FIRST REPORTED USE OF VAGUS NERVE STIMULATION IN BATTEN DISEASE: A CASE REPORT
Abstract number :
3.298
Submission category :
Year :
2002
Submission ID :
1454
Source :
www.aesnet.org
Presentation date :
12/7/2002 12:00:00 AM
Published date :
Dec 1, 2002, 06:00 AM
Authors :
Anand W. Mehendale. Neurobiology Research Unit, Phoenix Medical Associates, Kerrville, TX
RATIONALE: One of eight variants of neuronal ceroid lipofuscinous (NCL), Batten disease, an autosomal recessive disorder, affects lysosomal storage. Loss of vision, epilepsy, and cognitive/motor regression characterize NCLs. The incidence rate varies by country from 0.1 to 7 per 100,000 live births.
A male patient, aged 15 years with a history of Batten disease, scoliosis corrected by surgery, and depression presented with medically refractory epileptic seizures. Patient was initially diagnosed at age 5 for Batten Disease and began having epileptic seizures at age 7. Caregiver has reported approximately 140 seizures per month of varying type: partial complex, generalized tonic-clonic, myoclonic, and grand mal. He was taking oxcarbazepine, clonazepam, and levetiracetam for seizures and paroxetine HCl and citalopram hydrobromide for depression. The patient was implanted with the NeuroCybernetic Prosthesis (NCP) system (Cyberonics, Inc.; Houston, Texas), which delivers vagus nerve stimulation (VNS).
In the 4 months since device was turned on, the caregiver has reported a marked decrease in seizures. At the most recent visit, the only seizure type reported was myoclonic jerks, of which only a few occurred. The patient[scquote]s caregiver has been successful in aborting seizures by applying the magnet furnished by the manufacturer. In addition, aggressive behavior, mood swings, and postictal state have markedly improved. Since implantation, the patient switched from Paxil to Celexa for depression. AEDs have remained constant.
METHODS: Case Report
RESULTS: To our knowledge, this report is the first known use of VNS in Batten disease. In this patient, VNS Therapy has been effective in reducing seizures and lessening aggressive tendencies and mood swings.
CONCLUSIONS: Ongoing studies suggest that VNS may also be effective over time for the treatment of depression, which is also symptomatic of Batten disease. Given the success of VNS in this patient with Batten disease, further investigation into the effectiveness of VNS for all variations of NCL disorders would be of great interest.