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 » Introduction
 » Case Report
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Table of Contents    
CASE REPORT
Year : 2015  |  Volume : 63  |  Issue : 6  |  Page : 911-914

Bilateral pallidal deep brain stimulation in idiopathic dystonic camptocormia


1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Date of Web Publication20-Nov-2015

Correspondence Address:
Dwarakanath Srinivas
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.170080

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 » Abstract 

Introduction: Camptocormia is seen with Parkinson's disease (PD), segmental or generalized dystonia, extensor myopathies, anterior horn cell disorders, and muscle disorders. In severe selected cases of PD with camptocormia and dystonic camptocormia, deep brain stimulation (DBS) has been tried with variable success. In this paper, the first case report of its kind from India, we report a case of dystonic camptocormia who underwent DBS and review the available literature.
Methods: A 42-year-old male, presented with dystonia of 5-year duration, that initially started with cervical dystonia and later progressed to severe disturbance of posture causing involuntary truncal flexion induced by standing or sitting. The camptocormia was completely relieved when sitting on a chair or lying down on the bed. Routine blood testing was normal. The workup for secondary dystonia including slit lamp examination for Kayser-Fleischer ring and serum copper studies did not reveal any abnormality. Magnetic resonance imaging of the brain was unremarkable. The electromyogram of the lumbar and thoracic paraspinal muscles was also normal.
Results: The patient was initially treated with multiple drugs and Botulinum A toxin which were ineffective. He underwent bilateral globus pallidus interna (GPi) DBS Over a period of 2 weeks; there was a mild reduction in the dystonia of the trunk and neck. The maximum improvement in dystonia, approximately 30% over baseline, was noted at 2 weeks postsurgery and over a further long-term follow-up, the improvement was 50% as determined by the sub-item (trunk) assessment of the Burke-Fahn-Marsden (BFM) dystonia score. Cervical dystonia improved by >90% in sub-item (neck) assessment of BFM scale.
Conclusions: In this report, we have shown the efficacy of GPi DBS in the treatment of drug refractory dystonia associated camptocormia. Although only reported for PD associated camptocormia, evaluation for truncal extensor myopathy is mandatory in these cases also to achieve a good outcome.


Keywords: Camptocormia; deep brain stimulation; globus pallidus interna


How to cite this article:
Yadav R, Ansari AZ, Surathi P, Srinivas D, Somanna S, Pal P. Bilateral pallidal deep brain stimulation in idiopathic dystonic camptocormia. Neurol India 2015;63:911-4

How to cite this URL:
Yadav R, Ansari AZ, Surathi P, Srinivas D, Somanna S, Pal P. Bilateral pallidal deep brain stimulation in idiopathic dystonic camptocormia. Neurol India [serial online] 2015 [cited 2019 Dec 13];63:911-4. Available from: http://www.neurologyindia.com/text.asp?2015/63/6/911/170080



 » Introduction Top


Camptocormia is a condition characterized by forward bending of the trunk and has been reported with Parkinson's disease (PD), multiple system atrophy, dystonia, abdominal segmental dystonia, Tourrete syndrome, myotonic dystrophy and other myopathies, amyotrophic lateral sclerosis, and drugs.[1],[2] Treatment options are limited and include physiotherapy, botulinum toxin injections, levodopa, and other drugs.[1] There has been an equivocal response to deep brain stimulation (DBS) of the subthalamic nucleus (STN) and globus pallidus interna (GPi) in patients of PD and camptocormia.[1],[2] However, the response in primary idiopathic camptocormia with dystonia has largely been encouraging in various small series and reports.[3],[4],[5]

In this report, we present a rare case of primary cervical dystonia with camptocormia highlighting the management options and the response to various modalities of treatment.


 » Case Report Top


A 42-year-old male patient presented with dystonia of 5-year duration, which initially started with intermittent, abnormal patterned, anterocollic and retrocollic movements of the head that later developed into dystonic spasms. The dystonia was progressive with involvement of the trunk after 2 years and was associated with severe disturbance of posture involving involuntary truncal flexion induced by standing or sitting. He had no discomfort when seated or supine, but on walking, he developed a dramatic thoracolumbar flexion associated with pain in the lower abdomen and back. The patient's neurological examination showed cervical dystonia with moderate anterocollis and retrocollis. He had mild thoracic kyphosis while being seated and standing, but otherwise exhibited no abnormal anterior or lateral flexion. He was examined after walking for about 30s; he had the onset of thoracolumbar flexion that progressed for another 30s to reach a maximum flexion. This degree of flexion was intolerably painful that caused him to stop walking and forced his trunk upright. He would then resume walking, and the pattern would repeat [Video 1]. The rectus abdominis muscles were palpably tight during flexion. The camptocormia was completely relieved when sitting on chair or on lying down in bed. Routine blood testing including the hemogram with peripheral smear, liver and renal functions, calcium, phosphorus, serum ammonia and lactate, creatine phosphokinase and lactate dehydrogenase, was normal; the workup for secondary dystonia, including slit lamp examination for  Kayser-Fleischer ring More Details, was negative. Magnetic resonance imaging (MRI) of the brain was unremarkable, and MRI of the thoracic and lumbar spine were notable for mild degenerative changes. He was treated with trihexphenidil (up to 12 mg/day), clonazepam (up to 1.5 mg/day), baclofen (up to 30 mg/day), levodopa (up to 300 mg/day), and tetrabenazine (75 mg/day). He did not respond to any of these medications. He also received botulinum toxin A injections to the rectus abdominis muscles (initially 200 units) and later also to bilateral iliopsoas units, which were effective for only a transient period.

