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  • Stereotactic lesional surgery for the treatment of tremor in multiple sclerosis: a prospective case-controlled study.

    8 December 2017

    The effect of stereotactic lesional surgery for the treatment of tremor in multiple sclerosis was examined in a prospective case-controlled study. Surgery was not undertaken in 33 patients (72% of 46 cases referred for stereotactic surgery), two of whom died within 4 months of referral. Twenty-four multiple sclerosis patients were included in the study; 13 underwent surgery and were matched against 11 controls on the basis of age, sex, expanded disability system scores (EDSS) and disease duration. Assessments were carried out at baseline/preoperatively, and then 3 and 12 months later; these included accelerometric and clinical ratings of tremor, spirography, handwriting, a finger-tapping test, nine-hole peg test, tremor-related disability, general neurological examination, Barthel Activities of Daily Living (ADL) Index of general disability, EDSS, a 0-4 ataxia scale, Mini-Mental State (MMS) examination, speech and swallowing assessments and grip strength. Postoperative MRI scans demonstrated that tremor could be attenuated by lesions centred on the thalamus in seven cases, on the zona incerta in five cases and in the subthalamic nucleus in one case. Two patients developed hemiparesis and in two cases epilepsy recurred. Two surgical patients and one control patient died between the 3 and 6 months assessments. Both groups had a significant deterioration in EDSS but not Barthel ADL Index scores at 1 year, but the difference between the groups was not significant. Similarly, no differences between the groups' rates of deterioration of speech or swallowing or MMS were found. Significant improvements in contralateral upper limb postural (P2) and kinetic tremors, spiral scores and head tremor were detected at 3 and 12 months after surgery (but not handwriting or nine-hole peg test performance). Tremor-related disability and finger-tapping speed were also significantly better 12 months after surgery, the latter having significantly worsened for the control group. A 3 Hz 'filter' for postural (P2) upper limb tremor was detected by accelerometry/spectral analysis above which tremor was always abolished and at or below which some residual tremor invariably remained. Criteria for selecting multiple sclerosis patients for this form of surgery are discussed.

  • Possible seizure suppression via deep brain stimulation of the thalamic ventralis oralis posterior nucleus.

    12 December 2017

    Surgical treatment of intractable epilepsy with deep brain stimulation (DBS) has been shown to be of therapeutic benefit in some patients and with the recent publication of a randomised control study its use is likely to increase in the future. We describe a patient who developed a focal epileptic seizure within a few seconds of momentarily turning off the DBS stimulator in the nucleus ventralis oralis posterior, with which she was successfully treated for tremor. The seizure was the result of a newly diagnosed primary brain tumor. We suggest that the nucleus ventralis oralis posterior may be another thalamic target of DBS in epilepsy.

  • A block to pre-prepared movement in gait freezing, relieved by pedunculopontine nucleus stimulation.

    12 December 2017

    Gait freezing and postural instability are disabling features of Parkinsonian disorders, treatable with pedunculopontine nucleus stimulation. Both features are considered deficits of proximal and axial musculature, innervated predominantly by reticulospinal pathways and tend to manifest when gait and posture require adjustment. Adjustments to gait and posture are amenable to pre-preparation and rapid triggered release. Experimentally, such accelerated release can be elicited by loud auditory stimuli--a phenomenon known as 'StartReact'. We observed StartReact in healthy and Parkinsonian controls. However, StartReact was absent in Parkinsonian patients with severe gait freezing and postural instability. Pedunculopontine nucleus stimulation restored StartReact proximally and proximal reaction times to loud stimuli correlated with gait and postural disturbance. These findings suggest a relative block to triggered, pre-prepared movement in gait freezing and postural instability, relieved by pedunculopontine nucleus stimulation.

  • Post-deep brain stimulation--gradual non-stimulation dependent decrease in strength with attenuation of multiple sclerosis tremor.

    12 December 2017

    Tremor in multiple sclerosis is considered to be a persistent and progressive sign. We describe five patients with multiple sclerosis in whom upper limb tremor severity gradually decreased over a period of several years after deep brain stimulation. In every case this attenuation of tremor was accompanied by increasing pyramidal weakness in the relevant upper limb. In two patients this attenuation of tremor remained after stimulation was permanently switched off. In one other patient, where upper limb strength remained normal, tremor severity gradually worsened in spite of continuing stimulation. There was a highly significant difference (p = 0.0007) between the changes in intention tremor severities when the arms with increasing pyramidal weakness (n = 9) were compared to those in which normal strength was retained throughout follow-up period (n = 3); intention tremor decreased in the former and increased in the latter by means of -3.66 and +4.0 points of a 0-10 tremor scale respectively. There was also a significant correlation (0.699; p = 0.0359) between decreasing upper limb strength and decreasing intention tremor severity for the upper limbs of patients that had undergone contralateral DBS.

  • Physiological and harmonic components in neural and muscular coherence in Parkinsonian tremor.

