Deep brain stimulation for treatment of refractory Tourette syndrome: long-term follow-up.

BACKGROUND: Eighteen patients with severe and refractory Tourette Syndrome underwent bilateral thalamic deep brain stimulation. The surgical procedures and stimulation processes of the cohort were reported in 2008; the 2 year follow-up was reported in 2009. The aim of the research is the assessment of long-term outcome (5-6 years) on tics, obsessional behaviours, anxiety, mood, and on the overall general health of the patients and their general satisfaction. METHOD: In this study, all 18 of the original patients will be discussed, pre- and post-DBS, according to our protocol using standardized objective schedules, as well as the clinical impressions of both clinicians and patients. As there were no substantial nor statistical differences on measures of cognitive functioning between pre-DBS and 2 year follow-up, we decided not to continue this aspect of the formal assessment, particularly as there were also no clinical indications. RESULTS: At 5-6 year follow-up, there was a significant reduction in tic severity (p < 0.001), and significant improvements in obsessive compulsive behaviours (p = 0.003), anxiety (p < 0.001) and depressive (p < 0.001) symptoms. Patients, in general, required less medication for tics, co-morbid conditions and/or co-existent psychopathologies. The long-term outcome/satisfaction were not unanimous between patients and the medical team. CONCLUSIONS: At long-term follow-up, DBS was very successful in terms of a significant improvement in tics and also a significant reduction in the potentially disabling symptoms of obsessionality, anxiety and depression. However, compared with our more positive overall results at 2 years, these later results demonstrate long-term difficulties as follows: non-compliance, long-term complications , and the differences in the opinions between the (a) medical, (b) the surgical teams and (c) the post-DBS patients as to their outcome/satisfaction with the procedures. Our experience highlights the need for controlled studies, for long-term follow up, and the need to improve the selection of patients for DBS.

A computational model to compare different investment scenarios for mini-stereotactic frame approach to deep brain stimulation surgery.

UNLABELLED: Deep brain stimulation (DBS) alleviates symptoms of many neurological disorders by applying electrical impulses to the brain by means of implanted electrodes, generally put in place using a conventional stereotactic frame. A new image guided disposable mini-stereotactic system has been designed to help shorten and simplify DBS procedures when compared to standard stereotaxy. A small number of studies have been conducted which demonstrate localization accuracies of the system similar to those achievable by the conventional frame. However no data are available to date on the economic impact of this new frame. AIM: The aim of this paper was to develop a computational model to evaluate the investment required to introduce the image guided mini-stereotactic technology for stereotactic DBS neurosurgery. METHODS: A standard DBS patient care pathway was developed and related costs were analyzed. A differential analysis was conducted to capture the impact of introducing the image guided system on the procedure workflow. The analysis was carried out in five Italian neurosurgical centers. RESULTS: A computational model was developed to estimate upfront investments and surgery costs leading to a definition of the best financial option to introduce the new frame. Investments may vary from Euro 1.900 (purchasing of Image Guided [IG] mini-stereotactic frame only) to Euro 158.000.000. Moreover the model demonstrates how the introduction of the IG mini-stereotactic frame doesn't substantially affect the DBS procedure costs.

Thalamic deep brain stimulation for treatment-refractory Tourette syndrome: two-year outcome.

BACKGROUND: Eighteen patients with severe and refractory Tourette syndrome (TS) underwent bilateral thalamic deep brain stimulation (DBS). OBJECTIVE: To assess the long-term outcome on tics, behavioral symptoms, and cognitive functions in the largest case series of thalamic DBS for TS to date. METHODS: In this prospective cohort study, 15 of the original 18 patients were evaluated before and after surgery according to a standardized protocol that included both neuropsychiatric and neuropsychological assessments. RESULTS: In addition to marked reduction in tic severity (p = 0.001), 24-month follow-up ratings showed improvement in obsessive-compulsive symptoms (p = 0.009), anxiety symptoms (p = 0.001), depressive symptoms (p = 0.001), and subjective perception of social functioning/quality of life (p = 0.002) in 15 of 18 patients. There were no substantial differences on measures of cognitive functions before and after DBS. CONCLUSIONS: At 24-month follow-up, tic severity was improved in patients with intractable Tourette syndrome (TS) who underwent bilateral thalamic deep brain stimulation. Available data from 15 of 18 patients also showed that neuropsychiatric symptoms were improved and cognitive performances were not disadvantaged. Controlled studies on larger cohorts with blinded protocols are needed to verify that this procedure is effective and safe for selected patients with TS. LEVEL OF EVIDENCE: This study provides class IV evidence that bilateral thalamic deep brain stimulation reduces global tic severity measured 24 months after implantation in patients with severe intractable Tourette syndrome.

Symptomatic pineal cyst: case report and review of the literature.

