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At least 1,603 records · Page 89Linked to original sources
PubMed2006

Longitudinal study of bone and calcium metabolism and fracture incidence in spinocerebellar degeneration.

Little is known about bone and calcium metabolism and fracture incidence in spinocerebellar degeneration (SCD) despite frequent falls and immobilization. To address bone and calcium metabolism and fracture incidence in SCD, we conducted a 10-year prospective study in a cohort of adult patients with SCD. Bone mineral density (BMD) and serum levels of ionized calcium, parathyroid hormone, 25-hydroxyvitamin D, and pyridinoline cross-linked carboxy-terminal telopeptide of type I collagen (ICTP) were followed in 110 patients with SCD for 10 years. Age-matched healthy volunteers (n = 110) served as controls. At baseline, the SCD patients had a low BMD with high levels of serum ionized calcium and ICTP which correlated with the degree of immobilization (Barthel index). Over 10 years, serum 25-hydroxyvitamin D decreased to the osteomalacic level (<5 ng/ml), and calcium and ICTP further increased in accordance with a decreased Barthel index score. The BMD decreased by 15.2% in men and by 24.6% in women. The incidence of fractures in the patients was significantly higher as compared with the control group (men 8/49 vs. 1/42, p = 0.0428; women 16/49 vs. 2/48, p = 0.0026). Over 10 years, the BMD was significantly reduced in the SCD patients, particularly in women, which increased the risk of a fracture. Vitamin D deficiency due to sunlight deprivation, increased bone resorption due to immobilization, and frequent falls are probable causes of osteoporosis and fractures in these patients. Hypovitaminosis D and increased bone resorption may be corrected readily by the routine use of vitamin D supplements together with bisphosphonate.

Aged↗
PubMedDate not supplied

Vocal fold paresis in Shy-Drager syndrome.

Twelve patients with Shy-Drager syndrome (SDS) presenting symptoms of multiple nervous system atrophy and orthostatic hypotension were examined for laryngeal movement disorders and vocal impairment in speech. Vocal fold abductor paresis was found in 11 patients and was bilateral in 10. Speech task performance was recorded in SDS patients, Parkinson patients and age- and sex-matched controls. Trained listeners with inter-rated reliability greater than or equal to .85 judged each recording on 20 attributes while blind to speaker identity. SDS patients had a breathy and strained voice quality, reduced loudness, monopitch and monoloudness, imprecise consonants, variations in rate and rate-slowing, suggesting a flaccid type of dysarthria. In comparison with Parkinson patients, SDS patients had excess vocal hoarseness, intermittent glottal fry and a slow and deliberate speaking rate. Orthostatic hypotension, laryngeal stridor, hoarseness, intermittent glottal fry and slow speech rate were found to be discriminating symptoms of SDS.

Aged↗
PubMed2003

Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA/NOMID, MWS, and FCU.

NALP proteins are recently identified members of the CATERPILLER (CARD, transcription enhancer, R(purine)-binding, pyrin, lots of LRR) family of proteins, thought to function in apoptotic and inflammatory signaling pathways. Mutations in the CIAS1 gene, which encodes a member of the NALP (NACHT-, LRR-, and PYD-containing proteins) family, the cryopyrin/NALP3/PYPAF1 protein, expressed primarily in phagocytic cells, were recently found to be associated with a spectrum of autoinflammatory disorders. These include chronic infantile neurologic cutaneous and articular (CINCA) syndrome (also known as neonatal-onset multisystem inflammatory disease [NOMID]), Muckle-Wells syndrome (MWS), and familial cold urticaria (FCU). We describe herein 7 new mutations in 13 unrelated patients with CINCA syndrome and identify mutational hotspots in CIAS1 on the basis of all mutations described to date. We also provide evidence of genotype/phenotype correlations. A 3-dimensional model of the nucleotide-binding domain (NBD) of cryopyrin suggested that this molecule is structurally and functionally similar to members of the AAA+ protein family of ATPases. According to this model, most of the mutations known to affect residues of the NBD are clustered on one side of this domain in a region predicted to participate in intermolecular contacts, suggesting that this model is likely to be biologically relevant and that defects in nucleotide binding, nucleotide hydrolysis, or protein oligomerization may lead to the functional dysregulation of cryopyrin in the MWS, FCU, and CINCA/NOMID disorders.

