Role of Dynamic Magnetic Resonance Imaging in Hirayama Disease, a Rare Motor Neuron Disease.

Introduction Hirayama disease (HD) is a benign self-limiting motor neuron disease, most commonly occurring in young males. The disease has an insidious onset that affects T1, C8, and C7 myotomes. HD is more common in Japan and Asian countries, and rare in the western population. Magnetic resonance imaging (MRI) is the best technique for the diagnosis of this entity. Early diagnosis is important as the patients can be advised to limit neck flexion movements to arrest the progression of the disease. Any clinically suspected case of Hirayama disease should undergo flexion MRI as conventional neutral MRI may miss findings in a few cases. The purpose of the present study is to evaluate the usefulness of flexion MR imaging and laminodural space (LDS) measurement in young patients with clinical and electroneuromyography (ENMG) definite Hirayama disease. Materials and methods This is a retrospective observational study of 15 patients with clinical and ENMG definite Hirayama disease who were referred to the Department of Radiology. These patients underwent MRI of cervical spine in neutral position and with neck flexion of 30°-40°. In neutral MRI, atrophy and T2-weighted hyperintensities in the cord were noted. In flexion MRI, the maximum forward shifting of the posterior dural sac, also known as the LDS, was noted along with other parameters. Observation The mean age of the study population was 21 ± 3.36 years. Out of 15 patients, 14 were males and one was female; 14 patients (93.3%) had an involvement of unilateral upper extremity while one patient (6.6%) had asymmetric bilateral involvement. Straightening of cervical spinal curvature and cord atrophy was seen in 14 (93.3%) and 12 (80%) patients, respectively, on neutral position MRI. Intramedullary cervical cord T2-weighted hyperintensities were noted in eight patients (53.3%). Loss of the dural attachment and forward shifting of the posterior dural sac with prominent posterior epidural space was noted in all patients (100%). At the maximum forward shift of cord, the LDS ranged from 3.1 to 7.0 mm, with a mean of 5.38 ± 1.13 mm. Epidural flow voids were noted in 86.6% of cases. Conclusion Flexion MRI plays a very important role in confirming the diagnosis of Hirayama disease in clinically suspected cases. Anterior displacement of posterior dura matter and widening of LDS is noted in all cases in our study. Even though findings like cord atrophy and T2 hyperintensities are seen in conventional neutral MRI, these findings are not seen in all cases. So flexion MRI increases diagnostic confidence by showing increased LDS, which is a characteristic finding in Hirayama disease.

Correlation of Trigeminopontine Angle with Severity of Trigeminal Neuralgia due to Neurovascular Conflict over Medial Aspect of Nerve: Can We Prognosticate the Reduction in Pain in Patients on Medical Management?

Background Neurovascular conflicts (NVCs) are one of the major causative factors in patients presenting with trigeminal neuralgia (TN). We found a wide range of variation in degrees of acute angle formed between medial border of trigeminal nerve and anterior border of pons in patients with TN, i.e., medial trigeminopontine angle (mTPA), and tried to find its correlation with pain severity due to NVC over the medial aspect of nerve (mNVC). Aims and Objectives To correlate mTPA measurement with severity of TN due to mNVC. To calculate the reduction in pain in patients kept on medical management and its correlation with mTPA. Materials and Methods This was a retrospective observational study conducted between May 2018 and October 2020. A total of 41 patients presenting with TN and showing corresponding NVC were included in the study. Out of the total cases with NVC, 30 cases showed NVC over the medial surface of the nerve. All the patients were evaluated on MAGNETOM Skyra 3T magnetic resonance imaging (MRI; Siemens). Using the two-line Cobb angle method, the trigeminopontine angle was calculated. Pretreatment pain intensity and posttreatment pain relief of each patients were assessed by using the numeric rating scale (NRS) with numbers from 0 to 10 ("no pain" to "worst pain imaginable"). Relevant clinical details regarding pre- and posttreatment pain score, after a standard treatment plan of 600 mg of oxcarbazepine for 2 weeks, were collected. Results Patients showing response of more than or equal to 50% (≥50%) are considered as "good response" and those with response of less than 50% (<50%) are considered as "poor response." In our study with trigeminopontine angle threshold of 45 degrees, 7 out of 8 (87.5%) patients with >45° mTPA showed poor response and 15/22 (68.2%) patients with ≤45° showed good response to medical management for TN with statistical significance difference with a p -valve of 0.007. Conclusion We found a negative correlation between the mTPA and percentage pain relief in patients kept on medical management and realized that mTPA measurement could become an important tool for prognosticating pain relief for patients of TN on medical therapy; however, more evidence and multicentric studies are required for the same.

Pharmaco Penile Duplex Ultrasonography in the Evaluation of Erectile Dysfunction.

INTRODUCTION: The National Institute of Health defined 'erectile dysfunction' as the persistent inability to achieve and/or to maintain an erection for a satisfactory sexual performance. In last few years, the concept of erectile dysfunction has evolved from that of a disorder referred to as 'impotence' which used to be considered predominantly psychogenic to that of 'Erectile Dysfunction' (ED), a well understood physiologic result of multiple risk factors, both psychological and organic. The most common cause of organic erectile dysfunction is vasculogenic causes. Doppler evaluation of cavernosal arteries after intracavernosal injection of Papaverine is particularly useful in the evaluation of vasculogenic causes. AIM: To define the role of intracavernosal injection of Papaverine in the evaluation of vasculogenic causes of erectile dysfunction that includes arterial insufficiency and veno occlusive nature. MATERIALS AND METHODS: Pharmaco Penile Duplex Ultrasonography (PPDU) was done using a linear broadband phased array transducer (7-12 MHz) on a E-Saote MyLab 60 ultrasound colour Doppler system on 73 patients over a period of three years. Informed consent was taken from all patients. Visual grading score for erection, Cavernosal Artery Diameter (CAD), PSV (Peak Systolic Velocity), EDV (End Diastolic Velocity), RI (Resistive Index), AT (Acceleration Time) and dorsal vein changes were obtained in all patients following intracavernosal injection of Papaverine. RESULTS: Visual grading for erectile response was E0 in one patient, E1 in 11 patients, E2 in 9 patients, E3 in 7 patients, E4 in 4 patients and E5 in 41 patients. Eighteen patients were diagnosed as having arterial insufficiency, three patients were diagnosed as having venous insufficiency and two patients showed indeterminate results. CONCLUSION: In our study, Papaverine induced PPDU proved to be highly accurate and excellent method for assessing patients with erectile dysfunction.

Cerebral Aspergillus arteritis with bland infarcts: a report of two patients with poor outcome.

Two patients with cerebrovascular aspergillosis, in the form of arteritis, thrombosis and bland infarcts are reported. One patient had systemic lupus erythematosus with disseminated aspergillosis in lungs, kidneys and brain. The other patient was immunocompetent and had sphenoid sinusitis. Both the patients were diagnosed at autopsy only, despite extensive imaging and laboratory studies. High index of clinical suspicion and early aggressive antifungal therapy are required since definite diagnostic modalities are not available.