Asymptomatic cysts of the pineal region are common incidental findings in adults. In contrast, symptomatic pineal cysts are rare and their management is not well defined. We present the case of a 39-year-old woman suffering from intracranial hypertension, with visual disturbance and mild papilledema. The MR images showed a voluminous cyst of the pineal region responsible for an obstructive hydrocephalus. Endoscopic treatment with the aid of computerized neuronavigation consisting in third ventriculostomy and fenestration of the cyst was performed. Intracranial hypertension symptoms resolved in 24 hours. The one year follow-up cerebral MR images demonstrated the normalization of ventricular size with patency of the aqueduct of Sylvius. Reviewing the literature demonstrates that the endoscopic approach represents a minimally invasive and safe procedure in the treatment of symptomatic pineal cysts.

Deep brain stimulation in 18 patients with severe Gilles de la Tourette syndrome refractory to treatment: the surgery and stimulation.

BACKGROUND: There have been several reports of successful deep brain stimulation (DBS) for the treatment of severe Gilles de la Tourette syndrome (GTS). METHOD: 18 cases of GTS who were resistant to at least 6 months of standard and innovative treatments, as well as to psychobehavioural techniques, underwent DBS. DBS was placed bilaterally in the centromedian-parafascicular (CM-Pfc) and ventralis oralis complex of the thalamus. Patients were evaluated after surgery, with immediate and formal assessments at least every 3 months, including "on-off" and "sham off" in the first nine patients. RESULTS: All patients responded well to DBS, although to differing degrees. The duration of follow-up assessments ranged from 3 to 18 months. The comorbid symptoms of obsessive-compulsive behaviour, obsessive-compulsive disorder, self-injurious behaviours, anxiety and premonitory sensations decreased after treatment with DBS. There were no serious permanent adverse effects. CONCLUSIONS: DBS is a useful and safe treatment for severe GTS. The results of ours and previous DBS reports suggest that the CM-Pfc and ventralis oralis complex of the thalamus may be a good DBS target for GTS.

Effect of bilateral subthalamic electrical stimulation in Parkinson's disease.

BACKGROUND: Bilateral high frequency subthalamic stimulation has been reported to be effective in the treatment of Parkinson's disease and levodopa-induced dyskinesias. To analyze the results of this surgical procedure we critically reviewed 17 parkinsonian patients with advanced disease complicated by motor fluctuations and dyskinesias. METHODS: Between January 1998 and June 1999 these 17 consecutive patients (age 48-68 years; illness duration 8-27 years) underwent bilateral stereotactically guided implantation of electrodes into the subthalamic nucleus in the Department of Neurosurgery of the Istituto Nazionale Neurologico "C. Besta." Parameters used for continuous high-frequency stimulation were: frequency 160 Hz, pulse width 90 microsec, mean amplitude 2.05 +/- 0.45 V. Parts II and III of the UPDRS were used to assess motor performance before and after operation by the neurologic team. The follow-up ranged between 6 and 18 months. RESULTS: At latest examination, mean UPDRS II and III scores had improved by 30% (on stimulation, off therapy) with mean 50% reduction in daily off time. Peak dyskinesias and early morning dystonias also improved in relation to therapy reduction. Side effects were persistent postoperative supranuclear oculomotor palsy and postural instability in one case, worsened off-medication hypophonia in three, and temporary nocturnal confusion episodes in three. Postoperative MRI revealed a clinically silent intracerebral haematoma in one case. One electrode required repositioning. CONCLUSIONS: Continuous high frequency STN stimulation is an effective treatment for advanced PD. A functionally useful and safe electrode placement can be performed without microrecording.

Acute effects of bilateral subthalamic nucleus stimulation on clinical and kinematic parameters in Parkinson's disease.

After implantation with subthalamic stimulators, nine patients with advanced Parkinson's disease were studied on the task of tracing out, as accurately as possible, the four corners of a square with the dominant hand. The task was performed in four treatment conditions: on stimulation-off medication, off stimulation-off medication, off stimulation-on medication, and on stimulation-on medication. Movement times and peak velocities improved significantly only in the on stimulation-on medication condition compared to off stimulation-off medication. The improvement in clinical parameters with stimulation only (relative to off stimulation off medication) was of borderline significance, while consistent and significant clinical improvement was only obtained with addition of medication (on medication-on stimulation). This study provides quantitative evidence of the effect of subthalamic stimulation on kinematic measures in Parkinson's disease (PD) and suggests that combined treatment (medication and stimulation) is superior to either treatment alone.

Image guided neuroendoscopy for third ventriculostomy.