The Burke-Fahn-Marsden (BFM) Evaluation Scale for dystonia [3] was used for his pre- and post-operative objective assessment. After an informed and written consent, he was taken up for bilateral GPi DBS.

Surgical procedure

Frame fixation using the Leksell G stereotactic frame (Elekta Instruments, Stockholm, Sweden) was performed under local anesthesia. An MRI was then performed (1.5-T; Siemens Aera, Erlangen, Germany). Both T1-weighted (three-dimension (3D) magnetization-prepared rapid gradient-echo (MP- RAGE) sequence with 1 mm slice thickness) and T2-weighted images were taken. The images were transferred to the Medtronic Framelink (R) planning system, and planning was performed. The posteroventral pallidal GPi (PVP-GPi) was targeted using the direct method in relation to the optic tract. The target is situated just above the top of the optic tract at the midpoint of the optic tract, in the mid-commissural plane. Both the procedures were performed under general anesthesia, as severe dystonia did not permit surgery under local anesthesia. Postoperatively, we performed a CT scan, and these images were fused with the preoperative plan for checking for the accuracy of the electrode position. The programming was started 2 weeks after the surgical procedure.

Postoperative evaluation and course

Over a period of 6 weeks, there was a mild reduction in the dystonia of the trunk and neck. The maximum improvement in the dystonia, approximately 30% over baseline, was noted at 2 months postsurgery [Table 1]. At the end of 1-year, and at 15 months of follow-up, the patient had about 90% improvement in the sub-item neck assessment, and about 60% improvement in the total Burke-Fahn-Marsden Dystonia Rating Scale (BFMRDS) score [Video 2]. The stimulation parameters at 15 months follow-up were GPi right: C +1-3.8V 210 ms/180 Hz; GPi left: C +9-3.8V 210 ms/180 Hz.
Table  1: Serial Burke-Fahn-Marsden dystonia scores showing the improvement following bilateral GPi DBS for generalized dystonia

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 » Discussion Top


Our patient was a case of generalized dystonia with camptocormia. He showed a significant improvement on BFMRDS rating scale on the most involved items and about 50% improvement in the sub-item trunk assessment [Table 1]. In the available literature, there is limited experience in utilizing DBS in cases of dystonic camptocormia [Table 2].
Table  2: DBS in patients with dystonic camptocormia in literature

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In a series of 7 patients of camptocormia of mixed etiology, Capelle et al., showed that 4 patients with dystonia and 3 with PD underwent bilateral GPi DBS, and two underwent bilateral STN DBS.[3] These patients were assessed by the BFM scale in patients with dystonic type, and by the Unified Parkinson's Disease Rating Scale in patients with PD. In patients with dystonia with camptocormia, who underwent GPi DBS, there was a mean improvement of 53% in BFM motor score and 63% in the subscore for trunk at a mean follow-up of 14 months. In the 3 patients of PD, there was either no improvement or only a mild improvement in camptocormia, in those patients who underwent STN DBS but a moderate improvement in patients who underwent GPi DBS.[3] In another series of 3 patients of dystonic camptocormia by Reese et al., there was a significant response to bilateral GPi-DBS within weeks in the postoperative period that was sustained till about 3 years of follow-up.[6] There was an improvement of 82% as rated on the BFMRDS and 89% in the sub-item "trunk." Hagenacker et al., reported the findings of 2 patients having a generalized dystonia with camptocormia who underwent GPi DBS.[5] The first patient had 75% improvement and the second patient had 87% improvement in the subscore of truncal flexion, which persisted independent of the improvement in generalized dystonia. Sakas et al., also noted similar significant outcomes in two patients having drug-resistant dystonic camptocormia who underwent GPi DBS and suggested that the specific oscillatory activity in GPi is vital for the maintenance of erect posture.[7] In the other reports by Fukaya et al., and Nandi et al., similar observations have been found.[4],[9]

