Pallidal Deep Brain Stimulation in Patients with Prior Bilateral Pallidotomy and Selective Peripheral Denervation for Treatment of Dystonia.

INTRODUCTION: Deep brain stimulation (DBS) of the globus pallidus internus has become an accepted treatment for severe isolated idiopathic and inherited dystonia. Patients who had other forms of surgery earlier, such as radiofrequency lesioning or selective peripheral denervation, however, usually are not considered candidates for DBS. OBJECTIVE: The aim of this study was to evaluate the long-term outcome of pallidal DBS in a rare subgroup of patients who had undergone both pallidotomy and selective peripheral denervation previously with a waning effect over the years. METHODS: Pallidal DBS was performed according to a prospective study protocol in 2 patients with isolated idiopathic dystonia, and patients were followed for a period of at least 6 years. RESULTS: Both patients benefitted from long-lasting amelioration of dystonia after pallidal DBS, which was comparable to that of patients who did not have previous surgeries. In a 62-year-old female with cervical dystonia both the Burke-Fahn-Marsden (BFM) and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) motor scores were improved at follow-up 8 years after surgery (50 and 39%). In a 32-year-old male with generalized dystonia, the BFM motor and disability scores showed marked improvement at 6.5 years of follow-up (82 and 66%). CONCLUSIONS: Pallidal DBS can yield marked and long-lasting improvement in patients who underwent both pallidotomy and selective peripheral denervation earlier. Therefore, such patients, in general, should not be excluded from DBS.

The dimensions of "failed back surgery syndrome": what is behind a label?

BACKGROUND: The term failed back surgery syndrome (FBSS) has been criticized for being too unspecific and several studies have shown that a variety of conditions may underlie this label. The aims of the present study were to describe the specific symptoms and to investigate the primary and secondary underlying causes of FBSS in a contemporary series of patients who had lumbar spinal surgery before. METHODS: We used a multilevel approach along three different axes defining symptomatic, morphological, and functional pathology dimensions. RESULTS: Within the study period of 3 years, a total of 145 patients (74 f, 71 m, mean age 51a, range 32-82a) with the external diagnosis of FBSS were included. Disk surgery up to 4 times and surgery for spinal stenosis up to 3 times were the commonest index operations. Most often, the patients complained of low back pain (n = 126), pseudoradicular pain (n = 54), and neuropathic pain (n = 44). Imaging revealed osteochondrosis (n = 61), spondylarthrosis (n = 48), and spinal misalignment (n = 32) as the most frequent morphological changes. The majority of patients were assigned at least to two different symptomatic subcategories and morphological subcategories, respectively. According to these findings, one or more functional pathologies were assigned in 131/145 patients that subsequently enabled a specific treatment strategy. CONCLUSIONS: FBSS has become rather a vague and imprecisely used generic term. We suggest that it should be avoided in the future both with regard to its partially stigmatizing connotation and its inherent hindering to provide individualized medicine.

Body weight gain in patients with bilateral deep brain stimulation for dystonia.

In patients with Parkinson's disease, significant weight gain following chronic deep brain stimulation (DBS) has been reported. Recently, relevant weight gain could be demonstrated also following subthalamic nucleus DBS in patients with primary cervical dystonia. Prospective analyses of body weight changes following DBS in patients with dystonia, however, have not been published so far. We aimed to analyse the changes of body weight following DBS in patients with dystonia. The body mass index (BMI) of 17 consecutive patients with segmental or generalised dystonia (mean age 54.6 ± 16.1 years) treated with bilateral DBS of the globus pallidus internus (GPi) (n = 14) or the thalamic ventral intermediate nucleus (n = 3) was measured preoperatively (pre-OP) and at three follow-up (FU) time points post-DBS surgery (FU1 = 7 months, FU2 = 17 months, FU3 = 72 months). All patients benefited from marked improvement in their dystonia. The mean BMI pre-OP (SD) was 22.5 (±3.7) kg/m(2) and increased stepwise to 24.0 (±3.3) kg/m(2) at FU1, 24.4 (±3.7) kg/m(2) at FU2 and 24.9 (±3.7) kg/m(2) at FU3 (p < 0.05 at all three FUs compared to pre-OP). Relative BMI increase and improvement of dystonia were correlated (p = 0.025). Chronic bilateral GPi DBS in patients with dystonia is associated with significant body weight gain, in particular during the first 6 months post-OP. This probably is a result of improvement of dystonic motor symptoms and recovery of eating dysfunction rather than a target-specific phenomenon.

