A Case of Idiopathic Intracranial Hypertension Complicated with both Infratentorial and Supratentorial Cortical Superficial Siderosis: Novel Imaging Findings on Intravoxel Incoherent Motion Magnetic Resonance Imaging Offering Clues to Pathophysiology

TL;DR

A rare case of idiopathic intracranial hypertension combined with cortical superficial siderosis is reported, showing how MRI can help understand the condition and guide treatment.

Contribution

This is the first reported case of IIH with both infratentorial and supratentorial cortical superficial siderosis, offering new insights into the disease's pathophysiology.

Findings

MRI revealed prominent superficial siderosis on both the cerebellar surface and cerebral cortex.

Intracranial pressure decreased after treatment with acetazolamide, improving symptoms and optic nerve swelling.

The case suggests a possible causal link between IIH and superficial siderosis.

Abstract

The pathology of idiopathic intracranial hypertension (IIH), a disease characterized by papillary edema and increased intracranial pressure (IICP), is not yet understood; this disease significantly affects quality of life due to symptoms including vision loss, headache, and pulsatile tinnitus. By contrast, superficial siderosis (SS), a disorder in which hemosiderin is deposited on the surface of the cerebral cortex and cerebellum, potentially causes cerebellar ataxia or hearing loss. So far, no cases of IIH with infratentorial and supratentorial cortical SS have been reported. Herein, we report a case of a 31-year-old woman with obesity who developed this condition. The patient suddenly developed headache and dizziness, had difficulty walking, and subsequently became aware of diplopia. Fundus examination revealed bilateral optic nerve congestive papillae and right eye abducens disturbance. Head magnetic resonance imaging (MRI) showed prominent SS on the cerebellar surface and cerebral cortex. Lumbar puncture revealed IICP of 32 cmH2O, consistent with the diagnostic criteria for IIH, and treatment with oral acetazolamide was started; subsequently, the intracranial pressure decreased to 20 cmH2O. Her abduction disorder disappeared, and the swelling of the optic papilla improved. She was now able return to her life as a teacher without any sequelae. SS is caused by persistent slight hemorrhage into the subarachnoid space. In this case, both infratentorial and supratentorial cortical superficial SS was observed. Although cases of IIH complicated by SS are rare, it should be kept in mind that a causal relationship between IIH and SS was inferred from our case. Our findings also suggest that cerebrospinal fluid dynamic analysis using MRI is effective in diagnosing IIH and in determining the efficacy of treatment.

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