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.

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.

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.

Long-term results of percutaneous retrogasserian thermorhizotomy for "essential" trigeminal neuralgia: considerations in 1000 consecutive patients.

Long-term results (average follow-up, 9.3 years) obtained in 1000 consecutive patients suffering from cryptogenetic ("essential") trigeminal neuralgia treated with percutaneous thermorhizotomy are analyzed. Pain relief was obtained in 95% of the treated patients. Permanent morbidity was as follows: masseter weakness in 105 patients; oculomotor palsies in 5 patients; weakening of the corneal reflex in 197 patients, 6 of whom requested an ocular operation for keratitis; and painful dysesthesia in 52 patients, 15 of whom developed a painful anesthesia syndrome. There was a recurrence rate of 18.1%, and a correlation between postoperative sensory deficit and the cure rate was found. These results are discussed and compared to the results obtained with different techniques.

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.

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.

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.

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.

Familial frontal lobe oligodendroglioma. Case report.

Two cases of frontal bilateral oligodendroglioma invading the corpus callosum occurred in a 56-year old man and his 32-year old son. CT images of both patients are presented.

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.

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.

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.

Stereotactic neurosurgery in the treatment of tremor.

The results of stereotactical thalamotomy in 40 adult patients suffering from tremor of different etiology are presented. A combination of lesions of VOA-VOP-ZI seems to be optimal. Early results have been excellent in 63%, good in 23%, fair in 6% and poor in 8%. Long-term follow-up showed a negative shift with 40% excellent, 6% good and 54% poor results. In Parkinson disease with predominant tremor relief of this invalidating symptom can be achieved. But L-dopa therapy must be continued and surgical treatment does not stop the general disease progression.

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.

Treatment of deep brain abscesses by stereotactic implantation of an intracavitary device for evacuation and local application of antibiotics.

Complete recovery from deep brain abscesses was achieved in four patients treated by a specialized stereotactic method. In one patient the lesion was in the right thalamus, in two patients within the brain stem and in one case in the right rolandic cortex. The technique consists in the stereotactic implantation of a chronic intracavitary catheter connected to a subcutaneous reservoir to allow postoperative multiple evacuations and local antibiotic irrigations. Serial CT scan examinations guided the timing of intracavitary treatment and the removal of the catheter. No recurrence developed. The diagnostic and therapeutic advantages of this stereotactic technique are emphasized.

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.

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.