Objective: CASE REPORT

Background: The dystonia like others movement disorder may be included in neurodegenerative ataxia syndrome, in addition to cerebellar dysfunction. The pallidal stimulation has demonstrated to improve the dystonia associated with other neurological disorders. This is a case report about the effect of bilateral pallidal stimulation in the dystonia, in a patient with neurodegenerative ataxia.

Methods: A 26-years-old man had 17 years of incoordination, dysarthria, ataxic gait of slow progressive with frequent trauma. At 23 starts with repetitive movements and hyperextension of the right elbow associated to pain and functional limitation. Two years later compromises the contralateral limb, less severe but disabling. The gait is severely affected by dystonia rather than by the ataxia, thus requiring wheelchair. Perinatal history: instrumental delivery by cephalo-pelvic disproportion, normal neurodevelopment. Personal: mild cognitive impairment, sensory neuropathy axonal, severe thoracic scoliosis which underwent surgery. Family: negative. No consanguineous parents. Physical exam: dysarthria, generalized arreflexia, hypopalesthesia, dysmetria and bilateral adiadochokinesia, dystonia hyperextended upper limb resting and action. Ataxic gait with severe instability associated his dystonia. Scale ataxia (SARA): 22/42. Brain MRI showed cerebellar and brainstem atrophy. The diagnosis of neurodegenerative ataxia associated to other neurological manifestations, was made. The patient underwent bilateral implantation in the internal globus pallidus of constant-voltage, four-contacts, rechargeable deep brain stimulation (DBS) system.

Results: A rapid and sustained improvement in dystonia follow-up to 18 months. The patient was assessed at baseline (Dystonia scale of Burke: movement 25,5 and disabilities 24) and 18 months after leads implantation (Dystonia scale of Burke: movement 6,5 and disabilities 16) with improvement 75% in movement and 33% in disability. No complications related to surgery. Complications related to chronic stimulation were a slight worsening of dysarthria and instability.

Conclusions: The DBS improves movement and disability in a patient with dystonia multifocal associated to neurodegenerative ataxia, with an acceptable safety profile.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/effectiveness-of-bilateral-pallidal-deep-brain-stimulation-in-a-patient-with-dystonia-associated-to-neurodegenerative-ataxia-follow-up-to-18-months/