The aim of this study was to determine whether the resistive index in the anterior cerebral artery, as measured by transcranial Doppler ultrasonography without and with pressure provocation, predicts the need for cerebrospinal fluid drainage in hydrocephalic children. Both without and with pressure provocation, the resistive index was significantly higher (P > 0.05) in patients with raised intracranial pressure compared with control group patients and dropped significantly after drainage. With receiver operating characteristic analysis, the optimal cutoff point between normal and abnormal resistive index values was determined at 0.71 without pressure provocation and at 0.90 with pressure provocation. The addition of the pressure provocation test improved accuracy from 81 to 91%, mainly by improving specificity. In conclusion, transcranial Doppler ultrasonography with pressure provocation accurately identifies hydrocephalic children who require cerebrospinal fluid drainage procedures.
To evaluate the effectiveness of eptinezumab in patients with migraine after 6 and 12 months.
Background:
Patients who switched from subcutaneous CGRP mAbs to intravenous CGRP mAb (i.e., eptinezumab) are refractory to most preventive medications. While several real-world studies reported positive outcomes within 6 months of eptinezumab use, the eptinezumab usage pattern and treatment response after 1-year remain unknown.
Design/Methods:
This single-center retrospective study examined the first 30 subjects who received eptinezumab at the Jefferson Headache Center. We collected demographics, reasons for the switch, and usage pattern/response to eptinezumab after 6 and 12 months. The generalized estimating equation (GEE) test adjusted for age, sex, and BMI was used for repeated measure analysis. Missing data were not included.
Results:
We reviewed 30 patients (25 female, age 52.3±12.6, BMI 28.3±6.1) who experienced headaches for 26.8±17.8 years and switched to eptinezumab. At the index date, all subjects received a 100mg regimen and reported a mean monthly headache (MHD) of 24.6±8.6 and average pain intensity (API) of 6.2±1.8. After 6 months, 23 subjects continued, and 14 (61%) received a 300mg regimen. They had an MHD of 24.5±9.4 (n=16) and API of 5.7±1.4 (n=14). After 12 months, 15 subjects continued, and 10 (71.4%) were on a 300mg regimen. These subjects had an MHD of 22.5±10.5 (n=12) and API of 5.5±1.3 (n=12). The main reasons for eptinezumab discontinuation were inadequate response (n=6/15; 40%), intolerance (n=3/15; 20%), and insurance (n=5/15, 33%). There were insignificant reductions at 6 and 12 months of MHD (−0.71, 95%CI −3.42 to 2.00, p=0.61; −1.36, 95%CI −4.52 to 1.81, p=0.40) and API (−0.46, 95%CI −1.17 to 0.24, p=0.20; −0.63, 95%CI −1.38 to 0.13, p=0.11).
Conclusions:
In our study, patients may sustain no apparent benefit upon switching to eptinezumab after 6 or 12 months, reflecting the refractory nature of this study population. More prospective studies are needed. Disclosure: Miss Casaletto has nothing to disclose. Dr. Yang has nothing to disclose. Mr. Waters has nothing to disclose. Mr. Curran has nothing to disclose. Mr. Lowe has nothing to disclose. An immediate family member of Dr. Yuan has received personal compensation for serving as an employee of Merck. Dr. Yuan has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Trillen Medical. The institution of Dr. Yuan has received research support from NIH. Dr. Yuan has received publishing royalties from a publication relating to health care. Dr. Yuan has received personal compensation in the range of $0-$499 for serving as a Grant reviewer with NIH.
Polysplenia is a complex congenital syndrome. It forms one part of the spectrum of viscero-atrial situs abnormalities, along with asplenia (Ivemark's syndrome) and situs inversus. Lymphoma has been reported in cases of “ectopic” or “wandering” spleen, but not, to our knowledge, in polysplenia. We report the case of a 42-year-old patient who was a polysplenic with Hodgkin's disease.
Primary angiitis of the central nervous system (PACNS) is a rare, idiopathic vasculitis diagnosed most frequently in adults. We describe 2 children presenting with hemiplegia from PACNS treated with cyclophosphamide. Diagnosis in one child was based on abnormal angiography. Oral, but not intravenous (IV), cyclophosphamide was effective in preventing progressive weakness. The second child had unremarkable angiography, but brain biopsy revealed vasculitis; IV cyclophosphamide prevented further weakness. Both cases highlight the importance of early diagnosis and treatment.
We describe 5 children who meet criteria for primary angiitis of the central nervous system (PACNS). All patients presented with headache and/or focal neurologic deficits and exhibited clinical and/or radiographic evidence of disease progression. Two patients had disease progression prior to combined treatment with cyclophosphamide and corticosteroids; one progressed while receiving intravenous cyclophosphamide and stabilized after a change to daily oral dosing; one progressed after discontinuing therapy after less than 12 months and improved after retreatment; and one progressed on steroid therapy alone but was lost to followup. Children who have frequent or severe headaches or focal neurologic deficits should be carefully evaluated and those meeting criteria for PACNS should be treated aggressively.
