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Multi-phase computed tomography (CT) images have gained significant popularity in the diagnosis of hepatic disease. There are several challenges in the liver segmentation of multi-phase CT images. (1) Annotation: due to the distinct contrast enhancements observed in different phases (i.e., each phase is considered a different domain), annotating all phase images in multi-phase CT images for liver or tumor segmentation is a task that consumes substantial time and labor resources. (2) Poor contrast: some phase images may have poor contrast, making it difficult to distinguish the liver boundary. In this paper, we propose a boundary-enhanced liver segmentation network for multi-phase CT images with unsupervised domain adaptation. The first contribution is that we propose DD-UDA, a dual discriminator-based unsupervised domain adaptation, for liver segmentation on multi-phase images without multi-phase annotations, effectively tackling the annotation problem. To improve accuracy by reducing distribution differences between the source and target domains, we perform domain adaptation at two levels by employing two discriminators, one at the feature level and the other at the output level. The second contribution is that we introduce an additional boundary-enhanced decoder to the encoder–decoder backbone segmentation network to effectively recognize the boundary region, thereby addressing the problem of poor contrast. In our study, we employ the public LiTS dataset as the source domain and our private MPCT-FLLs dataset as the target domain. The experimental findings validate the efficacy of our proposed methods, producing substantially improved results when tested on each phase of the multi-phase CT image even without the multi-phase annotations. As evaluated on the MPCT-FLLs dataset, the existing baseline (UDA) method achieved IoU scores of 0.785, 0.796, and 0.772 for the PV, ART, and NC phases, respectively, while our proposed approach exhibited superior performance, surpassing both the baseline and other state-of-the-art methods. Notably, our method achieved remarkable IoU scores of 0.823, 0.811, and 0.800 for the PV, ART, and NC phases, respectively, emphasizing its effectiveness in achieving accurate image segmentation. [ABSTRACT FROM AUTHOR]

Context: Movement disorders represent a common presentation in pediatrics and are often a source of clinical and diagnostic dilemmas. In this review, we provide an overview of common causes along with simplified clinical approach and management options for major movement disorders. Sources: This narrative review is based on contemporary evidence and personal experience. Medline was searched for recent advances, current understanding and consensus on classification, clinical features, diagnosis and treatment. Results: Movement disorders are classified as hyperkinetic and hypokinetic disorders, the latter being rare in childhood. The hyperkinetic disorders include dystonia, chorea, athetosis, tics and tremor, stereotypies, myoclonus, startle syndromes and functional disorders. Some movement disorders can be benign and developmental. A large proportion of conditions are genetic in origin with a guarded prognosis. Some of the conditions may be post-infectious, immune-mediated or drug induced. Multiple types of movement disorders are present in many conditions. The age at onset, type and distribution of abnormal movements and presence of associated neurological and systemic features help in narrowing the differential diagnosis. The pharmacotherapy of movement disorders is complex and evolving. Conclusion: A synopsis of movement disorders presenting in pediatric age has been provided, incorporating the latest evidence. A simplified approach for clinical diagnosis has been developed for dystonia and chorea. [ABSTRACT FROM AUTHOR]

Background: Nerve conduction studies are considered to be the gold standard for diagnosing secondary tarsal tunnel syndrome (sTTS), but their utility in the diagnosis of idiopathic tarsal tunnel syndrome (iTTS) is largely unknown. Objective: We sought to investigate the value of motor nerve conductions studies (MNCS) in the diagnosis of clinically suspected iTTS. Materials and Methods: Twenty-six (52 limbs) adult patients of clinically suspected iTTS were subjected to motor nerve conductions of posterior tibial nerve, and its branches and motor conduction parameters were compared with those of 45 healthy controls. Results: Symptoms were bilateral in 70% (P = 0.02), with heel pain in 95% of symptomatic limbs. MNCS was abnormal in 32 (80%) of symptomatic limbs and 8 (66.6%) of asymptomatic limbs (P = 0.004). Out of electrophysiologically abnormal nerves (n = 67), the pathological process could be identified in all the nerves with abnormal MNCS (P = 0.02). Probable demyelination was seen in 58.2% of the electrophysiologically abnormal nerves. Discussion: The present study shows that iTTS are gender and Body Mass Index neutral with bilateral symptoms being common. Tinel's sign was inconsistent. Heel pain did not correlate with abnormal inferior calcaneal nerve conductions. Motor nerve conduction study was abnormal in a significant number of symptomatic limbs. "Probable demyelination" was more frequent in symptomatic limbs. Conclusion: MNCS is significantly abnormal in symptomatic limbs of subjects with iTTS. Demyelination is slightly more common than axonopathy in iTTS. With a sensitivity of 80% and specificity of 33.3%, MNCS seems to be useful as a screening tool in clinically suspected iTTS. This study is Level II: Lesser quality randomized controlled trial or prospective comparative study. [ABSTRACT FROM AUTHOR]