Bilateral deep brain stimulation for cervical dystonia in patients with previous peripheral surgery.

BACKGROUND: There are no data available concerning whether patients with cervical dystonia who have recurrent or new symptoms after peripheral denervation surgery benefit similarly from pallidal deep brain stimulation compared with patients who receive primarily pallidal stimulation. METHODS: Data on 7 cervical dystonia patients with recurrent or progressive dystonia after peripheral denervation who underwent pallidal stimulation were prospectively collected. Deep brain stimulation was performed in Mannheim/Hannover, Germany, or in Umea, Sweden. To the subgroup from Mannheim/Hannover, a second group of patients without previous peripheral surgery was matched. Assessments included the Toronto Western Spasmodic Torticollis Rating Scale and the Burke-Fahn-Marsden dystonia rating scale, as well as the Tsui scale in the Swedish patients. RESULTS: The 4 patients from Mannheim/Hannover experienced sustained improvement from pallidal stimulation by a mean of 57.5% according to the Toronto Western Spasmodic Torticollis Rating Scale (P < .05) and by a mean of 69.5% according to the Burke-Fahn-Marsden dystonia rating scale (P < .05) at long-term follow-up of 40.5 months. The patients from Umea had a mean Tsui score of 7 prior to surgery and a mean score of 3 at the mean follow-up of 8 months (62.5%). In the matched group the Toronto Western Spasmodic Torticollis Rating Scale improved by 58.8% and the Burke-Fahn-Marsden dystonia rating scale by 67% (P < .05) at long-term follow-up (mean, 41.5 months). CONCLUSIONS: Patients who had prior peripheral surgery for cervical dystonia experience improvement from subsequent pallidal stimulation that is comparable to that of de novo patients.

Stereotactic drainage of empyema of the cavum septi pellucidi et vergae.

BACKGROUND: Persistent midline ventricular cavae may only rarely cause clinical symptoms. Exceptionally, empyemas may develop in these cavae. Optimal treatment has been defined only poorly so far. METHODS: Here, we report successful treatment of a bacterial empyema in the cavum septi pellucidi et vergae due to sphenoid sinus sinusitis in a 36-year-old woman by stereotactic puncture and drainage of the empyema and long-term administration of antibiotics. RESULTS AND CONCLUSIONS: Stereotactic puncture and drainage accompanied by antibiotic therapy result in beneficial outcome in the long term. Transcallosal interhemispheric approaches and free-hand techniques should be discouraged since, according to previously published reports, they may result in severe morbidity or mortality in this condition.

Micrographia induced by pallidal DBS for segmental dystonia: a subtle sign of hypokinesia?

Recently parkinsonism has been reported as a rare side effect of globus pallidus internus (GPi) deep brain stimulation (DBS) for dystonia. In the present systematic prospective study in 11 patients with segmental dystonia not affecting distal arm function, we could demonstrate significant changes in handwriting characterized by mild micrographia following GPi-DBS. We propose that this finding reflects GPi-DBS-induced disturbances of basal ganglia function in terms of a mild hypokinetic syndrome, as a result of outflow alterations in pallido-thalamo-cortical pathways.

Subcutaneous peripheral neurostimulation for the treatment of severe chronic poststernotomy neuralgia.

