Type II Lepra Reaction With Idiopathic Intracranial Hypertension: A Case Report With Challenges in Diagnosis and Management

Introduction: Leprosy-related type II lepra reaction (T2LR) is a rare immune-mediated complication that typically treated with glucocorticoids and thalidomide. Idiopathic intracranial hypertension (IIH) is marked by increased intracranial pressure leading to symptoms like headache and vision loss. Here we report a rare case of concomitant T2LR and IIH. Here, we reported a woman with leprosy T2LR with idiopathic intracranial hypertension.

Case presentation: A 35-year-old woman with leprosy who developed T2LR received regular glucocorticoid and thalidomide treatment for 2 years. She subsequently experienced progressive vision loss, which was initially misdiagnosed as optic neuritis. After high-dose glucocorticoid treatment, her condition worsened. The patient was later found to have elevated intracranial pressure and was ultimately diagnosed with idiopathic intracranial hypertension. She was treated with cerebrospinal fluid diversion, which relieved her symptoms. Although the exact cause of her vision loss and intracranial hypertension was unclear, it was speculated to be related to T2LR. The patient was mildly obese and had received high-dose corticosteroids, both of which are known risk factors for intracranial hypertension. T2LR is associated with increased proinflammatory cytokine levels, which can enhance choroid plexus permeability, potentially leading to intracranial hypertension.

Discussion: IIH and T2LR are both rare conditions, and evidence suggests that inflammatory mediators in T2LR may increase choroid plexus permeability, raising intracranial pressure. In this case, mild obesity, female sex, and prolonged corticosteroid therapy for T2LR likely increase the risk for IIH. Long-term glucocorticoid use is associated with IIH, as it may increase intracranial pressure through local cortisol activation in the choroid plexus. The patient’s misdiagnosis of optic neuritis, due to a lack of typical IIH symptoms, delayed appropriate treatment and led to vision loss. This case emphasizes the importance of considering IIH in patients on chronic corticosteroids presenting with unusual vision changes.

Conclusion: While this case may represent an extreme instance, it highlights a broader issue: prolonged steroid use can increase intracranial pressure to varying degrees, and not all patients with elevated intracranial pressure experience headaches. Clinicians should be aware of this possibility in patients undergoing long-term steroid treatment and conduct thorough evaluations to detect such complications.