Anomalies of aortic arch are a common occurrence. Such anomalies of right sided aortic arch with its various branching patterns are of clinical importance. Rarer anomalies include isolation (deficient connection) of either left subclavian artery or left common carotid artery; that is, they do not have their origin from aorta or its major branches. We present a case of an 18-year-old male who presented with gradual onset pulsatile swelling with bruit in neck on left side and was evaluated by CT brain and neck angiography. CT angiography revealed right sided aortic arch with aberrant left subclavian artery and isolated left common carotid artery. Very few cases of such an anomaly have been documented in the literature but none in an adult. [ABSTRACT FROM AUTHOR]

Background and aims Joint hypermobility when associated with symptoms in the absence of systemic rheumatologic disease is termed as benign joint hypermobility syndrome ( BJHS). BJHS is often an under-recognised and a poorly managed entity. Indian studies on BJHS are very few and none have been carried out in any of the service rheumatology centres. Hence this retrospective study was carried out at a tertiary medical institute of the Indian Army to assess the varied clinical profile of BJHS. Methods All patients consecutively diagnosed as BJHS at the rheumatology clinic of the Army Hospital (Research and Referral) Delhi from May 2010 to May 2011 were included in the study. Their age, sex, presenting features, clinical profile, laboratory and radiological parameters were studied. Results The mean age of these patients was 30 ± 5.71 years with a median duration of symptoms of 42 (06-120) months. There were 45 males and 39 females (male : female = 1.15 : 1.00). The median Beighton's score in these patients was 6/9 (range 4-9). Most of our patients were military personnel (43/84), and all had knee joint pain with evidence of degenerative changes in 19 and synovitis in two patients. Eleven patients including nine military personnel had evidence of soft tissue rheumatism with associated fibromyalgia in four and anxiety disorder in one. Out of 18 patients with a Beighton's score of ≥ 7, nine had incidental findings of lateral head tilt on frontal observation. There was evidence of carpal tunnel syndrome in a patient with wrist synovitis and one patient had associated skin laxity without features of Ehlers-Danlos syndrome. Conclusion BJHS is often under-recognized in clinical practice and is usually missed because of a lack of awareness. A high index of clinical suspicion to diagnose this entity is essential due to its associated morbidities, especially among those exposed to strenuous physical activities. [ABSTRACT FROM AUTHOR]

Background: Acute hypokalemic paralysis, characterized by acute flaccid paralysis is primarily a calcium channelopathy, but secondary causes like renal tubular acidosis (RTA), thyrotoxic periodic paralysis (TPP), primary hyperaldosteronism, Gitelman's syndrome are also frequent. Objective: To study the etiology, varied presentations, and outcome after therapy of patients with hypokalemic paralysis. Materials And Methods: All patients who presented with acute flaccid paralysis with hypokalemia from October 2009 to September 2011 were included in the study. A detailed physical examination and laboratory tests including serum electrolytes, serum creatine phosphokinase (CPK), urine analysis, arterial blood gas analysis, thyroid hormones estimation, and electrocardiogram were carried out. Patients were further investigated for any secondary causes and treated with potassium supplementation. Result: The study included 56 patients aged 15-92 years (mean 36.76 ± 13.72), including 15 female patients. Twenty-four patients had hypokalemic paralysis due to secondary cause, which included 4 with distal RTA, 4 with Gitelman syndrome, 3 with TPP, 2 each with hypothyroidism, gastroenteritis, and Liddle's syndrome, 1 primary hyperaldosteronism, 3 with alcoholism, and 1 with dengue fever. Two female patients were antinuclear antibody-positive. Eleven patient had atypical presentation (neck muscle weakness in 4, bladder involvement in 3, 1 each with finger drop and foot drop, tetany in 1, and calf hypertrophy in 1), and 2 patient had respiratory paralysis. Five patients had positive family history of similar illness. All patients improved dramatically with potassium supplementation. Conclusion: A high percentage (42.9%) of secondary cause for hypokalemic paralysis warrants that the underlying cause must be adequately addressed to prevent the persistence or recurrence of paralysis. [ABSTRACT FROM AUTHOR]

