The prevalence of TTR cardiac amyloidosis among patients undergoing bone scintigraphy.

BACKGROUND: Radiolabeled bisphosphonates bone scintigraphy is highly sensitive in detecting transthyretin (TTR) cardiac amyloidosis; data on the true prevalence of cardiac involvement in TTR amyloidosis are lacking. METHODS AND RESULTS: This retrospective observational, monocentric study aims to estimate the prevalence of positive bone scan suspect for TTR cardiac amyloidosis among an all-comers population who underwent a bone scintigraphy. ECG, echocardiography and clinical status of patients with unexpected cardiac uptake (Perugini score 2-3) who underwent bone scintigraphy with [99mTc]-HDP or [99mTc]-DPD at San Luigi Gonzaga University Hospital between January 2015 and May 2020 have been collected. The prevalence of bone scintigraphy suspect for cardiac involvement was 0.54% (23/4,228). The bone scintigraphy was mainly performed using [99mTc]-HDP (82.9%) and the dominant indication for the test was oncology in the 47.9% of cases. 8 Subjects had a history of neuropathy (34.8%) and 5 of carpal tunnel syndrome (21.7%). 11 Patients suffered a previous episode of heart failure (48%) while 5 patients (21.7%) were totally asymptomatic, without any sign or symptom before the bone scintigraphy making the nuclear examination crucial for an early diagnosis of TTR amyloidosis. CONCLUSION: Bone scintigraphy allows suspecting TTR amyloidosis in a pre-clinical stage of the disease in an all-comers population of patients undergoing bone scintigraphy mainly for oncology reasons.

Use of the gonadotropin-releasing hormone (GnRH) stimulation test to monitor gonadal function in intact adult male cats.

The gonadotropin-releasing hormone (GnRH) stimulation test is a common procedure used to investigate normality of the pituitary-gonadal axis in mammals. There is very little information on the technique, its efficacy and side effects in small animals and in particular no information for male cats. In dogs, such test is performed by intravenous (IV) administration. With cats, the number of times the animal needs to be restrained for blood sampling should be the least possible. The purpose of this study was to assess efficacy and side effects of the GnRH stimulation test in tomcats comparing the IV with the intramuscular (IM) route of administration. A GnRH stimulation test was performed in eight adult tomcats through IM or IV administration of 50 µg gonadorelin. The response of the pituitary-gonadal axis was assessed by measuring serum testosterone on blood samples collected prior to and 1 hr following treatment. When considering each single group of cats, the post-stimulation serum testosterone values were significantly higher than the pre-treatment ones (p < .05). When comparing the two groups of cats, basal testosterone concentrations did not differ, and also post-GnRH testosterone concentrations did not differ. In conclusion, in the cats of our study, the GnRH stimulation test produced the same results following the IM or the IV route of administration. Therefore, in tomcats, the IM route can be considered as effective as the IV one and should be preferred when doing a GnRH test.

Proton and carbon NMR spectroscopic analysis of rat liver postnatal development.

BACKGROUND: In the present paper compositional changes of rat liver from birth to adult age were investigated by proton and carbon nuclear magnetic resonance (NMR) spectroscopy. METHODS: The water-soluble and organic (lipid) fraction of pooled liver tissue from rats aged 1,7, 21 days, and 5 months were extracted according to Folch's method and analysed by proton and carbon nuclear magnetic resonance spectroscopy at 500 MHz. Phospholipids separated by chromatography from the organic fraction were also analysed. RESULTS: In proton spectra of the water-soluble fraction several metabolites were identified. Developmental changes in the ratio between betaine and choline and between 3-hydroxybutyrate and several other organic acids were shown. In proton spectra of the total organic fraction, signals from fatty acids chain, phophocholine and glycerol were assigned unambiguously. Phospholipids fatty acid acyl chain length, mean unsaturation and mean polyunsaturation increased from birth to weaning. The relative amounts of unsaturated fatty acids did not show obvious changes during the investigated period. CONCLUSIONS: This study shows that NMR spectroscopy is a useful tool to investigate postnatal developmental changes in the liver chemical constituents with a minimum of preparative procedures.

Polyunsaturated fatty acids mapping by (1)H MR-chemical shift imaging.

Parametric mapping of polyunsaturated fatty acids (PUFA) distribution in adipose tissues was obtained by (1)H chemical shift imaging (CSI). A matrix of spectra, acquired with a CSI sequence having two spatial and one spectroscopic dimension, was processed with ad hoc algorithms. The protocol was applied to phantoms containing different lipids in which the degree of polyunsaturation was determined by high-resolution nuclear magnetic resonance (NMR). High correlation (R(2) = 0.998) between degrees of polyunsaturation given by our protocol and that measured by high-resolution NMR was found. The thoracic region of rats was also examined. Parametric maps of the polyunsaturation degree were obtained for the brown adipose tissue and the white axillary fat: the first deposit was found more polyunsaturated than the second. Finally, in vivo mapping of the inguinal region of the rat was produced that allowed us to individuate PUFA-rich areas in adipose tissue. This work demonstrates the feasibility of PUFA imaging in vivo.

NMR spectroscopic analysis of rat brain development: in vitro proton and carbon studies of whole tissue and its phospholipid fraction.