OBJECTIVE: Poststernotomy pain frequently develops after sternotomy in thoracic and cardiovascular surgery, and may affect patients' quality of life. In some cases of severe poststernotomy neuralgia, pharmacologic therapy does not provide adequate relief. CASE REPORT: We report on a 42- year-old woman who underwent sternotomy for aortic and mitral valve replacement. She developed severe chronic poststernotomy neuralgia that was refractory to medical treatment. After local anesthesia markedly but only transiently alleviated pain, we considered the option of subcutaneous peripheral neurostimulation (SPNS). Plate electrodes were implanted bilaterally in the parasternal region at the site of maximal pain. After a period of test stimulation, the electrodes were connected to a dual-channel implantable pulse generator. SPNS induced paresthesias in the painful area. Revision surgery was necessary twice because of electrode migration. Chronic SPNS markedly alleviated pain (visual-analog scale, VAS, 9/10 preoperatively, 2/10 postoperatively) and allodynia (VAS 9/10 preoperatively, 2/10 postoperatively) at the last available follow-up, 15 months postoperatively. CONCLUSIONS: SPNS may be a viable treatment option in patients with severe chronic poststernotomy neuralgia.

Stereotactic versus endoscopic surgery in periventricular lesions.

OBJECT: Endoscopic and stereotactic surgery have gained widespread acceptance as minimally invasive tools for the diagnosis of intracerebral pathologies. We investigated the specific advantages and disadvantages of each technique in the assessment of periventricular lesions. METHOD: This study included a retrospective series of 70 patients with periventricular lesions. Endoscopic surgery was performed in 17 patients (mean age, 37 years; range, 4 months-78 years) and stereotactic biopsy in 55 patients (mean age, 63 years; range, 23-80 years), including two patients who underwent both procedures. RESULTS: Hydrocephalus was present in 13/17 patients in the endoscopic group (77%) and in 11/55 patients in the stereotactic group (20%). Diagnosis was achieved in all patients in the endoscopic group and in all but one patient in the stereotactic group, in whom histological diagnosis was obtained by endoscopic biopsy during a second operation. In the endoscopic group, additional procedures performed included ventriculostomy (2/17), cyst fenestration (3/17), endoscopic shunt revision (3/17) and placement of Rickham reservoirs or external cerebrospinal fluid drains (6/17). Adverse events occurred in one patient after endoscopy (chronic subdural hematoma) and in two patients after stereotactic surgery (one mild hemiparesis and one transitory paresis of the contralateral leg). CONCLUSIONS: Endoscopic and stereotactic surgery have distinct advantages and disadvantages in approaching periventricular lesions. The advantages of endoscopy encompass the possibility to perform additional surgical procedures during the same session (e.g. tumour reduction, third ventriculostomy, fenestration of a cyst). The visual control reduces the hazard of injury to anatomical structures and allows for a better control of bleeding although there is a considerable blind-out in such situations. The advantages of stereotactic surgery include a smaller approach and precise planning of the trajectory. It is usually performed under local anaesthesia. Both methods provide a safe and efficient therapeutic option in periventricular lesions with low surgical-related morbidity.

Chronic deep brain stimulation in patients with tardive dystonia without a history of major psychosis.

Tardive dystonia usually occurs with a delay after neuroleptic exposure in patients with major psychosis. A subgroup of patients, however, is given such medication for "mild depression" or "neurasthenia." Tardive dystonia, in general, may respond favorably to pallidal deep brain stimulation (DBS). Nevertheless, it remains unclear thus far whether or not similar beneficial outcome is achieved with pallidal DBS in different subgroups of patients with tardive dystonia. Four women (mean age 59 years at surgery) underwent stereotactic pallidal DBS in the frame of an observational study. Tardive dystonia occurred secondary to medication with fluspirilene and haloperidol, and injection of long-acting depot neuroleptics prescribed for mild depression or "nervousness." Assessment included the Burke-Fahn-Marsden (BFM) scale preoperatively and at 12 months follow-up. Extended follow-up was available at a mean of 27.3 months postoperatively (range 16-36 months). There were no surgically related complications. All 4 patients experienced sustained statistically significant benefit from pallidal DBS. Mean improvement at 12 months was 77% for the BFM motor score (range, 45-91%; P = 0.043), and 84% at the last available follow-up (range, 70-91%; P = 0.03). This was paralleled by improvement of the BFM disability score. Chronic pallidal DBS in patients with tardive dystonia without a history of major psychosis provides sustained improvement which is similar to that in other subgroups of patients with tardive dystonia. This effect is stable on extended follow-up for up to 3 years.

