ABSTRACT
Camptocormia is an abnormal posture with marked flexion of thoracolumbar spine that abates in the recumbent position. Camptocormia has been described in various neurological (Parkinsonism), muscular (myopathy), psychogenic or orthopedic disorders. There are several hypotheses that can explain this impaired posture but they are usually related to the concomitant pathologies. We report the first case of a patient with confirmed myotonic dystrophy addressed to our medical center for impaired posture who underwent extensive medical exams and explorations because of a myotonic hand. Axial weakness and muscle atrophy, validated by CT-scan imaging, are discussed independent of the concomitant pathology (Parkinson, myopathy).
Subject(s)
Back Pain/physiopathology , Muscle Weakness/physiopathology , Muscular Atrophy/physiopathology , Myotonic Dystrophy/physiopathology , Postural Balance/physiology , Spinal Diseases/physiopathology , Back Pain/etiology , Diagnosis, Differential , Disability Evaluation , Female , Hand/pathology , Hand/physiopathology , Humans , Middle Aged , Muscle Weakness/etiology , Muscle, Skeletal/pathology , Muscle, Skeletal/physiopathology , Muscular Atrophy/etiology , Muscular Atrophy/pathology , Myotonic Dystrophy/complications , Myotonic Dystrophy/pathology , Spinal Diseases/etiology , Spinal Diseases/pathologySubject(s)
Aortic Diseases/complications , Atherosclerosis/complications , Echocardiography, Transesophageal , Embolism/etiology , Intracranial Embolism/etiology , Magnetic Resonance Imaging , Toes/blood supply , Aged , Aortic Diseases/diagnostic imaging , Atherosclerosis/diagnostic imaging , Cerebral Infarction/diagnosis , Cerebral Infarction/etiology , Embolism/pathology , Humans , Ischemia/etiology , Ischemia/pathology , MaleABSTRACT
In aged humans, diminished mineralocorticoid receptor (MR)-mediated feedback in the brain could contribute to impaired feedback regulation of the hypothalamic-pituitary-adrenal (HPA) axis, but no study specifically compared young and old individuals with regard to MR function. We examined 10 healthy young (mean age +/- SD [standard deviation] 26.1 +/- 2.9 years) and 10 elderly men (68.3 +/- 4.7 years) at the nadir of cortisol levels (2:00 pm-9:00 pm) when HPA activity is mainly controlled by the MR. After pretreatment with 3 g metyrapone to minimize the impact of basal endogenous cortisol secretion, participants received orally, in randomized order on two separate occasions, either 0.5 mg of the MR agonist fludrocortisone or placebo. Fludrocortisone significantly decreased maximum adrenocorticotropic hormone (ACTH) and cortisol concentrations in both groups. ACTH and cortisol values after fludrocortisone were significantly higher in older men compared with young men. Our results implicate that a decrease in MR-mediated negative feedback contributes to the diminished feedback activity in older humans.
Subject(s)
Fludrocortisone/pharmacology , Hypothalamo-Hypophyseal System/drug effects , Mineralocorticoids/pharmacology , Pituitary-Adrenal System/drug effects , Receptors, Mineralocorticoid/agonists , Adrenocorticotropic Hormone/blood , Adult , Aged , Aged, 80 and over , Analysis of Variance , Area Under Curve , Humans , Hydrocortisone/blood , Hypothalamo-Hypophyseal System/physiology , Male , Metyrapone/administration & dosage , Middle Aged , Pituitary-Adrenal System/physiologyABSTRACT
Whereas animal studies have shown a clear inhibitory effect of hippocampal mineralocorticoid receptors (MR) on hypothalamic-pituitary-adrenal (HPA) axis activity, investigations in humans revealed equivocal results. To further clarify the influence of MR in HPA activity we studied 10 healthy men during the circadian nadir of HPA activity (14:00 to 21:00) after pre-treatment with 3 g metyrapone to minimize the impact of basal endogenous cortisol secretion. On three separate occasions, in a placebo-controlled design, subjects received in a randomized order either 0.5 mg fludrocortisone p.o. or 0.2 mg aldosterone i.v. or placebo. Fludrocortisone exerted a significant inhibition of ACTH, cortisol and 11-desoxycortisol (p < 0.05), whereas no such effect was observed after aldosterone or placebo. These preliminary data suggest that MR are involved in the inhibition of the HPA axis during the circadian nadir of glucocorticoid concentrations in humans.
