The authors report the case of an adult patient with irreducible fixed dystonia (inward rotation) of the right foot that arose after cardioembolic ischemia of the left putamen and globus pallidus externus. Given the resistance of such symptomatology to all of the attempted conservative treatments (including botulinum toxin), the authors decided to perform deep brain stimulation, positioning the intracerebral electrode in the left internal capsule at the level of the motor fibers controlling the right foot, as confirmed by intraoperative electromyography. After the intervention, the patient was able to perform voluntary movements of outward rotation and abduction in the right foot and begin gait rehabilitation. Deep brain stimulation of the posterior limb of the internal capsule could be an alternative target used to treat poststroke fixed dystonic conditions.
Angelo Franzini, Giuseppe Messina, Carlo Marras, Franco Molteni, Roberto Cordella, Paola Soliveri and Giovanni Broggi
Angelo Franzini, Giuseppe Messina, Roberto Cordella, Carlo Marras and Giovanni Broggi
The aim of this study was to review the indications for and results of deep brain stimulation (DBS) of the posterior hypothalamus (pHyp) in the treatment of drug-refractory and severe painful syndromes of the face, disruptive and aggressive behavior associated with epilepsy, and below-average intelligence. The preoperative clinical picture, functional imaging studies, and overall clinical results in the literature are discussed.
All patients underwent stereotactic implantation of deep-brain electrodes within the pHyp. Data from several authors have been collected and reported for each clinical entity, as have clinical results, adverse events, and neurophysiological characteristics of the pHyp.
The percentage of patients with chronic cluster headache who responded to DBS was 50% in the overall reported series. The response rate was 100% for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and for chronic paroxysmal hemicrania, although only 2 patients and 1 patient, respectively, have been described as having these conditions.
None of the 4 patients suffering from refractory neuropathic trigeminal pain benefited from the procedure (0% response rate), whereas all 5 patients (100%) affected with refractory trigeminal neuralgia (TN) due to multiple sclerosis (MS) and undergoing pHyp DBS experienced a significant decrease in pain attacks within the first branch of cranial nerve V. Six (75%) of 8 patients presenting with aggressive behavior and mental retardation benefited from pHyp stimulation; 6 patients were part of the authors' series and 2 were reported in the literature.
In carefully selected patients, DBS of the pHyp can be considered an effective procedure for the treatment of refractory trigeminal autonomic cephalalgias, aggressive behavior, and MS-related TN in the first trigeminal branch. Only larger and prospective studies along with multidisciplinary approaches (including, by necessity, neuroimaging studies) can lead us to better patient selection that would reduce the rate of nonresponders.
Paolo Ferroli, Marco Schiariti, Roberto Cordella, Carlo Boffano, Simone Nava, Emanuele La Corte, Claudio Cavallo, Dario Bauer, Melina Castiglione, Morgan Broggi, Francesco Acerbi and Giovanni Broggi
Surgery of brainstem lesions is increasingly performed despite the fact that surgical indications and techniques continue to be debated. The deep pons, in particular, continues to be a critical area in which the specific risks related to different surgical strategies continue to be examined. With the intention of bringing new knowledge into this important arena, the authors systematically examined the results of brainstem surgeries that have been performed through the lateral infratrigeminal transpontine window.
Between 1990 and 2013, 29 consecutive patients underwent surgery through this window for either biopsy sampling or for removal of a deep pontine lesion. All of this work was performed at the Department of Neurosurgery of the Istituto Nazionale Neurologico "Carlo Besta", in Milan, Italy. A retrospective analysis of the findings was conducted with the intention of bringing further clarity to this important surgical strategy.