    12 December 2017

    OBJECTIVE: To differentiate physiological from harmonic components in coherence analysis of the tremor-related neural and muscular signals by comparing power, cross-power and coherence spectra. METHODS: Influences of waveform, burst-width and additional noise on generating harmonic peaks in the power, cross-power and coherence spectra were studied using simulated signals. The local field potentials (LFPs) of the subthalamic nucleus (STN) and the EMGs of the contralateral forearm muscles in PD patients with rest tremor were analysed. RESULTS: (1) Waveform had significant effect on generating harmonics; (2) noise significantly decreased the coherence values in a frequency-dependent fashion; and (3) cross-spectrum showed high resistance to harmonics. Among six examples of paired LFP-EMG signals, significant coherence appeared at the tremor frequency only, both the tremor and double tremor frequencies and the double-tremor frequency only. CONCLUSIONS: In coherence analysis of neural and muscular signals, distortion in waveform generates significant harmonic peaks in the coherence spectra and the coherence values of both physiological and harmonic components are modulated by extra noise or non-tremor related activity. SIGNIFICANCE: The physiological or harmonic nature of a coherence peak at the double tremor frequency may be differentiated when the coherence spectra are compared with the power and in particular the cross-power spectra.

  • Phantom limb pain relieved with different modalities of central nervous system stimulation: a clinical and functional imaging case report of two patients.

    12 December 2017

    Phantom limb pain (PLP) is a type of neuropathic pain syndrome that has evoked much interest in scientific and clinical fields. The condition is often intractable and severely debilitating. Though there are anecdotal reports in the literature of successful management of individual cases with brain and spinal cord stimulation, it has not been possible to develop a system of management that is consistently successful, mainly due to the paucity of basic neurophysiological data about PLP and its pathways in the central nervous system (CNS). Functional imaging offers a way of collecting information about the basic mechanisms and pathways of PLP from patients without the excessive risk of more invasive penetrating electrode studies or the questionable reliability of animal data. There have been very few studies that have looked at the direct effect of CNS stimulation on regional brain activation and correlation with the pain state. We describe two cases of PLP that have been satisfactorily treated with CNS stimulation (motor cortex and then periventricular gray in one and spinal cord in the other) and have been subjected to single photon emission computed tomography (SPECT) studies when in pain and then during stimulation with recorded pain relief. We found that regardless of the site of stimulation in the CNS, pain relief is associated with blood flow changes in similar areas of the brain, mainly the parietal and cingulate cortex and also in the thalamic nuclei and the central gray matter. Further studies of this kind should reveal more about the complex mechanisms of PLP and other forms of neuropathic pain.

  • Unilateral and bilateral pallidotomy for idiopathic Parkinson's disease: a case series of 115 patients.

    12 December 2017

    Lesioning of the internal pallidum is known to improve the symptoms of idiopathic Parkinson's disease (PD) and alleviate dyskinesia and motor fluctuations related to levodopa therapy. The benefit obtained contralateral to a single lesion is insufficient in some cases when symptoms are bilaterally disabling. However, reports of unacceptably high rates of adverse effects after bilateral pallidotomy have limited its use in such cases. We report on the outcome of unilateral (UPVP) and bilateral (BPVP) posteroventral pallidotomy in a consecutive case series of 115 patients with PD in the United Kingdom and Australia. After 3 months, UPVP resulted in a 27% reduction in the off medication Part III (motor) Unified Parkinson's Disease Rating Scale score and abolition of dyskinesia in 40% of cases. For BPVP, these figures were increased to 31% and 63%, respectively. Follow-up of a smaller group to 12 months found the motor scores to be worsening but benefit to dyskinesia and activities of daily living was maintained. Speech was adversely affected after BPVP, although the change was small in most cases. Unilateral and bilateral pallidotomy can be performed safely without microelectrode localisation. Bilateral pallidotomy appears to be more effective, particularly in reducing dyskinesia; in our experience, the side effects have not been as high as reported by other groups.

  • Early postoperative management of DBS in dystonia: programming, response to stimulation, adverse events, medication changes, evaluations, and troubleshooting.

    7 December 2017

    Early postoperative management in deep brain stimulation-treated patients with dystonia differs from that of patients with essential tremor and Parkinson's disease, mainly due to the usually delayed effects of deep brain stimulation and the heterogenous clinical manifestation and etiologies of dystonia. The present chapter summarizes the available data about and concentrates on practical clinical aspects of early postoperative management in deep brain stimulation-treated patients with dystonia.

  • Long-Term management of DBS in dystonia: response to stimulation, adverse events, battery changes, and special considerations.

    12 December 2017

    Multiple independent case series have documented sustained benefit of bilateral pallidal deep brain stimulation (DBS) up to 3 years in patients with primary dystonia. Growing evidence exists for positive outcomes extending up to 10 years. The beneficial effects from DBS are usually reported to be stable, thus requiring little long-term modifications of the parameters of stimulation. Speech and swallowing abnormalities are less responsive than other dystonic symptoms. Symptom exacerbation after initial benefit has been reported in a few cases. It is not known whether this is related to potential tolerance or habituation to stimulation or to progression of the underlying disease. Failures of pallidal DBS, at least in primary dystonia patients, should not be accepted without further re-evaluation of each individual case, including possible revisions of the electrode location. Both hardware- and stimulation-related adverse effects, including insufficient relief of speech function, have been reported in the long-term. Despite early reports suggesting that hardware problems might be more frequent in dystonia, more recent studies did not confirm these observations. In patients with severe segmental (e.g., axial) or generalized dystonia, sudden cessation of stimulation may become a medical emergency and should be anticipated changing the neurostimulator before its natural end of life.