Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease.

Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND). Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample. Results: Nineteen patients, who constituted 20.6% of 92 patients of clinical and flexion MRI confirmed Hirayama disease were found to have bilateral symmetrical wasting and weakness of distal upper limb muscles at the time of presentation. Mean ± standard deviation (SD) age of onset of the disease process was 21.7 ± 3.8 years with mean ± SD duration of illness of 3.6 ± 1.3 years. MRI revealed lower cervical cord flattening in 13 (68.4%) patients which was symmetrical in 6 (31.6%) patients and asymmetrical in 7 (36.8%) patients. In the majority of these patients, T2-weighted images (T2WI) cervical cord hyperintensities were found extending from C5 to C6 vertebral level. Seven (36.8%) patients in our study showed bilateral symmetric T2WI hyperintensities in anterior horn cells (AHC). Conclusion: Bilateral symmetrical involvement of Hirayama disease is an uncommon presentation. Symmetrical cervical cord flattening, T2WI cord and/or bilateral AHC hyperintensities were the major MRI findings detected. Flexion MRI demonstrated similar findings in both bimelic amyotrophy and classical unilateral amyotrophy. However, flexion MRI produced some distinguishing features more typical for bilateral symmetrical Hirayama disease which help to differentiate it from other MNDs.

Spectrum of MRI Appearance of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in Primary Amenorrhea Patients.

INTRODUCTION: Mayer-Rokitansky-Kuster-Hauser (MRKH) synd-rome is a malformation in female genital tract due to interrupted embryonic development of para-mesonephric ducts leading to uterine and proximal vagina aplasia or hypoplasia. AIM: To analyse the MRI findings in females suspected of MRKH syndrome in a primary amenorrhea workup. MATERIALS AND METHODS: A cross-sectional study comprising of 11 patients of MRKH syndrome presented in a tertiary care centre from March 2016 to February 2017 evaluated in 1.5 Tesla MRI scanner. RESULTS: Normal positioned small vestigial uterus demonstrated in two patients (18.2%) and complete uterine and proximal vaginal agenesis were noted in nine patients (81.8%). The mean volume of rudimentary right uterine bud was 2.26 mL ±3.3 (SD) and left uterine bud was 1.27 mL±1.1 (SD). The mean volume of right ovary was 4.74 mL±2.58 (SD) and left ovary was 4.65 mL±2.2 (SD). The mean developed vaginal length was 26.2 mm±6.34 (SD). CONCLUSION: The rudimentary uterine buds associated with MRKH syndrome always maintained caudal relationship with ovary. Ovaries or rudimentary uterine buds can be ectopic and should be recognized with MRI before undergoing fertility treatment. Following MRI diagnosis, surgery allows patients to have sexual function with possible attainment of reproduction after assisted reproduction technique or surrogacy.

Extra-pituitary Cerebral Anomalies in Pediatric Patients of Ectopic Neurohypophysis: An Uncommon Association.

CONTEXT: Ectopic neurohypophysis (EN) refers to an interrupted, nonvisualized, and thinned out pituitary stalk with ectopic location of the posterior pituitary gland. Concurrent extra-pituitary cerebral and extra-cranial anomalies have been rarely reported in patients of EN. AIM: The aim of this study was to evaluate the magnetic resonance imaging (MRI) findings of extra-pituitary cerebral anomalies in pediatric patients of EN. SETTINGS AND DESIGN: A hospital-based cross-sectional study was conducted in a tertiary care center. SUBJECTS AND METHODS: The study group comprised eight pediatric patients of EN associated with extra-pituitary cerebral or vascular anomalies. Clinical and biochemical assessment was done in all patients. RESULTS: Out of the total eight patients with EN, MRI showed interrupted pituitary stalk in five patients (62.5%) and nonvisible pituitary stalk in three patients (37.5%). Ectopic posterior pituitary bright spot was demonstrated in median eminence in six patients (75%), faintly visualized in one patient (12.5%) and nonvisualized in another one patient. Statistical significant association was noted between pituitary gland height and patient's body height with the pituitary gland volume (P < 0.001). Varied extra-pituitary cerebral anomalies encountered in our patients ranged from isolated anomalies such as optic nerve hypoplasia in three patients (37.5%), corpus callosum dysplasia in four patients (50%), agyria-pachygyria complex in two patients (25%), and intracranial vascular anomalies in two patients to syndromic association of tuberous sclerosis in one patient. CONCLUSION: Identifying and reporting of associated extra-pituitary cerebral anomalies in patients with EN are crucial in assessing the overall neurological outcome of such patients.

Role of Cross Sectional Imaging in Isolated Chest Wall Tuberculosis.

