and in whom further invasive myelographic examinations are warranted before considering targeted therapy.

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Atypical Presentations of Intracranial Hypotension: Comparison with Classic Spontaneous Intracranial Hypotension

Cited by 45 publications

References 19 publications

The splenium of the corpus callosum: embryology, anatomy, function and imaging with pathophysiological hypothesis

The splenium of the corpus callosum: embryology, anatomy, function and imaging with pathophysiological hypothesis

Awakening from a medical mystery: one patient’s experience of being undiagnosed

Assessing Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension With a Scoring System Based on Brain Magnetic Resonance Imaging Findings

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