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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-four selected low-risk preterm and 10 full-term infants were followed in a prospective study of the evolution of neurological signs in the first year of life and to consider the prognostic value of transient neurological abnormalities in relation to learning disabilities at school age. We report the results of the assessments made during the first 12 months. The assessment at 3, 6, 9 and 12 months was made using the Amiel-Tison neurological schedule and the Griffiths developmental scale. A high percentage of our low-risk premature infants (69% of 37-34 weeks gestational age and 75% of 33-27 weeks gestational age group) showed transient neurological anomalies, whereas no major neurological sequelae, i.e., cerebral palsies or mental impairment, were found. Moreover, in our series, the syndrome of transient dystonia associated with low birth weight, already described by other authors in samples not selected for risk, was not observed. The psychological development of all our infants was within normal limits at every assessment. Nevertheless, a significantly decreased performance at the sixth month in both preterm groups, particularly in the group of low gestational age, was found, and its significance will be clarified by further follow-up studies of early school age.
Ital J Neurol Sci 1983 Sep
PMID:Neurological and psychological development of low-risk preterm infants in the first year of life. 619 19

A prospective study was undertaken of the outcome at 1 year in 129 preterm infants of less than 34 weeks gestation (range 27 to 34 weeks) who underwent detailed neurologic assessment and ultrasound scanning in the neonatal period and again at 40 weeks postmenstrual age, and an independent neurodevelopmental assessment at 12 months chronologic age. Of the 129 infants, 37 (29%) had ultrasound evidence of periventricular hemorrhage. At 40 weeks postmenstrual age the infants were classified as neurologically normal, abnormal, or borderline on the basis of the neurologic examination. Of the 62 infants considered normal at 40 weeks, 57 (91%) were assessed as normal at one year, compared to only 14 (35%) of the 39 infants considered abnormal (P less than 0.001). Ten (85%) of the 12 normal infants with associated periventricular hemorrhage were normal at 1 year, compared to 47 (94%) of the 50 normal infants without periventricular hemorrhage, whereas 5 (25%) of 20 abnormal infants with associated periventricular hemorrhage and 9 (47%) of the 19 without periventricular hemorrhage were normal at 1 year. There was no direct correlation in individual cases between the severity of neurologic deficit and the presence or severity of periventricular hemorrhage. Infants with a cluster of abnormal signs were more likely to have later dystonia or cerebral palsy than those with marked hypotonia but no other abnormality.
J Pediatr 1984 Sep
PMID:Correlation of neurologic assessment in the preterm newborn infant with outcome at 1 year. 647 Aug 69

We examined 61-year-old identical twin women of Jewish extraction with a probable autosomal recessive form of torsion dystonia. The dystonia in each was relatively mild and discovered only because a young relative developed dystonia. The twins were said to be discordant for dystonia, but personal evaluation led to the diagnosis of dystonia in both. Their slow course, with prolonged spontaneous remission in one twin, is in contrast to that described in most published reports. Although similar in mode of onset and initial course, the twins were dissimilar in age at onset, influence of pregnancy, diurnal variation in symptoms, need for medication, later course, and degree of disability at age 61. Normal plasma levels of norepinephrine and dopamine-beta-hydroxylase are consistent with autosomal recessive hereditary torsion dystonia. The importance of personal evaluation of key family members in establishing the correct genetic basis for a heterogeneous group of disorders, such as the hereditary dystonias, is stressed.
Ann Neurol 1984 Sep
PMID:Dystonia in 61-year-old identical twins: observations over 45 years. 648 39

The activity of nerve growth factor (NGF) in the salivary glands and in the sciatic nerve was compared between normal mice and mice affected by either of three neurological mutations by the use of a biological assay. No evidence was obtained for defects in amount or activity of NGF associated with the sprawling or splotch mutations. A reduction in the NGF content was found in salivary glands and sciatic nerve in homozygous dystonia musculorum mice. It is pointed out that the low amounts of NGF in dtJ/dtJ mice is likely to be a consequence of the general disturbances in development seen in this mutant rather than the specific cause for the neurological disorder.
Neurosci Lett 1984 Sep 07
PMID:Nerve growth factor in three neurologically deficient mouse mutants. 649 18

