Deep brain stimulation (DBS) in the treatment of dystonic tremor is well established with targets including the venterointermediate nucleus (VIM), globus pallidus interna (GPi). More recently the subthalmic nucleus (STN) and zona incerta (ZI) have emerged as promising targets [[1]Crowell J.L. Shah B.B. Surgery for dystonia and tremor.Curr Neurol Neurosci Rep. 2016; 16: 22Crossref PubMed Scopus (15) Google Scholar]. DBS targeting the centromedian nucleus (CMN) has been found to be a safe and effective treatment for refractory generalised epilepsy [[2]Valentin A. Garcia Navarrete E. Chelvarajah R. Torres C. Navas M. Vico L. et al.Deep brain stimulation of the centromedian thalamic nucleus for the treatment of generalized and frontal epilepsies.Epilepsia. 2013; 54: 1823-1833Crossref PubMed Scopus (165) Google Scholar]. There is no body of evidence in the literature where dystonic tremor and refractory generalised epilepsy have been managed simultaneously and successfully by stimulation of the VIM. A 58-year-old right-handed female with juvenile myoclonic epilepsy (JME) from the age of twelve and positive family history of epilepsy suffered refractory seizures despite some control over the years with various combinations of anti-epileptic drugs in an epilepsy clinic. She suffered 2–3 seizures weekly. She first noticed a mild postural tremor her early 50's. Initially attributed to her valproate (VPA) use, it was cautiously reduced however her tremor did not improve but progressed over the years. Examination revealed an asymmetric tremor of the hands and mild dystonic head tremor. She was commenced on Levetiracetam (LVM) and referred to the movement disorders clinic as the tremor became intrusive into her activities of daily living. Further exam in the movement disorders clinic few months later, demonstrated a mild dystonic head tilt with an associated 'yes, yes' head tremor. She had evidence of some facial tremor but no jaw tremor and no voice tremor. In the limbs, a mild dystonic posturing of the left hand and coarse bilateral postural tremor grade 4/10 were observed. The tremor increased further on goal directed movements to 5/10 [[3]Bain P.G. Findley L.J. Atchison P. Behari M. Vidailhet M. Gresty M. Rothwell J.C. Thompson P.D. Marsden C.D. Assessing tremor severity.J Neurol Neurosurg Psychiatry. 1993; 56: 868-873Crossref PubMed Scopus (308) Google Scholar]. There was also a degree of postural tremor in her legs. Handwriting and spiral drawings were affected bilaterally (Fig. 1a). There was no rest component to the tremor, extra pyramidal or cerebellar features. She was diagnosed with a late onset and medically refractory dystonic tremor; now concurrent with her severe and refractory epilepsy. Her medications were adjusted; LVM was tapered and her VPA was slightly increased to 500 mg BD. She was referred to the functional surgery multidisciplinary meeting to consider DBS therapy for her tremor (Fig. 2).Fig. 2Images demonstrating her DBS lead implantation with VIM/ ZI target.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Months later, her condition progressed with further functional impairment impacting her ability to brush her teeth, eat and drink without spillage. She required significant assistance with her activities of daily living and reported social embarrassment and isolation. DBS leads were implanted bilaterally (Boston Scientific 8 contact) targeting the VIM and ZI. Within weeks of her DBS being switched on she had significant reduction in her tremor severity. At three year follow up review, she confirmed dramatic reduction into her seizure frequency (2 seizures in 3 years). The tremor was still controlled such that she can feed herself with demonstrable improvement in her Archimedes spirals (Fig. 1b) The limitations in this presentation include the fact that the diagnosis of epilepsy and observed response to DBS is based on clinical grounds. We have no historical EEG to justify assessment for electrophysiological resolution of seizure activity following implantation. Another feature is that her tremor did not worsen because of VPA alone as there were clear dystonic features present. The authors presumed the progression of the tremulous condition to be caused by a separate disease entity, namely late onset action/dystonic tremor. We are aware of a not too dissimilar case reported from Japan where DBS was effective in managing both Parkinson's disease and epilepsy (Hoshida et al.). We conclude that it can be reasonably assumed, that DBS targeting the VIM/ ZI successfully controlled our patient's refractory generalised epilepsy and disabling dystonic tremor. It adds merit to the argument that DBS can prove fruitful as a single intervention in managing two, co-existing and medically refractory conditions. None to declare.
