Pediatric brainstem abscess successfully treated with stereotactic aspiration: illustrative case.

BACKGROUND: Pediatric brainstem abscesses are rare entities that account for 1% of all brain abscesses and, when diagnosed, constitute a neurosurgical emergency. OBSERVATIONS: A previously healthy 11-year-old male presented with several days of worsening headache, confusion, and ataxia. Brain magnetic resonance imaging (MRI) revealed a midbrain and pons lesion. The patient subsequently had a rapid neurological decline with loss of consciousness and brainstem function. Follow-up MRI revealed significant enlargement of the brainstem lesion with extension into the pons, midbrain, and thalamus, with greater concerns for an abscess rather than a tumor or an inflammatory process. He was taken for an emergent stereotactic aspiration of the abscess, and broad-spectrum antibiotics were initiated. He had neurological improvement, which subsequently declined 5 days later with brain MRI revealing an increase in the brainstem abscess, which required a second stereotactic aspiration. After rehabilitation, he made a significant neurological recovery. LESSONS: Pediatric brainstem abscesses are rare pathologies, and a high index of suspicion is needed in patients presenting with a brainstem lesion mimicking tumor but with rapid neurological decline despite no other evidence of infection or infectious/inflammatory markers. Stereotactic aspiration is required for large lesions to target the antibiotic treatment and as an adjunct to broad-spectrum antibiotics.

Vertebral osteomyelitis due to Salmonella Poona in a healthy adolescent female.

We present a case of vertebral osteomyelitis in a previously healthy, adolescent Caucasian female athlete. After months of lower back pain, spinal imaging demonstrated phlegmon and suspected osteomyelitis of the L4 vertebral body. A bone biopsy was obtained, and microbiologic cultures yielded pure growth of Salmonella enterica subsp. enterica serovar Poona (S. Poona), a member of the nontyphoid Salmonella group associated with food-borne gastroenteritis in the United States. This case represents the first reported association of S. Poona with osteomyelitis and is interesting in that the infection developed in a patient without traditional risk factors for invasive Salmonella disease (i.e. sickle cell disease). This case highlights the importance of keeping a broad differential diagnosis for lower back pain and emphasizes the value of obtaining specimens for microbiologic culture to aid in diagnosing non-traditional and potentially emerging bacterial pathogens.

Are Clowns Good for Everyone? The Influence of Trait Cheerfulness on Emotional Reactions to a Hospital Clown Intervention.

Trait cheerfulness predicts individual differences in experiences and behavioral responses in various humor experiments and settings. The present study is the first to investigate whether trait cheerfulness also influences the impact of a hospital clown intervention on the emotional state of patients. Forty-two adults received a clown visit in a rehabilitation center and rated their emotional state and trait cheerfulness afterward. Facial expressions of patients during the clown visit were coded with the Facial Action Coding System. Looking at the total sample, the hospital clown intervention elicited more frequent facial expressions of genuine enjoyment (Duchenne smiles) than other smiles (Non-Duchenne smiles), and more Duchenne smiles went along with more perceived funniness, a higher level of global positive feelings and transcendence. This supports the notion that overall, hospital clown interventions are beneficial for patients. However, when considering individual differences in the receptiveness to humor, results confirmed that high trait cheerful patients showed more Duchenne smiles than low trait cheerful patients (with no difference in Non-Duchenne smiles), and reported a higher level of positive emotions than low trait cheerful individuals. In summary, although hospital clown interventions on average successfully raise the patients' level of positive emotions, not all patients in hospitals are equally susceptible to respond to humor with amusement, and thus do not equally benefit from a hospital clown intervention. Implications for research and practitioners are discussed.

Safety and efficacy of rapid (1,000 mg in 1 hr) intravenous iron dextran for treatment of maternal iron deficient anemia of pregnancy.

Maternal iron deficiency anemia (IDA) is associated with risk of adverse perinatal outcomes. Oral iron is recommended to reverse anemia, but has gastrointestinal toxicity and frequent non-adherence. Intravenous (IV) iron is reserved for intolerance of, or unresponsiveness to, oral therapy, malabsorption, and severe anemia (1% with hemoglobin [Hgb] levels <7 g/dL). With rare (<100 per one million) adverse events (AEs) ability to infuse a sufficient dose of low molecular weight iron dextran (LMWID) over 60 min, LMWID is an attractive option. This study demonstrated safety and efficacy of rapid IV infusion of 1,000 mg LMWID to gravidas with moderate to severe IDA. An observational treatment study of 1,000 mg LMWID administered over 1 hr for IDA in 189 consecutive, unselected second and third trimester gravidas after oral iron failure was conducted. All received a test dose of 25 mg LMWID and were monitored for AEs during the 60-min infusion. No premedication was administered unless more than one drug allergy or asthma was present in which case IV methylprednisolone was administered. All were followed through pregnancy and delivery. Monitored parameters included Hgb, mean corpuscular volume, serum ferritin, and percent transferrin saturation. About 189 subjects received 1,000 mg LMWID. No serious AEs occurred. About 2% experienced transient infusion reactions. Hgb improved by 1-1.9 g/dL in 82% and ≥2 g/dL in 24%. Second trimester treatment was not associated with greater Hgb improvement than third trimester treatment. Anemia resolved in 95%. Administration of a single large dose of IV LMWID was effective, safe, and convenient. Am. J. Hematol. 91:590-593, 2016. © 2016 Wiley Periodicals, Inc.