The lateral infratrigeminal transpontine window was exposed through 4 different approaches: 1) classic retrosigmoid (15 cases), 2) minimally invasive keyhole retrosigmoid (10 cases), 3) translabyrinthine (1 case), and 4) combined petrosal (3 cases). No deaths occurred during the entire clinical study. The surgical complications that were observed included hydrocephalus (2 cases) and CSF leakage (1 case). In 6 (20.7%) of 29 patients the authors encountered new neurological deficits during the immediate postoperative period. All 6 of these patients had undergone lesion removal. In only 2 of these 6 patients were permanent sequelae observed at 3 months follow-up. These findings show that 93% of the patients studied did not report any permanent worsening of their neurological condition after this surgical intervention.
This retrospective study supports the idea that the lateral infratrigeminal transpontine window is both a low-risk and safe corridor for either biopsy sampling or for removal of deep pontine lesions.
Francesco Acerbi, Morgan Broggi, Marica Eoli, Elena Anghileri, Claudio Cavallo, Carlo Boffano, Roberto Cordella, Lucia Cuppini, Bianca Pollo, Marco Schiariti, Sergio Visintini, Chiara Orsi, Emanuele La Corte, Giovanni Broggi and Paolo Ferroli
Fluorescein, a dye that is widely used as a fluorescent tracer, accumulates in cerebral areas where the blood-brain barrier is damaged. This quality makes it an ideal dye for the intraoperative visualization of high-grade gliomas (HGGs). The authors report their experience with a new fluorescein-guided technique for the resection of HGGs using a dedicated filter on the surgical microscope.
The authors initiated a prospective Phase II trial (FLUOGLIO) in September 2011 with the objective of evaluating the safety of fluorescein-guided surgery for HGGs and obtaining preliminary evidence regarding its efficacy for this purpose. To be eligible for participation in the study, a patient had to have suspected HGG amenable to complete resection of the contrast-enhancing area. The present report is based on the analysis of the short- and long-term results in 20 consecutive patients with HGGs (age range 45–74 years), enrolled in the study since September 2011.
In all cases fluorescein (5–10 mg/kg) was injected intravenously after intubation. Tumor resection was performed with microsurgical technique and fluorescence visualization by means of BLUE 400 or YELLOW 560 filters on a Pentero microscope.
The median preoperative tumor volume was 30.3 cm3 (range 2.4–87.8 cm3). There were no adverse reactions related to fluorescein administration. Complete removal of contrast-enhanced tumor was achieved in 80% of the patients. The median duration of follow-up was 10 months. The 6-months progression-free survival rate was 71.4% and the median survival was 11 months.
Analysis of these 20 cases suggested that fluorescein-guided technique with a dedicated filter on the surgical microscope is safe and allows a high rate of complete resection of contrast-enhanced tumor as determined on early postoperative MRI. Clinical trial registration no.: 2011-002527-18 (EudraCT).
Carlo Efisio Marras, Michele Rizzi, Flavio Villani, Giuseppe Messina, Francesco Deleo, Roberto Cordella and Angelo Franzini
Hypothalamic hamartomas (HHs) are developmental malformations associated with a range of neurological problems, including intractable seizures. There is increasing evidence of the epileptogenicity of the hamartoma and of the inhomogeneous distribution of the epileptic abnormalities within the malformation. The management strategy for treatment and results differ according to the insertion plane and the extension of the malformation into the hypothalamus. Cases characterized by extensive involvement of the hypothalamus are particularly challenging.
The authors describe the case of a patient with drug-resistant epilepsy and a large hypothalamic hamartoma with an extensive area of attachment. The patient underwent implantation of 2 deep brain electrodes. The intraoperative recording showed a synchronous interictal epileptic discharge in the left temporal lobe and on the left side of the lesion. The patient was treated with chronic high-frequency stimulation. No side effects due to the stimulation were reported. At 18 months' follow-up, a reduction in complex partial seizure frequency was reported, but no significant reduction in overall seizure frequency was noticed (p = 0.14, t-test).
The authors report on neurophysiological studies of the relationship between HH and epilepsy, and also discuss the literature on chronic high-frequency stimulation, including its rationale and the results of chronic stimulation of various targets for the treatment of drug-resistant epilepsy due to HH.