Stuttering induced by thalamic deep brain stimulation for dystonia.

We report a patient in whom deep brain stimulation of the ventral intermediate nucleus of the thalamus (Vim) for treating dystonia reversibly induced stuttering at suboptimal stimulation parameters. Adjustments of stimulation parameters resulted in excellent control of dystonic motor symptoms and complete resolution of speech dysfluency. This is the first report on stuttering as an adverse effect of Vim stimulation which is primarily used to treat tremors of various etiologies.

Microvascular decompression of the anterior inferior cerebellar artery for intermediate nerve neuralgia.

The authors present the case of a 63-year-old woman with a 5-year history of intractable paroxysmal 'atypical' otofacial pain. The patient's pain attacks were not typical for either trigeminal or vagoglossopharyngeal neuralgia. Surgical exploration via a suboccipital retromastoid craniotomy showed vascular compression of the nervus intermedius by the anterior inferior cerebellar artery and the patient's pain was successfully managed with microvascular decompression.

Less is more: adaptation of voltage after battery replacement in deep brain stimulation for dystonia.

BACKGROUND: To maintain the efficacy of deep brain stimulation (DBS) on dystonic symptoms, slight incremental increase in voltage may be necessary over years after a steady state has been reached following the initial programming of optimal settings. So far however, no data are available regarding the adjustment of voltage after implantable pulse generator (IPG) replacement to achieve sustained optimal control of dystonia with the least side effects. METHODS: We analyzed stimulation settings before and after IPG replacement for battery depletion (n = 61) in 18 patients with chronic DBS of the globus pallidus internus or the ventral intermediate nucleus of the thalamus for dystonia. RESULTS: The stimulation intensity could be significantly reduced by 24.8% after IPG replacement. The amount of voltage reduction was equal between bipolar and monopolar stimulation modes (24.9 vs. 24.3%, p = 0.89) and significantly correlated with the magnitude of stimulation intensity before IPG replacement (ρ = 0.429, p = 0.001). CONCLUSIONS: After IPG replacement, in patients with DBS for segmental dystonia the voltage can be reduced by approximately 25%. This phenomenon might be explained by a gradual decrease in the electrical energy effectively delivered by the IPG in the course of the lifetime of the battery or neuroplastic processes in particular in the period around battery replacement.

Treatment of recurrent trigeminal neuralgia due to Teflon granuloma.

Recurrent trigeminal neuralgia after microvascular decompression (MVD) may be due to insufficient decompression, dislocation of the implant to pad the neurovascular contact, or the development of granuloma. Here, we report on our experience with Teflon granuloma including its treatment and histopathological examination. In a series of 200 patients with trigeminal neuralgia MVD was performed with Teflon felt according to Jannetta's technique. In three patients with recurrent facial pain Teflon granuloma was found to be the cause for recurrence. In each instance, the granuloma was removed for histopathological examination. Mean age at the first procedure was 62.3 years and at the second procedure 66.3 years. Recurrence of pain occurred between 1 and 8.5 years after the first procedure. MRI scans demonstrated local gadolineum enhancement in the cerebellopontine angle, and CT scans showed local calcification. Intraoperatively dense fibrous tissue was found at the site of the Teflon granuloma. Histopathological examination revealed foreign body granuloma with multinuclear giant cells, collagen-rich hyalinized scar tissue, focal hemosiderin depositions, and microcalcifications. The Teflon granuloma was completely removed, and a new Teflon felt was used for re-decompression. Patients were free of pain after the second procedure at a mean of 40.3 months of follow-up. Teflon granuloma is a rare cause for recurrent facial pain after MVD. Small bleeding into the Teflon felt at surgery might trigger its development. A feasible treatment option is surgical re-exploration, nerve preserving removal of the granuloma, and repeat MVD.