The patient we describe had cerebellar ataxia, slow eye movements, myoclonus, facial dystonia and signs of spinal cord and peripheral nerve involvement. The patient's mother, brother and sister died from the same disease. Neuropathological examination revealed lesions of olivo-ponto-cerebellar atrophy (OPCA) associated with spinal cord degenerative changes characteristic of Menzel's hereditary ataxia. Although myoclonus was similar to Hunt's dyssynergia cerebellaris myonica, pathological findings did not show significant involvement of the dentate nucleus or superior cerebellar peduncle and physiopathological hypotheses for myoclonus are discussed. Slow eye movement is emphasized in the propositus and we suggest that it could be specific of one type of OPCA. Its pathological significance is discussed, but a primitive and unique involvement of the paramedian pontine reticular formation is unlikely.
J Neurol Sci 1983 Sep
PMID:Menzel's hereditary ataxia with slow eye movements and myoclonus. A clinico-pathological study. 663 53

We studied six patients with clinical and radiographic evidence of rostral brainstem lesion and bilateral blepharospasm. Two patients also had other facial dystonic movements. Four patients suffered rostral brainstem strokes, and two had multiple sclerosis. None had been treated with antipsychotic drugs prior to the onset of blepharospasm. Medical treatment was helpful in two patients, and bilateral selective facial nerve section was used in another patient. Possible pathogenic mechanisms are discussed.
Neurology 1983 Sep
PMID:Blepharospasm associated with brainstem lesions. 668 64

We have examined 29 subjects with writers' cramp (and 4 with typists' and one with pianists' cramp) and have noted two major groupings, simple and dystonic. We have observed spread from one to the other. We have seen repeatedly, in patients with isolated simple writers' cramp certain subtle physical signs which are found also in other basal ganglia diseases. We have noted also the frequent association of other features of segmental and generalized dystonia in patients with dystonic writers' cramp. We have demonstrated that patients with isolated writers' cramp have no higher an incidence of psychiatric disturbance, as judged by formal Present State Examination, than the normal population. We conclude that isolated writers' cramp is a physical illness rather than a psychological disturbance, and that it is a focal dystonia.
Brain 1982 Sep
PMID:Writers' cramp-a focal dystonia. 710 63

Eight cases of persistent dystonia appearing one to 14 years after non-progressive cerebral insults are described. Five were due to perinatal anoxia, one to trauma, and two to cerebral infarction. This phenomemon of delayed-onset dystonia has not been described previously, although review of earlier literature reveals several probable examples. Delayed-onset dystonia due to perinatal anoxia is an important diagnostic alternative to dystonia musculorum deformans for dystonia occurring in childhood.
J Neurol Neurosurg Psychiatry 1980 Sep
PMID:Delayed-onset dystonia in patients with "static" encephalopathy. 719 39

Appearance of dystonic movements in four epileptics children, while carbamazepine administration, is the object of this publication. Human material is formed by four patients in pediatric ages of 4.5, 5, 6 and 7 years old, which presented dystonia after the administration of carbamazepine. Clinical, neuroradiological, psychometric and bioelectric explorations were performed, also serum determinations of anticonvulsant levels. Three of the children presented serious psychomotor retardation. The electroencephalograms showed no uniform changes. Serum levels of carbamazepine were in therapeutics limits. From this personal experience and bibliography data it is deduced that dystonia is not a toxic phenomena, establishing the possibility of an interaction between carbamazepine and dopaminergic system.
An Esp Pediatr 1980 Sep
PMID:[Dystonia and carbamazepine (author's transl)]. 746 96

There may be insufficient awareness of dopa responsive dystonia (DRD), which has a characteristic diurnal variation of symptoms. Two children are reported in whom the diagnosis of DRD was missed. The first was thought to have hysteria and the second hereditary spastic paraparesis. A full history is vital for the diagnosis of this important treatable syndrome.
Arch Dis Child 1995 Sep
PMID:Dopa responsive dystonia. 749 70


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