Adolescent↗
PubMed2003

Anti-ganglioside antibodies in idiopathic and hereditary cerebellar degeneration.

Cerebellar degeneration has been associated with gluten sensitivity and celiac disease. Patients with celiac disease may have neuropathy and antibodies to gangliosides. The authors investigated the presence of antiganglioside antibodies in 22 patients with hereditary and nonhereditary ataxia and found 64% reactive in a novel agglutination test.

Aged↗
PubMed2006

Voglibose inhibits postprandial hypotension in neurologic disorders and elderly people.

The authors examined blood pressure, glucose, insulin, and neurotensin before and after intake of 75 g glucose with or without voglibose in 28 neurologic patients and 20 healthy controls. Voglibose significantly prevented hypotension and neurotensin increment after glucose intake and had no influence on glucose or insulin increment. These results suggest that voglibose benefits postprandial hypotension.

Adult↗
PubMed1999

The treatment of neurogenic orthostatic hypotension with 3,4-DL-threo-dihydroxyphenylserine: a randomized, placebo-controlled, crossover trial.

OBJECTIVE: To study the therapeutic effect and mechanism of action of 3,4-DL-threodihydroxyphenylserine (DL-DOPS) in neurogenic orthostatic hypotension. METHODS: The blood pressure (BP) response to an orthostatic challenge on DL-DOPS was compared with that of placebo in a randomized, double-blind, placebo-controlled, crossover trial in 10 patients. The mechanism of action of DOPS was studied by measuring forearm vascular resistance and changes in supine and upright plasma DL-DOPS and norepinephrine levels. The effect of DL-DOPS on the quality of life was determined by questionnaire. RESULTS: DL-DOPS increased the supine (p<0.001) and upright (p<0.05) systolic blood pressure (SBP) and diastolic blood pressure (DBP) (both p<0.01). The peak SBP on DL-DOPS in the supine position occurred 300 minutes after ingestion of the medication. The increase in BP was accompanied by an increase in plasma levels of norepinephrine and DL-DOPS in both the supine and upright positions after DL-DOPS ingestion (p<0.0001). There was a trend toward improvement in symptoms of orthostatic intolerance. CONCLUSION: DL-DOPS improved features of neurogenic orthostatic hypotension in patients with central and peripheral autonomic nervous system disease. There was an increase in plasma norepinephrine. No major side effects occurred.

Adult↗
PubMed2002

Brain aconitase activity is not decreased in progressive supranuclear palsy.

The novel finding of decreased activity of aconitase, a key Krebs cycle enzyme highly sensitive to oxidative damage, in cybrid cell lines using mitochondrial DNA from patients with progressive supranuclear palsy (PSP) implies an enzyme abnormality in brain. However, the authors found that postmortem brain aconitase activity is normal in PSP. This suggests that patients with PSP do not have systemic aconitase deficiency and that data derived from cybrid cell models of neurodegenerative disorders might not always predict similar changes in human brain.

Aconitate Hydratase↗
PubMed2000

Carbon disulfide.

Explore the source record for details and available documents.

Carbon Disulfide↗
PubMed1996

Putaminal Iron Deposition in HIV Infection.

PURPOSE: T2 shortening (hypointensity) in magnetic resonance (MR) images of the putamen, which may be associated with iron deposition, only occurs in normal subjects over the age of 60 years. Increased or premature putaminal iron deposition may be related to brain injury. We sought to determine the correlation between MR putaminal hypointensity in HIV-infected patients and brain iron deposition. METHODS: Eleven T2-weighted axial MR scans were retrospectively rated for the extent of putaminal hypointensity from patients who also had neuropathological examination for the extent of putaminal iron disposition. Correlations between MR putaminal hypointensity and brain iron were obtained. RESULTS: Neuropathological examination in 9 of 10 patients with putaminal hypointensity demonstrated putaminal iron deposition, predominantly in a perivascular pattern. CONCLUSIONS: Premature putaminal iron deposition occurs in patients with HIV infection and may be detected by MR imaging.

HIV Infections↗