To present a challenging diagnosis and management of a man with spontaneous intracranial hypotension, hematologic comorbidities, and no apparent CSF leakage.
Spontaneous Intracranial Hypotension (SIH) is a rare neurological condition typically attributed to a low cerebrospinal fluid (CSF) volume due to leaks along the neuroaxis. To the best of our knowledge, no cases of patients with SIH and comorbidities increasing the risks of thromboses or hemorrhage, treated with Epidural Blood Patches, have been reported, complicating diagnostic and therapeutic decision-making in these cases.
A 48-year-old man with a previous history of Antiphospholipid Syndrome (APS), autoimmune thrombocytopenia, recurrent deep venous thrombosis, and pulmonary embolisms was admitted to the hospital due to a severe headache. The headache was bifrontal with occipital radiation, reaching an intensity of 10/10. Medical treatment commenced with caffeine and acetaminophen, resulting in a partial alleviation of symptoms. A lumbar puncture was contraindicated due to anticoagulation and thrombocytopenia. Imaging studies with CT and cranial MRI with gadolinium revealed a 7mm descent of the cerebellar tonsils and classical signs of intracranial hypotension, with no evidence of cerebrospinal fluid leakage.
As the patient deteriorated, he presented with hypoxia and severe headaches, increasing the risk of life-threatening brain herniation. Risks and benefits were evaluated, and it was decided to switch from acenocoumarol to enoxaparin. Despite the limited options for diagnosis and management, the patient was successfully treated with Blinded Epidural Blood Patches with fluoroscopy. At the nine-month follow-up, significant clinical and radiological improvement was documented.
Although SIH is often linked to CSF leakage, we present an exceptionally rare case involving a male patient with APS and SIH, in the absence of apparent CSF leakage. Due to his medical condition and a history of recurrent thromboses, both the diagnostic process and the management of SIH proved to be highly complex.