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Drug-induced hyperthermia with rhabdomyolysis in CLN3 disease
Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Region Västra Götaland, Sahlgrenska University Hospital, Department of Pediatrics, Gothenburg, Sweden.
Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Region Västra Götaland, Sahlgrenska University Hospital, Department of Pediatrics, Gothenburg, Sweden.
Örebro University, School of Medical Sciences. Centre for Clinical Research and Education, County Council of Värmland, Sweden.
Pediatric Clinic, Blekinge Hospital, Karlskrona, Sweden.
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2022 (English)In: European journal of paediatric neurology, ISSN 1090-3798, E-ISSN 1532-2130, Vol. 39, p. 74-78Article in journal (Refereed) Published
Abstract [en]

CLN3 disease (MIM# 204200), the most prevalent of the neuronal ceroid lipofuscinoses (NCL), is an autosomal recessive disorder with juvenile onset characterized by blindness, epilepsy, dementia, psychiatric manifestations, and motor deterioration. Problems related to behavior, emotions and thought are among the main features. Antidepressant and antipsychotic drugs have been employed with variable results. Neuroleptic malignant syndrome (NMS) has previously been described in two patients with NCL, one with CLN3 disease and one with adult onset NCL of unclear genetic origin. Our aims were to describe the occurrence of drug-induced hyperthermia in pediatric patients with CLN3 disease from West and South Sweden and to delineate the range of associated clinical features. Our study identified four patients presenting with seven episodes of severe drug-induced hyperthermia and either NMS-like or Serotonin syndrome (SS)-like features. Possibly provoking drugs were risperidone, clozapine, olanzapine, haloperidol, quetiapine, and sertraline. The course was atypical, frequently prolonged, associated with rhabdomyolysis and status dystonicus, and resulted in the death of three of the patients. Our study points to a vulnerability to drug-induced hyperthermia in patients with CLN3 disease which we believe could be underreported. Interestingly the proposed pathophysiological mechanisms behind NMS and SS on one hand and CLN3 on the other hand seem to converge in a common mechanism involving dysregulation of the sympathetic nervous system.

Place, publisher, year, edition, pages
Elsevier, 2022. Vol. 39, p. 74-78
Keywords [en]
CLN3, Juvenile neuronal ceroid lipofuscinosis, Neuroleptic malignant syndrome, Rhabdomyolysis, Serotonin syndrome
National Category
Neurology
Identifiers
URN: urn:nbn:se:oru:diva-99645DOI: 10.1016/j.ejpn.2022.06.007ISI: 000813006200003PubMedID: 35716526Scopus ID: 2-s2.0-85132515985OAI: oai:DiVA.org:oru-99645DiVA, id: diva2:1672522
Note

Funding agency:

Queen Silvia Children's Hospital Research Foundation ALFGBG-718681

Available from: 2022-06-20 Created: 2022-06-20 Last updated: 2022-08-18Bibliographically approved

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Wikström, Sverre

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