Schipani, Elke

Dermatoneuro syndrome (DNS) is a rare diagnosis of exclusion characterised by fever, altered mental status and seizures in patients with scleromyxoedema. We present a complex case of a patient presenting with a fever, coma, seizures, rigidity and deep T2 fluid-attenuated inversion recovery hyperintensities in the context of both scleromyxoedema and influenza A with extensive, negative infectious workup. This case highlights the shared clinical features of DNS and influenza-associated encephalopathy, the latter being the eventual diagnosis for our patient. We review the DNS literature focusing on presentation, adequacy of investigation, treatment and prognosis. Finally, we provide an initial workup for patients with suspected DNS, including nasopharyngeal influenza PCR.

Abstract BACKGROUND Meningiomas are the most common primary intracranial tumors, typically managed with surgical resection and/or radiation therapy. Systemic therapy options are limited and reserved for cases refractory to conventional interventions. Cyclin dependent kinase (CDK) proteins are key cell cycle regulators. CDK inhibitors are clinically available, approved for treatment of breast cancer and in clinical trials for meningioma. METHODS We present a case where a patient with incidentally-discovered intracranial meningioma undergoing CDK inhibitor therapy with ribociclib for breast cancer experienced a radiographic response. RESULTS A 73-year-old female was diagnosed with estrogen receptor (ER) positive, progesterone receptor (PR) positive, HER2 negative metastatic invasive ductal carcinoma of the breast. She was treated with estrogen receptor antagonist fulvestrant and CDK 4/6 inhibitor ribociclib. MRI of the brain performed for disease staging demonstrated an extraaxial, dural-based, homogenously enhancing mass along the left posterior petrous ridge consistent with a meningioma. Two-month interval scan demonstrated a marginal decrease in size of the mass (15.6x18.3 mm to 15.4x17.5 mm). Systemic disease related to breast cancer concurrently improved. Two years later, with ongoing fulvestrant and ribociclib therapy, a repeat MRI brain demonstrated further decrease in size of the meningioma (13.4x16.8 mm). CONCLUSIONS Hormonally-directed therapies previously demonstrated limited benefit for meningiomas despite PR/ER positivity reported for these tumors. However, CDK 4/6 inhibitors abemaciclib and ribociclib have demonstrated activity in treatment of recurrent grade 2/3 meningiomas. The radiographic response noted in our case suggests there may be a role for systemic therapy even in treatment-naive cases that may not be amenable to surgical resection or radiation therapy.

Background Recent studies show cervical artery dissection (CeAD) is equally common between sexes, and that the incidence of CeAD has risen at a greater rate in females than males. In this population-based study, we identify sex differences in patients diagnosed with spontaneous and traumatic CeAD. Methods 144 patients with spontaneous or traumatic CeAD were studied for sex differences in medical comorbidities, presenting symptoms and outcomes. Results Females were more likely to carry a diagnosis of migraine, while males were more likely to have hyperlipidaemia. Females were more likely to present with neck pain, males with stroke. Females were significantly more likely to develop recurrent dissections in the study period. Conclusions These findings underscore the importance of understanding CeAD through the lens of sex differences and may explain the significant rise in the diagnosis of CeAD in females. These findings support the importance of considering sex-specific risk factors and medical comorbidities with sex predilection in the diagnosis and management of CeAD. Furthermore, it emphasises the importance of female patients understanding risk factors and presenting signs that should prompt evaluation for CeAD.