Extracranial Vertebral Artery Aneurysm Presenting as a Chronic Cervical Mass Lesion

Background. Aneurysms of the extracranial vertebral artery are rare and can provide a diagnostic and therapeutic challenge. Methods. We reviewed the clinical history of a patient presenting with cervical radiculopathy, who harboured an extracranial vertebral artery aneurysm eroding the cervical spine. Results. CT Angiography and MR Angiography set the diagnosis, by revealing a left C5-C6 vertebral artery aneurysm with cervical root impingement. Bony reconstruction depicted enlargement of the C6 transverse foramen and a marked enlargement of the C6-C7 intravertebral foramen. The lesion was treated by intravascular proximal vertebral artery occlusion. Conclusions. Extracranial vertebral artery aneurysms require a high index of clinical suspicion. This is the first report of a vertebral artery pseudoaneurysm presenting with bony erosion, which supports a less minacious portrayal of vertebral artery aneurysms

Malignant neuroleptic syndrome following deep brain stimulation surgery: a case report

<p>Abstract</p> <p>Background</p> <p>The neuroleptic malignant syndrome is an uncommon but dangerous complication characterized by hyperthermia, autonomic dysfunction, altered mental state, hemodynamic dysregulation, elevated serum creatine kinase, and rigor. It is most often caused by an adverse reaction to anti-psychotic drugs or abrupt discontinuation of neuroleptic or anti-parkinsonian agents. To the best of our knowledge, it has never been reported following the common practice of discontinuation of anti-parkinsonian drugs during the pre-operative preparation for deep brain stimulation surgery for Parkinson's disease.</p> <p>Case presentation</p> <p>We present the first case of neuroleptic malignant syndrome associated with discontinuation of anti-parkinsonian medication prior to deep brain stimulation surgery in a 54-year-old Caucasian man.</p> <p>Conclusion</p> <p>The characteristic neuroleptic malignant syndrome symptoms can be attributed to other, more common causes associated with deep brain stimulation treatment for Parkinson's disease, thus requiring a high index of clinical suspicion to timely establish the correct diagnosis. As more centers become eligible to perform deep brain stimulation, neurologists and neurosurgeons alike should be aware of this potentially fatal complication. Timely activation of the deep brain stimulation system may be important in accelerating the patient's recovery.</p

Pitfalls in the diagnosis of a tumefactive demyelinating lesion: A case report

<p>Abstract</p> <p>Introduction</p> <p>In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. We report a patient with a space-occupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as Balo concentric sclerosis and a glioma. This case report highlights important diagnostic clues in the differential diagnosis of Balo concentric sclerosis.</p> <p>Case presentation</p> <p>A 20-year-old Caucasian woman with acute onset of left-sided weakness and numbness was admitted to hospital with neurologic signs of left-sided hemiparesis and hypoesthesia. Brain magnetic resonance imaging showed a mass lesion of abnormal signal intensity with concentric enhancing rings in the right parietal lobe, without perifocal edema. The characteristic concentric pattern detected on the magnetic resonance images was highly suggestive of Balo disease, and corticosteroids were administered. Evoked potentials, cerebrospinal fluid analysis, and magnetic spectroscopy findings were not specific, and glioma was also included in the differential diagnosis. A stereotactic biopsy was not diagnostic.</p> <p>After one month the patient showed moderate clinical improvement, and during 12 months follow-up, no further relapses occurred. In the follow-up magnetic resonance imaging, the concentric pattern had completely disappeared, and only a low-signal, gliotic lesion remained.</p> <p>Conclusion</p> <p>We hope this case presentation will advance our understanding of clinical and radiologic appearance of Balo concentric sclerosis, which is a rare demyelinating disease. Although this is a specific entity, it has a broader clinical impact across medicine, because it must be differentiated from other space-occupying lesions in the central nervous system.</p

Factor XIII deficiency as a potential cause of supratentorial haemorrhage after posterior fossa surgery

Postoperative intracranial haemorrhage can be a dramatic event, carrying significant morbidity and mortality. Bleeding at sites remote from the operation area represents a small percentage of haemorrhages whose aetiology remains unclear (Harders et al. Acta Neurochir (Wien) 74(1-2):57-60, 1985). We present the case of a 60-year-old patient who underwent posterior fossa craniotomy for the removal of a space-occupying lesion and suffered supratentorial haemorrhage soon after the operation. A thorough postoperative investigation revealed low levels of factor XIII (FXIII), the factor mainly responsible for fibrin clot stabilisation. We suggest that reduced FXIII activity may be an important but preventable predisposing factor to remote postoperative haemorrhage in neurosurgical patients

Constant-Current Deep Brain Stimulation of the Globus Pallidus Internus in the Treatment of Primary Dystonia by a Novel 8-Contact (Octrode) Lead