Aim: Nailfold capillaroscopy (NFC) is a simple, non-invasive method with exceptional predictive value for the analysis of microvascular abnormalities, especially in systemic sclerosis (SSc) but remains underutilized due to cost factors of the nailfold videocapillaroscope, lack of expertise and availability issues. The aim of this study was to establish the utility of an inexpensive digital microscope to study NFC changes in SSc in correlation with disease subsets and extent of skin involvement. Methods: Twenty-two diffuse cutaneous SSc (DSS), 20 limited cutaneous SSc (LSS) patients and 42 controls were evaluated with NFC using a digital microscope at 30× and 100× magnification. Digital micrographs were used to study qualitative and quantitative changes in microvasculature. Results: The capillary density was significantly less in all cases of SSc as compared to controls (5.3 ± 1.4 vs. 8.7 ± 1.2; P < 0.00001). Disorganized architecture was much more prevalent in DSS versus LSS (86.4% vs. 25%). The vascular deletion score (VDS) was significantly higher in DSS as compared to LSS ( P < 0.0001). Scleroderma pattern (SP) was seen in 18 (81.9%) and 15 (75%) of patients with DSS and LSS, respectively. Only 4% of normal subjects showed non-specific pattern and none showed SP. The mean modified Rodnan skin score (MRSS) was positively correlated with vascular deletion score ( r = 0.572; P < 0.001) and negatively with capillary density ( r = −0.8; P < 0.001). Conclusion: Nailfold capillaroscopy changes in SSc are related to disease subset and MRSS. NFC with digital microscope is a simplified, inexpensive, outpatient procedure with results comparable to previous studies. [ABSTRACT FROM AUTHOR]

. Despite the use of standardized definitions, widely varying prevalence estimates of electrocardiographic (ECG) features related to arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) have been reported in different cohorts. This study was aimed at examining the variability in the ECG interpretation resulting from the same reader, different readers, and using different ECG-resolutions. Blinded to other clinical data, 2 readers examined quantitative and qualitative ECG features of 20 (10 ARVD/C) randomly selected individuals. ECGs were recorded at standard-speed (SS) and double-speed-double-amplitude (DS) settings. The SS ECGs were scanned, magnified 4×, and evaluated using electronic calipers (EL). One reader repeated all measurements. For both readers, the intraclass correlation coefficient (ICC) for the measurement of QRS duration was good between conventional and electronic evaluation [DS vs EL: Reader 1-0.64 (0.52-0.73); Reader 2-0.67 (0.55-0.76)][SS vs EL: Reader 1-0.60 (0.47-0.70); Reader 2-0.60 (0.47-0.70)]. Using the same resolution, the intrareader ICC was good for SS [0.70 (0.59-0.78)], DS [0.85 (0.80-0.90)], and EL [0.70 (0.69-0.83)] resolutions, but deteriorated for interreader comparisons [0.50 (0.36-0.62), 0.75 (0.66-0.82), and 0.75 (0.66-0.82), respectively]. For qualitative parameters, the intra- and interreader agreement was inconsistent for all but 2 parameters. Both readers were in perfect agreement while interpreting right precordial T-wave inversion [κ= 1] and right bundle branch block morphology (RBBB) [κ= 0.83 (0.5-1.0)] even when using SS resolution. Right precordial t-wave inversion and RBBB are the only ECG parameters that can be detected consistently even using the conventionally used ECG-resolution. The substantial variability in evaluation of other parameters is not improved even with the use of higher resolutions. (J Cardiovasc Electrophysiol, Vol. 22, pp. 561-568 May 2011) [ABSTRACT FROM AUTHOR]

Background: Parietal block, defined as intra right ventricular (RV) conduction slowing, is a major diagnostic criterion for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Objective: We evaluated the utility of total RV endocardial activation duration (EAD) measured by 3-dimensional electroanatomic mapping during sinus rhythm in the diagnosis of ARVD/C. Methods: Twenty-five consecutive patients with frequent left bundle branch block morphology premature ventricular complexes who underwent electroanatomic mapping as a part of the evaluation for ARVD/C were included in the study. All patients were evaluated using standard protocol that included electrocardiogram (ECG), signal-averaged ECG, Holter monitoring, echocardiography, and magnetic resonance imaging. Invasive testing was performed as indicated. Total RV EAD was measured as the time interval between the onset of RV activation to the latest activated region in the RV. Results: The mean age of the study subjects was 38 ± 11 years, and 32% were men. Fourteen subjects were diagnosed with ARVD/C using task force criteria, and the remainder had idiopathic ventricular tachycardia. Although the surface QRS durations were similar, the total RV EAD was significantly prolonged in ARVD/C compared with idiopathic VT (83.9 ± 10 ms vs. 50.8 ± 7 ms, P <.001). None of the idiopathic VT subjects had RV EAD of >65 ms. RV EAD also showed significant negative correlation with RV ejection fraction. Conclusion: Total RV EAD obtained by 3-dimensional electroanatomic mapping is a sensitive marker of intra-RV conduction delay in ARVD/C, and a total RV EAD of >65 ms accurately differentiates ARVD/C from idiopathic VT. [Copyright &y& Elsevier]

