New antidepressive and antipsychotic drugs in juvenile neuronal ceroid lipofuscinoses — a pilot study

Backman, Maria L; Åberg, L.; Aronen, Eeva T.; Santavuori, Pirkko

doi:10.1053/ejpn.2000.0455

Cited by 20 publications

(18 citation statements)

References 9 publications

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“…This perseverative style has been described previously in JNCL (Hofman et al 1999), but more work is needed to distinguish it from what is seen in many other conditions, including developmental delay, autism, and some psychiatric and neurological disorders (Bodfish et al 2000). Finally, some obsessive fears were apparently provoked by visual hallucinations, which have been previously reported in JNCL (Lanska and Lanska 1993, Santavuori et al 1993, Bäckman et al 2001. Cognitively impaired JNCL children may be unable to differentiate between frightening hallucinations and real events, and consequently may ruminate about these fearful perceptions, despite parental reassurances.…”

Section: Discussionmentioning

confidence: 51%

“…Behavioral and psychiatric problems in JNCL have also been described, including aggression, anxiety, depression, and hallucinations (Boustany and Filipek 1993, Lauronen et al 1999, Bäckman et al 2001. A partly retrospective survey of two JNCL groups reported many patients with aggression, mood disturbances, and hallucinations or delusions (Santavuori et al 1993).…”

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confidence: 99%

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Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis

Adams

Blieck

Mink

et al. 2006

Developmental Medicine &Amp; Child Neurology

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We obtained information about the behavioral, psychiatric, and functional status of 26 children (13 males, 13 females) with juvenile neuronal ceroid lipofuscinosis (JNCL; mean age 12y 3mo [SD 3y 4mo]; range 6y 9mo to 18y 8mo). Twenty-five children had visual impairment and 18 were known to have a positive seizure history before enrollment. Parents completed the Child Behavior Checklist, Scales of Independent BehaviorRevised, and a structured interview to assess obsessivecompulsive symptoms. Participants exhibited a broad range of behavioral and psychiatric problems, rated as occurring frequently and/or as severe in more than half of the sample. Males and females did not differ with regard to the number of behavioral and psychiatric problems. Children were also limited in their ability to perform activities of daily living, including self-care, hygiene, socialization, and other age-appropriate tasks. Results provide a quantitative baseline for behavioral and psychiatric problems and functional level in JNCL, against which further decline can be measured. Longitudinal assessment of behavioral and psychiatric symptoms and functional abilities is continuing and will provide much-needed data on the natural history of JNCL.

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Section: Discussionmentioning

confidence: 51%

mentioning

confidence: 99%

Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis

Adams

Blieck

Mink

et al. 2006

Developmental Medicine &Amp; Child Neurology

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“…Psychotic, affective, and schizophreniform features are managed with citalopram, risperidone, olanzapine, or quetiapine. 9 Reports of decreased D1 dopamine transporter density in JNCL by PET, 1, 80 along with similar findings in one of the Cln3 −/− mouse models, 95 suggest that JNCL patients may have a dysregulation of the dopamine system, offering a rationale for a trial of dopaminergic medications. 3 …”

Section: Treatmentmentioning

confidence: 92%

Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) and the Eye

Bozorg

Ramirez-Montealegre

Chung

et al. 2009

Survey of Ophthalmology

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Juvenile neuronal ceroid lipofuscinoses (JNCL) or Batten disease is the most common type of NCL in the United States and Europe. This devastating disorder presents with vision failure and progresses to include seizures, motor dysfunction, and dementia. Death usually occurs in the third decade, but some patients die before age twenty. Though the mechanism of visual failure remains poorly understood, recent advances in molecular genetics have improved diagnostic testing and suggested possible therapeutic strategies. The ophthalmologist plays a crucial role in both early diagnosis and documentation of progression of JNCL. We update Batten disease research, particularly as it relates to the eye, and present various theories on the pathophysiology of retinal degeneration.

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“…While families wait for disease-modifying treatment, we must also consider interventions that could mitigate the everyday impact of dementia-related symptoms. Pharmacologic treatments have been evaluated; 14 Finnish children with juvenile Batten disease were placed on specific antidepressant or antipsychotic medications; approximately 70% were deemed to have a “good or satisfactory” response 40 . However, conclusions are limited in this study which was not randomized — all children received medication — and clinical outcome was determined qualitatively by parents and treating clinicians in an unblinded fashion.…”

Section: Looking Forward – Challenges and Opportunitiesmentioning

confidence: 99%

Neurobehavioral Features and Natural History of Juvenile Neuronal Ceroid Lipofuscinosis (Batten Disease)

Adams

Mink

2013

J Child Neurol

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Juvenile neuronal ceroid lipofuscinosis is a childhood-onset neurodegenerative disease with prominent symptoms comprising a pediatric dementia syndrome: intellectual decline, mood and behavioral impairments, and loss of adaptive skills. We review the history of neurobehavioral features in juvenile neuronal ceroid lipofuscinosis and the work of the University of Rochester Batten Center to characterize the extent and progression of neurobehavioral symptoms over disease course, and discuss the relevance of neurobehavioral studies as an aid to understanding the clinical phenotype of juvenile Batten disease and potential targets for intervention.

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New antidepressive and antipsychotic drugs in juvenile neuronal ceroid lipofuscinoses — a pilot study

Cited by 20 publications

References 9 publications

Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis

Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis

Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) and the Eye

Neurobehavioral Features and Natural History of Juvenile Neuronal Ceroid Lipofuscinosis (Batten Disease)

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