OBJECTIVE: To evaluate bilateral constant-current globus pallidus internus (GPi) deep brain stimulation using an 8-contact lead. METHODS: This prospective, open-label, single-center pilot study of 10 patients assessed the feasibility of delivering bilaterally constant-current GPi deep brain stimulation with a novel 8-channel lead to treat primary dystonia using standard scales as outcome measures. RESULTS: Patients included 4 men and 6 women with a mean age of 35.8 years +/- 9.2 (range, 27-49 years). Mean age of onset was 18.5 years +/- 9.1 (range, 8-35 years), and mean disease duration was 17.3 years (range, 7-27 years). All had primary dystonia (8 generalized dystonia, 1 segmental dystonia, 1 focal dystonia). The primary variable was determined as 50% reduction in dystonia symptoms from baseline to the 6-month follow-up, as defined by the Burke-Fahn-Marsden Dystonia Rating Scale. Six patients (60.0%) achieved &gt;50% reduction in Burke-Fahn-Marsden Dystonia Rating Scale score and were classified as responders at the 6-month follow-up. Five of these 6 responders (83.3%) sustained that response through the assessment at the end of the first year. Constant-current stimulation was associated with significant improvement in pain and quality of life in all patients. Nearly 84% of the overall improvement occurred by the end of first month after stimulation onset, documenting an early response to treatment. Axial symptoms responded the best. CONCLUSIONS: Constant-current GPi deep brain stimulation proved safe and efficacious for treatment of primary dystonia. Motor scores improved by 54%, mostly within the first month. No phenotype-specific stimulation could be achieved, despite the capability of the new lead to stimulate specific loci within the GPi

Spinal synovial cysts: pathogenesis, diagnosis and surgical treatment in a series of seven cases and literature review

This study is designed based on the retrospective analysis of patients treated in the Neurosurgical Department of two major hospitals and review of the literature. The aim of this study is to evaluate the efficacy of surgery and address controversial issues in the treatment of symptomatic lumbar intraspinal synovial cysts. Spinal juxtafacet cysts (synovial and ganglion cysts) are a rare cause of low back and radicular leg pain. Although the relevant reports in the international literature are increasing, the controversy about conservative versus surgical treatment and the need for concomitant fusion still exists. Data from seven patients (age range 58-69 years, mean age 61 years) with low back and radicular leg pain due to a lumbar facet joint cyst were retrospectively analyzed. Demographic data, cyst level, presence of concomitant local pathology, treatment and results of treatment were recorded. A follow-up of at least 6 months (range 6-48 months) was conducted and results were noted. All patients had back pain, while five also experienced unilateral radicular leg pain and one had bilateral leg pain. One patient had neurogenic claudication. MRI identified the cyst and highlighted underlying pathology in all cases. All patients underwent surgical cyst excision. No fusion was performed. Post-operatively, all patients showed a total resolution of symptoms with sustained benefit at final evaluation. Review of the literature revealed a trend towards surgery, as this is correlated to a more favorable outcome compared with conservative treatment. Fusion should be performed on a case-by-case basis only. Surgery is a safe and effective treatment choice in this increasingly appearing ailment. A prospective, randomized trial should clarify issues under debate

Effect of constant-current pallidal deep brain stimulation for primary dystonia on cognition, mood and quality of life: Results from a prospective pilot trial

Objective: Knowledge on the effects of DBS on cognitive functions is limited and no data exists on the effects of constant-current DBS (CC-GPi-DBS), which appears to prevail over constant-voltage stimulation. Our aim was to prospectively assess the effect of Constant-Current-GPi-DBS, using an 8-contact lead, on cognition, mood and quality of life. Patients and Methods: Ten patients aged 27-49 underwent prospective neuropsychological assessment using dedicated tests. Various cognitive domains (intelligence, executive functions, memory, attention, visuo-spatial perception, verbal intelligence) as well as emotional state and quality of life were examined preoperatively and 1, 6 and 12 months after continuous constant-current DBS. Results: Patients performed preoperatively below average on information processing speed, phonemic verbal fluency and working memory. At 6-months there was an improvement in phonemic verbal fluency (p &lt; .05), which was retained at 12-months postoperatively (p = .05). Results also showed marginal improvement in the Trail Making-A test (p = .051) and the Stroop colour-word test (p &lt; .05). Despite improvement in Quality of Life (Physical and Mental Component improved by 32.42% and 29.46% respectively), patients showed no discernible change in anxiety and depression status. Conclusions: CC-GPi-DBS for primary dystonia has no discernible negative impact on cognition and mood. If anything, we noted an improvement of certain cognitive functions

Restoration of erect posture in idiopathic camptocormia by electrical stimulation of the globus pallidus internus

The authors report on 2 young patients who developed drug-resistant idiopathic dystonic camptocormia (bent spine) and were treated successfully by deep brain stimulation (DBS) of the globus pallidus internus (GPi). The first patient, a 26-year-old woman, suffered for 3 years from such severe camptocormia that she became unable to walk and was confined to bed or a wheelchair. The second patient, a 21-year-old man, suffered for 6 months from less severe camptocormia; he was able to walk but only for short distances with a very bent spine, the arms in a parallel position to the legs, and the hands almost approaching the floor to potentially support him in case of a forward fall. Within a few days following DBS, both patients experienced marked clinical improvement. At most recent follow-up (44 months in one case and 42 in the other), the patients’ ability to walk upright remained normal. Similar findings have only been reported recently in a few cases of camptocormia secondary to Parkinson disease or tardive dyskinesia. On the basis of the experience of these 2 idiopathic cases and the previously reported cases of secondary camptocormia with a favorable response to GPi DBS, the authors postulate that specific patterns of oscillatory activity in the GPi are vital for the maintenance of erect posture and the adoption of bipedal walking by humans. (DOI: 10.3171/2010.3.JNS09981