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Cryopyrin-associated periodic syndrome is a hereditary inflammatory disorder encompassing a wide spectrum of clinical phenotypes. This disorder has been associated with mutation of the NLRP3 gene coding for cryopyrin, which leads to overproduction of interleukin-1. The condition causes relapsing bouts of inflammation involving multiple organ systems, including the central nervous system, and, if untreated, can lead to long-term debilitating effects. A literature review revealed only a few brief descriptions of magnetic resonance imaging findings in cryopyrin-associated periodic syndrome patients. We describe serial magnetic resonance imaging findings in an 11-year-old female with clinically diagnosed cryopyrin-associated periodic syndrome who presented with intermittent headaches, progressive sensorineural hearing loss, fevers, and abdominal pain. The magnetic resonance imaging showed progressively worsening low T2 signal in the cochlea, cochlear enhancement, and leptomeningeal enhancement. We also describe some previously unreported findings in this syndrome, including cranial nerve and cauda equina enhancement.

The sphenoid sinus is an uncommon location for protrusion of a meningocele. When this does occur, it nearly always presents with leakage of cerebrospinal fluid through the nasal cavity. We present a case of a 38-year-old female found to have a meningocele protruding into the left sphenoid sinus, who presented with intractable headache but no CSF rhinorrhea. The lesion was discovered on computed tomography angiography, which was performed in order to rule out intracranial pathology as the etiology of her headache. Prior imaging, including pre- and post-contrast MRI, demonstrated the fluid within the sphenoid sinus, but did not reveal the communication through a defect in the base of the skull. Thus, it was assumed to be strictly related to sinus disease in the past. Our case represents a phenomenon whereby meningoceles protruding through the basilar skull into the sphenoid sinus or any other location are potentially misdiagnosed due to poor visualization of the osseous defect and lack of awareness of this entity.