Increased Vertebral Gallium-68-DOTATATE Activity on Positron Emission Tomography-Computed Tomography in Merkel Cell Carcinoma: Not Always Metastasis.

Merkel cell carcinoma (MCC) is a rare neuroendocrine dermal malignancy seen in elderly light-skinned individuals, associated with immunosuppression and Merkel cell polyomavirus infection. As a neuroendocrine tumor, the recurrence and metastasis of MCC can be evaluated using positron emission tomography-computed tomography (PET-CT) with the Gallium-68-DOTATATE (Ga-68-DOTATATE) radiotracer, which has demonstrated increased sensitivity to neuroendocrine metastases when compared to F-18 fluorodeoxyglucose (FDG). Here, we present the case of a patient with known metastatic MCC with a new, abnormal focus of increased radiotracer activity in the thoracic spine on Ga-68-DOTATATE PET-CT suspected to represent a metastatic lesion. Further evaluation with MRI revealed a benign vertebral hemangioma, highlighting the limitations of this radiotracer in the setting of benign spinal lesions. Multimodality imaging findings of metastatic MCC and potential pitfalls of Ga-68-DOTATATE PET-CT staging are discussed.

MRI features associated with rapid disease activity in clinically isolated syndrome patients at high risk for multiple sclerosis.

Clinically isolated syndrome (CIS) is a central nervous system inflammatory and demyelinating event that lasts at least 24 h and can represent the first episode of relapsing-remitting multiple sclerosis. MRI is an important imaging tool in the diagnosis and longitudinal monitoring of CIS progression. Accurate differential diagnosis of high-risk versus low-risk CIS is important because high-risk CIS patients could be treated early. Although a few studies have previously characterized CIS and explored possible imaging predictors of CIS conversion to MS, it remains unclear which amongst the commonly measured MRI features, if any, are good predictors of rapid disease progression in CIS patients. The goal of this review paper is to identify MRI features in high-risk CIS patients that are associated with rapid disease activity within 5 years as measured by clinical disability.

MRI features associated with high likelihood of conversion of radiologically isolated syndrome to multiple sclerosis.

Radiologically isolated syndrome (RIS) is the asymptomatic precursor to clinically isolated syndrome, relapsing-remitting multiple sclerosis (MS) or primary progressive MS. RIS is frequently diagnosed when an individual gets an MRI for an unrelated medical issue, such as headache or trauma. Treating RIS patients is controversial, but physicians may be inclined to offer prophylactic treatment for high-risk RIS patients. Identifying imaging and clinical features associated with high likelihood of early clinical conversion may prove helpful to identify a high-risk subset for potential MS therapy. The goal of this paper is to review current literatures to identify imaging and clinical features that predict early (within 5 years) conversion from RIS to MS.

Ectopic craniopharyngioma of the fourth ventricle in a patient with Gardner syndrome.

Ectopic craniopharyngioma is uncommon and a craniopharyngioma confined purely within the fourth ventricle is extremely rare. We report a craniopharyngioma of the fourth ventricle in a 20-year-old man with Gardner syndrome. Imaging characteristics of craniopharyngiomas and fourth ventricle lesions are discussed with a review of the literature regarding the pathogenesis of craniopharyngiomas and the possible association with Gardner syndrome.

Report from a quality assurance program on patients undergoing the MILD procedure.

OBJECTIVES: To characterize trends in pain and functional outcomes and identify risk factors in patients with lumbar spinal stenosis (LSS) and neurogenic claudication undergoing the "Minimally Invasive Lumbar Decompression" (MILD) procedure. DESIGN: Retrospective observational cohort study. SETTING: Academic multidisciplinary pain center at Stony Brook Medicine. SUBJECTS: Patients undergoing the MILD procedure from October 2010 to November 2012. METHODS: De-identified perioperative, pain and function related data for 50 patients undergoing MILD were extracted from the Center for Pain Management's quality assessment database. Data included numerical rating scale (NRS), symptom severity and physical function (Zurich Claudication Questionnaire), functional status (Oswestry Disability Index [ODI]), pain interference scores (National Institutes of Health Patient-Reported Outcomes Measurement Information System [PROMIS]), and patients' self-reported low back and lower extremity pain distribution. RESULTS: No MILD patient incurred procedure-related complications. Average NRS scores decreased postoperatively and 64.3% of patients reported less pain at 3 months. Clinically meaningful functional ODI improvements of at least 20% from baseline were present in 25% of the patients at 6 months. Preliminary analysis of changes in PROMIS scores at 3 months revealed that pre-MILD "severe" lumbar canal stenosis may be associated with high risk of "no improvement." No such impact was observed for NRS or ODI outcomes. CONCLUSION: Overall, pain is reduced and functional status improved in LSS patients following the MILD procedure at 3 and 6 months. Given the small sample size, it is not yet possible to identify patient subgroups at risk for "no improvement." Continued follow-up of longer-term outcomes appears warranted to develop evidence-based patient selection criteria.