Third ventriculostomy has become an increasing popular procedure for the treatment of hydrocephalus of different aetiologies. Between october 1997 and october 1998, 17 patients (12 females, 5 males; 12-82 year-old; mean age 43) underwent image-assisted endoscopic third ventriculostomy for hydrocephalus at the Istituto Nazionale Neurologico "C.Besta" of Milano. There was no mortality and no long term morbidity. Neuronavigation has been found useful in selecting the safest trajectory to the target avoiding any traction on the foramen of Monro related structures and allowing the necessary mobility for fine adjustments under visual and "tactile" control when choosing the safest point to perform the stoma. According to our experience neuro-endoscopy and neuronavigation seems to be complementary in reaching easy, safe and successful results in the treatment of hydrocephalus of different origins.

Reversal of thalamic hand syndrome by long-term motor cortex stimulation.

The authors describe a case of complete recovery from the so-called "thalamic hand" syndrome following chronic motor cortex stimulation in a 64-year-old man suffering from poststroke thalamic central pain. As of the 2-year follow-up examination, the patient's dystonia and pain are still controlled by electrical stimulation. It is speculated that a common mechanism in which the thalamocortical circuit loops are rendered out of balance may sustain hand dystonia and central pain in this case of thalamic syndrome. To the authors' knowledge this is the first reported case of its kind.

Microvascular decompression for trigeminal neuralgia: comments on a series of 250 cases, including 10 patients with multiple sclerosis.

OBJECTIVE: To examine surgical findings and results of microvascular decompression (MVD) for trigeminal neuralgia (TN), including patients with multiple sclerosis, to bring new insight about the role of microvascular compression in the pathogenesis of the disorder and the role of MVD in its treatment. METHODS: Between 1990 and 1998, 250 patients affected by trigeminal neuralgia underwent MVD in the Department of Neurosurgery of the "Istituto Nazionale Neurologico C Besta" in Milan. Limiting the review to the period 1991-6, to exclude the "learning period" (the first 50 cases) and patients with less than 1 year follow up, surgical findings and results were critically analysed in 148 consecutive cases, including 10 patients with multiple sclerosis. RESULTS: Vascular compression of the trigeminal nerve was found in all cases. The recurrence rate was 15.3% (follow up 1-7 years, mean 38 months). In five of 10 patients with multiple sclerosis an excellent result was achieved (follow up 12-39 months, mean 24 months). Patients with TN for more than 84 months did significantly worse than those with a shorter history (p<0.05). There was no mortality and most complications occurred in the learning period. Surgical complications were not related to age of the patients. CONCLUSIONS: Aetiopathogenesis of trigeminal neuralgia remains a mystery. These findings suggest a common neuromodulatory role of microvascular compression in both patients with or without multiple sclerosis rather than a direct causal role. MVD was found to be a safe and effective procedure to relieve typical TN in patients of all ages. It should be proposed as first choice surgery to all patients affected by TN, even in selected cases with multiple sclerosis, to give them the opportunity of pain relief without sensory deficits.

Long-term follow-up of germinoma after stereotactic biopsy and brain radiotherapy: a cell kinetics study.

The primary aim of this study is to report the long-term outcome of pineal and suprasellar germinoma after stereotactic biopsy and whole brain radiotherapy. The second purpose is to report an investigation of the biological features and cell kinetics of this peculiar and enigmatic brain tumour. Of 34 supratentorial germ cell tumours diagnosed and treated between 1980 and 1993, 20 patients were found to be affected by true germinoma localized in the pineal and/or suprasellar regions. The diagnosis was achieved by stereotactic biopsy in all cases. In 14 patients, the potential proliferative activity of the tumour was investigated by (3H)thymidine in vitro binding and labelling index determination. Chorionic gonadotropin, alpha-fetoprotein and embryonal carcinoma antigen were negative in the cerebrospinal fluid of these patients. All but 1 patient underwent whole brain radiotherapy. Clinical and neuroradiological follow-up ranged between 3 and 13 years (mean 8). Complete clinical and neuroradiological recovery was achieved in all patients after treatment. Fatal recurrences owing to neuraxis dissemination occurred in three cases. The labelling index in the whole series ranged between 0.1 and 5% (median 2.5). Only syncytiotrophoblastic cells had proliferative activity, while none of the lymphoid-like cells showed thymidine labelling.

Transillumination in minimally invasive surgery for carpal tunnel release. Technical note.

An alternative technique for performing minimally invasive release of carpal tunnel syndrome is described. The suggested methodology is based on transillumination of the carpal tunnel during surgery. The advantages of the technique are discussed and compared with other available surgical procedures including endoscopy. The authors also describe preliminary operative results in 50 consecutive patients.

A neurophysiological method for the evaluation of motor performance in spastic walking patients.

Intrathecal baclofen is at present the best treatment for severe spasticity of various etiologies. In walking patients affected by severe spasticity a careful evaluation of the motor performance is needed for a correct indication for this treatment. The examination should focus on the delicate balance between spasticity and voluntary muscle activation which is crucial for an improvement of motor performance during gait. Seven patients have been neurophysiologically evaluated by the use of a Cibex apparatus measuring torque and movement velocity of the lower limbs simultaneously with static and dynamic recordings of the EMG.

