Gastroparesis and Gastroparesis Syndromes as Neuromuscular Disorders.

Gastroparesis syndromes (GpS) are a spectrum of disorders presenting with characteristic symptoms increasingly recognized as being gastrointestinal (GI) neuromuscular disorders (NMDs). This review focuses on GpS as a manifestation of neurologic disorders of GI NMD. GpS can be associated with systemic abnormalities, including inflammatory, metabolic, and serologic disorders, as well as autoimmune antibodies via nerve and muscle targets in the GI tract, which can be treated with immunotherapy, such as intravenous immunoglobulin. GpS are associated with autonomic (ANS) and enteric (ENS) dysfunction. Disorders of ANS may interact with the ENS and are the subject of continued investigation. ENS disorders have been recognized for a century but have only recently begun to be fully quantified. Anatomic structural changes in the GI tract are increasingly recognized in GpS. Detailed descriptions of anatomic changes in GpS, and their correlation with physiologic findings, have opened a new era of investigation. The management of GpS, when viewed as GI NMD, has shifted the paradigms of both diagnosis and treatment. This article concludes with current approaches to GpS directed at underlying neuromuscular pathology.

Baseline Characteristics and Predictive Factors of Intravenous Immunoglobulin Response in Drug and Device Refractory Gastroparesis Symptoms.

INTRODUCTION: Intravenous immunoglobulin (IVIG) has been shown in a small pilot series to be helpful for some patients with gastroparesis that is refractory to drugs, devices, and surgical therapies. Many but not all patients have serologic neuromuscular markers. We hypothesize that those patients with serologic markers and/or longer duration of therapy would have better responses to IVIG. MATERIALS AND METHODS: We studied 47 patients with a diagnosis of gastroparesis and gastroparesis-like syndrome that had all failed previous therapies including available and investigational drugs, devices, and/or pyloric therapies. Patients had a standardized 12-week course of IVIG, dosed as 400 mg/kg per week intravenously. Symptom assessment was done with Food and Drug Administration (FDA) compliant traditional patient-reported outcomes. Success to IVIG was defined as 20% or greater reduction in average symptom scores from baseline to the latest evaluation. RESULTS: Fourteen patients (30%) had a response, and 33 (70%) had no response per our definition. Patients responding had a higher glutamic acid decarboxylase 65 positivity (64% vs. 30%, P =0.049, missing=3) and longer duration of therapy (>12 wk/continuous: 86% vs. 48%, P =0.09). CONCLUSIONS: In this moderately sized open-label series of refractory patients with gastroparesis symptoms treated with IVIG, 30% of patients responded. While serologic markers and extended therapies show a trend to greater response, neither was statistically significant, except for glutamic acid decarboxylase 65 which showed a higher positivity rate in responders. We conclude that a clinical trial of IVIG may be warranted in severely refractory patients with gastroparesis symptoms.

Impact of Clostridioides Difficle Infection and its Therapy on Nutritional Status.

PURPOSE OF REVIEW: Clostridiodes difficile infection (CDI) is a leading nosocomial cause of increased morbidity and mortality in hospitalized patients and the presentation can vary from asymptomatic infection to severe fulminant colitis and sepsis. It can significantly impact nutritional status in hospitalized patients and lead to longer length of stay with increased morbidity and mortality. RECENT FINDINGS: An interplay of various intrinsic and extrinsic factors such as systemic inflammation, diarrheal losses, and impact of isolation influence the nutritional status of patients with CDI. While diarrheal losses can lead to dehydration and electrolyte disturbances, isolation can further hamper adequate nutritional support and make early signs of malnutrition overlooked. Similar detrimental impacts on nutritional status can also be observed in other bacterial and viral colonic infections. While prompt diagnosis and early treatment is crucial to prevent mortality, emphasis on nutritional rehabilitation can help reduce morbidity and promote recovery in CDI. Initiation of early feeding in critically sick patients with close monitoring for early signs of malnutrition promotes favorable outcomes.

Role of non-invasive multimodality imaging in autoimmune pericarditis.

Systemic autoimmune diseases are an important cause of pericardial involvement and contribute to up to ∼22% cases of pericarditis with a known aetiology. The underlying mechanism for pericardial involvement varies with each systemic disease and leads to a poor understanding of its management. Multimodality imaging establishes the diagnosis and determines the type and extent of pericardial involvement. In this review, we elaborate upon various pericardial syndromes associated with different systemic autoimmune and autoinflammatory diseases and the multitude of imaging modalities that can be used to further characterize autoimmune pericardial involvement. Lastly, these forms of pericarditis have a greater likelihood of recurrence, and clinicians need to understand their unique treatment approaches to improve patient outcomes.

Cortical Venous Thrombosis Complicating Tubercular Meningitis.

Cortical venous thrombosis (CVT) mostly occurs due to a hypercoagulable state. Infection- related CVT is very rare and is a medical emergency associated with a very high mortality rate. It has been described with bacterial and fungal infections; but only a few isolated case reports of CVT related to tubercular meningitis (TBM) exist. Only six documentations of CVT, as a complication of TBM, exist in the literature. Here, we report a case of a young girl with TBM who developed CVT as a complication. Key Word: Tuberculosis, Meningitis, Cerebral venous thrombosis.

Priapism as a Debut Presentation of Chronic Myeloid Leukemia.

Chronic myeloid leukemia (CML) is a chronic myeloproliferative disorder that usually presents with high white blood cell counts and massive splenomegaly. Priapism is a rare manifestation of CML and is mostly due to hyperleukocytosis. Its debut appearance as a sign of hematological dyscrasia is a rare event. Priapism occurring in a setting of any leukemia is both a medical and a urological emergency that requires immediate local therapy, symptomatic treatment, cytoreductive therapy and early initiation of targeted therapy. This case report describes priapism as an unusual presentation of CML and its importance in the work-up and management of patients presenting with priapism.