Juvenile Parkinsonism Treated With Levodopa

Abstract

We describe three patients with early onset of parkinsonism. Two of these were sisters and showed a reduction in homovanillic acid (HVA), 5-hydroxytryptamine (5-HT), and 5-hydroxyindole acetic acid (5-HIAA) levels in the CSF. In the third patient, parkinsonism developed, which was probably related to chickenpox encephalitis. All three patients responed favorably to levodopa therapy.

Progressive dystonia with marked diurnal fluctuation

“…The CSF HVA concentration in dystonia musculorum deformans has been reported to be normal or low [8]. Sachdev et a1 [30] have recently reported 2 female siblings with juvenile parkinsonism whose CSF contained no measurable HVA or SHIAA. It is not clear from the report whether the CSF was obtained by routine lumbar puncture or at pneumoencephalography.…”

Parkinsonismo juvenil: considerações a respeito de 10 casos

“…Segundo Jellinger, os dois primeiros poderiam ser incluídos no grupo das atrofias palidais. Alguns autores, como Martin e col. 8 e Sachdev e col. 10 descrevem, sob a denominação de parkinson juvenil, casos de síndromes parkinsonianas de tipo pós-encefalítico instaladas em pacientes jovens. O critério diagnóstico por nós adotado é fundamentalmente clínico, e o. casos aqui incluídos têm como denominador comum uma síndrome parkinsoniana instalada antes dos trinta anos.…”

Juvenile Parkinsonism: a term in search of an identity

“…However, if one drops the requirement for pathological verification of diagnosis, it is not as difficult to find reports of relatively pure Parkinsonism in juveniles. Interestingly, more than half of these patients have positive family histories (Mjones, 1949; Martin et al ., 1971; Sachdev et al ., 1977; Carlier and Dubru, 1979; Narabayashi et al ., 1986; Quinn et al ., 1987; Ishikawa and Miyatake, 1995; Schrag et al ., 1998), raising the question as to whether they may harbor a Parkin mutation. Indeed, even in those individuals without a family history (a total of about 20 such cases have been reported) (Naidu et al ., 1978; Clough et al ., 1981; Gershanik and Leist, 1986; Narabayashi et al ., 1986; Muthane et al ., 1994; Schrag et al ., 1998) a Parkin disorder cannot be ruled out since, as it is an autosomal recessive disorder, not all affected individuals will have an obvious family history in any case.…”