Safety and efficacy of total dose infusion of 1,020 mg of ferumoxytol administered over 15 min.

For the majority of patients with iron deficiency anemia (IDA), a full course of intravenous (IV) iron is 1 g. Most IV irons require 5-10 administrations of 100-300 mg. We have successfully employed 1 g low molecular weight iron dextran over 1 hr. For further convenience for patients and physicians, we explored the administration of 1.02 g of ferumoxytol over 15 min instead of the approved 2 × 510 mg injections. Sixty patients with IDA, (hemoglobin <11 g/dL, transferrin saturation [TSAT] ≤20%, and ferritin <100 ng/mL) with an inadequate response or intolerance to oral iron, received 1020 mg ferumoxytol over 15 min. Vital signs were measured for 1 hr. Adverse events (AEs) were collected via telephone at 1, 2, and 7 days. Follow-up visits occurred at 4 and 8 weeks for efficacy assessments. The primary endpoint was safety and tolerability. Secondary efficacy endpoints included mean change in hemoglobin, TSAT, and red cell distribution width. No serious adverse events (SAEs) occurred. Fifty-eight patients received the planned dose. Twenty-six out of sixty (43.3%) patients reported AEs of which 13 were mild and transient during infusion. All resolved within minutes. Fourteen patients reported self-limited arthralgias, myalgias, and/or headache within 24-48 hr. At Baseline, the mean hemoglobin was 9.4 g/dL. The mean increments at Week 4 and 8 were 2.1 and 2.6 g/dL, respectively. Ferumoxytol, administered as 1.02 g infusion over 15 min was well tolerated with no SAEs and demonstrated excellent efficacy. If corroborated in future studies this represents an improved method of treating IDA.

Neuroimaging of lipid storage disorders.

Lipid storage diseases, also known as the lipidoses, are a group of inherited metabolic disorders in which there is lipid accumulation in various cell types, including the central nervous system, because of the deficiency of a variety of enzymes. Over time, excessive storage can cause permanent cellular and tissue damage. The brain is particularly sensitive to lipid storage as the contents of the central nervous system must occupy uniform volume, and any increases in fluids or deposits will lead to pressure changes and interference with normal neurological function. In addition to primary lipid storage diseases, lysosomal storage diseases include the mucolipidoses (in which excessive amounts of lipids and carbohydrates are stored in the cells and tissues) and the mucopolysaccharidoses (in which abnormal glycosylated proteins cannot be broken down because of enzyme deficiency). Neurological dysfunction can be a manifestation of these conditions due to substrate deposition as well. This review will explore the modalities of neuroimaging that may have particular relevance to the study of the lipid storage disorder and their impact on elucidating aspects of brain function. First, the techniques will be reviewed. Next, the neuropathology of a few selected lipid storage disorders will be reviewed and the use of neuroimaging to define disease characteristics discussed in further detail. Examples of studies using these techniques will be discussed in the text.

The prevalence and impact of restless legs syndrome on patients with iron deficiency anemia.

Restless Legs Syndrome (RLS) a common, under-recognized disorder disrupts sleep and diminishes quality of life. Despite a clear relation between low peripheral iron and increased prevalence and severity of RLS, the prevalence and clinical significance of RLS in iron-deficient anemic (IDA) populations is unknown. In this study all new patients referred for anemia to a community-based hematology practice over a 1-year period (March 2011-2012) were included if they had IDA and no RLS treatment. Patients completed a validated questionnaire identifying RLS, blood tests, and a sleep-vitality questionnaire (SVQ). Patients with RLS were compared to patients with no RLS for differences on SVQ, blood tests, baseline characteristics, and sleep quality. Three hundred forty-three patients were evaluated and 251 (89.2% female, average age of 45.6 years) included in the study. The prevalence of clinically significant RLS (RLS sufferers) was 23.9%, nine times higher than the general population. IDA-RLS sufferers reported poorer quality of sleep, decreased sleep time, increased tiredness, and decreased energy during the day compared to patients with IDA without RLS. Blood tests did not relate to RLS diagnosis but RLS was less likely for African-American than Caucasian patients. Clinically significant RLS occurs commonly with IDA producing much greater disruption of sleep and shorter sleep times than does IDA alone. This indicates the need for identification of RLS with IDA and consideration of appropriate therapeutic interventions for this sizeable subgroup: either aggressive iron treatment to reduce the RLS symptoms or medications for RLS or both.

Neurotransmitter abnormalities and response to supplementation in SPG11.

OBJECTIVE: To report the detection of secondary neurotransmitter abnormalities in a group of SPG11 patients and describe treatment with l-dopa/carbidopa and sapropterin. DESIGN: Case reports. SETTING: National Institutes of Health in the Undiagnosed Disease Program; Children's National Medical Center in the Myelin Disorders Bioregistry Program. PATIENTS: Four SPG11 patients with a clinical picture of progressive spastic paraparesis complicated by extrapyramidal symptoms and maculopathy. INTERVENTIONS: L-Dopa/carbidopa and sapropterin. RESULTS: 3/4 patients presented secondary neurotransmitter abnormalities; 4/4 partially responded to L-dopa as well as sapropterin. CONCLUSIONS: In the SPG11 patient with extrapyramidal symptoms, a trial of L-dopa/carbidopa and sapropterin and/or evaluation of cerebrospinal fluid neurotransmitters should be considered.