piperidines and Dystonic-Disorders

We report a case of Pisa syndrome (PS) due to the acetylcholinesterase inhibitor donepezil which may have been precipitated by pharmacokinetic interactions with commonly used medications. PS is defined as a reversible lateral bending of the trunk with a tendency to lean to one side. This is a rare but very distressing complication with this commonly used medication which was not initially recognised, leading to increasing disability for the patient and significant carer stress. Cessation of donepezil and modulation of potential interacting medications resulted in complete resolution.

Topics: Aged, 80 and over; Cholinesterase Inhibitors; Donepezil; Drug Interactions; Dystonic Disorders; Humans; Indans; Male; Omeprazole; Piperidines; Polypharmacy; Postural Balance; Proton Pump Inhibitors; Risk Factors

Deep brain stimulation (DBS) has become accepted therapy for intractable dystonia and other movement disorders. The accurate placement of DBS electrodes into the globus pallidus internus is assisted by unimpaired microelectrode recordings (MERs). Many anesthetic and sedative drugs interfere with MERs, requiring the patient to be awake for target localization and neurological testing during the procedure. In this study, a novel anesthetic technique was investigated in pediatric DBS to preserve MERs.. In this paper, the authors describe a sedative/anesthetic technique using ketamine, remifentanil, dexmedetomidine, and nicardipine in 6 pediatric patients, in whom the avoidance of GABAergic stimulating drugs permitted excellent surgical conditions with no detrimental effects on intraoperative MERs. The quality of the MERs, and the frequency of its use in making electrode placement decisions, was reviewed.. All 6 patients had good-quality MERs. The data were of sufficient quality to make a total of 9 trajectory adjustments.. Microelectrode recordings in pediatric DBS can be preserved with a combination of dexmedetomidine and ketamine, remifentanil, and nicardipine. This preservation of MERs is particularly crucial in electrode placement in children.

Topics: Adolescent; Anesthesia; Anesthetics, Combined; Anesthetics, Dissociative; Antihypertensive Agents; Child; Deep Brain Stimulation; Dexmedetomidine; Dystonia; Dystonic Disorders; Female; Humans; Hypnotics and Sedatives; Ketamine; Male; Microelectrodes; Neurophysiological Monitoring; Nicardipine; Piperidines; Remifentanil; Sleep; Wakefulness

Neurodegeneration associated with pantothenate kinase deficiency is an autosomal recessive condition caused by mutations in the pantothenate kinase 2 gene (PANK2). Clinical characteristics include progressive motor impairment and dementia. Medical treatment is limited and the dystonia tends to be refractory, making stereotactic surgery with placement of deep-brain electrodes an option that is being adopted with greater frequency in these patients. We report the case of a 32-year-old woman with severe dystonia associated with PANK2 protein deficiency. The patient was scheduled for stereotactic bilateral placement of electrodes in the medial globus pallidus, guided by computed tomography and under general anesthesia, to treat the debilitating dystonia and generalized stiffness associated with her condition. Anesthesia was maintained with propofol, rocuronium and remifentanil in perfusion during the intervention, which was uneventful. After the procedure, the patient was transferred to the intensive care unit and sedation was provided with remifentanil to allow slow, gradual emergence from anesthesia. The patient was discharged from hospital after placement of the implanted pulse generator, and subsequent follow-up showed improvement of the dystonia.

Topics: Adult; Androstanols; Anesthesia, Intravenous; Deep Brain Stimulation; Dystonic Disorders; Female; Globus Pallidus; Humans; Intubation, Intratracheal; Muscle Rigidity; Neuromuscular Nondepolarizing Agents; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Piperidines; Preanesthetic Medication; Propofol; Radiography, Interventional; Remifentanil; Rocuronium

Glutaric aciduria type 1 (GA-1) is an inborn error of metabolism that results from a deficiency of glutaryl-CoA dehydrogenase. This disorder mainly manifests in early childhood and most patients with this condition develop a dystonic-dyskinetic syndrome. We report the anesthetic management of two sisters with GA-1, aged 30 and 17 months respectively at the time of surgery, who presented with macrocephaly and psychomotor delay. The children required CSF shunting procedures for hydrocephalus and subdural fluid collections, which were performed under total intravenous anesthesia with propofol and remifentanil.

Topics: Anesthesia; Anesthetics, Intravenous; Cerebrospinal Fluid Shunts; Child, Preschool; Dystonic Disorders; Female; Glutarates; Glutaryl-CoA Dehydrogenase; Head; Humans; Hydrocephalus; Infant; Metabolism, Inborn Errors; Piperidines; Propofol; Psychomotor Disorders; Remifentanil; Siblings