Abstract

We present the case of a 41-year-old female with Relapsing-Remitting Multiple Sclerosis (RRMS) who developed Idiopathic Intracranial Hypertension (IIH) following steroid tapering. After initial treatment with high-dose corticosteroids for an MS relapse, the patient presented with acute onset headache, nausea, vomiting, and raised Intracranial Pressure (ICP) shortly after tapering to 10 mg/day of oral steroids. Cerebrospinal Fluid (CSF) analysis revealed an elevated opening pressure of 33 cm H₂O. This case highlights the rare but significant risk of IIH following corticosteroid tapering in the context of multiple sclerosis. Early recognition and management are crucial for patient outcomes.

Introduction

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is characterized by elevated intracranial pressure without an identifiable cause such as mass lesions, infection, or venous thrombosis. It predominantly affects obese women of childbearing age but has been reported in other populations, including patients on corticosteroid therapy. Steroid tapering has been implicated in IIH development, and this case demonstrates the onset of IIH after tapering oral corticosteroids in a patient with Multiple Sclerosis (MS). While MS its introduce been conclusively associated with IIH, the use of high-dose corticosteroids to manage acute MS relapses introduces a risk factor. We discussed the mechanisms, clinical presentation, and management of steroid tapering-induced IIH in this patient.

Informed Consent

Written informed consent was obtained from the patient. A copy of the written consent is available for review by the Editor of this journal.

Case Presentation

A 41-year-old female, previously diagnosed with Relapsing-Remitting Multiple Sclerosis (RRMS), was admitted to our hospital on December 17, 2024, with a 4-day history of subacute onset bilateral lower limb numbness, tingling, and unsteady gait, deviating to the left side, especially when closing her eyes. Three months prior, she had experienced a similar episode of bilateral lower limb numbness, which resolved spontaneously. She denied any cranial nerve symptoms, visual complaints, motor weakness, or sphincteric disturbances. There was no history of recent infections, fevers, or vaccinations preceding her presentation. Neurological examination normal cranial nerves assessment with unremarkable fundus examination. Motor assessment showed Normal muscle power in all limbs, with hypertonia in the right lower limb and exaggerated reflexes bilaterally, more prominent on the left side, with positive adductor and patellar reflexes. Plantar reflexes were mute bilaterally. Sensory examination showed sensory level till level of C4 with impaired position and vibration sense at the big toes bilaterally. Romberg’s test was Positive. EDSS was 6 (ambulant with unilateral support because of unsteadiness). MRI of the brain, orbit, and cervical spine with contrast showed multiple non-enhancing demyelinating plaques in periventricular, juxtacortical, subcortical, and cervical regions involving fewer than three segments. CSF analysis: Showed 17 lymphocytes, normal protein and glucose, negative culture, and virology PCR. CSF oligoclonal bands were positive, IgG index 0.7, CSF kappa free light chains (KFLC) 2.8 (elevated). Anti-AQP4 antibodies and Anti-MOG antibodies were negative. Optical coherence tomography (OCT) showed mild disc pallor on the right side. Visual evoked potentials (VEP) showed prolonged P100 latency on the left eye (119 ms). Vasculitis screen and virology screen were negative.

Hospital Course and Treatment

The patient was diagnosed with an MS relapse and started on 1 g of intravenous methylprednisolone daily for five days. Following steroid treatment, she was transitioned to oral steroids (60 mg/day) with a gradual taper over one month. Despite steroid therapy, her symptoms showed only minimal improvement. Due to unavailability of plasma exchange, the patient was offered transfer to a tertiary center for this treatment but refused. Subsequently, intravenous immunoglobulin (IVIG) was initiated for five days, and she started on her first dose of ocrelizumab for long-term disease modification. The patient continued regular physiotherapy for balance and gait rehabilitation. However, after tapering her oral steroids to 10 mg/day, she returned to the emergency department after 3 weeks from discharge with acute onset nausea, vomiting, and severe postural headache that increase when lying flat. On examination, she exhibited signs of raised intracranial pressure, and a lumbar puncture revealed an elevated opening pressure of 33 cm H₂O. CSF analysis was normal with no evidence of infection. Patient was treated by therapeutic lumbar puncture and started on acetazolamide 500 mg bd for two weeks with marked improvement of her symptoms then was tapered down successfully. She was advised to avoid rapid tapering of steroids in the future. Regular follow-up with neurology was planned to monitor her MS and intracranial pressure.

Discussion

Steroid tapering has been implicated in the development of IIH, though it remains a rare complication. Corticosteroids, particularly when used in high doses, are known to reduce inflammation and intracranial pressure. Rapid tapering or withdrawal of steroids, however, may lead to a rebound in intracranial pressure. In our case, tapering the patient’s oral steroids from 60 mg/day to 10 mg/day likely contributed to the development of IIH, with a CSF opening pressure of 33 cm H₂O. Steroid-induced IIH has been previously reported in the literature. According to Diaz et al., rebound intracranial hypertension can occur following corticosteroid tapering, especially in patients receiving long-term high-dose steroids for autoimmune or inflammatory diseases [1]. Additionally, Mollan et al. reviewed cases of steroid-induced IIH, noting that a rapid decrease in steroid dosage might disrupt the normal regulation of cerebrospinal fluid dynamics [2]. In MS patients, corticosteroids are frequently employed to manage acute relapses, as seen in this patient. While IIH itself is not commonly associated with MS, the use of corticosteroids in high doses, followed by rapid tapering, poses a risk for the development of IIH [3,4]. The exact mechanism remains unclear, but disruption of glucocorticoid-mediated CSF homeostasis is a potential contributing factor.

Conclusion

This case demonstrates a rare occurrence of steroid taperinginduced idiopathic intracranial hypertension in a patient with multiple sclerosis. Given the potential for raised intracranial pressure following corticosteroid therapy, clinicians should be cautious when tapering steroids in MS patients and monitor for symptoms of IIH. Early recognition and management are essential for preventing complications associated with elevated ICP.

References

1. Diaz JM, Jabbari B (2001) Steroid-induced pseudotumor cerebri: Case report and review of the literature. Clin Neuropharmacol 24(6): 316-319.
2. Mollan SP, Ali F, Hassan-Smith G, Botfield H, Sinclair AJ (2020) Steroid-induced idiopathic intracranial hypertension (SIH): A review of the literature. J Neuroophthalmol 40(3): 412-421.
3. Ball AK, Clarke CE (2006) Idiopathic intracranial hypertension. Lancet Neurol 5(5): 433-442.
4. Radhakrishnan K, Ahlskog JE, Garvey MJ, Kurland LT (1993) Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol 50(1): 78-80.