2003
DOI: 10.1023/a:1025916518457
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Neuroleptic malignant syndrome in juvenile neuronal ceroid lipofuscinosis associated with low‐dose risperidone therapy

Abstract: We present a patient with juvenile neuronal ceroid lipofuscinosis who developed a neuroleptic malignant syndrome when treated for hallucinations with a very low dose of risperidone, an atypical neuroleptic medication with usually few extrapyramidal side-effects. The loss of dopaminergic neurons in this condition may make these patients more vulnerable to this severe adverse effect.

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Cited by 11 publications
(4 citation statements)
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“…While three of them developed NMS-like features (#2-4), one patient presented with predominantly SS-like features (#1). To our knowledge, NMS-like features have only been described in one previous patient with CLN3 disease [16] while there has been no prior description of SS-like features or other descriptions of rhabdomyolysis in CLN3 disease.…”
Section: Discussionmentioning
confidence: 91%
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“…While three of them developed NMS-like features (#2-4), one patient presented with predominantly SS-like features (#1). To our knowledge, NMS-like features have only been described in one previous patient with CLN3 disease [16] while there has been no prior description of SS-like features or other descriptions of rhabdomyolysis in CLN3 disease.…”
Section: Discussionmentioning
confidence: 91%
“…Another theory to explain the pathophysiological mechanisms behind NMS is the dopaminergic hypothesis given the relationship between NMS and dopamine agonistic drugs [19]. In comparison, adolescent patients with CLN3 have a pronounced loss of dopaminergic neurons [20] and it has been suggested that such a vulnerable dopaminergic system could predispose them to extrapyramidal side-effects including NMS [16]. A third possible cause of NMS could be a link to calcium-mediated disruption of the musculoskeletal system similar to malignant hyperthermia [21].…”
Section: Discussionmentioning
confidence: 99%
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“…Delusions, as well as visual and auditory hallucinations, have been reported . The association of psychosis with dementia is common, and a propensity toward neuroleptic malignant syndrome has been reported …”
Section: Resultsmentioning
confidence: 99%