INTRODUCTION: Isolated chest wall tuberculosis though a rare entity, the incidence of it has been on rise among immunocompromised population making it an important challenging diagnosis for the physicians. Its clinical presentation may resemble pyogenic chest wall abscess or chest wall soft tissue tumour. Sometimes it is difficult to detect clinically or on plain radiograph. AIM: The present study was conducted with an aim to evaluate the common sites and varying appearances of isolated chest wall tuberculosis. MATERIALS AND METHODS: A hospital based cross-sectional retrospective study was conducted in Assam Medical College and Hospital, a tertiary care centre in North East India. The study group comprise of 21 patients (n=15 male and n=6 females) with isolated chest wall tuberculosis without associated pulmonary or spinal involvement who were subjected to Computed Tomography/Magnetic Resonance Imaging (CT/MRI) of the thorax following initial Ultrasonogram (USG) evaluation of the local site. Pathological correlation was done from imaging guided sampling of the aspirate or surgery. RESULTS: Variable sites of involvement were seen in the chest wall in our patients (n=21), with chest wall abscess formation being the most common presentation and rib being the most common bony site affected in the thoracic cage. Bony sclerosis was noted in 11 patients (52.4%), periosteal reaction in 10 patients (47.6%) and sequestration in five patients (23.8%). CT/MRI not only localized the exact site and extent of the abscesses which facilitated guided aspirations, but also helped in detecting typical bony lesions thereby, differentiating from pyogenic osteomyelitis besides ruling out associated pulmonary or pleural involvement in such patients. CONCLUSION: Cross-sectional imaging plays an important role by giving a wholesome picture of both soft tissue and bony pathology. It is important to have adequate understanding of the radiologic manifestations of the chest wall involvement and complications of tuberculosis to facilitate diagnosis and in assessing response to treatment on follow up in patients.

Characterisation of musculoskeletal tumours by multivoxel proton MR spectroscopy.

OBJECTIVE: The purpose of this study is to evaluate the role of multi-voxel proton MR spectroscopy in differentiating benign and malignant musculoskeletal tumours in a more objective way and to correlate the MRS data parameters with histopathology. METHODS AND MATERIALS: A hospital-based prospective study was carried out comprising 42 patients who underwent MRI examinations from 1 July 2013 to 30 June 2014. After routine sequences, single-slice multi-voxel proton MR spectroscopy was included at TE-135 using the PRESS sequence. The voxel with the maximum choline/Cr ratio was used for analysis of data in 32 patients. The strength of association between the MR spectroscopy findings and the nature of tumour and histopathological grading were assessed. RESULTS: Of the 42 patients, the MR spectra were not of diagnostic quality in 10. In the remaining 32 patients, 12 (37.5%) had benign and 20 (62.5%) malignant tumours. The mean choline/Cr ratio was 6.97 ± 5.95 (SD) for benign tumours and 25.39 ± 17.72 (SD) for malignant tumours. In our study statistical significance was noted between the choline/Cr ratio and the histological nature of musculoskeletal tumours (p = 0.002) assessed by unpaired t-test. The choline/Cr ratio and histological grading were also found to be significant (p = 0.001) when assessed by one-way ANOVA test. CONCLUSIONS: Multi-voxel MR spectroscopy showed a higher choline/Cr ratio in malignant musculoskeletal tumours than in benign ones (p = 0.002). The choline/Cr ratio and histological grading of musculoskeletal tumours also showed statistical significance (p = 0.001).

Magnetic Resonance Imaging Analysis of Caudal Regression Syndrome and Concomitant Anomalies in Pediatric Patients.

OBJECTIVE: The aim of this study was to evaluate the magnetic resonance imaging (MRI) findings of caudal regression syndrome (CRS) and concomitant anomalies in pediatric patients. MATERIALS AND METHODS: A hospital-based cross-sectional retrospective study was conducted. The study group comprised 21 pediatric patients presenting to the Departments of Radiodiagnosis and Pediatric Surgery in a tertiary care hospital from May 2011 to April 2016. All patients were initially evaluated clinically followed by MRI. RESULTS: In our study, 21 pediatric patients were diagnosed with sacral agenesis/dysgenesis related to CRS. According to the Pang's classification, 2 (9.5%) patients were Type I, 5 (23.8%) patients were Type III, 7 (33.3%) patients were Type IV, and 7 (33.3%) patients were of Type V CRS. Clinically, 17 (81%) patients presented with urinary incontinence, 6 (28.6%) with fecal incontinence, 9 patients (42.9%) had poor gluteal musculatures and shallow intergluteal cleft, 7 (33.3%) patients had associated subcutaneous mass over spine, and 6 (28.6%) patients presented with distal leg muscle atrophy. MRI showed wedge-shaped conus termination in 5 (23.8%) patients and bulbous conus termination in 3 (14.3%) patients above the L1 vertebral level falling into Group 1 CRS while 7 (33.3%) patients had tethered cord and 6 (28.6%) patients had stretched conus falling into Group 2 CRS. CONCLUSION: MRI is the ideal modality for detailed evaluation of the status of the vertebra, spinal cord, intra- and extra-dural lesions and helps in early diagnosis, detailed preoperative MRI evaluation and assessing concomitant anomalies and guiding further management with early institution of treatment to maximize recovery.

