title: A casuistic rationale for the treatment of spastic and myocloni in a childhood neurodegenerative disease: neuronal ceroid lipofuscinosis of the type Jansky-Bielschowsky.
abstract: The casuistic of a child suffering from late infantile neuronal ceroid lipofuscinosis (NCL) of the type Jansky-Bielschowsky aims to provide a description of possible therapeutic options for the severe spastic and the debilitating myocloni that occur within the context of this disorder. Moreover, it also should include a discussion of potential indications for the application of delta 9-Tetrahydrocannabinol (THC) (Dronabinol, Marinol) in childhood.
authors.affiliation: Brunnenstrasse 54, D-34537 Bad Wildungen, Germany. Ruelor@t-online.de
authors.forename: Ruediger
authors.initials: R
authors.lastname: Lorenz
mesh_terms: D018712:Analgesics, Non-Narcotic / Q000627:therapeutic use; D014151:Anti-Anxiety Agents / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D000975:Antioxidants / Q000627:therapeutic use; D001569:Benzodiazepines; D002675:Child, Preschool; D000078306:Clobazam; D003241:Consanguinity; D018450:Disease Progression; D013759:Dronabinol / Q000627:therapeutic use*; D004569:Electroencephalography; D006801:Humans; D007555:Isoxazoles / Q000627:therapeutic use; D008297:Male; D008550:Melatonin / Q000627:therapeutic use; D009128:Muscle Spasticity / Q000188:drug therapy* / Q000209:etiology; D018482:Muscle, Skeletal / Q000473:pathology; D009207:Myoclonus / Q000188:drug therapy* / Q000209:etiology; D009472:Neuronal Ceroid-Lipofuscinoses / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D018696:Neuroprotective Agents / Q000627:therapeutic use; D018697:Nootropic Agents / Q000627:therapeutic use; D010166:Palliative Care; D010889:Piracetam / Q000627:therapeutic use*; D012640:Seizures / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome; D000078305:Zonisamide
chemical_list: D018712:Analgesics, Non-Narcotic; D014151:Anti-Anxiety Agents; D000927:Anticonvulsants; D000975:Antioxidants; D007555:Isoxazoles; D018696:Neuroprotective Agents; D018697:Nootropic Agents; D001569:Benzodiazepines; D000078306:Clobazam; D000078305:Zonisamide; D013759:Dronabinol; D008550:Melatonin; D010889:Piracetam