236 research outputs found

    Striatal and nigral neuron subpopulations in rigid Huntington's disease: Implications for the functional anatomy of chorea and rigidity-akinesia

    Full text link
    Neuropeptide immunohistochemistry was used to test several hypotheses of the anatomical bases of chorea and rigidity-akinesia. To test the hypothesis that elevated concentration of striatal somatostatin causes chorea, we visually compared the density of striatal neurons containing somatostatin and neuropeptide Y in brains affected by choreic or rigid-akinetic Huntington's disease (HD). The density of these neurons was elevated in both rigid-akinetic and choreic HD specimens with an apparently normal total number of these neurons, indicating that elevated somatostatin concentration, by itself, does not lead to chorea. We tested the hypothesis that rigid-akinetic HD results from deficient dopaminergic nigrostriatal neurotransmission by examining tyrosine hydroxylase–immunoreactive (TH-IR) neurons in the substantia nigra. In rigid-akinetic HD brains, there was no obvious reduction of nigral TH-IR neurons, indicating that rigid-akinetic HD is probably not due to loss of nigral dopaminergic neurons. Finally, we also examined the status of striatal projection neurons and found near total loss of all striatal neurons projecting to the lateral globus pallidus, medial globus pallidus, and substantia nigra in brains affected by rigid-akinetic HD in contrast to the preservation of neurons projecting to the medial globus pallidus in choreic HD. These results are consistent with the hypothesis that chorea results from preferential loss of striatal neurons projecting to the lateral globus pallidus and that rigid-akinetic HD is a consequence of the additional loss of striatal neurons projecting to the medial segment of the pallidum.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/50333/1/410270403_ftp.pd

    The history of written language disorders: reexamining Pitres’ case (1884) of pure agraphia

    Get PDF
    The first clinical description of pure agraphia was reported by the French neurologist Pitres in 1884. Pitres used the case study evidence to argue for modality-specific memory representations and the localization of writing. This article reviews Pitres’s contribution to the study of acquired writing disorders, the components of writing models and the cerebral localization which subserve writing, in light of the views entertained by his contemporaries and current authors. Although numerous cases have been reported throughout this century, the view that writing can be impaired while other language functions and motor activities remain intact is still challenged

    Ocjena radne sposobnosti pacijenta s Wilsonovom bolesti - prikaz bolesnika

    Get PDF
    Wilson’s disease (WD) is a rare, progressive autosomal recessive disorder characterised by impaired transport and excessive accumulation of copper in the liver, brain, and other tissues. The disease is diagnosed based on clinical manifestations and screening tests results. Work ability assessment of patients with WD is based on the analysis of liver, kidney, neurological, and cognitive impairments, and takes into account patient’s level of education. This article presents a case with a 48-year-old male patient, who was admitted for work ability assessment due to polymorphic symptoms. The patient had been working as a salesman for 28 years. A detailed interview and examination by occupational health and other medical specialists revealed that the patient had been suffering from Wilson’s disease from the age of 13, and had now developed hepatic manifestations (compensated liver cirrhosis with portal hypertension), neurological manifestations (dystonia, dysarthria, muscle weakness, vertigo), and psychiatric manifestations (depression, insomnia, cognitive impairment) of the disease, including problems partially caused by long-lasting treatment with copper chelating agents (neurological and haematological manifestations). There were no ocular manifestations of Wilson’s disease (Kayser-Fleischer rings or sunflower cataract). The patient was assessed as having drastically diminished general work ability, dominantly due to neurological and psychiatric impairments caused by Wilson’s disease.Wilsonova je bolest rijetka, progresivna autosomno recesivna bolest karakterizirana poremećajem transporta bakra i posljedičnim prekomjernim nakupljanjem bakra u jetri, mozgu i drugim tkivima i organima. Dijagnoza bolesti postavlja se na osnovi kliničkih manifestacija bolesti i nalaza laboratorijskih ispitivanja. Ocjena radne sposobnosti pacijenata s Wilsonovom bolesti zasniva se na analizi postojanja oĆĄtećenja i stupnja oĆĄtećenja hepatičkih, neuroloĆĄkih, bubreĆŸnih i kognitivnih funkcija, kao i na analizi stupnja obrazovanja pacijenata. Prikazan je slučaj D. M., 48-godiĆĄnjeg pacijenta, koji je primljen zbog polimorfnih tegoba na bolničko ispitivanje radi ocjene radne sposobnosti. Pacijent je radio kao prodavač posljednjih 28 godina. Nakon detaljne anamneze i pregleda koje su obavili specijalisti medicine rada i drugi specijalisti utvrđeno je da pacijent boluje od Wilsonove bolesti od 13. godine ĆŸivota i da u ovom trenutku ima izraĆŸene hepatične manifestacije (kompenzirana ciroza jetre s portalnom hipertenzijom), neuroloĆĄke manifestacije (distonija, dizartrija, miĆĄićna slabost, vrtoglavica) i psihijatrijske manifestacije (depresija, nesanica, kognitivno oĆĄtećenje) Wilsonove bolesti, kao i da su prisutne tegobe djelomično uzrokovane dugotrajnom upotrebom kelatne terapije (neuroloĆĄki i hematoloĆĄki poremećaji). Nisu uočene karakteristične očne promjene Wilsonove bolesti (Kayser-Fleischerov prsten, katarakta u obliku suncokreta). Ocjenom radne sposobnosti utvrđeno je da pacijent ima drastično smanjenu radnu sposobnost preteĆŸno zbog neuroloĆĄkih i psihičkih poremećaja u sklopu Wilsonove bolesti

    Safety of ultrasonography in pregnancy : WHO systematic review of the literature and meta-analysis

    Get PDF
    Objective In the context of the planned International Society of Ultrasound in Obstetrics and Gynecology- World Health Organization multicenter study for the development of fetal growth standards for international application, we conducted a systematic review and meta-analysis to evaluate the safety of human exposure to ultrasonography in pregnancy. Methods A systematic search of electronic databases, reference lists and unpublished literature was conducted for trials and observational studies that assessed short- and long-term effects of exposure to ultrasonography, involving women and their fetuses exposed to ultrasonography, using B-mode or Doppler sonography during any period of pregnancy, for any number of times. The outcome measures were: (1) adverse maternal outcome; (2) adverse perinatal outcome; (3) abnormal childhood growth and neurological development; (4) non-right handedness; (5) childhood malignancy; and (6) intellectual performance and mental disease. Results The electronic search identified 6716 citations, and 19 were identified from secondary sources. A total of 61 publications reporting data from 41 different studies were included: 16 controlled trials, 13 cohort and 12 case-control studies. Ultrasonography in pregnancy was not associated with adverse maternal or perinatal outcome, impaired physical or neurological development, increased risk for malignancy in childhood, subnormal intellectual performance or mental diseases. According to the available clinical trials, there was a weak association between exposure to ultrasonography and non-right handedness in boys (odds ratio 1.26; 95% CI, 1.03-1.54). Conclusion According to the available evidence, exposure to diagnostic ultrasonography during pregnancy appears to be safe.publishersversionPeer reviewe
    • 

    corecore