We report a 35-year-old man who presented with abnormal movement of hand upon extension at wrist joint in an outstretched arm. He was diagnosed and treated elsewhere as having action-induced tremors. On examination, he had action-induced clonus at wrist joint with bilateral corticospinal tract features in the form of weakness, spasticity and extensor plantars. Evaluation also revealed cervical compressive myelopathy. The purpose of presenting this case report is to highlight this rare clinical sign which can be mistaken for action-induced tremors. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 40-year-old woman who was admitted to the hospital because of corpus callosal hemorrhage with intraventricular extension. Also discusses the diagnostic removal of ventricular blood and hydrocephalus through computed tomography (CT) and applying fibrinolytic therapy with intraventricular alteplace.

Background: Dopa responsive dystonia is characterized by progressive disabling dystonia, diurnal variation and a dramatic response to Levodopa. Case characteristics: Two siblings presented with regression of motor milestones and hypertonia in lower limbs. History of diurnal variation was present in elder sibling. Outcome: Both responded dramatically to Levodopa. The genomic DNA analysis of elder sibling revealed a novel mutation. Message: A trial of Levodopa should be considered in a child with motor regression with diurnal variation, in the presence of extrapyramidal features. [ABSTRACT FROM AUTHOR]

The article discusses subacute sclerosing panencephalitis (SSPE), which is a progressive, fatal encephalitis of children and adolescents caused by persistent infection of immune resistant measles virus. In the case of a 14-year-old with acute onset rapidly progressive cognitive decline and myoclonic jerks of 15 days duration, magnetic resonance imaging (MRI) showed hypointensities on T1W image with corresponding hyperintensities on T2W image and T2 flair.

A 32-year-old man presented with atypical left hemifacial spasm (HFS) with MRI showing compression of left facial nerve at the root exit zone. HFS rarely presents atypically with onset in orbicularis oris and later spreading upward to involve the orbicularis oculi as was seen in our case. Atypical presentation of hemifacial spasm is rare and vertebral artery dolichoectasia as the underlying aetiology, like in our case is even rarer and has not been described in literature so far. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to determine the extent of left ventricular (LV) involvement in individuals predisposed to developing arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), and to investigate novel morphologic variants of ARVD/C. Background: The discovery of desmosomal mutations associated with ARVD/C has led researchers to hypothesize equal right ventricular (RV) and LV affliction in the disease process. Methods: Thirty-eight (age 30 ± 17 years; 18 males) family members of 12 desmosomal mutation-carrying ARVD/C probands underwent genotyping and cardiac magnetic resonance imaging (CMR). The CMR investigators were blinded to clinical and genetic data. Results: Twenty-five individuals had mutations in PKP2, DSP, and/or DSG2 genes. RV abnormalities were associated with the presence of mutation(s) and with disease severity determined by criteria (minor = 1; major = 2) points for ARVD/C diagnosis. The only LV abnormality detected, the presence of intramyocardial fat, was present in 4 individuals. Each of these individuals was a mutation carrier, whereas 1 had no previously described ARVD/C-related abnormality. On detailed CMR, a focal “crinkling” of the RV outflow tract and subtricuspid regions (“accordion sign”) was observed in 60% of the mutation carriers and none of the noncarriers (p < 0.001). The sign was present in 0%, 37%, 71%, and 75% of individuals who met 1, 2, 3, and 4+ criteria points, respectively (p < 0.01). Conclusions: Despite a possible LV involvement in ARVD/C, the overall LV structure and function are well preserved. Independent LV involvement is of rare occurrence. The accordion sign is a promising tool for early diagnosis of ARVD/C. Its diagnostic utility should be confirmed in larger cohorts. [Copyright &y& Elsevier]