Rapid response of parkinsonian tremor to STN-DBS changes: direct modulation of oscillatory basal ganglia activity?

Although deep brain stimulation (DBS) of the subthalamic nucleus (STN) has proved to be effective for tremor and other cardinal symptoms in Parkinson's disease (PD), the precise mechanisms of action of DBS are still unclear. We analyzed the time course of resting tremor amplitude and frequency during discontinuation and subsequent reinitiation of STN-DBS in nine PD patients, using a computerized three-dimensional motion analysis combined with surface electromyography. Following discontinuation of STN-DBS, resting tremor amplitude rapidly increased, reaching maximum amplitude after 2 min (mean +/- 95%CI: 34.3 +/- 13.8 mm; P < 0.01), subsequently stabilizing at a medium level. Reinitiation of stimulation after 30 min resulted in rapid, nearly complete suppression of tremor activity within 1 min (1.4 +/- 1.3 mm; P < 0.01) and, furthermore, increased tremor frequency within a few seconds in seven of nine patients. These findings support the hypothesis that STN-DBS acts by direct interference with the neurotransmission of basal ganglia networks involved in tremor.

Chronic deep brain stimulation for segmental dystonia.

Fourteen consecutive patients with segmental dystonia underwent chronic deep brain stimulation (DBS) surgery in the frame of a prospective study protocol. Twelve patients received chronic pallidal stimulation, while 2 patients with prominent dystonic tremor received chronic thalamic ventrointermediate nucleus stimulation. Twelve patients had primary dystonia, and 2 patients secondary dystonia. The Burke-Fahn-Marsden dystonia rating scale (BFM motor) showed a mean relative improvement of 57.3% at the first follow-up (FU1, mean 7 months) and 57.8% at the second follow-up (FU2, mean 16 months). The mean BFM scores were 34.9 +/- 17.7 preoperatively, 14.9 +/- 11.7 at FU1, and 14.8 +/- 10.3 at FU2. Scores of the disability subscale improved by 43% at FU1 and 36% at FU2. Improvement was comparatively less in those patients with secondary dystonia. Dysarthria was a limitation of DBS in 4 patients when using high voltage. Overall, chronic DBS is a very effective treatment option for medically refractory segmental dystonia.

Impact of surgical treatment on tremor due to posterior fossa tumors.

OBJECT: The object of this study was to investigate the impact of surgical treatment on tremor caused by posterior fossa tumors. METHODS: The authors performed a retrospective evaluation of 6 cases involving patients with tremors due to posterior fossa tumors. Patients who had been treated with neuroleptic medication or had a family history of movement disorders were excluded. All patients had postural or kinetic tremors. Tremor was mainly unilateral. The study group included 5 women and 1 man. Mean age at surgery was 59 years. Five patients underwent total or subtotal tumor resection, and 1 patient underwent stereotactic biopsy only. The histological diagnosis was epidermoid tumor in 2 patients, metastasis in 2 others, and vestibular schwannoma and low-grade glioma in 1 each. RESULTS: Two patients had no improvement of tremor, postoperatively. In both of these patients the tumor (low-grade glioma in 1, metastasis in the other) involved the dentate nucleus directly. In the other patients, a compressive effect on the dentate nucleus or the dentatothalamic pathways was present without invasion of the cerebellar structures, and immediate or gradual amelioration of the tremor was observed postoperatively. CONCLUSIONS: The prognosis of tremor due to posterior fossa tumors appears to depend mainly on the involvement of tremor-generating structures. The prognosis appears to be favorable in those patients with compression of these substrates, whereas primary invasion by tumor has a poor prognosis. Caution must be used in generalizing the findings of this study because of the small number of cases in the series.

Failure of long-term nerve root stimulation to improve neuropathic pain.

