Cystic adventitial disease is a rare disorder that occurs in peripheral arteries. Calf claudication caused by compression of the popliteal artery is a typical presentation of this disease. This is a report of two cases of occluded popliteal artery decompression by percutaneous ultrasound-guided cyst aspiration. In both cases, decompression of the artery was achieved with a significant decrease in the size of adventitial cysts and restoration of flow. Both patients reported complete resolution of symptoms and no calf pain 5 years after the procedure. MR findings and resolution of symptoms in these two patients show the efficacy of percutaneous adventitial cyst aspiration in a 5-year follow-up.

Background Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by a biallelic mutation in the SMN1 gene, resulting in progressive muscle weakness and atrophy. Nusinersen is the first disease-modifying drug for all SMA types. We report on effectiveness and safety data from 120 adults and older children with SMA types 1c-3 treated with nusinersen. Methods Patients were evaluated with the Hammersmith Functional Motor Scale Expanded (HFMSE; n = 73) or the Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND; n = 47). Additionally, the Revised Upper Limb Module (RULM) and 6-minute walk test (6MWT) were used in a subset of patients. Patients were followed for up to 30 months of nusinersen treatment (mean, SD; 23, 14 months). Subjective treatment outcomes were evaluated with the Patients Global Impression–Improvement (PGI-I) scale used in all patients or caregivers at each follow-up visit. Results An increase in the mean HFMSE score was noted at month 14 (T14) (3.9 points, p < 0.001) and month 30 (T30) (5.1 points, p < 0.001). The mean RULM score increased by 0.79 points at T14 (p = 0.001) and 1.96 points (p < 0.001) at month 30 (T30). The mean CHOP-INTEND increased by 3.6 points at T14 (p < 0.001) and 5.6 points at month 26 (p < 0.001). The mean 6MWT improved by 16.6 m at T14 and 27 m at T30 vs. baseline. A clinically meaningful improvement in HFMSE (≥ 3 points) was seen in 62% of patients at T14, and in 71% at T30; in CHOP INTEND (≥ 4 points), in 58% of patients at T14 and in 80% at T30; in RULM (≥ 2 points), in 26.6% of patients at T14 and in 43.5% at T30; and in 6MWT (≥ 30-meter increase), in 26% of patients at T14 and in 50% at T30. Improved PGI-I scores were reported for 75% of patients at T14 and 85% at T30; none of the patients reporting worsening at T30. Adverse events were mild and related to lumbar puncture. Conclusions In our study, nusinersen led to continuous functional improvement over 30-month follow-up and was well tolerated by adults and older children with a wide spectrum of SMA severity.

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Purpose Intrathecal injection of nusinersen is an approved treatment of spinal muscular atrophy (SMA). CT-guided injection is a method of nusinersen administration in patients with severe scoliosis, in whom standard lumbar puncture is not feasible. The injections are repeated every 4 months for life, and accumulated radiation doses absorbed by the patient can increase the risk of cancer. In this study, we present the results of CT-guided intrathecal nusinersen injections with an ultra-low radiation dose protocol. Methods Eighteen patients (15 adults and three children) in whom standard lumbar puncture was not feasible due to severe scoliosis or spinal stabilization were included in this retrospective study. The first 23 injections were performed with a standard radiation dose protocol and the next 42 injections with an ultra-low-dose protocol. The radiation doses, measured as total dose length product (DLP), were acquired and compared between the protocols. Results Injections were successful in 100% of patients with both ultra-low-dose and standard protocols. The radiation dose, measured as DLP, was 111.2–1100.7 (Me = 248.1) mGy*cm for the standard protocol. For the ultra-low-dose protocol, the dose range was 5.0–54.4 (Me = 26.7) mGy*cm, which was significantly lower than with the standard protocol (p < 0.001, η2 = 0.67). Conclusion Radiation doses can be significantly decreased in the CT-guided injection of nusinersen. The proposed protocol allows for effective CT-guided intrathecal nusinersen administration in patients with SMA and severe scoliosis.

Objectives: The objectives of the study were to evaluate adrenal radiofrequency ablation (RFA) as a method of treatment in patients with severe adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome, among whom bilateral adrenalectomy is not a suitable option. Material and Methods: Five patients with ACTH-dependent Cushing syndrome underwent RFA of both adrenal glands. Four of them presented with Cushing disease unsuccessfully treated with pituitary surgery and medical therapy, while one patient had ACTH-dependent Cushing syndrome due to pancreatic endocrine tumor with liver metastases. All patients were disqualified from adrenalectomy due to morbid obesity or lack of consent. Results: A technical success was obtained in all cases, with only one re-intervention necessitated by a cooling effect of the inferior vena cava. Despite pre-procedural adrenergic blockade, severe hypertension was noted during the procedure in three cases, this being treated immediately using direct-acting vasodilators. No complications occurred otherwise. In all cases, significant improvement of clinical symptoms was observed, as well as marked decreases in levels of serum cortisol, free urine cortisol, and dehydroepiandrosterone sulfate. Conclusion: Bilateral RFA under CT-guidance is technically feasible and clinical improvement can be achieved using the method. In patients disqualified from adrenal surgery, RFA might be considered as an alternative method of ACTH-dependent Cushing syndrome treatment.

Intraosseous lipoma is very rare, usually benign tumor of flat bones. However, the localization in skull bones is described in sporadic cases. The differential diagnosis includes end stage of infection, infarct lesions, intraosseous meningioma, angiolipoma, or myxofibrous tumors. We report a patient with intraosseous lipoma located in the sphenoid bone. The diagnosis was established due to the characteristic radiological features. According to the history of seizures, the lesion was removed via endoscopic endonasal approach. Histopathological examination showed adipocytes. The patient underwent control neuroimaging studies.

SummaryBackgroundTo investigate the effect of gadoxetic acid disodium (Gd-EOB-DTPA) on T2 relaxation times and apparent diffusion coefficient (ADC) values of the liver and focal liver lesions on a 1.5-T system.Material/MethodsMagnetic resonance (MR) studies of 50 patients with 35 liver lesions were retrospectively analyzed. All examinations were performed at 1.5T and included T2-weighted turbo spin-echo (TSE) and diffusion-weighted (DW) images acquired before and after intravenous administration of Gd-EOB-DTPA. To assess the effect of this hepatobiliary contrast agent on T2-weighted TSE images and DW images T2 relaxation times and ADC values of the liver and FLLs were calculated and compared pre- and post-injection.ResultsThe mean T2 relaxation times of the liver and focal hepatic lesions were lower on enhanced than on unenhanced T2-weighted TSE images (decrease of 2.7% and 3.6% respectively), although these differences were not statistically significant. The mean ADC values of the liver showed statistically significant decrease (of 4.6%) on contrast-enhanced DW images, compared to unenhanced images (P>0.05). The mean ADC value of liver lesions was lower on enhanced than on unenhanced DW images, but this difference (of 2.9%) did not reach statistical significance.ConclusionsThe mean T2 relaxation times of the liver and focal liver lesions as well as the mean ADC values of liver lesions were not significantly different before and after administration of Gd-EOB-DTPA. Therefore, acquisition of T2-weighted and DW images between the dynamic contrast-enhanced examination and hepatobiliary phase is feasible and time-saving.

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