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Table of Contents    
CASE REPORT
Year : 2022  |  Volume : 70  |  Issue : 2  |  Page : 757-759

Spinal Cord Stimulation Improved Freezing of Gait and Hypokinetic Dysarthria of a Patient with Dopamine-Resistant Multiple System Atrophy-Parkinsonian Type


Trauma Center, First Affiliated Hospital of Kunming Medical University, Kunming, China

Date of Submission24-May-2021
Date of Decision29-Sep-2021
Date of Acceptance04-Oct-2021
Date of Web Publication3-May-2022

Correspondence Address:
Dr. Xingjian Gong
No.295, Xichang Road, Kunming, Yunnan
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.344653

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 » Abstract 


Background: Multiple system atrophy parkinsonian type (MSA-P) patients with resistance to dopamine have highly limited treatment options. This calls for further study of spinal cord stimulation (SCS) as a potential nondopaminergic therapy to improve motor and speech functions of patients with dopamine-resistant parkinsonism.
Case Presentation: A 58-year-old male with MSA-P had hypokinetic dysarthria, freezing of gait (FOG), and spinal disc herniation with refractory back pain. SCS was used to treat his refractory back pain. Serendipitously, after the surgery, the patient reported not only a reduction in pain but also rapid improvement of FOG and hypokinetic dysarthria.
Conclusion: SCS has been found in some cases to improve FOG and hypokinetic dysarthria. It is necessary to further study the potential of and the mechanism behind SCS as a potential nondopaminergic therapy to improve motor and speech functions of patients with dopamine-resistant parkinsonism.


Keywords: Freezing of gait, hypokinetic dysarthria, multiple system atrophy, spinal cord stimulation
Key Message: Multiple system atrophy parkinsonian type patients have highly limited treatment options due to their resistance to dopamine. Spinal cord stimulation deserves further study as a potential alternative to dopaminergic therapies to improve motor and speech functions of patients with dopamine-resistant parkinsonism.


How to cite this article:
Gong H, Liu Y, Zhu X, Gong X. Spinal Cord Stimulation Improved Freezing of Gait and Hypokinetic Dysarthria of a Patient with Dopamine-Resistant Multiple System Atrophy-Parkinsonian Type. Neurol India 2022;70:757-9

How to cite this URL:
Gong H, Liu Y, Zhu X, Gong X. Spinal Cord Stimulation Improved Freezing of Gait and Hypokinetic Dysarthria of a Patient with Dopamine-Resistant Multiple System Atrophy-Parkinsonian Type. Neurol India [serial online] 2022 [cited 2022 Jul 3];70:757-9. Available from: https://www.neurologyindia.com/text.asp?2022/70/2/757/344653




Multiple system atrophy - parkinsonian type (MSA-P) is a neurodegenerative disorder that affects the autonomic functions of the human body. Freezing of gait (FOG) and hypokinetic dysarthria are symptoms that are commonly seen in MSA-P. Patients with these symptoms do not respond equally well to dopaminergic therapies. FOG and dysarthria can severely reduce a patient's quality of life and add significant burdens to patients' families and society. Therefore, effective alternative therapies for patients with dopamine resistance are called for.

We report a case of rapid improvement of FOG and hypokinetic dysarthria after using spinal cord stimulation (SCS).


 » Case Presentation Top


A 58-year-old male was diagnosed with PD 4 years ago after he started experiencing severe resting tremor of his upper-right limb, slow hand-and-leg movement, small and shuffling steps, drooling, difficulty with opening his right eye, and occasional speech impairment. He took 250 mg of levodopa, 1 mg of pramipexole, and trihexyphenidyl daily with no noticeable improvement.

At admission, the patient presented with severe upper-right limb action tremor, hypokinetic dysarthria, hypomimia, bradykinesia, FOG [Appendix Video], refractory back pain, and hypertonia of both legs with a grade-4 muscle tone on the modified Ashworth Scale (MAS). Oculomotor examinations suggested that the patient had normal pupillary light reflex, normal eye movements. Moreover, he had disappeared forehead lines, right side Hoffmann (+), left slide Hoffmann (-), Babinski (-), and bilateral Kernig (-). The patient was able to complete the finger-to-nose test and the heel-to-shin test very slowly. On the New Freezing of Gait Questionnaire (NFOG-Q), a self-reportable test that measures the severity of FOG, the patient scored 26 out of 28, where higher scores correspond to severer FOG. Clinical examinations indicated that the patient's blood pressure was 124/83 mm Hg standing up and 104/70 mm Hg lying down, which suggested orthostatic hypotension. Color Doppler ultrasound results indicated that the patient had urinary retention at around 141 mL [Figure 1]. Notably, the patient had undergone transurethral resection of the prostate. From his MRI, our radiologist suggested that the patient had spinal disc herniation, which likely caused his refractory back pain.
Figure 1: Color Doppler Ultrasound image indicated that the patient had a urinary retention of 141 mL

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Because the patient reported a fast-declining efficacy of levodopa, his response to levodopa treatment was assessed using the MDS-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), which evaluates different aspects of PD-like motor functions. A baseline score was first measured. Then, the patient was challenged once with levodopa at 1.5 times the regular dosage. MDS-UPDRS score was measured every 30 min for 6 h. The highest improvement in score improvement was 12%, indicating a limited levodopa efficacy.

We determined that this patient was misdiagnosed as PD. His Parkinsonian symptoms, together with L-dopa-resistance, high urinary retention, and high orthostatic hypotension, suggest that the patient had MSA-P. Deep brain stimulation (DBS) and dopaminergic medications are ineffective in patients with L-dopa resistance.