When considering Deep Brain Stimulation (DBS) surgical treatment of dystonia syndromes, it is important to consider multiple aspects of the disease and its presentation. It is crucial to know if the dystonia is idiopathic, inherited or acquired as well as focal, segmental or generalised. Careful phenotyping of idiopathic as well as inherited dystonias and accurate diagnosis of acquired dystonias informs the decision-making process for patients and clinicians by providing them with useful predictors of outcomes of the proposed surgery. Here, we provided a review of the current literature, highlighted the areas where evidence is scarce and suggested future directions for research.
ObjectivesPercutaneous balloon compression (PBC) can be offered to medically refractory patients with trigeminal neuralgia who are unsuitable for microvascular decompression. Its associated with up to 4% risk of anaesthesia dolorosa which increases with duration and severity of compression and is more common with repeat procedures. We audited our outcomes for this procedure over last 7 years.DesignRetrospective audit of prospectively collected data.SubjectsAll patients undergoing PBC at our centre.MethodsTheatre and radiology records reviewed to identify patients. Case notes and radiology reviewed for history, diagnosis, details of procedure, immediate symptom relief, complications, further procedures and last follow up. Descriptive, comparative Kaplan Meir analysis undertaken.ResultsTotal 93 patients (4 b/l), 165 procedures. Average follow up 36 months. 24 patients had MS, 17 patients had atypical pain. All except 4 patients had good immediate pain relief. No patient developed anaesthesia dolorosa, 2 patients had transient diplopia, 1 maxillary hematoma and 1 infection. 56 experienced recurrence, 43 needed further surgical intervention. 25 PBC twice, 11 thrice, 4 four times and 1 five times. Average time to first recurrence=32 months. 85% pain free at 1 year and 70% at 2 years.ConclusionsPBC is an effective procedure. With conservative approach, this procedure can be safely repeated.
BACKGROUND:Parkinson's disease (PD) is a debilitating illness associated with considerable impairment of quality of life and substantial costs to health care systems. Deep brain stimulation (DBS) is an established surgical treatment option for some patients with advanced PD. The EARLYSTIM trial has recently demonstrated its clinical benefit also in patients with early motor complications. We sought to evaluate the cost-effectiveness of DBS, compared to best medical therapy (BMT), among PD patients with early onset of motor complications, from a United Kingdom (UK) payer perspective.METHODS:We developed a Markov model to represent the progression of PD as rated using the Unified Parkinson's Disease Rating Scale (UPDRS) over time in patients with early PD. Evidence sources were a systematic review of clinical evidence; data from the EARLYSTIM study; and a UK Clinical Practice Research Datalink (CPRD) dataset including DBS patients. A mapping algorithm was developed to generate utility values based on UPDRS data for each intervention. The cost-effectiveness was expressed as the incremental cost per quality-adjusted life-year (QALY). One-way and probabilistic sensitivity analyses were undertaken to explore the effect of parameter uncertainty.RESULTS:Over a 15-year time horizon, DBS was predicted to lead to additional mean cost per patient of £26,799 compared with BMT (£73,077/patient versus £46,278/patient) and an additional mean 1.35 QALYs (6.69 QALYs versus 5.35 QALYs), resulting in an incremental cost-effectiveness ratio of £19,887 per QALY gained with a 99% probability of DBS being cost-effective at a threshold of £30,000/QALY. One-way sensitivity analyses suggested that the results were not significantly impacted by plausible changes in the input parameter values.CONCLUSION:These results indicate that DBS is a cost-effective intervention in PD patients with early motor complications when compared with existing interventions, offering additional health benefits at acceptable incremental cost. This supports the extended use of DBS among patients with early onset of motor complications.
