Creutzfeldt-Jakob Disease May Present Early With Unusual Bulbar Predominance.

INTRODUCTION: Creutzfeldt-Jakob disease (CJD) is a prion protein disorder of significant consequence and currently incurable. Diagnosis can be challenging early in the disease course. CJD can present in many ways but often fits a pattern of cognitive problems, cerebellar disturbance, behavioral/psychological changes, and perhaps myoclonus. CASE REPORT: We herein present the case of a 69-year-old White male with subacute progressive bulbar and limb weakness over ten weeks period. Early on, he was diagnosed with amyotrophic lateral sclerosis versus autoimmune-related bulbar neuropathy and treated as such. However, he continued to deteriorate clinically that prompted another admission, upon readmission, his cerebrospinal fluid RTQuick and 14-3-3 from the National Prion Disease Pathology Surveillance Center (NPDPSC) did eventually return positive. Hence he was diagnosed with CJD. CONCLUSIONS: CJD may present with progressive bulbar symptoms similar to acute inflammatory demyelinating polyradiculoneuropathy (MF variant), motor neuron disease, or autoimmune brainstem encephalitis. It becomes even higher on the differentials especially with no response to immunotherapy.

Variation in the Phototherapy Practices and Irradiance of Devices in a Major Metropolitan Area.

BACKGROUND: Phototherapy (PT) is widely used to prevent and treat severe hyperbilirubinemia and its associated risks for both acute and chronic bilirubin encephalopathy. Intensive PT, recommended for inpatient treatment of hyperbilirubinemia in term and near-term infants, is defined as having a spectral irradiance of ≥30 µW/cm2/nm. OBJECTIVES: We aimed to assess local PT practices by measuring the irradiance of PT devices in local neonatal intensive care units and newborn nurseries. METHODS: The irradiance footprint, including maximum irradiance at the center of the footprint, of 39 PT devices in 7 area hospitals was measured according to current practice in these facilities. RESULTS: The mean ± SD (range) footprint irradiance was 20.7 ± 5.8 (8.8-29.4) µW/cm2/nm. The mean ± SD maximum irradiance at the footprint center for all devices at a mean clinically used treatment distance of 33.1 ± 9.3 (25.5-60.0) cm was 27.8 ± 7.0 (14.7-42.0) µW/cm2/nm. Sixty-two percent of the devices did not meet the minimum recommended spectral irradiance for intensive PT. For the sites without irradiance-based protocols, the maximum irradiance of the devices (n = 33) at the treatment distances was 25.8 ± 6.1 µW/cm2/nm. CONCLUSIONS: Despite established PT guidelines, local protocols and practices vary. Based on an assessment of 7 local hospitals, intensive PT was suboptimal for 62% of devices. Straightforward changes, such as decreasing the distance between an infant and the light source and establishing a consistent irradiance-based protocol, could substantially improve the quality of the intervention.