Delayed recurrence of spontaneous intracranial hypotension syndrome mimicking a Chiari I malformation: Case report with a review of the literature

Introduction: Cerebellar tonsils descent seen on brain MRI is, along with other findings, a recognized radiological sign of possible spontaneous intracranial hypotension (SIH). The short-term outcome of SIH is usually favorable with symptoms improvement and reversibility of the low-lying tonsils. Nevertheless, data on the long-term outcome are lacking or inconsistent.

Case report: A 32-year-old woman presented to her general practitioner with a six months history of non-specific headaches. An MRI brain with gadolinium showed a 12mm tonsillar descent with no other remarkable findings. Headaches were initially managed conservatively as migraines. Following the onset of progressive upper back and shoulder pain at rest, nausea, photophobia and fogging in her vision, the patient was referred to our Department with a suspicion of symptomatic Chiari I malformation. After an in-depth anamnesis, it emerged a previous history of SIH, 14 years earlier, successfully treated conservatively in another center. A whole spine MRI confirmed the suspicion of recurrent SIH showing an anterior cervico-thoracic epidural fluid collection. The patient underwent an epidural blood patch with complete resolution of the symptoms and radiological signs.

Discussion: To our knowledge, this case is the first report of delayed recurrence of a SIH successfully treated conservatively over 10 years earlier. The etiopathogenesis and management of this rarity with literature review is discussed.

Conclusion: An isolated cerebellar tonsil descent with no other remarkable findings on brain MRI and a previous history of SIH should always alert the clinician of a possible late recurrence of a CSF leak and avoid unnecessary Chiari I malformation surgical procedures.