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Pathophysiology And Management Of Spontaneous Intracranial Hypotension--A Review, Nadir Ali Syed, Farhan Arshad Mirza, Aqueel Hussain Pabaney, Rameez-Ul-Hassan

Section of Neurology

Spontaneous Intracranial Hypotension is a syndrome involving reduced intracranial pressure secondary to a dural tear which occurs mostly due to connective tissue disorders such as Marfans Syndrome, and Ehler Danlos Syndrome. Patients with dural ectasias leading to CSF leakage into the subdural or epidural space classically present with orthostatic headaches and cranial nerve deficits mostly seen in cranial nerves V-VIII. Diagnosis of SIH is confirmed with the aid of neuroimaging modalities of which Cranial MR imaging is most widely used. SIH can be treated conservatively or with epidural blood patches which are now widely being used to repair dural tears, …

Spontaneous Dural Tear Leading To Intracranial Hypotension And Tonsillar Herniation In Marfan Syndrome: A Case Report, Aqueel H. Pabaney, Farhan A. Mirza, Nadir A. Syed, Humera Ahsan

Section of Neurology

Background:We describe the case of a 38 year old male with Marfan syndrome who presented with orthostatic headaches and seizures. Case Presentation: The Patient was diagnosed with Spontaneous Intracranial Hypotension secondary to CSF leaks, objectively demonstrated by MR Myelogram with intrathecal contrast. Epidural autologus blood patch was administered at the leakage site leading to significant improvement.

Conclusion:

Our literature search shows that this is the second reported case of a Marfan Patient presenting with symptomatic spontaneous CSF leaks along with tonsillar herniation.