Lipoprotein(a) Testing Trends in a Large Academic Health System in the United States.

Background Despite its high prevalence and clinical significance, clinical measurement of lipoprotein(a) is rare but has not been systematically quantified. We assessed the prevalence of lipoprotein(a) testing overall, in those with various cardiovascular disease (CVD) conditions and in those undergoing cardiac testing across 6 academic medical centers associated with the University of California, in total and by year from 2012 to 2021. Methods and Results In this observational study, data from the University of California Health Data Warehouse on the number of individuals with unique lipoprotein(a) testing, unique CVD diagnoses (using International Classification of Diseases, Tenth Revision [ICD-10], codes), and other unique cardiac testing were collected. The proportion of total individuals, the proportion of individuals with a given CVD diagnosis, and the proportion of individuals with a given cardiac test and lipoprotein(a) testing any time during the study period were calculated. From 2012 to 2021, there were 5 553 654 unique adults evaluated in the University of California health system, of whom 18 972 (0.3%) had lipoprotein(a) testing. In general, those with lipoprotein(a) testing were more likely to be older, men, and White race, with a greater burden of CVD. Lipoprotein(a) testing was performed in 6469 individuals with ischemic heart disease (2.9%), 836 with aortic stenosis (3.1%), 4623 with family history of CVD (3.3%), 1202 with stroke (1.7%), and 612 with coronary artery calcification (6.1%). For most conditions, the prevalence of testing in the same year as the diagnosis of CVD was relatively stable, with a small upward trend over time. Lipoprotein(a) testing was performed in 10 753 individuals (1.8%) who had lipid panels, with higher rates with more specialized testing, including coronary computed tomography angiography (6.8%) and apolipoprotein B (63.0%). Conclusions Lipoprotein(a) testing persists at low rates, even among those with diagnosed CVD, and remained relatively stable over the study period.

Clinical-radiological-pathological correlation of cavernous sinus hemangioma: Incremental value of diffusion-weighted imaging.

AIM: To elucidate the clinical, magnetic resonance imaging (MRI), pathological features of these lesions and asses the incremental value of diffusion-weighted imaging (DWI) in diagnosing them. METHODS: Fifteen consecutive patients (11 females and 4 males; mean age 40.93 years; age range 13-63 years) with cavernous sinus hemangiomas (CSH) who underwent examination between November 2008 and May 2016 were included for the analysis. MRI, clinical and surgical findings of each patient was retrospectively reviewed. DWI were also analysed and mean-apparent diffusion coefficient (ADC) value was calculated. Eleven patients underwent surgical removal of the lesion and 2 patients had biopsy only. Diagnosis of CSH was confirmed histologically in 13 patients. RESULTS: Eleven patients (73%) presented with headaches and 10 (66%) had cranial nerve involvement. Extra cavernous sinus extension was noted in 14 (94%). Surgery was performed in 13 (87%) and post-operative radiation was given to 4 (28%) patients. Thirteen patients remained asymptomatic on follow up. Three conspicuous imaging features were highly suggestive of the diagnosis: Lack of diffusion restriction (100%), homogeneous hyperintensity on T2 weighted image sequences (93.3%) and intense post-contrast enhancement (100%). The mean ADC was 1.82 × 10-3 ± 0.2186 cm2/s. CONCLUSION: T1-weighted hypointensity with homogeneous hyperintensity on T2-weighted sequences, intense enhancement and absence of hemosiderin within the lesion on GRE sequence favour the diagnosis. Facilitated diffusion on DWI differentiates CSH from other solid cavernous sinus lesions and significantly improves the diagnostic accuracy, a critical factor for planning surgery.

Acute multifocal neuropathy following cocaine inhalation.

