Non-diabetic Euglycemic Ketoacidosis in an Adult Patient With Spinal Muscular Atrophy Type II.

Spinal muscular atrophy (SMA) is a rare neuromuscular disease that develops as a result of the degeneration of the anterior horn cells in the spinal cord and lower brainstem motor nuclei, resulting in progressive muscle weakness and atrophy. While the initial presentation of this disease involves diffuse muscular atrophy at an early age, patients with an established diagnosis and later-stage disease often present with gastrointestinal symptoms related to metabolic imbalances. Here, we examine the case of an adult patient with SMA type II who presented with complaints of 12 hours of intractable nausea and vomiting. The patient was found to be in euglycemic ketoacidosis (EKA), an uncommon, but not unheard of, metabolic derangement in SMA patients with severely decreased muscle mass.

Fatal Disseminated Coccidioidomycosis in Cirrhosis: A Case Series.

PURPOSE: Coccidioidomycosis is endemic to the Southwest United States and Mexico. In this case series we describe 3 cases, occurring in the Southwest United States, of patients with disseminated coccidioidomycosis infection in cirrhosis, all with a miliary pattern present on chest imaging. METHODS: This case series was performed conducting a review of patients' electronic health records and thorough review of the literature for coccidioidomycosis infection in patients with liver disease. RESULTS: Three patients with different etiology of liver disease with Model for End-stage Liver Disease - Sodium (MELD-Na) scores >20 had chest imaging findings indicative of a miliary pattern on presentation. Each patient subsequently had extensive infectious disease workup that showed evidence of disseminated coccidioidomycosis. All 3 patients clinically worsened and eventually died. CONCLUSIONS: This case series highlights the severity of disseminated coccidioidomycosis in patients with cirrhosis in an endemic area, as well as potential early clues such as miliary patterns on chest imaging. A review of the literature found a significant connection among potential mechanisms describing why patients with cirrhosis have such adverse outcomes in the setting of disseminated coccidioidomycosis, including cirrhosis-associated immune dysfunction and genetic defects in immune functioning.

Physostigmine in Anticholinergic Poisoning: An Old Antidote With Resurgence.

Physostigmine is a cholinesterase inhibitor used therapeutically in patients with anticholinergic delirium that is so severe that intubation may be required for airway protection. Given that a wide variety of medications have anticholinergic properties, and the current standard of care is typically a central nervous system depressant, hospitalizations are often lengthy and normally require a critical-care level of attention. Despite this, physostigmine is often underutilized and poorly understood in the clinical setting. We report a case of a 43-year-old female who presented to the emergency department one hour after ingesting approximately 150 tablets of diphenhydramine in a suicide attempt. She was treated with benzodiazepines with minimal success, and airway protection became imminent as her mentation continued to decline. Through the use of physostigmine, we were able to save this patient from endotracheal intubation and the potential complications of mechanical ventilation.

Large Left Ventricular Thrombus in a Patient with Systemic and Venous Thromboembolism Secondary to Protein C and S Deficiency.

58-year-old Hispanic female presented with an altered mental status. A CT scan of the head demonstrated multiple scattered infarcts and a large right temporal lobe infarct. We also diagnosed the patient with right popliteal and femoral vein thrombosis, bilateral pulmonary embolism, and a transient right radial artery occlusion. Her 12-lead EKG showed lateral ST elevation. Emergent coronary angiogram revealed normal coronaries. Echocardiogram demonstrated a large mobile mass attached to the anterolateral free wall with overall normal contractility of the left ventricle. The patient underwent surgical embolectomy to prevent further systemic embolization. Coagulability workup returned positive for protein C and S deficiency. The patient did well after surgery. Following her surgery, we initiated chronic oral anticoagulation. The presentation with intracardiac thrombus in a normal heart should raise a concern of a probable thrombophilia.

T2 mapping of the heart with a double-inversion radial fast spin-echo method with indirect echo compensation.

BACKGROUND: The abnormal signal intensity in cardiac T2-weighted images is associated with various pathologies including myocardial edema. However, the assessment of pathologies based on signal intensity is affected by the acquisition parameters and the sensitivities of the receiver coils. T2 mapping has been proposed to overcome limitations of T2-weighted imaging, but most methods are limited in spatial and/or temporal resolution. Here we present and evaluate a double inversion recovery radial fast spin-echo (DIR-RADFSE) technique that yields data with high spatiotemporal resolution for cardiac T2 mapping. METHODS: DIR-RADFSE data were collected at 1.5 T on phantoms and subjects with echo train length (ETL) = 16, receiver bandwidth (BW) = ±32 kHz, TR = 1RR, matrix size = 256 × 256. Since only 16 views per echo time (TE) are collected, two algorithms designed to reconstruct highly undersampled radial data were used to generate images for 16 time points: the Echo-Sharing (ES) and the CUrve Reconstruction via pca-based Linearization with Indirect Echo compensation (CURLIE) algorithm. T2 maps were generated via least-squares fitting or the Slice-resolved Extended Phase Graph (SEPG) model fitting. The CURLIE-SEPG algorithm accounts for the effect of indirect echoes. The algorithms were compared based on reproducibility, using Bland-Altman analysis on data from 7 healthy volunteers, and T2 accuracy (against a single-echo spin-echo technique) using phantoms. RESULTS: Both reconstruction algorithms generated in vivo images with high spatiotemporal resolution and showed good reproducibility. Mean T2 difference between repeated measures and the coefficient of repeatability were 0.58 ms and 2.97 for ES and 0.09 ms and 4.85 for CURLIE-SEPG. In vivo T2 estimates from ES were higher than those from CURLIE-SEPG. In phantoms, CURLIE-SEPG yielded more accurate T2s compared to reference values (error was 7.5-13.9% for ES and 0.6-2.1% for CURLIE-SEPG), consistent with the fact that CURLIE-SEPG compensates for the effects of indirect echoes. The potential of T2 mapping with CURLIE-SEPG is demonstrated in two subjects with known heart disease. Elevated T2 values were observed in areas of suspected pathology. CONCLUSIONS: DIR-RADFSE yielded TE images with high spatiotemporal resolution. Two algorithms for generating T2 maps from highly undersampled data were evaluated in terms of accuracy and reproducibility. Results showed that CURLIE-SEPG yields T2 estimates that are reproducible and more accurate than ES.

Trimethoprim-sulfamethoxazole induced rhabdomyolysis.

Empiric antibiotic usage is very common in clinical practice and Trimethoprim-Sulfamethoxazole (TMP-SMX) is one such antibiotic used extensively in primary care practice. Some patients experience serious adverse effects to the antibiotics that markedly increase the morbidity and the cost of medical care. We describe one such patient, a previously healthy 40-year-old Hispanic female who developed myositis and rhabdomyolysis secondary to TMP-SMX. To the best of our knowledge, this is the first report of TMP-SMX-induced rhabdomyolysis in an immunocompetent host.

Macrodactylia fibrolipomatosis presenting as a small bowel obstruction.

A patient presented with progressive abdominal distention, discomfort, nausea, vomiting, and constipation. The patient also had congenital macrodactyly of the third, fourth, and fifth digits of the left hand. He [corrected] was diagnosed with macrodactylia fibrolipomatosis with multiple small bowel lipomas and complete small bowel obstruction. Macrodactylia fibrolipomatosis is a rare condition characterized by benign fibrofatty infiltration involving the soft tissues of the distal arm or leg with associated dactylomegaly of the associated digits. This patient had a rare presentation that included extensive small bowel lipomatosis.