Microstructural Integrity of Peripheral Nerves in Charcot-Marie-Tooth Disease: An MRI Evaluation Study.

BACKGROUND: Charcot-Marie-Tooth (CMT) disease is diagnosed through clinical findings and genetic testing. While there are neurophysiological tools and clinical functional scales in CMT, objective disease biomarkers that can facilitate in monitoring disease progression are limited. PURPOSE: To investigate the utility of diffusion tensor imaging (DTI) in determining the microstructural integrity of sciatic and peroneal nerves and its correlation with the MRI grading of muscle atrophy severity and clinical function in CMT as determined by the CMT neuropathy score (CMTNS). STUDY TYPE: Prospective case-control. SUBJECTS: Nine CMT patients and nine age-matched controls. FIELD STRENGTH/SEQUENCE: 3 T T1 -weighted in-/out-of phase spoiled gradient recalled echo (SPGR) and DTI sequences. ASSESSMENT: Fractional anisotropy (FA), axial diffusivity (AD), radial diffusivity (RD), and mean diffusivity (MD) values for sciatic and peroneal nerves were obtained from DTI. Muscle atrophy was graded according to the Goutallier classification using in-/out-of phase SPGRs. DTI parameters and muscle atrophy grades were compared between CMT and controls, and the relationship between DTI parameters, muscle atrophy grades, and CMTNS were assessed. STATISTICAL TESTS: The Wilcoxon Signed Ranks test was used to compare DTI parameters between CMT and controls. The relationship between DTI parameters, muscle atrophy grades, and CMTNS were analyzed using the Spearman correlation. Receiver operating characteristic (ROC) analyses of DTI parameters that can differentiate CMT from healthy controls were done. RESULTS: There was a significant reduction in FA and increase in RD of both nerves (P < 0.05) in CMT, with significant correlations between FA (negative; P < 0.05) and RD (positive; P < 0.05) with muscle atrophy grade. In the sciatic nerve, there was significant correlation between FA and CMTNS (r = -0.795; P < 0.05). FA and RD could discriminate CMT from controls with high sensitivity (77.8-100%) and specificity (88.9-100%). DATA CONCLUSION: There were significant differences of DTI parameters between CMT and controls, with significant correlations between DTI parameters, muscle atrophy grade, and CMTNS. Level of Evidence 2 Technical Efficacy Stage 2 J. MAGN. RESON. IMAGING 2021;53:437-444.

A Double-Blind Randomized Controlled Trial Investigating the Most Efficacious Dose of Botulinum Toxin-A for Sialorrhea Treatment in Asian Adults with Neurological Diseases.

This study aims to determine the most efficacious dose of Botulinum neurotoxin type A (BoNT-A) in reducing sialorrhea in Asian adults with neurological diseases. A prospective, double-blind randomized controlled trial was conducted over 24 weeks. Thirty patients with significant sialorrhea were randomly assigned to receive a BoNT-A (Dysport(®)) injection into the submandibular and the parotid glands bilaterally via an ultrasound guidance. The total dose given per patient was either BoNT-A injection of (i) 50 U; (ii) 100 U; or (iii) 200 U. The primary outcome was the amount of saliva reduction, measured by the differential weight (wet versus dry) of intraoral dental gauze at baseline and at 2, 6, 12, and 24 weeks after injection. The secondary outcome was the subjective report of drooling using the Drooling Frequency and Severity Scale (DFS). Saliva reduction was observed in response to all BoNT-A doses in 17 patients who completed the assessments. Although no statistically significant difference among the doses was found, the measured reduction was greater in groups that received higher doses (100 U and 200 U). The group receiving 200 U of Dysport(®) showed the greatest reduction of saliva until 24 weeks and reported the most significant improvement in the DFS score.

Uterine arteriovenous malformation.

Uterine arteriovenous malformation (AVM) is a rare condition, with fewer than 100 cases reported in the literature. Despite it being rare, it is a potentially life-threatening condition. This case report describes a 33-year-old woman who presented with secondary post-partum hemorrhage. Transabdominal ultrasound (US) of the pelvis showed increased vascularity with multidirectional flow of the uterus and a prominent vessel, located on the left lateral wall. She also had retained product of conception, which complicated the diagnosis. A uterine artery angiogram confirmed an AVM in the fundal region with an early draining vein. Embolisation of the AVM was performed successfully.

Jaundice and life-threatening hemobilia: an uncommon presentation of choledochal cyst.

Hemobilia with jaundice as a result of cholestasis and bleeding from choledochal cyst is uncommon. Ascertaining the diagnosis is often challenging and delayed diagnosis can lead to significant consequences due to hemodynamic instability, particularly in elderly patients. Although surgery remains the definitive treatment modality, interventional radiology for hemostasis has been increasingly recognized as an option. In this manuscript, we described two Malaysian cases of jaundice and hemobilia associated with choledochal cysts and the challenges related with clinical diagnosis and management.

Benefits of 3D rotational DSA compared with 2D DSA in the evaluation of intracranial aneurysm.

RATIONALE AND OBJECTIVES: The aim of this study was to compare conventional two-dimensional (2D) digital subtraction angiography (DSA) with three-dimensional (3D) rotational DSA in the investigation of intracranial aneurysm in terms of detection, size measurement, neck diameter, neck delineation, and relationship with surrounding vessels. A further aim was to compare radiation dose, contrast volume, and procedural time between the two protocols. MATERIALS AND METHODS: Thirty-five patients who presented with subarachnoid bleeds on computed tomography and were suspected of having intracranial aneurysms underwent conventional 2D DSA followed by 3D DSA. The 3D digital subtraction angiographic images were displayed as surface shaded display images. Aneurysm detection, sac size, neck diameter, neck delineation, and relationship of aneurysm to the surrounding vessels analyzed from the two protocols were compared. Radiation dose, contrast volume, and procedural time for both examinations were also compared. RESULTS: Three-dimensional DSA detected 44 aneurysms in 31 patients, with negative findings seen in four patients. A false-negative detection rate of 6.8% (three of 44) for 2D DSA was noted. There was no significant difference in aneurysm size between 3D and 2D DSA. The sizes of aneurysm necks were found to be significantly larger in 3D DSA than on 2D DSA. The aneurysm neck and relationship to surrounding vessels were significantly better demonstrated on 3D DSA than on 2D DSA. Radiation dose (entrance surface dose), contrast use, and procedural time with 3D DSA were significantly less than with 2D DSA. CONCLUSIONS: Three-dimensional DSA improves the detection and delineation of intracranial aneurysms, with lower radiation dose, less contrast use, and shorter procedural time compared to 2D DSA. The size of the aneurysm neck on 3D DSA tended to be larger than on 2D DSA.

A rare case of primary infiltrating neuroendocrine carcinoma of the breast.

Primary neuroendocrine carcinoma of the breast is a very rare malignant tumor. There are not many cases reported in the English literature since it was first documented in 1983. Reports on the imaging features, in particular the ultrasonographic features of this rare tumor are scarce. Herein, we report a case of aggressive primary infiltrating neuroendocrine carcinoma of the breast, masquerading as an inflammatory breast condition in a 22-year-old young lady, perhaps the youngest case ever reported in the English literature. We discuss the imaging features and highlight the Doppler ultrasonographic findings of this rare breast carcinoma. This is the first documentation on Doppler ultrasonographic findings of primary neuroendocrine carcinoma of the breast in the literature.