SCS was used to treat the patient's debilitating back pain. A permanent spinal cord stimulator including electrodes and power supply was implanted under local anesthesia into the dorsal epidural space of the spinal canal in-between T9 and T11 [Figure 2]. A range of electric stimulation parameters was tested to achieve the best pain relief, and we landed at 300 μs/20 Hz/1.6 V. The patient stopped dopaminergic medication after the surgery. On the day following the SCS implantation, the patient complained of a sense of tinkling and atony on both feet and reported a significantly alleviated back pain. Interestingly, the patient also reported a reduction in the frequency and severity of FOG [Appendix Video], which was supported by a score of 21 on the NFOG-Q test, reduced from 26, and a grade-1 MSA muscle tone, reduced from grade 4. He also showed a clear recovery from hypomimia for he was able to make different facial expressions like smile at ease. In the following week, the patient was able to talk clearly at a regular volume for more than ten minutes. The patient scored 20 on the NFOG-Q a month after the surgery, and 16 three months after the surgery.
Figure 2: Spinal Cord Stimulation electrodes are implanted into the dorsal epidural space of the spinal canal in-between T9 and T11

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 » Discussion Top


FOG and hypokinetic dysarthria can severely reduce a patient's quality of life and add significant burdens to patients' families and society. FOG occurs in 30%–70% of patients with Parkinson's disease and Parkinsonism,[1] and as many as 89% of patients with Parkinsonism are reported to have some form of speech disorder or other, such as hypophonia and dysphonia.[2]

The pathophysiology of FOG and hypokinetic dysarthria is not well understood. Studies have shown that both FOG and hypokinetic dysarthria in Parkinsonian patients can be a result of malfunctions within basal ganglia, and dopaminergic therapies, such as levodopa and DBS, can improve both symptoms.[3],[4] Yet on Parkinsonian patients with dopamine resistance, dopaminergic therapies failed to achieve any significant long-term efficacy, as many studies have shown that several heterogeneous nondopaminergic mechanisms could be at play in causing FOG and hypokinetic dysarthria.[2],[5] The lack of effective treatment options for Parkinsonian patients with dopamine resistance demands further investigations into the mechanisms of dopamine resistance and possible therapies.

SCS has long been used to treat chronic pain when pain-relief medications fail. This case supports the findings from some previous studies[6],[7] and suggests that SCS could also be a potential therapy for dopamine-resistant FOG and hypokinetic dysarthria. However, the mechanism of how spinal cord stimulation treats FOG and hypokinetic dysarthria is unclear. Parkinsonian speech disorders may be attributable to defective basal ganglionic control.[3],[8] SCS could help with hypokinetic dysarthria by inhibiting pathological neuronal firing in basal ganglia.[9] SCS is also advantageous in that the implantation surgery is less risky than STN-DBS and requires only local anesthesia.

In this case, we saw a rapid improvement of FOG and hypokinetic dysarthria on an MSA-P patient who is resistant to dopaminergic treatments after using SCS. These positive outcomes were found to be long-lasting and further improved over time as SCS treatment continued. Further study on how SCS improves FOG and hypokinetic dysarthria is called for as it would help with the development of new therapies that would help increase treatment options for patients unsuitable for dopaminergic medications and DBS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Choi S-M, Jung H-J, Yoon G-J, Kim BC. Factors associated with freezing of gait in patients with Parkinson's disease. Neurol Sci 2019;40:293-8.  Back to cited text no. 1
    
2.
Dashtipour K, Tafreshi A, Lee J, Crawley B. Speech disorders in Parkinson's disease: Pathophysiology, medical management and surgical approaches. Neurodegener Dis Manag 2018;8:337-48.  Back to cited text no. 2
    
3.
Leopold NA, Kagel MC. Laryngeal deglutition movement in parkinson's disease. Neurology 1997;48:373-5.  Back to cited text no. 3
    
4.
Lewis SJG, Barker RA. A pathophysiological model of freezing of gait in Parkinson's disease. Parkinsonism Relat Disord 2009;15:333-8.  Back to cited text no. 4
    
5.
Gao C, Liu J, Tan Y, Chen S. Freezing of gait in Parkinson's disease: Pathophysiology, risk factors and treatments. Transl Neurodegener 2020;9. doi: 10.1186/s40035-020-00191-5.  Back to cited text no. 5
    
6.
Zhang Y, Song T, Zhuang P, Wang Y, Zhang X, Mei S, et al. Spinal cord stimulation improves freezing of gait in a patient with multiple system atrophy with predominant parkinsonism. Brain Stimulat 2020;13:653-4.  Back to cited text no. 6
    
7.
Pinto de Souza C, Hamani C, Oliveira Souza C, Lopez Contreras WO, Dos Santos Ghilardi MG, Cury RG, et al. Spinal cord stimulation improves gait in patients with Parkinson's disease previously treated with deep brain stimulation. Mov Disord Off J Mov Disord Soc 2017;32:278-82.  Back to cited text no. 7
    
8.
Takakusaki K, Habaguchi T, Ohtinata-Sugimoto J, Saitoh K, Sakamoto T. Basal ganglia efferents to the brainstem centers controlling postural muscle tone and locomotion: A new concept for understanding motor disorders in basal ganglia dysfunction. Neuroscience 2003;119:293-308.  Back to cited text no. 8
    
9.
Santana MB, Halje P, Simplício H, Richter U, Freire MAM, Petersson P, et al. Spinal cord stimulation alleviates motor deficits in a primate model of Parkinson's disease. Neuron 2014;84:716-22.  Back to cited text no. 9
    


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