Symptomatic Spontaneous Intracranial Hypotension Caused by a Cerebrospinal Fluid Leak at the C1-C2 Level of the Spine Sealed With an Epidural Blood Patch-Case Series and Review of Literature.

Abstract

Background: Spontaneous intracranial hypotension (SIH) is a disorder caused by low cerebrospinal fluid (CSF) pressure due to a CSF leak located anywhere within the neuroaxis. Risk factors include trauma, degenerative disc disease, and especially connective tissue disorders. In most cases, SIH presents with orthostatic headache, worsening when upright and characteristic radiological features as diffuse pachymeningeal enhancement on MRI [4, 6]. There are a lot of publications concerning treatment of CSF leaks located in the lumbar and thoracic spine, but only scant information regarding treatment and its outcomes of CSF leaks in the upper cervical spine, e.g., C1-2.

Case series: Three women presented with persistent headaches, worsening when standing, lasting weeks to months. Two reported cervical pain, one had double vision, and all experienced dizziness. Symptoms resolved in recumbency. All had connective tissue disorders: patient 1 had systemic lupus erythematosus (SLE) and Sjögren's syndrome, patient 2 had polymyositis, and patient 3 was under observation for SLE. The MRI showed typical SIH features, and the cervical spine MRI was unremarkable except for spinal longitudinal epidural collection (SLEC) in two patients. CT-myelography identified CSF leaks at C1-2 in all cases. Epidural blood patching (EBP) at C1-2 under CT guidance resolved symptoms, with only one patient experiencing a brief post-procedural headache. At 3 months' follow-up, all patients remained symptom-free. Repeated CT-myelography in one patient showed no residual CSF leak.

Conclusions: SIH is a very rare and frequently misdiagnosed condition. Administration of EBP at the C1-2 level of the spine is troublesome; therefore, it should be performed under guidance of computed tomography. All three patients were discharged symptom free within 24-72 h.