Surgical Treatments for Movement Disorders

Published on 03/03/2015 by admin

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Last modified 22/04/2025

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43 Surgical Treatments for Movement Disorders

Surgical therapies for certain movement disorders are important treatment modalities, particularly in medically refractory cases where the patient has become significantly disabled. Early on, thousands of surgically induced brain lesions were performed between 1950 and 1970 after a serendipitous surgical “mistake” led to loss of a classic Parkinson disease (PD) tremor in one patient. Very rapidly an initial enthusiasm developed for this therapeutic modality. However, the introduction of levodopa in 1966 led to a significant cessation in the development of more sophisticated surgical treatment for PD. Subsequently our understanding of the physiology of movement disorders and our ability to better assess baseline and outcome data in these patients have markedly improved since the initial historical period. Concomitantly, it became clear that medical management would not provide long-term resolution of the classic PD in many patients. Today PD primarily includes mostly the idiopathic subset in contrast to the combined idiopathic as well as the postencephalitic variants present when surgical therapeutic methodologies were in their infancy. Currently there are several operations performed for the rather few neurologic disorders that are treated effectively by surgery (Table 43-1). These include very specific intentional destructive lesions targeting specific basal ganglia sites as well as deep brain stimulation (DBS) within the basal ganglia and thalamus.

Table 43-1 Summary of Current Best Procedures for Movement Disorders*

Disorder Procedures
Essential (familial) tremor Vim Thalamic DBS or
Thalamotomy (unilateral)
Parkinson disease DBS either to the STN or
Gpi or
Pallidotomy (unilateral)
Dystonia Gpi DBS

* DBS, deep brain stimulation; Gpi, globus pallidus pars interna; STN, subthalamic nucleus; Vim, ventralis intermedius.

Patient Selection

Patients who are candidates for DBS are typically refractory to standard medical therapy that included multiple trials with varying dosages and combinations of pharmacotherapy. Once a diagnosis of PD is confirmed, expeditious medication trials are encouraged, in order that years of potential benefit from subsequent surgery are not lost if the patient eventually becomes a medication failure. Ideally the surgeon prefers to consider patients whose clinical severity has not progressed toward end stages where surgical intervention becomes less appropriate. However, in most centers, DBS and lesion placement for PD are performed as late as age 80 years. This is predicated on the patient still evidencing good results to preoperative neuropsychological testing that demonstrates no more than minimal signs of dementia. Similar criteria are applied when contemplating DBS treatment for tremor per se.

Dystonia, in contrast, typically involves children as young as ages 6–8 years. Although adequate genetic testing is helpful for diagnosis, many dystonia patients are DYT-1 negative. The presence or absence of genetic confirmation does not determine which child will benefit from DBS.

Decisions to pursue DBS for patients with variable clinical presentations, including severe essential tremor, PD, or dystonia, are clinical ones. Patients are initially selected from those who have failed various medication protocols. The neurosurgeon then considers the potential contribution of the invasive procedure to the patient’s overall quality of life as well as its potential acute and chronic side effects and thus its relative risk benefits.

General Procedure

The operations are performed stereotactically, typically with the help of a frame affixed to the head of the patient (Fig. 43-1). This frame serves to create a space with x, y, and z coordinates that the head, and thus the brain, lies within. As such, any particular location within that space can be targeted using devices to place and hold a probe tip, recording electrode, or stimulating electrode at the desired coordinates. These systems are highly accurate, provided the surgeon is specifically trained and able to precisely focus the probe target within just 1 mm of an intended therapeutic site deep within the appropriate target brain structures including the basal ganglia, thalamus, or subthalamic brain structures. A system of stereotactic targeting software is used to define readily appreciated common brain structures as initial reference points. These systems typically use measurements based on the location of the anterior and posterior commissures.

During the intraoperative procedure, the patients are kept awake and monitored by a clinical neurophysiologist. As the probe is advanced, specific cell types are verified physiologically by having a clinical neurophysiologist make microelectrode recordings along the probe’s trajectory toward the intended subcortical target. Cells in certain specific areas have reproducible physiologic signatures vis-à-vis their firing rates and patterns. The utilization of these physiologic markers helps validate the exact location of the electrode (see Fig. 43-1).There are major albeit relatively uncommon risks associated with these procedures, the most significant being a debilitating or fatal intracerebral hemorrhage; currently, these complications still occur in approximately 0.5–3% of cases.

Operations Performed For Movement Disorders

Deep Brain Stimulation

In the early 1990s, reports of the initial attempts to control tremor, utilizing high-frequency deep brain stimulation (DBS) of the thalamus’s Vim region, began to surface. Interestingly, this new approach developed before the resurgence of interest in pallidotomy. This procedure has continued to gather a following among movement disorder experts. DBS is currently the predominant treatment for medically refractory tremor (typically benign essential tremor, but also tremor-predominant PD).

Subsequently stimulation techniques were then reported in both the globus pallidus (Gpi) and the subthalamic nucleus (STN) for other indications. DBS has now also been tried in several other areas of the brain, though most experience is with stimulation in the Gpi and STN for PD and dystonia. Excellent relief of major parkinsonian symptoms, including rigidity, bradykinesia, tremor, and akinesia, can be achieved using DBS in the majority of patients with true PD. More recent publications, providing longer-term follow-up perspectives comparing either sham surgical controls or good historical validation are now available. These demonstrate a persistent superior efficacy of DBS for PD, tremor, and dystonia over destructive lesions such as thalamotomy or pallidotomy.

In all instances when indicated, DBS four-contact electrodes may be placed bilaterally. This also includes settings where prior thalamotomies or pallidotomies have been unsuccessful. However, the stimulating electrodes need to be placed in a healthy area and not one previously lesioned. The electrodes are brought out through the brain and secured at the skull edge. A battery is placed in a small subcutaneous pocket just under the clavicle and tunneled to the electrode with a connecting wire (see Fig. 43-1).

Patients with dystonia are increasingly receiving DBS as a primary therapeutic modality. The consensus to date indicates that Gpi DBS is the best site to achieve therapeutic success. Although these results are especially applicable for genetically positive DYT-1 patients, similar encouraging results are also documented in individuals with secondary dystonias. Thus, Gpi DBS has the potential to relieve most dystonic posturing, particularly in any or all four limbs. This modality is also proving to be valuable wherein there is dystonia of either the neck musculature and speech muscles. Drawbacks for utilization of DBS include the somewhat higher risks for infection and the need to reprogram the stimulation parameters, often over several months in the postoperative period.