There are conflicting reports on the beneficial effects of PD associated camptocormia. Improvement has been reported by Lyons et al., Asahi et al., Thani et al., and Sako et al.[7],[10],[11],[12] However, no improvement after surgery has been observed by Umemura et al., in 6 out of 18 patients of PD associated Camptocormia.[2] Allert et al., and Asahi et al., had similar observations.[10],[13] In a series of 4 patients of PD and camptocormia, Asahi et al., reported that the lack of improvement of camptocormia in PD was related to the severity of paraspinal muscle degeneration.[10] One patient in their series did not improve as he had paraspinal muscle degeneration.[10] Allert et al., have also made similar observations in a patient with PD, who had truncal erector myopathy and did not respond to DBS.[13] They suggested caution in choosing DBS as a treatment in cases of PD with camptocormia as the extensor myopathy may not lead to sufficient relief.[13] Hence, it is imperative that both imaging and electrophysiological assessments are utilized to rule out any extensor truncal myopathy in patients with camptocormia.[14]

Our patient, during the immediate postoperative phase, had a remarkable improvement in the generalized dystonia. The jerky episodes of forward bending were reduced, and the duration for which he could stand erect increased markedly. The response to his geste antagoniste improved. After a month, he was able to drive a two-wheeler and went back to work. He also had improvement in anxiety, anger outbursts, and had significant weight gain.


 » Conclusion Top


In this case report, the first from India, we have shown the efficacy of GPi DBS in the treatment of drug refractory dystonia associated camptocormia. Although only reported for PD associated camptocormia, evaluation for truncal extensor myopathy is mandatory in these cases also to achieve a good outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 » References Top

1.
Finsterer J, Strobl W. Presentation, etiology, diagnosis, and management of camptocormia. Eur Neurol 2010;64:1-8.  Back to cited text no. 1
    
2.
Umemura A, Oka Y, Ohkita K, Yamawaki T, Yamada K. Effect of subthalamic deep brain stimulation on postural abnormality in Parkinson disease. J Neurosurg 2010;112:1283-8.  Back to cited text no. 2
    
3.
Capelle HH, Schrader C, Blahak C, Fogel W, Kinfe TM, Baezner H, et al. Deep brain stimulation for camptocormia in dystonia and Parkinson's disease. J Neurol 2011;258:96-103.  Back to cited text no. 3
    
4.
Fukaya C, Otaka T, Obuchi T, Kano T, Nagaoka T, Kobayashi K, et al. Pallidal high-frequency deep brain stimulation for camptocormia: An experience of three cases. Acta Neurochir Suppl 2006;99:25-8.  Back to cited text no. 4
    
5.
Hagenacker T, Gerwig M, Gasser T, Miller D, Kastrup O, Jokisch D, et al. Pallidal deep brain stimulation relieves camptocormia in primary dystonia. J Neurol 2013;260:1833-7.  Back to cited text no. 5
    
6.
Reese R, Knudsen K, Falk D, Mehdorn HM, Deuschl G, Volkmann J. Motor outcome of dystonic camptocormia treated with pallidal neurostimulation. Parkinsonism Relat Disord 20:176-179.  Back to cited text no. 6
    
7.
Lyons M, Boucher O, Patel N, Birch B, Evidente V. Long-term benefit of bilateral subthalamic deep brain stimulation on camptocormia in Parkinson's disease. Turk Neurosurg 2012;22:489-92.  Back to cited text no. 7
    
8.
Sakas DE, Stavrinou LC, Boviatsis EJ, Stathis P, Themistocleous M, Gatzonis S. Restoration of erect posture by deep brain stimulation of the globus pallidus in disabling dystonic spinal hyperextension. J Neurosurg 2010;112:1279-82.  Back to cited text no. 8
    
9.
Nandi D, Parkin S, Scott R, Winter JL, Joint C, Gregory R, et al. Camptocormia treated with bilateral pallidal stimulation. J Neurosurg 2002;97:461-6.  Back to cited text no. 9
    
10.
Asahi T, Taguchi Y, Hayashi N, Hamada H, Dougu N, Takashima S, et al. Bilateral subthalamic deep brain stimulation for camptocormia associated with Parkinson's disease. Stereotact Funct Neurosurg 2011;89:173-7.  Back to cited text no. 10
    
11.
Thani NB, Bala A, Kimber TE, Lind CR. High-frequency pallidal stimulation for camptocormia in Parkinson disease: Case report. Neurosurgery 2011;68:E1501-5.  Back to cited text no. 11
    
12.
Sako W, Nishio M, Maruo T, Shimazu H, Matsuzaki K, Tamura T, et al. Subthalamic nucleus deep brain stimulation for camptocormia associated with Parkinson's disease. Mov Disord 2009;24:1076-9.  Back to cited text no. 12
    
13.
Allert N, Miskiewicz AA, Coenen VA. Deep brain stimulation for Parkinson's disease and camptocormia? A case report. Fortschr Neurol Psychiatr 2011;79:97-101.  Back to cited text no. 13
    
14.
O'Riordan S, Paluzzi A, Liu X, Aziz T, Nandi D, Bain P. Camptocormia response to bilateral globus pallidus interna stimulation in three patients. Mov Disord 2009;24(Suppl 1):489  Back to cited text no. 14
    



 
 
    Tables

  [Table 1], [Table 2]



 

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