The developmental patterns of the rat brain at several postnatal time points were investigated in Folch (chloroform-methanol) extracts. The chloroform- (lipid-containing) and water-soluble (cytosolic) fractions of whole-tissue extracts and the phospholipid fraction separated from the organic fraction by chromatography were analysed by means of high-resolution (1)H and (13)C nuclear magnetic resonance (NMR) spectroscopy. Analysis of the cytosolic fraction showed the changing patterns of several brain metabolites during postnatal maturation, in full agreement with data obtained from perchloric acid extracts. (1)H NMR spectroscopy of the phospholipid fraction allowed for quantitative evaluation of fatty acid acyl chain length, mean unsaturation and mean polyunsaturation. It was found that both mean unsaturation and polyunsaturation are lower in adult brain phospholipids than during the first 3 postnatal weeks. (13)C NMR spectroscopy of the same fraction showed that the molar percentage of C(18) fatty acids (oleic, linoleic, linolenic) in brain phospholipids is similar at all the investigated time points. These results indicate that the combination of Folch extraction with simple chromatographic procedures and NMR analysis yields useful data to define the chemical maturation of the brain.

Transient global amnesia and migraine in young people.

Two cases of transient global amnesia (TGA) in a 16-year-old boy and in a 13-year-old girl are reported; both occurred during competitive sport and were associated with migraine. TGA in young people could provide crucial information on the still equivocal pathogenesis, a vascular thromboembolic hypothesis being untenable in such cases. A migrainous mechanism is likely to underlie TGA in young people and "pure' TGA in general. TIA or stroke, like epilepsy or other pathological conditions, should concern the differential diagnosis of transient amnestic episodes rather than the pathogenesis of TGA.

Dermatomal somatosensory evoked potentials in the diagnosis of lumbosacral radiculopathies.

The diagnostic sensitivity of dermatomal somatosensory evoked potentials (DSEPs) was evaluated in a homogeneous group of fifteen patients with low back pain due to isolated lumbosacral radiculopathy. The normative values from L3 to S1 were defined, as were the parameters of abnormality. In seven patients with pseudoradicular or referred leg pain, a negative neurological examination and images indicative of protrusion or paramedian disc hernia, the DSEPs were normal in six cases and slightly altered in one. In eight patients with deafferentation leg pain, clinical signs of root damage and images indicative of an intraforaminal hernia, the DSEPs showed varying degrees of alteration which could generally be correlated to the severity of the clinical picture. This method can therefore be useful in differentiating pain due to stimulation of the receptors of the osteoarticular structures or the root sheath, in which the DSEPs are normal, from pain due to direct root damage, in which the DSEPs are altered. This functional evaluation of anatomical damage provides further information which may help to achieve a better therapeutic approach.

Neurophysiological and urodynamic examinations in the functional assessment of the spinal cord below the injury site.

We examined the characteristics of specific components of the evoked electrospinogram (EESG) in response to tibial nerve stimulation in 28 patients with traumatic injury of the dorsal and cervical spinal cord. The data were correlated with clinical, urodynamic and additional neurophysiological findings. In the majority of patients (82%), 11 with complete and 12 with partial spinal cord lesions, the lumbar components of the EESG were normal. In 4 of these patients with complete lesion above T2 the dorsal EESG was absent. In 5 cases (18%), the lumbosacral EESG was altered in the presence of an atypical clinical syndrome characterized by persistent urinary retention associated with lower leg atrophy and reduced tendon jerks. In these cases, MRI provided evidence of an unexpected sacral lesion. Unlike the dorsal EESG reflecting the afferent dorsal column volley, the lumbar components of the EESG are usually unaffected by interruption of the cord pathways; these last events thus originate from segmental neuronal activity, the involvement of which bears witness to additional lumbosacral damage.

October 1942: a strange epidemic paralysis in Saval, Verona, Italy. Revision and diagnosis 50 years later of tri-ortho-cresyl phosphate poisoning.

In the autumn of 1942 a strange epidemic paralysis started in Saval, at that time a country area but now part of the city of Verona. The epidemic went on for several months and affected 41 people, all working as owners or labourers on the same farm. Some of the farm animals (chickens, horses, cattle, pigs) also became ill. About 20 patients were admitted to the nearby city hospital. The outbreak was diagnosed as polyneuritis with a probable viral cause. Fifty years later, seven people with sequelae of the disease were examined. The most severe cases present a spastic paraplegia and lower leg muscle atrophy without sensory impairment, resembling an amyotrophic lateral sclerosis "frozen" for 50 years. The clinical syndrome can now be attributed confidently to organophosphate induced delayed polyneuropathy. All the epidemiological data obtained from the survivors were evaluated and a careful review of the literature was made. Contamination of the ground from a rubbish dump near the farmyard would explain the epidemiology of the Saval outbreak.

Atypical syndromes caudal to the injury site in patients following spinal cord injury. A clinical, neurophysiological and MRI study.

Some patients with a cervical or a high thoracic spinal cord injury show atypical signs and symptoms such as atrophy of lower leg muscles, reduced tendon jerks or urinary retention, either persisting from the acute phase or developing in the course of time. A series of 35 patients was prospectively examined from this point of view with neurophysiological, urodynamic and MRI studies. Seven patients (20%) presented atypical findings; in two there was an isolated areflexic bladder, but no signs of functional or structural sacral damage were found. In five patients (14.2%) a definite cause for the sacral lesion could be detected. The syndrome caudal to the level of the lesion is neither obvious at the beginning nor immutable in time. Treatment requires to be adapted to the changing patterns of somatic and visceral activity.