OBJECT: Stimulation of dorsal nerve roots or dorsal root ganglia was reported to alleviate neuropathic pain in selected patients during the early postoperative period. A prospective study was initiated to investigate long-term outcome in patients with neuropathic pain of the lower extremities or groin who were treated with selective nerve root stimulation. METHODS: The study included patients with dermatomally distributed neuropathic pain who were > 18 years of age and in whom the pain was refractory to medical treatment. The patients were prospectively evaluated using a visual analog scale (VAS) for pain and ratings for quality of life, activities of daily living, and depression preoperatively, and after defined intervals postoperatively. Implantation of electrodes was performed via foraminotomy or interlaminar fenestration in an awake procedure. An implantable pulse generator (IPG) was implanted in a second operation after successful test stimulation performed over several days. RESULTS: Three patients were included in the study before it was stopped. The mean maximum pain score preoperatively was 9.3. All patients had successful test stimulation with > 50% pain relief prior to implantation of the IPG (mean maximum VAS Score 3.6). The beneficial effect, however, was lost within the next few months despite adjustment of stimulation settings. With higher amplitudes, side effects such as pain attacks or motor phenomena occurred. They disappeared after stopping stimulation, but neuropathic pain recurred to its full extent. The study was stopped 18 months after the first implantation, when the third and last IPG of this series was explanted. Due to the overall short-term effect of stimulation, no relevant changes in ratings for quality of life, activities of daily living, or depression were detected. CONCLUSIONS: Spinal nerve root stimulation proved to be effective on short-term follow-up in 3 patients with neuropathic pain in a dermatomal distribution. Long-term stimulation, however, was disappointing because of the loss of effectiveness and the occurrence of side effects.

Cavernous angioma of the geniculate ganglion.

Intracranial extraaxial cavernous angiomas are rare vascular malformations. Their occurrence at the geniculate ganglion of the facial nerve within the temporal bone is exceptional. The authors describe a 35-year-old man who developed a slowly progressing facial palsy. Initial cranial MR imaging showed no pathological findings, but 2 years later another MR examination detected a small tumor located at the geniculate ganglion of the facial nerve. The tumor was removed via a subtemporal approach. Histological examination revealed a cavernous angioma. Even small cavernomas located at the geniculate ganglion of the facial nerve may result in facial palsy. Isolated facial palsy in a young person should be monitored closely using imaging studies even if the initial imaging study is negative. Early decompression of the facial nerve may help to preserve its function.

Occipital nerve stimulation for refractory occipital pain after occipitocervical fusion: expanding indications.

BACKGROUND: Occipital nerve stimulation is being used for various pain syndromes. Here, we expand its use for the treatment of refractory occipital pain after occipitocervical fusion. CASE DESCRIPTION: We describe a case of occipital neuralgia in a 60-year-old man following posterior occipitocervical fusion. The maximum pain intensity was rated 9/10 on the visual analogue scale (VAS). Since pain proved to be refractory to analgetic medication, two quadripolar electrodes (Resume II, Medtronic) were implanted in the occipital region to stimulate the occipital nerve bilaterally. The patient experienced a dramatic response during test stimulation for 10 days with externalized electrodes, and a pacemaker (Synergy, Medtronic) was connected to the electrodes. While on chronic stimulation (bipolar 6 V, 210 mus, 130 Hz) improvement of pain was maintained, reflected by a decrease in the VAS score to 1/10 at 12 months of follow-up. CONCLUSION: Occipital nerve stimulation for medical refractory occipital neuralgia after occipitocervical fusion is an effective method expanding the indications for its use.

Neurogenic pulmonary edema in a fatal case of subarachnoid hemorrhage.

Neurogenic pulmonary edema (NPE) is caused by a variety of central nervous system lesions and may appear as a subclinical complication. The fulminant form of NPE is always life-threatening. Many pathophysiologic mechanisms have been implicated in the development of NPE, but the exact interaction remains unknown. We report a case of a fulminant NPE with fatal consequences associated with a subarachnoid hemorrhage. Treatment focuses on ventilatory support and measures to reduce intracranial pressure.