Diabetic Lumbosacral Radiculoplexus Neuropathy (Diabetic Amyotrophy).

Diabetic lumbosacral radiculoplexus neuropathy, also known as diabetic amyotrophy, has a characteristic course of sudden onset of unilateral pain in the thigh and hip, which may spread to the other side in weeks to months and proceeds with progressive lower extremity weakness, often resulting in the inability to walk unassisted. The syndrome is typically monophasic, and most patients will recover at least to some degree. Less typical features include lack of pain, distal predominant weakness, absence of diabetes, and upper extremity involvement. This article provides a series of interesting cases to highlight the diagnostic challenges and discusses management decision making.

Magnetic resonance imaging correlates with electrical impedance myography in facioscapulohumeral muscular dystrophy.

INTRODUCTION: Electrical impedance myography (EIM) has been proposed as a noninvasive biomarker of muscle composition in facioscapulohumeral muscular dystrophy (FSHD). Here we determine the associations of EIM variables with muscle structure measured by MRI. METHODS: We evaluated 20 patients with FSHD at two centers, comparing EIM measurements (resistance, reactance, and phase at 50, 100, and 211 kHZ) recorded from bilateral vastus lateralis, tibialis anterior, and medial gastrocnemius muscles to MRI skin and subcutaneous fat thickness, MRI T1-based muscle severity score (T1 muscle score), and MRI quantitative intramuscular Dixon fat fraction (FF). RESULTS: While reactance and phase both correlated with FF and T1 muscle score, 50 kHz reactance was most sensitive to muscle structure alterations measured by both T1 score (ρ = -0.71, P < .001) and FF (ρ = -0.74, P < .001). DISCUSSION: This study establishes the correlation of EIM with structural MRI features in FSHD and supports further evaluation of EIM as a potential biomarker in FSHD clinical trials.

Satisfactory Response With Achieving Maintenance Low-Dose Prednisone in Generalized Myasthenia Gravis.

OBJECTIVES: To estimate the satisfactory response rate (SR%) with achieving maintenance, low-dose prednisone in acetylcholine receptor antibody-positive generalized myasthenia gravis. METHODS: In this retrospective study, we estimate the SR% as defined by (remission/minimal manifestations status for at least 6 months using 7.5 mg or less of prednisone daily, for maintenance treatment at 2, 4, and 6 years after symptoms onset) for patients who were not taking steroid-sparing immunosuppressant (SSI) as a primary outcome and for patients taking an SSI as a secondary outcome. RESULTS: Forty-five patients were not taking an SSI at 2 years, 34 patients at 4 years, and 17 patients at 6 years; SR% was 44.4%, 64.7%, and 58.8%, respectively. Thirty-six patients were taking an SSI at 2 years, 22 patients at 4 years, and 15 patients at 6 years; the SR% was 50.0%, 45.4%, and 66.7%, respectively. CONCLUSIONS: Nearly half of the generalized myasthenia gravis patients who were not taking an SSI achieved an SR.

Late-onset, praxis-induced myoclonic epilepsy.

Praxis-induction of seizures is an interesting subset of reflex epilepsy in which seizures are induced by higher mental activities associated with the use of part of the body. Reflex traits have often been described in patients with juvenile myoclonic epilepsy. We report a patient presenting with praxis-induced myoclonic epilepsy at a late age. Ictal myoclonus was triggered by building a bird house and captured by video-polygraphic EEG recording. At 39 years old, the patient's age at onset of epilepsy was consistent with the syndrome of adult myoclonic epilepsy. Our case supports the notion of adult myoclonic epilepsy with possible occurrence of praxis-activation of seizures, as has been noted with the other idiopathic generalised epilepsies. [Published with videosequences].

Plant protein intake is associated with fibroblast growth factor 23 and serum bicarbonate levels in patients with chronic kidney disease: the Chronic Renal Insufficiency Cohort study.

BACKGROUND: Protein from plant, as opposed to animal, sources may be preferred in chronic kidney disease (CKD) because of the lower bioavailability of phosphate and lower nonvolatile acid load. STUDY DESIGN: Observational cross-sectional study. SETTING AND PARTICIPANTS: A total of 2,938 participants with CKD and information on their dietary intake at the baseline visit in the Chronic Renal Insufficiency Cohort Study. PREDICTORS: Percentage of total protein intake from plant sources (percent plant protein) was determined by scoring individual food items using the National Cancer Institute Diet History Questionnaire (DHQ). OUTCOMES: Metabolic parameters, including serum phosphate, bicarbonate (HCO3), potassium, and albumin, plasma fibroblast growth factor 23 (FGF-23), and parathyroid hormone (PTH), and hemoglobin levels. MEASUREMENTS: We modeled the association between percent plant protein and metabolic parameters using linear regression. Models were adjusted for age, sex, race, diabetes status, body mass index, estimated glomerular filtration rate, income, smoking status, total energy intake, total protein intake, 24-hour urinary sodium concentration, use of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, and use of diuretics. RESULTS: Higher percent plant protein was associated with lower FGF-23 (P = .05) and higher HCO3 (P = .01) levels, but not with serum phosphate or parathyroid hormone concentrations (P = .9 and P = .5, respectively). Higher percent plant protein was not associated with higher serum potassium (P = .2), lower serum albumin (P = .2), or lower hemoglobin (P = .3) levels. The associations of percent plant protein with FGF-23 and HCO3 levels did not differ by diabetes status, sex, race, CKD stage (2/3 vs. 4/5), or total protein intake (≤0.8 g/kg/day vs. >0.8 g/kg/day; P-interaction >.10 for each). LIMITATIONS: This is a cross-sectional study; determination of percent plant protein using the Diet History Questionnaire has not been validated. CONCLUSIONS: Consumption of a higher percentage of protein from plant sources may lower FGF-23 and raise HCO3 levels in patients with CKD.