Background While there have been technical advances in endovascular treatment options for a wide variety of intracranial aneurysms not all aneurysms are suitable for coil embolization. Wide neck aneurysms at a bifurcation have remained technically challenging and coil retention is a valid concern. However, the PulseRider® (Pulsar Vascular, San Jose, CA USA) was recently introduced as an adjunctive device specifically designed to provide a scaffold at the neck of bifurcation aneurysms. The technology is specifically designed for complex bifurcations aneurysms to provide neck reconstruction and coil support. It provides coverage only where it is needed allowing for a low metal to artery ratio; this along with intraluminal patency is a significant advantages over current Y-stenting. Methods This is a review of data on eight (8) patients that had anterior and two (2) that had posterior circulation aneurysms. There were eight (8) females and two (2) males and the average age was 51 (range 41 to 62). The aneurysms were referred to as wide-neck when the dome/neck ratio was 1.5 and/or neck length was 4 mm. Results Overall, 10 patients were treated with the PulseRider device. Of the 10 patients treated two (2) had basilar artery aneurysms that had ruptured more than six (6) years prior and required retreatment. One patient had subarachnoid hemorrhage prior to treatment of a ruptured basilar apex and left middle cerebral artery (MCA) aneurysms but treatment with the PulseRider was for a right unruptured MCA. One patient with an Acom that had ruptured and was treated six (6) months prior had recurrence on follow-up. An unsuccessful attempted clipping was followed by endovascular treatment with PulseRider and coils. One other patient had residual aneurysm on one (1) week follow-up angiogram post craniotomy and was then treated with PulseRider and coils. All but one patient were started on dual anti-platelet therapy (Aspirin and Clopidogrel) prior to endovascular treatment. Because a decision was made to use the PulseRider intra-operatively in one patient there was an adjustment in heparin dosage and intravenous (IV) Aspirin 500 mg. was givenFollow-up angiography was done at 6 months on 2 patients and complete or near-complete (>95%) embolization was achieved in all patients and the branch vessels remained patent. The PulseRider adapted easily to the geometry of the aneurysm and the branch vessels allowing placement inside the aneurysm, in the branch vessels or in a hybrid manner with one side of the arch in the aneurysm and the other in a branch vessel. There was technical success related to placement of the device in all cases. All procedures were completed without complications. There were no neurological deficits. Conclusion Although this is a small series preliminary results are quite promising with good angiographic occlusion of the aneurysms. It will be necessary however, to obtain long term results on a larger series of patients in order to prove the safety and efficacy of this device as a treatment option. Disclosures H. Nahser: 6; C; support for Travel to meetings by various companies. A. Chandran: 6; C; Travel expenses to conferences by various companies. M. Puthuran: 6; C; travel expenses to conferences by various companies. P. Eldridge: 6; C; travel expenses to conferences by various companies. T. Patankar: 6; C; travel expenses to conferences by various companies. T. Goddard: 6; C; Travel expenses to conferences by various companies.
Dural arteriovenous fistulae at the falx and at the an terior and posterior margin of the tentorium cerebelli drain into cortical veins exclusively and their most common mode of presentation is haemorrhage. The concept of dural fistulae that involve smaller sinuses at the tent and falx was introduced by PICARD (1990, Journal of Neuroradiologie 17,161–181). We have retrospectively analysed a series of patients treated in between 2002 and 2012. 12 were found to be located at the falx and 30 located at the tent. One patient with an unruptured fistula died before treatment because of a haemorrhage. All other patients except one were treated initially with embolisation via feeding meningeal arteries with Onyx. This lead to radioanatomic cure in all but sixth patients. In the completely obliterated we observed that during this single feeder injection of Onyx 20 or 34 led to penetration to the origin of the first draining vein at the tentorial or falcine sinus and could then, with continued injection, retrogradely penetrate into the distal parts of the other feeding arteries leading to complete arterial disconnection from the venous side. In two patients for safety reasons to block retrograde embolisation of the internal carotid artery via the artery of Bernasconi, the internal carotid artery was temporarily blocked with a balloon at the origin of this branch during injection. One patient primarily and 3 others were then successfully occluded by surgery, the remaining by an endovascular transvenous approach with injection of Onyx. No lethal or complications with persistent disabling morbidity were recorded. Disclosures H. Nahser: 6; C; Support for Travel to meetings by various companies. M. Puthuran: 6; C; Support for Travel by various companies. A. Chandran: None. M. Javadpour: 6; C; Support for Travel to meetings by various companies. P. Eldridge: 6; C; support for Travel to meetings by various companies.
Two patients with Parkinson's disease (PD) treated successfully with subthalamic nucleus deep brain stimulation (STN-DBS) for 3-4 years are reported, who demonstrated a persistent improvement following removal of STN-DBS for late infection. Possible hypotheses are discussed--whether a microlesioning effect or a disease-modifying effect of STN-DBS, though neither adequately explain this phenomenon.