Traumatic vs Spontaneous Cerebrospinal Fluid Hypotension Headache: Our experience in a series of 137 cases.

OBJECTIVES: To analyze and compare differences between epidemiological and clinical aspects, as well as radiologic findings and treatment, in a series of adult patients with traumatic intracranial hypotension (TIH) and spontaneous intracranial hypotension (SIH) treated at our institution in order to identify predictors of recurrence. BACKGROUND: Cerebrospinal fluid hypotension headache (CSF-HH) is often caused by orthostasis and relieved by recumbency. Etiology can be either traumatic or spontaneous. Indirect signs of CSF hypotension are often observed on brain MRI. The most common therapeutic approach is conservative management and, when necessary, the use of an epidural blood patch. METHODS: Medical history and brain MRI of adult patients consulting our institution with a diagnosis of CSF-HH between January 2010 and March 2019, were retrospectively reviewed. Clinical criteria as per the International Classification of Headache Disorders, 3rd edition, were applied. Presence of typical MRI findings were assessed by two experienced neuroradiologists, previously informed of patients' clinical characteristics. Patients were divided into two different groups, namely: Group A: Spontaneous Intracranial Hypotension (SIH) and Group B: Traumatic Intracranial Hypotension (TIH). Recurrence was defined as return of symptoms after one month of remission. In order to find predictors of recurrent intracranial hypotension the patients were divided into three groups: Recurrent Orthostatic headache (ROH); (33 cases; 25%); Non-Recurrent Orthostatic headache (NROH) (84; 61%) and Patients missing follow-up (20; 15%). The latter were excluded from the regression analysis. RESULTS: 137 patients with CSF-HH were identified: 80 traumatic (54 women, age 33.8 ±â€¯10.4 years) and 57 spontaneous (31 women, age 43.9 ±â€¯15.2 years). Median follow-up was 35 months (range: 8 months-9 years). Compared with TIH, patients with SIH showed lower frequency of orthostatic headache and higher frequency of aural fullness. Also, in patients with SIH, brain MRI showed a higher frequency of pachymeningeal enhancement, ventricular collapse, cisternal obliteration, posterior fossa crowding, brainstem distortion, and a more likely presence of subdural collections. Patients with SIH required an epidural blood patch treatment more often, showed higher recurrence rates, and were more prone to develop subdural hematomas. RECURRENCE: As more days elapsed between headache onset and clinical consultation, the presence of spontaneous intracranial hypotension, tinnitus, and thoraco-lumbo-sacral pain were all more common in patients with recurrence. Findings on brain MRI suggesting higher recurrence rates in patients included: ventricular collapse, brainstem distortion, and posterior fossa crowding. Patients treated with invasive therapy (epidural blood patch) presented a higher recurrence rate. In the multivariate regression analysis, the only independent predictor of recurrence after adjusting for age, sex and traumatic vs spontaneous cause of IH, was brainstem distortion diagnosed on MRI (OR 5.13, 95% CI: 1.2-21.7; p = 0.026). CONCLUSIONS: SIH and TIH can no longer be likened, since there is considerable variability in clinical presentation, imaging findings, response to treatment and recurrence rates. Anatomical abnormalities underlying SIH leaks are often complex and not simply a disruption of normal structures as encountered in TIH, which could explain why treatment success is poor and recurrence rates remain high.

Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system.

INTRODUCTION: To assess clinical and/or imaging features useful to distinguish between Susac syndrome (SuS) and primary angiitis of central nervous system (PACNS). METHODS: Multicenter retrospective analysis of two cohorts of Argentine patients diagnosed with SuS and PACNS. RESULTS: 13 patients diagnosed with SuS (6 women and 7 men, mean age 35 ±â€¯10 years) and 15 with PACNS (10 women and 5 men, mean age 44 ±â€¯18 years) were analyzed. Cognitive impairment (11 out of 13 patients vs. 5 out of 15, p = .006), ataxia (7 out of 13 vs. 2 out of 15, p = .042) and auditory disturbances (7 out of 13 vs. 0 out of 15, p = .003) were more frequent in SuS patients; whereas seizures were more frequent in PACNS patients (8 out of 15 vs. 1 out of 13, p = .035). On MRI, corpus callosum (CC) involvement was observed more often in SuS, with abnormalities in CC genu, in 13 out of 13 SuS patients vs. only 2 out of 15 PACNS patients (p < .001); in CC body these were present in 13 out of 13 SuS patients vs. 1 out of 15 PACNS patients, (p < .001); and in CC splenium in 12 out of 13 Sus patients vs. 1 of 15 PACNS, p < .001). Cortical lesions were more frequent in PACNS patients (10 out of 15 vs. 3 out of 13 SuS patients, p = .02), as were hemorrhages (5 out of 15 vs. 0 out of 13 SuS, p = .04) and multiple basal ganglia infarcts (7 out of 15 vs. 1 out of 13 Sus, p = .037). CONCLUSION: Specific clinical and/or MRI findings may help distinguish SuS from PACNS with potential therapeutic implications.