Thinning of the arterial media layer as a possible preclinical stage in HIV vasculopathy: a pilot study.

BACKGROUND AND PURPOSE: The purpose of this study was to determine if postmortem intracranial arteries from donors with HIV without stroke have thinner media layers compared with patients without HIV and without stroke. METHODS: Cross-sectional cuts from intracranial arteries were stained with van Gieson and hematoxylin and eosin. Arteries were examined for thickness of each arterial layer. Univariable and multivariable models were used for statistical analyses with probability values <0.05 considered significant. RESULTS: A total of 18 brains were analyzed, 5 with HIV and 13 without. Fifty-five arteries were collected, 15 from HIV brains and 40 from unaffected controls. In univariable analysis, in arteries from HIV-infected brains, the media to wall thickness ratio was smaller than in donors without HIV (0.496 versus 0.563, P=0.017). In multivariable analysis, HIV infection was the only independent predictor of smaller media ratios compared with the same-aged control subjects (P=0.049) but not with aged control subjects (P=0.081). CONCLUSIONS: In patients with HIV without clinical stroke, the media arterial layer is thinner than in patients without HIV. This suggests that a thinner media layer might be a preclinical stage in the development of HIV-related vasculopathy.

Correlates of osteoprotegerin and association with aortic pulse wave velocity in patients with chronic kidney disease.

BACKGROUND AND OBJECTIVES: Osteoprotegerin (OPG), a cytokine that regulates bone resorption, has been implicated in the process of vascular calcification and stiffness. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Serum OPG was measured in 351 participants with chronic kidney disease (CKD) from one site of the Chronic Renal Insufficiency Cohort Study. Cortical bone mineral content (BMC) was measured by quantitative computed tomography in the tibia. Multivariable linear regression was used to test the association between serum OPG and traditional cardiovascular risk factors, measures of abnormal bone and mineral metabolism, and pulse wave velocity. RESULTS: Higher serum OPG levels were associated with older age, female gender, greater systolic BP, lower estimated GFR, and lower serum albumin. OPG was not associated with measures of abnormal bone or mineral metabolism including serum phosphorus, albumin-corrected serum calcium, intact parathyroid hormone, bone-specific alkaline phosphatase, or cortical BMC. Among 226 participants with concurrent aortic pulse wave velocity measurements, increasing tertiles of serum OPG were associated with higher aortic pulse wave velocity after adjustment for demographics, traditional vascular risk factors, and nontraditional risk factors such as estimated GFR, albuminuria, serum phosphate, corrected serum calcium, presence of secondary hyperparathyroidism, serum albumin, and C-reactive protein or after additional adjustment for cortical BMC in a subset (n = 161). CONCLUSIONS: These data support a strong relationship between serum OPG and arterial stiffness independent of many potential confounders including traditional cardiovascular risk factors, abnormal bone and mineral metabolism, and inflammation.

Relationship of estimated GFR and coronary artery calcification in the CRIC (Chronic Renal Insufficiency Cohort) Study.

BACKGROUND: Coronary artery calcification (CAC) is associated with increased mortality risk in the general population. Although individuals with chronic kidney disease (CKD) are at markedly increased mortality risk, the incidence, prevalence, and prognosis of CAC in CKD are not well understood. STUDY DESIGN: Cross-sectional observational study. SETTING & PARTICIPANTS: Analysis of 1,908 participants who underwent coronary calcium scanning as part of the multiethnic CRIC (Chronic Renal Insufficiency Cohort) Study. PREDICTOR: Estimated glomerular filtration rate (eGFR) computed using the Modification of Diet in Renal Disease (MDRD) Study equation, stratified by race, sex, and diabetic status. eGFR was treated as a continuous and a categorical variable compared with the reference value of >60 mL/min/1.73 m(2). MEASUREMENTS: CAC detected using computed tomography (CT) using either an Imatron C-300 electron beam computed tomography (CT) scanner or multidetector CT scanner. CAC was computed using Agatston score as a categorical variable. Analyses were performed using ordinal logistic regression. RESULTS: We found a strong and graded relationship between lower eGFR and increasing CAC. In unadjusted models, ORs increased from 1.68 (95% CI, 1.23-2.31) for eGFR of 50-59 mL/min/1.73 m(2) to 2.82 (95% CI, 2.06-3.85) for eGFR <30 mL/min/1.73 m(2). Multivariable adjustment only partially attenuated the results (OR, 1.53; 95% CI, 1.07-2.20) for eGFR <30 mL/min/1.73 m(2). LIMITATIONS: Use of eGFR rather than measured GFR. CONCLUSIONS: We showed a graded relationship between severity of CKD and CAC independent of traditional risk factors. These findings support recent guidelines that state that if vascular calcification is present, it should be considered as a complementary component to be included in the decision making required for individualizing CKD treatment.