Understanding the pathophysiology of idiopathic intracranial hypertension (IIH): a review of recent developments.

Idiopathic intracranial hypertension (IIH) is a condition of significant morbidity and rising prevalence. It typically affects young people living with obesity, mostly women of reproductive age, and can present with headaches, visual abnormalities, tinnitus and cognitive dysfunction. Raised intracranial pressure without a secondary identified cause remains a key diagnostic feature of this condition, however, the underlying pathophysiological mechanisms that drive this increase are poorly understood. Previous theories have focused on cerebrospinal fluid (CSF) hypersecretion or impaired reabsorption, however, the recent characterisation of the glymphatic system in many other neurological conditions necessitates a re-evaluation of these hypotheses. Further, the impact of metabolic dysfunction and hormonal dysregulation in this population group must also be considered. Given the emerging evidence, it is likely that IIH is triggered by the interaction of multiple aetiological factors that ultimately results in the disruption of CSF dynamics. This review aims to provide a comprehensive update on the current theories regarding the pathogenesis of IIH.

Ophthalmoplegia Associated With Extramedullary Multiple Myeloma: Case Series From a Tertiary Cancer Center.

BACKGROUND: Multiple myeloma (MM) is a malignant disorder of plasma cells that results in tumor cells replacing the bone marrow. In extramedullary MM (EMM), however, tumor cells proliferate outside the bone marrow. EMM may produce ophthalmoplegia through direct invasion of the superior orbital fissure, cavernous sinus, and/or sphenoidal sinus. Several mechanisms have been proposed including cranial nerve palsies, direct infiltration of bone, extraocular muscle metastasis, myelomatous meningitis, and parenchymal or paraneoplastic effects. METHODS: We retrospectively reviewed the medical records of 7 patients at MD Anderson Cancer Center who suffered from ophthalmoplegia secondary to extramedullary MM between 2019 and 2021. We collected information regarding the symptoms, signs, radiographic and laboratory findings, management, complications, and prognosis of these patients throughout their disease course. RESULTS: Skull base MRI revealed 4 patients with ophthalmoplegias secondary to superior orbital fissure invasion, 2 patients with ophthalmoplegias secondary to cavernous sinus invasion, and 1 patient with ophthalmoplegia secondary to sphenoid sinus invasion. CONCLUSIONS: This is a case series describing 7 patients with ophthalmoplegias secondary to EMM. Our article is unique because of the size of the included cohort, which is large when compared with most English language publications detailing such ophthalmoplegias.

Metastatic Breast Cancer Presenting as Progressive Ophthalmoplegia 30 Years After Initial Cancer Diagnosis.

ABSTRACT: A 60 year-old woman presented with painless progressive ophthalmoplegia of the right eye. She had a history of left-sided breast carcinoma 30 years ago that was managed with mastectomy only, with appropriate serial follow-up investigations. On examination, her visual acuity was 20/400 in the right eye and 20/20 in the left. She had a right relative afferent pupillary defect. Ocular examination was significant for 2 mm of ptosis, complete ophthalmoplegia, and 2+ chemosis in the right eye. The left eye was normal. MRI of the brain and orbits showed bilateral retrobulbar infiltrative disease in the right eye greater than that in the left eye. Right orbitotomy and biopsy confirmed an infiltrative signet ring cell/histiocytoid carcinoma consistent with metastatic lobular breast carcinoma. Given that recurrence of breast cancer is most common during the second year after the initial disease and rarely reported beyond 20 years after the initial diagnosis, our patient's delayed recurrence 30 years after the treatment of initial disease is unusual and rare. However, it highlights the importance of including metastatic cancer in the differential diagnosis for ophthalmoplegia.