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  • Atypical forms  (1)
  • Drug induced movement disorder  (1)
  • Generalized dystonia  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 227 (1982), S. 239-247 
    ISSN: 1432-1459
    Keywords: Focal dystonia ; Segmental dystonia ; Generalized dystonia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Fünfzehn von idiopathischer — und zwar 7 von generalisierter und 8 von fokaler und segmentarischer — Dystonie befallenen Patienten unterzogen sich verschiedenen pharmakologischen Behandlungen mit kleinen Mengen Bromocriptine, Pymozide und Triesifenidile. Die Symptome wurden durch eine fortlaufende Punktzahl bezeichnet, so daß deren Schätzung von der Intensität des Symptoms „Dystonie“ in jedem einzelnen befallenen Körperteil abhing. Die Dystonien wurden durch Bromocriptine nicht bedeutend geändert. Pymozide führte zu einer geringeren, doch unbedeutenden, Besserung der dystonischen Symptome. Triesifenidile wirkte auf die generalisierten Dystonien, in Übereinstimmung mit einigen Literaturangaben. Die Veränderlichkeit der pharmakologischen Ergebnisse wurde auf die Verschiedenheit der dystonischen Syndrome zurückgeführt, unter denen man Fälle versammelt, die sich durch Anfangsalter, Sitz der dystonischen Symptome und Entwicklungsart voneinander unterscheiden.
    Notes: Summary A total of 15 patients affected by idiopathic dystonia (7 with generalized and 8 with focal or segmental dystonia) were subjected to therapy with bromocriptine at low doses, pimozide and trihexyphenidyl. The symptoms were evaluated by giving a progressive score in relation to the intensity of the dystonic symptom to each of the body segments involved by the dystonia. Bromocriptine did not significantly modify the dystonia. Pimozide showed a slight nonsignificant improvement of the dystonic symptoms. Trihexyphenidyl was effective in the generalized dystonias, in agreement with previous reports in the literature. The variation in the pharmacological results could be due to the diversity of the dystonic syndromes, which comprise cases that are different in age at onset, site of dystonic symptoms, and evolution.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neurological sciences 17 (1996), S. 157-159 
    ISSN: 1590-3478
    Keywords: Transient paroxysmal dystonia ; Cisapride ; Drug induced movement disorder ; Infancy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Presentiamo il caso di una bambina di 5 mesi, trattata sin dalla nascita con cisapride per la prevenzione di reflusso gastroesofageo, in quanto affetta da atresia congenita dell'esofago corretta chirurgicamente in 1° giornata di vita. Dal 3° mese la bambina presentò, con frequenza ingravescente, distonie accessuali non più verificatesi dalla sospensione del trattamento farmacologico. La precocità di comparsa della fenomenologia accessuale, la relazione temporale tra cessazione della stessa e sospensione del farmaco, nonché alcune caratteristiche semeiologiche sottolineate nel lavoro, portano ad attribuire alla cisapride un ruolo causale. I processi di sviluppo all'interno dello striato propri di questa età della vita, che comportano rapidi rimaneggiamenti strutturali e di equilibrio funzionale tra i principali neurotrasmettitori, hanno sicuramente rappresentato una situazione favorente nei confronti di un disturbo extrapiramidale transitorio.
    Notes: Abstract The case is reported of an infant presenting paroxysmal dystonia during cisapride theraphy. We suggest that this drug, a substituted benzamide, probably interfered with the age-related modification of striatal neurotransmitters, provoking extrapyramidal symptoms. Considering the widespread use of cisapride in early infancy for the treatment of gastrointestinal disorders, attention must be drawn to this possible side effect.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Neurological sciences 21 (2000), S. S57 
    ISSN: 1590-3478
    Keywords: Key words NCL ; Atypical forms
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The neuronal ceroid lipofuscinoses (NCL) are progressive neurodegenerative diseases occurring in infancy and adulthood. Atypical forms of these diseases have been described and are particularly represented in the late-infantile and juvenile onset groups. Recent progress in biochemistry and molecular genetics has identified some of these variants as separate disease entities while disclosing the phenotypic variability of some classic forms. We report the result of a retrospective analysis performed on a series of 27 NCL patients, 15 of which were atypical as to clinical and/or pathological findings. Most of such patients, belonging to the late-infantile onset group and displaying homogeneous clinical-pathological features, were suggestive for CLN6. The two atypical juvenile NCL patients had features which resembled the “protracted form” of the disease. Given their relative frequency, strict clinical and pathological criteria are still the most useful tools for identifying and characterizing atypical forms and for defining phenotype-genotype correlations.
    Type of Medium: Electronic Resource
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