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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 6 | Page : 1354-1355
Intrathecal baclofen pump in spinocerebellar degeneration
R Ramnarayan, Simon Herculus
Department of Neurosurgery, Consultant Neurosurgeon, New Hope Hospital, Kilpauk, Chennai, Tamil Nadu, India
|Date of Web Publication||11-Nov-2016|
Department of Neurosurgery, Consultant Neurosurgeon, New Hope Hospital, Kilpauk, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Ramnarayan R, Herculus S. Intrathecal baclofen pump in spinocerebellar degeneration. Neurol India 2016;64:1354-5
Spinocerebellar ataxia is an autosomal dominant progressive degenerative disease with several subtypes. This pathology is considered not treatable. Here, we present a patient with non-Friedriech ataxia who benefitted from intrathecal baclofen pump insertion. A 40-year-old male patient presented with severe limb ataxia, ophthalmoplegia, intention tremor, along with brisk reflexes. He had difficulty in writing, eating, and dressing. He required the support of one person while walking, which was progressive (now he required two persons' full support) for the last ten years. He underwent a trial of intrathecal baclofen injection and showed an immediate improvement. Therefore, after obtaining a proper informed consent, he underwent insertion of an intrathecal baclofen pump (Synchromed II Medtronic). He has been on follow up for two years and has shown improvement in many symptoms (50–60% according to the patient). His limb ataxia has become minimal and he has been able to walk without support (using a walking stick). He has been able to eat and drink by himself and unbutton his shirt. His eye movements have become full. The dose of baclofen is 150 g per day. Rossi et al., conducted a systematic review of the common clinical signs of autosomal dominant cerebellar ataxias. They analysed 1062 publications reviewing 12141 patients. Of these, 52% were male patients and the mean age at onset was 35 ± 11 years. The clinical presentation at the onest of manifestations in 3945 patients revealed gait ataxia as being the most frequent sign (68%), whereas overall, the non-ataxia symptom frequency was 50%. During the course of the disease, dysarthria (90%) and saccadic eye movement alterations (69%) were the most prevalent non-ataxia clinical findings. Trujillo-Martín et al., studied the effectiveness and safety of the available treatment alternatives for these ataxias. They found limited evidence for medical therapies and noted that quality studies to evaluate the safety and efficacy of treatments for most degenerative ataxias (DA) are scarce. No valid information on the actual value of physical rehabilitation and psychological support as treatment modalities was available. Bonouvrié et al., retrospectively studied the overall satisfaction of caregivers with intrathecal baclofen treatment in a group of children and adolescents with progressive neurological disorders. One patient, a 4-year-old child, had spinocerebellar ataxia and had reduced spasticity and ataxia at 6.8 years following the treatment. The caregiver gave a satisfaction score of 7/10.
To conclude, intrathecal baclofen could be an option in some cases of spinocerebellar degeneration. A trial injection will be the best guide.
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Conflicts of interest
There are no conflicts of interest.
| » References|| |
Rossi M, Perez-Lloret S, Doldan L, Cerquetti D, Balej J, Millar Vernetti P, et al
. Autosomal dominant cerebellar ataxias: A systematic review of clinical features. Eur J Neurol 2014;21:607-15.
Trujillo-Martín MM, Serrano-Aguilar P, Monton-Alvarez F, Carrillo-Fumero R. Effectiveness and safety of treatments for degenerative ataxias: A systematic review. Mov Disord 2009;24:1111-24.
Bonouvrié L, Van Schie P, Becher J, Van Ouwerkerk W, Vermeulen R. Satisfaction with intrathecal baclofen treatment in paediatric patients with progressive neurological disease. Dev Med Child Neurol 2008;50:636-8.