Low-grade glial tumors in basal ganglia and thalamus: natural history and biological reappraisal.

The natural history of 70 patients affected by low-grade astrocytomas was recorded after the histological diagnosis was obtained by serial stereotactic biopsy. Forty-three percent of these patients died within 3 years. The value of cell kinetics assessment at the time of stereotactic biopsy was investigated, and the labeling index percent may be considered the most accurate prognostic factor in these histologically homogeneous astrocytomas. It has been confirmed that the young age of patients predicts a more favorable course, but the value of this also seems to be linked to and dependent on cell kinetics. These data are discussed in view of the opportunity to perform more aggressive "cytoreductive" treatments in deep brain tumors when these indices support an expected poor prognosis.

Role of stereotactic biopsy in multifocal brain lesions: considerations on 100 consecutive cases.

One hundred patients affected by multifocal brain lesions were investigated by serial stereotactic biopsy. Systemic diseases and primary neoplasms elsewhere were previously ruled out. The histological diagnosis obtained in this series comprises malignant gliomas in 37% of patients; primary non-Hodgkin's brain lymphoma in 15%; metastatic brain tumours in 15% (no evidence of the primary tumour at the time of stereotactic surgery); low grade gliomas in 12%; infective diseases in 10% (including brain abscesses and multifocal viral encephalitis); and ischaemic lesions in 6%. In addition, two patients with germinomas, two with primitive neuroepithelial tumours, two with multiple telangiectases, and one with a teratoma were also included in this series. Histological findings obtained by stereotactic procedures guided the choice of treatment, avoiding the risks of blind treatments. Indications and future perspectives for stereotactic surgery in multifocal brain lesions are discussed with emphasis on advances in diagnostic and therapeutic tools.

Italian multicentric study on pain treatment with epidural spinal cord stimulation.

A multicentric study on the treatment of nonmalignant chronic pain with epidural spinal cord stimulation (SCS) has been carried out in 32 Italian centers devoted to pain therapy. Neurosurgical and anesthesiology units participated in this retrospective study. 410 of the eligible patients were enrolled in the protocol: 48% were male, 52% female. All patients underwent a screening test period (average 21 days) and 74% underwent the definitive implant. The diagnosis was failed back surgery syndrome in 45%, reflex sympathetic dystrophy in 15%, phantom limb pain in 14%, postherpetic neuralgia in 8%, peripheral nerve injury in 5%, others 13%. 84% received noninvasive unsuccessful treatment (10 tensor acupuncture). All had previous pharmacological therapy which was not always discontinued when SCS took place. Pain assessment had been done with the visual analog scale and verbal scale both subjectively and by the physician and nurses. Neuropsychological profile with minimal mental test or MMPI was obtained in 68% of the patients. These results were favorable (i.e. excellent or good; more than 50% reduction of pain) in 87% of the patients at the 3-month follow-up, 75% at the 6-month follow-up, 69% at the 1-year follow-up, and 58% at the 2-year follow-up. Complication rate was: dislocation of the electrocatheter 4%, technical problems 3%, infections of the system 2%. The results will be discussed in correlation with the different etiologies of the nonmalignant chronic pain syndrome.

The treatment of spasticity by intrathecal administration of baclofen: a preliminary personal experience.

The administration of baclofen, a GABAb agonist, by direct infusion into the CSF by means of a programmable device, may avoid the undesired side effects of the oral administration of both the same and other antispastic drugs while giving a marked reduction of spasticity. The preliminary results on 12 patients show the total efficacy of this procedure in reducing spasticity markedly.

Epidural stimulation for peripheral vascular disease: 10 years experience.

105 patients suffering from arteriosclerotic peripheral vascular disease (PVD) underwent epidural spinal stimulation in the last 10 years. Before 1986 the main indication was ischemic pain without any consideration of trophic lesions (19 pts) while afterward the selection of the patients was more accurate in terms of disease's stage. The analysis of the data shows a more favourable results in the second group. Our experience suggest to use this technique in the treatment of patients in Fontaine's stage III and IV without necrosis and large trophic lesions.

Trigeminal neuralgia: new surgical strategies.

Presentation of the results of treatment in trigeminal neuralgia, using percutaneous radiofrequency coagulation in 712 cases, percutaneous microcompression in 206 cases, and microvascular decompression in 22 cases. Based on the results the following management strategy is proposed: pts. 65 years or younger = percutaneous balloon compression or, if neuroradiological evidence of neurovascular compression) is given, microvascular decompression. Pts. elder than 65 years = thermorhizotomy. It may be repeated in case of recurrence. If the initial operation was percutaneous compression, the second one should be microvascular decompression or, depending on age or other clinical circumstances of the patient, thermorhizotomy.