High-dose cyclophosphamide for moderate to severe refractory multiple sclerosis: 2-year follow-up (investigational new drug No. 65863).

High-dose cyclophosphamide (HDC) is a chemotherapy treatment designed to eradicate autoreative B- and T-cells responsible for lymphocyte-mediated autoimmune illness while sparing the pluripotent blood stem cell of any ill effects. Multiple sclerosis (MS) is the most common inflammatory and demyelinating immune-mediated disorder of the central nervous system in young adults. Patients with moderate to severe, refractory MS, defined as an Expanded Disability Status Scale (EDSS) score of 3.5 or higher after two or more Food and Drug Administration-approved disease-modifying agents, received 200 mg/kg of cyclophosphamide over 4 days. For the next 2 years, quarterly EDSS score evaluations and biannual brain magnetic resonance imaging and neuro-ophthalmologic evaluations were obtained. Fifteen patients were evaluated for clinical response. During follow-up, one patient increased their baseline EDSS score by 1.0. EDSS score stability of decrease was realized in five of seven (71%) patients with relapsing-remitting MS and six of eight (75%) patients with secondary progressive MS. Four patients required additional immunomodulatory treatment after treatment. Treatment response was seen regardless of the baseline presence or absence of contrast lesion activity. HDC can effectively decrease symptoms, stop disease progression, and allow for disability regression in relapsing-remitting MS and secondary progressive MS patients. The most appropriate candidates for HDC, its duration of benefit, and the potential need for prophylactic preventative immune manipulation after HDC all require further investigation.

Reversible pituitary hyperplasia at birth in a macrosomic full-term baby boy.

Pituitary hyperplasia is generally associated with end-organ failure such as primary hypothyroidism, physiological changes such as puberty and pregnancy, or neoplasms secreting releasing factors. We present a full-term infant with an enlarged pituitary height of 8 mm at age 3 days despite a normal endocrinological evaluation. Repeat imaging at 5 months of age revealed a normal-size pituitary gland. To our knowledge, pituitary hyperplasia has not been described in a neonate with normal pituitary function.

Parietal lobe epileptic focus identified on SPECT-MRI fusion imaging in a case of epilepsia partialis continua.

Epilepsia partialis continua, or "Kozhevnikov syndrome," is a rare condition characterized by persistent localized motor seizures usually localized in a distant limb. It is most often seen in children under 16 years old without gender preference. We report a 12-year-old girl with epilepsia partialis continua who presented to the emergency room after the onset of seizures. Routine electroencephalography (EEG) was performed with no clear ictal localization. Magnetic resonance imaging (MRI) was unremarkable. Ictal single photon emission computed tomography (SPECT) images with Tc-99m exametazime fused with MRI images revealed a parietal lobe epileptic focus within the superior segment of the postcentral gyrus. This case illustrates a rare disorder with an unusual epileptic localization identified by SPECT-MRI fusion.

High-dose cyclophosphamide for moderate to severe refractory multiple sclerosis.

BACKGROUND: High-dose cyclophosphamide is active in immune-mediated illnesses. OBJECTIVE: To describe the effects of high-dose cyclophosphamide on severe refractory multiple sclerosis. DESIGN, SETTING, AND PATIENTS: Patients with multiple sclerosis with an Expanded Disability Status Scale (EDSS) score of 3.5 or higher after 2 or more Food and Drug Administration-approved disease-modifying therapy regimens were evaluated. INTERVENTIONS: Patients received 200 mg/kg of cyclophosphamide over 4 days. MAIN OUTCOME MEASURES: Patients had brain magnetic resonance imaging and neuro-ophthalmologic evaluations every 6 months and quarterly EDSS and quality-of-life evaluations for 2 years. RESULTS: Twelve patients were evaluated for clinical response (median follow-up, 15.0 months; follow-up range, 6-24 months). During follow-up, no patients increased their baseline EDSS scores by more than 1.0. Five patients decreased their EDSS scores by 1.0 or more (EDSS score decrease range, 1.0-5.0). Two of 11 patients had a single enhancing lesion at baseline; these lesions resolved after high-dose cyclophosphamide treatment. At 12 months, 1 patient showed 1 new enhancing lesion without a corresponding high-intensity T2-weighted or fluid-attenuated inversion recovery signal. Patients reported improvement in all of the quality-of-life parameters measured. Neurologic improvement involved changes in gait, bladder control, and visual function. Treatment response was seen regardless of the baseline presence or absence of contrast lesion activity. Patient quality-of-life improvement occurred independently of EDSS score changes. In this small group of patients with treatment-refractory multiple sclerosis, high-dose cyclophosphamide was associated with minimal morbidity and improved clinical outcomes. CONCLUSIONS: High-dose cyclophosphamide treatment in patients with severe refractory multiple sclerosis can result in disease stabilization, improved functionality, and improved quality of life. Further studies are necessary to determine the most appropriate patients for this treatment.