INTRODUCTION: We report a young man, not a habitual cocaine user, who developed an acute multifocal neuropathy following a second exposure to inhaled cocaine. METHODS: Case report. RESULTS: Clinical and electrophysiological findings suggested an acute multiple mononeuropathy following cocaine exposure. Imaging of the shoulder and pelvic girdles revealed multifocal denervation in selected proximal muscles. The patient was empirically treated with intravenous steroids to good effect. DISCUSSION: Cocaine use, although usually affecting the central nervous system, does produce peripheral nerve disease in rare instances. This unusual pattern of neurological involvement needs to be differentiated from the more common symptoms resulting from affection of the brain.

Adult lead encephalopathy.

The neurotoxicity of lead to the adult brain is less well-known than that seen in children. It may present as acute severe encephalopathy or as chronic cognitive and behavioural symptoms, either in isolation or with systemic features of lead intoxication. Magnetic resonance imaging findings in lead encephalopathy vary. The pathogenesis of lead encephalopathy and its effects on cognition both during development and in the adult are discussed. That encephalopathic symptoms do not correlate with blood lead levels and that they may persist after chelation are highlighted. As an illustration, we describe a patient with chronic cumulative lead intoxication, who presented with peripheral neuropathy, anaemia, and a 'lead line' on the gingiva. She had cognitive dysfunction with extensive subcortical and cerebellar white matter lesions on magnetic resonance imaging. An area of restricted diffusion in both frontal regions is likely to be due to active ongoing demyelinating at the 'leading edge' of the lesion. Although systemic features and the peripheral neuropathy improved with chelation, the encephalopathy showed only marginal change, with later appearance of a symmetric akinetic-rigid state.

Delayed chorea after recovery from a symmetric parkinsonian syndrome due to striatal myelinolysis.

Extrapontine myelinolysis (EPM) is rare and usually results from osmotic stress due to inappropriate correction of electrolyte disturbances. Parkinsonism is a relatively common manifestation of EPM, seen in 60% of patients. Dopaminergic therapy leads to good recovery in many patients. Delayed extrapyramidal manifestations, including hyperkinetic movement disorders such as chorea or dystonia, are postulated to result from aberrant neuronal regeneration or denervation supersensitivity following the initial osmotic insult. We present a patient with a symmetric parkinsonian syndrome due to EPM involving the striatum who made a good recovery with levodopa therapy, only to develop chorea five months later. The possible mechanisms underlying the genesis of these movement disorders in EPM is discussed.

More often striatal myelinolysis than pontine? A consecutive series of patients with osmotic demyelination syndrome.

INTRODUCTION: Osmotic demyelination syndrome (ODS) is increasingly recognized to involve extrapontine locations in addition to the better-known central pontine myelinolysis. AIMS: This study describes clinical and radiological features of consecutive patients with ODS detected over 1 year. METHODS: Patients fulfilling clinical and radiological criteria for ODS were prospectively enrolled and were followed with serial assessments during hospital stay and up to 253 days after discharge. RESULTS: Eight patients (five females) aged 24-89 years were enrolled, comprising 0·06% of all admissions to the general medical and neurology services. All had preceding hyponatraemia and hypokalaemia of various aetiologies. One patient developed ODS after postpartum pituitary haemorrhage, which has been reported only rarely. Sodium levels were corrected at maximal rates exceeding 8 mmol/l/day. Neurological symptoms attributed to ODS began 3-15 days after clinical improvement following sodium correction in four patients; the remainder did not show any intervening lucent interval. Fifty per cent were stuporous at admission, 50% had seizures, 62·5% had symmetric parkinsonism, and 75% had prominent primitive reflexes. Stretch reflexes were absent in 25% and normal or brisk in the remainder. Magnetic resonance imaging (MRI) showed symmetric striatal lesions in all patients, with concomitant pontine involvement in only 25%. Four patients had poor outcomes (modified Rankin score >3), with prognosis dependent on the presence of severe systemic illness, liver dysfunction, encephalopathy, seizures, and degree of disability upon discharge from hospital. Patients with parkinsonism responded to dopaminergic therapy, with chorea, dystonia, and depression as later developments. This series is remarkable for the high incidence of extrapontine lesions, much more common than pontine involvement.