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Introduction Spontaneous intracranial hypotension (SIH) is characterized by acute orthostatic headaches caused by a spinal cerebrospinal fluid (CSF) leak and/or CSF hypotension. This condition is well-documented in Marfan and Ehlers-Danlos syndromes but is rarely associated with connective tissue diseases. Diagnosis relies on neuroimaging and lumbar puncture, which reveal low CSF pressure. SIH has an annual incidence of 5 per 100,000 individualsMaterial and Methods Case 1A 27-year-old woman with a 7-year history of anti-Scl70 diffuse cutaneous systemic sclerosis (dcSSc) complicated by acral ulcers, interstitial lung disease and esophageal dysmotility, on vasoactive and immunosuppressive therapy, presented with tinnitus and holocranial gravitational headache relieved by clinostasis. Symptoms started 48 hours before hospital admission without fever, infection, or neurological deficits. Head CT showed no acute damage.She had recently discontinued immunosuppressive therapy for four weeks due to pyelonephritis. SIH was suspected and an epidural blood patch was performed. Brain MRI revealed diffuse pachymeningeal thickening above and below the tentorium with bilateral subdural hematomas, consistent with SIH. Eight weeks later, a second blood patch led to symptom resolution and normalization of MRI findings.Case 2A 57-year-old woman with a 6-year history of anti-Scl70 dcSSc with vascular and lung involvement, on vasoactive and immunosuppressive therapy, presented with tinnitus, diplopia and occipitonuchal headache worsening with upright posture. Symptoms followed gastroenteritis.Brain MRI revealed bihemispheric fluid collections on T2-weighted and FLAIR sequences with diffuse post-contrast meningeal enhancement, suggesting SIH. An epidural blood patch led to clinical and imaging resolution.Results Dural tears, meningeal diverticula, CSF-venous fistulae, and trauma are common causes of spontaneous intracranial hypotension (SIH). Hereditary connective tissue disorders, involving abnormalities in fibrillin or elastin, predispose individuals to dural weakening and CSF leaks.In systemic sclerosis (SSc), fibrillin-1 alterations and microangiopathy of the vasa nervorum may contribute to dural fragility. Both patients in this report demonstrated severe vascular involvement. Case 1 had refractory digital ulcers and capillary loss observed on nailfold capillaroscopy, while therapy discontinuation might have acted as a triggering factor.Infectious agents may also play a role. A 2023 case report suggested that COVID-19 infection, confirmed via PCR in subdural fluid, could induce SIH.Conclusions This report represents the third documented case of SIH in a patient with SSc. It underscores the potential underdiagnosis of SIH in this population, where headaches may be mistakenly attributed to other causes.Abstract P.378 Figure 1MRI with contrast, T1 sequence: post-contrast enhancement of the pachymeninges