A new analysis was conducted looking at pathophysiological relationships between pseudotumor cerebri (PTC) and concurrent comorbidities, with the analysis highlighting empty sella (ES) syndrome, polycystic ovary syndrome (PCOS), sensorineural hearing loss, and elevated inflammatory markers.¹

This research was led by Sara Zarei, MD, from the department of neurology at the Neurology Group in Pomona, California.

Zarei and colleagues’ research was designed to highlight the significance associated comorbidities to prevent complications and allow healthcare providers to become better equipped to optimize outcomes through targeted interventions and management, given the potential severity of certain related comorbidities.²

“The main objective of this case is to delve into the pathophysiological associations between PTC and concurrent comorbidities, including PCOS, sensorineural hearing loss, ES syndrome, and elevated inflammatory markers,” Zarei and colleagues wrote. “In the current medical literature, there is no documentation of another PTC case presenting the constellation of concurrent comorbidities observed in this patient.”

Background and Findings

The investigators noted that PTC—which is also known as idiopathic intracranial hypertension (IIH)—is known to be characterized by increased intracranial pressure without hydrocephalus or mass lesion, as well as normal cerebrospinal fluid (CSF) studies and neuroimaging.

The specific cause of PTC is known to be uncertain, though potential mechanisms for the condition include cerebral edema, impaired CSF absorption, elevated CSF production, and abnormal cerebral venous pressure gradients. Those experiencing it may report many different symptoms such as radicular pain, pulsatile tinnitus, pain in the back, visual issues, dizziness, blurred vision, pain in the neck, cognitive problems, and intermittent horizontal diplopia.

The investigators of this analysis involved a 32-year-old female subject who had been initially diagnosed with polycystic ovary syndrome (PCOS) as a result of chronic headaches and oligomenorrhea. While this individual had received maximum medical treatment as well as optic nerve sheath fenestration, she was found to have suffered complete bilateral vision loss.

The patient sought care 5 years afterward. She presented with left-sided hearing loss, tinnitus, joint pain, inflammatory markers which were elevated, and headaches. The research team involved in the analysis worked to look at the pathophysiology of such coexisting issues.

Overall, the subject was found to have displayed papilledema and an elevated opening pressure of 36 mm H2O during lumbar puncture. After the individual’s diagnosis, her treatment with 250 mg of Diamox twice per-day and Topamax 25 mg was promptly initiated.

Due to the patient’s case being notably complex, she was referred to an ophthalmologist and later a neurosurgeon for additional examination. Following many discussions, the subject made the decision to delay considering a VP shunting procedure.

She instead opted for medication management and an additional focused weight loss plan. The investigators found that follow-up interactions demonstrated that she had major improvement, including the resolution of her papilledema and substantial relief of visual disturbances.

The research team expressed the obvious value in acknowledgement of obesity’s substantial role in triggering a chronic proinflammatory state in patients’ bodies.

The subject’s positive response to this plan, the investigators noted, suggests there may be significant value to timely diagnosis and to timely intervention in IIH cases. They added that this may be especially true among those with PCOS and with a history of headaches, to prevent complications.

“A multidisciplinary approach involving neurologists, ophthalmologists, endocrinologists, and rheumatologists is essential for the comprehensive management of IIH and its associated comorbidities,” they wrote. “Further research and studies are needed to enhance our understanding of the underlying mechanisms and to identify more effective treatment strategies for this challenging condition.”

References

1. ^{Zarei S, Kamali S, Narinyan W, et al. Idiopathic intracranial hypertension associated with polycystic ovarian syndrome, sensorineural hearing loss, and elevated inflammatory markers that lead to bilateral blindness: A case report with literature review. Surg Neurol Int. 2023;14:399. Published 2023 Nov 17. doi:10.25259/SNI_670_2023}
2. ^{Dave SB, Subramanian PS. Pseudotumor cerebri: An update on treatment options. Indian J Ophthalmol. 2014. 62: 996-8.}