Tacrolimus for Treating Orbital and Cranial Form of Idiopathic Inflammatory Pseudotumors

Background@#and Purpose: Orbital and cranial form of idiopathic inflammatory pseudotumors (IIPs) are rare disorders with heterogeneous clinical presentations. Corticosteroids have been the first-line treatment for IIPs, but they are not always effective. @*Methods@#We reviewed the medical records of three patients with orbital or cranial form of IIP who were treated with tacrolimus as an adjuvant treatment. @*Results@#The three patients showed favorable outcomes with the addition of tacrolimus, which is a calcineurin inhibitor that inhibits T-cell activation and T-cell-dependent B-cell activation. @*Conclusions@#Tacrolimus may be a safe and effective immunosuppressant for refractory or relapsing form of orbital or cranial IIPs.

Cerebrospinal Fluid Levels of β-Amyloid 40 and β-Amyloid 42 are Proportionately Decreased in Amyloid Positron-Emission Tomography Negative Idiopathic Normal-Pressure Hydrocephalus Patients

BACKGROUND AND PURPOSE: Cerebrospinal fluid (CSF) biomarkers of Alzheimer's disease (AD) could be misleading in idiopathic normal-pressure hydrocephalus (iNPH). We therefore investigated the CSF biomarkers in 18F-florbetaben amyloid-negative positron-emission tomography (PET) [amyloid PET(−)] iNPH, amyloid-positive PET [amyloid PET(+)] AD, and cognitively normal (CN) subjects. METHODS: Ten amyloid PET(+) AD patients (56.7±5.6 years old, mean±standard deviation), 10 amyloid PET(−) iNPH patients (72.8±4.5 years old), and 8 CN subjects (61.2±6.5 years old) were included. We measured the levels of β-amyloid (Aβ)40, Aβ42, total tau (t-tau) protein, and phosphorylated tau (p-tau) protein in the CSF using enzyme-linked immunosorbent assays. RESULTS: The level of Aβ42 and the Aβ42/Aβ40 ratio in the CSF were significantly lower in AD than in iNPH or CN subjects. The Aβ40 level did not differ significantly between AD and iNPH (p=1.000), but it did between AD and CN subjects (p=0.032). The levels of both t-tau and p-tau were higher in AD than in iNPH or CN subjects. The levels of Aβ42, Aβ40, t-tau, and p-tau were lower in iNPH than in CN subjects, but there was no significant difference after controlling for age. CONCLUSIONS: Our results suggest that the mechanism underlying low CSF Aβ levels differs between amyloid PET(−) iNPH and amyloid PET(+) AD subjects. The lower levels of all CSF biomarkers in iNPH patients might be due to reduced clearances from extracellular fluid and decreased brain metabolism of the periventricular zone in iNPH resulting from glymphatic dysfunction.

Anti-N-methyl-D-aspartate Receptor Encephalitis Caused by a Mature Mediastinal Teratoma

An extra-ovarian teratoma has been reported in a few cases of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. A 25-year-old woman presented with psychiatric symptoms. We did not find an ovarian teratoma on initial examination, and her initial simple chest X-ray was normal. We incidentally found an abnormality on follow-up simple chest X-ray and diagnosed an anterior mediastinal teratoma. Therefore, in patients with suspected anti-NMDAR encephalitis, even if simple chest X-ray is normal, chest computed tomography should be performed to investigate a hidden teratoma.