Spectrum of Radiological Manifestations in Lymphoproliferative Malignancies with Unusual Extra Nodal Soft Tissue Involvement.

Lymphoproliferative malignancies constitute a wide spectrum of haematological malignancies and their prevalence is widely increasing. Non-Hodgkin lymphomas and Hodgkin disease, frequently involve extranodal soft tissue structures in the head and neck, thorax and abdomen. These malignancies may involve virtually any type of soft tissues to any extent; hence many different imaging manifestations are possible which may mimic other disorders. The imaging characteristics of extranodal lymphomatous soft tissue involvement are described and classified here according to the site of involvement in 6 cases (primary diseases with orbital, muscle, extra testicular, scalp, sinonasal and pachymeningeal/dural involvement). In majority of these cases at presentation we found a predominantly homogeneous soft tissue mass with mildly high attenuation on CT and a T2 intermediate signal on MRI at these sites without any manifestation of disease elsewhere but on follow-up two out of these six cases developed systemic disease elsewhere. Few consistent patterns were noticed on CT and MRI which might help to include lymphomas as an important differential diagnosis of soft tissue masses. Though a definitive diagnosis requires a biopsy (bone marrow, lymph node, or mass), and other laboratory tests, imaging primarily aims at staging of the disease and identification of new or recurrent disease.

Comparative Evaluation of Ultrasonography and Cross-sectional Imaging in Determining Gall Bladder Perforation in Accordance to Niemeier's Classification.

INTRODUCTION: Gall Bladder (GB) perforation, a rare but dreaded complication of acute cholecystitis and is associated with high mortality rate. Early detection of acute cases of GB perforation reduces the risk of biliary peritonitis and hence the associated mortality and morbidity. AIM: The purpose of the study was to make a comparative evaluation of the role of Cross-sectional imaging in GB perforation with base line investigation like sonography. Finally both modalities were compared in determining the type of perforation according to Niemeier's classification. MATERIALS AND METHODS: We retrospectively evaluated the Ultrasonography (USG), Computed Tomography (CT) and Magnetic Resonance Imagings (MRI) findings in patients of GB perforation with surgical correlation. RESULTS: We evaluated 17 patients of GB perforations over a time period of one year. USG was done in all patients. As cross-sectional modality only CT scan was done in 14 patients and MRI scan was done in four patients. Both CT and MRI scans were done in one patient. CONCLUSION: Cross-sectional imaging must not be delayed in suspected cases of GB perforations because it helps in establishing a quicker diagnosis, detecting complications and also helps in decision making related to management thereby reducing the morbidity and mortality associated with this condition.

Magnetic Resonance Imaging of Brain in Evaluation of Floppy Children: A Case Series.

Objective Hypotonia is a common clinical entity well recognized in pediatric age group, which demands experienced clinical assessment and an extensive array of investigations to establish the underlying disease process. Neuroimaging comes as great help in diagnosing the disease process in rare cases of central hypotonia due to structural malformations of brain and metabolic disorders and should always be included as an important investigation in the assessment of a floppy child. In this article, we discuss the MRI features of eight cases of central and two cases of combined hypotonia and the importance of neuroimaging in understanding the underlying disease in a hypotonic child.

Imaging in Rare and Atypical Sinonasal Masses: An Interesting Case Series.

Sinonasal tumours present a myriad of radiographic findings. While many of these tumours have been well described with regard to their typical sites of origin, age group and radiological appearance we have come across lesions in our daily practice which are exceedingly rare with regard to site of origin in sinonasal cavity. The radiological appearances of 4 such rare and unusual tumours arising in sinonasal region evaluated by cross sectional imaging (CT/MRI) have been illustrated in this article with a purpose to review the radio-pathological correlation of these tumours and to explain the utility of cross-sectional imaging CT and MRI in exploring diagnostic clues. Morphological features and radiological patterns of each tumour have been graded into mild, moderate and severe based on the extent of tumoural involvement. This review is intended to acquaint radiologists with the appearance of atypical sinonasal masses and their radiological appearance on cross sectional imaging to make an early diagnosis.

Radiographic patterns of soft tissue calcinosis in juvenile dermatomyositis and its clinical implications.

Juvenile Dermatomyositis (JDM) is a rare autoimmune inflammatory disease of muscles affecting children and adolescents with soft tissue calcification and varying systemic involvement. Though diagnosis is primarily by clinical, biochemical and histopathological tests, Imaging has unique significance from characterizing the calcinosis, detecting early changes in muscle in active phase of the disease, diagnosing potential complications,rule out other important differentials, guide biopsies ,and assessing the progress on follow up. Four distinct patterns of calcinosis have been described in relation to dermatomyositis which need to be differentiated from other aetiologies of soft tissue calcification and myopathies.