Pallidal deep brain stimulation in Huntington’s disease

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Erscheinungsjahr:
2014
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Beschreibung:
  • Objective:

    To evaluate the effect of pallidal deep brain stimulation (DBS) on motor symptoms in Huntington's disease (HD).
    Background:

    Pallidal DBS is a safe and well-established treatment for dyskinesias in Parkinson's disease and dystonia. In recent years it has also been used in patients with chorea but only limited experience exists so far.
    Methods:

    Here we report three months (case 3), one year (case 2) and three year (case 1) follow-up assessed with the Unified Huntington's Disease Rating Scale (UHDRS) after Globus pallidus internus (GPi) DBS in three HD patients with treatment refractory chorea.
    Results:

    Case 1: This 54 year old woman presented with generalized chorea, gait disturbances, cognitive decline and irritability (42 CAG repeats in the IT15-gene). Five years after diagnosis she had severe generalized chorea with axial involvement and repeated falls. Medication with risperidone and tetrabenazine was not tolerated due to side effects. Since high dose tiapride was ineffective she underwent bilateral GPi DBS. 40 Hz low frequency stimulation led to sustained improvement of motor symptoms for three years postoperatively (UHDRS preoperatively 61; 1 yrs postoperatively 57; 3 yrs postoperatively 54). Tiapride was discontinued. Case 2: This 35 year old woman presented with combined chorea, dystonia and parkinsonism as well as mood disturbances (46 CAG repeats in the IT15-gene). Four years into the disease she developed severe chorea and hypotonia so that she was unable to sit and walk without assistance. She had to be fixated in bed or on a chair due to several falls and head injuries (UHDRS 93). High dose tetrabenazine and tiapride were ineffective. At one-year follow up 40 Hz DBS significantly improved chorea and the patient was able to lie in bed without fixation, sit and walk without help (UHDRS 71). Tetrabenazine was withdrawn. Case 3: Seven years after diagnosis this 45 year old man presented with severe chorea of the extremities and trunk, which caused many injuries (45 CAG repeats in the IT15-gene). Higher doses of neuroleptics were not tolerated due to intolerable apathy. At three months follow up 40 Hz GPi DBS induced significant improvement of motor symptoms (UHDRS preoperatively 64; 3 months postoperatively 48).
    Conclusions:

    GPi DBS markedly improved motor symptoms in these HD patients with medically refractory chorea.
Lizenz:
  • info:eu-repo/semantics/restrictedAccess
Quellsystem:
Forschungsinformationssystem des UKE

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oai:pure.atira.dk:publications/ab8e4c38-87cc-40f1-8465-0c6caa42c284