Eosinophilic fasciitis with subjacent myositis.

INTRODUCTION: Eosinophilic fasciitis (EF) is a rare disorder that can present with muscle symptoms that mimic other neuromuscular diseases. METHODS: We report the case of a 43-year-old woman with chronic muscle aches, tightness, and stiffness with hypertrophied, well-defined muscles despite physical inactivity, and thickened skin with reduced elasticity and discoloration. RESULTS: Except for mild peripheral eosinophilia, laboratory studies, including blood count, electrolytes, paraneoplastic panel, muscle enzymes, thyroid function, and serum protein electrophoresis, were normal. Nerve conduction studies and needle electromyography were normal. Magnetic resonance imaging of the thighs demonstrated superficial and deep fascial thickening with T2 hyperintensity and post-gadolinium enhancement. Fascial and muscle biopsy demonstrated an inflammatory exudate in the perimysium and endomysium with fragmented perimysial connective tissue and thickened, inflamed fascia. EF was diagnosed. The patient was treated with methotrexate and prednisone followed by improvement of muscle stiffness and tightness. CONCLUSION: EF should be considered when patients present with muscle pain or enlarged muscles. Muscle Nerve 56: 525-529, 2017.

Epilepsy treatment in adults and adolescents: Expert opinion, 2016.

INTRODUCTION: There are over twenty anti-seizure medications and anti-seizure devices available commercially in the United States. The multitude of treatment options for seizures can present a challenge to clinicians, especially those who are not subspecialists in the epilepsy field. Many clinical questions are not adequately answered in double-blind randomized controlled studies. In the presence of a knowledge gap, many clinicians consult a respected colleague with acknowledged expertise in the field. Our survey was designed to provide expert opinions on the treatment of epilepsy in adults and adolescents. METHOD: We surveyed a group of 42 physicians across the United States who are considered experts based on publication record in the field of epilepsy, or a leadership role in a National Association of Epilepsy Centers comprehensive epilepsy program. The survey consisted of 43 multiple-part patient scenario questions and was administered online using Redcap software. The experts provided their opinion on 1126 treatment options based on a modified Rand 9-point scale. The patient scenarios focused on genetically-mediated generalized epilepsy and focal epilepsy. The scenarios first focused on overall treatment strategy and then on specific pharmacotherapies. Other questions focused on treatment of specific patient populations (pregnancy, the elderly, patients with brain tumors, and post organ transplant patients), epilepsy patients with comorbidities (renal and hepatic disease, depression), and how to combine medications after failure of monotherapy. Statistical analysis of data used the expert consensus method. RESULTS: Valproate was considered a drug of choice in all genetically-mediated generalized epilepsies, except in the population of women of child-bearing age. Ethosuximide was a drug of choice in patient with absence seizures, and levetiracetam was a drug of choice in patients with genetic generalized tonic-clonic seizures and myoclonic seizures. Lamotrigine, levetiracetam and oxcarbazepine were considered drugs of choice for initial treatment of focal seizures. Lamotrigine and levetiracetam were the drugs of choice for women of child-bearing age with either genetic generalized epilepsy or focal epilepsy. Lamotrigine and levetiracetam were the drugs of choice in the elderly population. Lamotrigine was preferred in patients with co-morbid depression. Levetiracetam was the drug of choice in treating patients with hepatic failure, or who have undergone organ transplantation. Compared to the 2005 and 2001 surveys, there was increased preference for the use of levetiracetam and lamotrigine, and decreased preference for the use of phenytoin, gabapentin, phenobarbital and carbamazepine. DISCUSSION: The study presented here provides a "snapshot" of the clinical practices of experts in the treatment of epilepsy. The experts were very often in agreement, and reached consensus in 81% of the possible responses. However, expert opinion does not replace the medical literature; instead, it acts to supplement existing information. Using the study results is similar to requesting an expert consultation. Our findings suggest options that the clinician should consider to achieve best practice.