Impressive neurological recovery following C-spine dislocation in a patient on long term immunosuppression.

We report excellent neurological improvement in a patient with C6/C7 dislocation following a high speed road traffic accident. This case in particular is unusual because the patient was the recipient of an organ transplant during childhood and was therefore on long term immunosuppressant medication at the time of injury. In this report we reflect on the role of steroid use in traumatic spinal cord injury and put our case within the context of current evidence and this unusual clinical scenario.

Idiopathic intracranial hypertension and bariatric surgery: a literature review and a presentation of two cases.

OBJECTIVE: There is paucity of data concerning the impact of bariatric surgery on idiopathic intracranial hypertension (IIH). We therefore present the clinical course of two cases that were managed at our centre, which is a regional referral centre for bariatric surgery and present a literature review of patients with IIH who underwent bariatric surgery for the treatment of obesity. METHOD: Two patients were referred for bariatric surgery who were both morbidly obese and were diagnosed with IIH. One patient underwent bariatric surgery; the other patient's weight was managed conservatively. We followed up both patients for 2 years. PubMed was searched for published studies of patients with IIH undergoing bariatric surgery. RESULTS: The patient that underwent weight loss surgery achieved sustained weight loss from 108kg to 87kg and gross resolution of their symptoms. The patient whose weight was managed conservatively lost weight initially and then put the weight back on. Her IIH symptoms persisted despite CSF shunt therapy. The literature review identified 12 original studies, containing 51 patients. Weight loss after bariatric surgery was reported for 84% of patients and improvement or resolution of IIH symptoms was reported for 84% of patients. CONCLUSION: In light of the literature review and our experience, bariatric surgery appears to be a potential treatment option for IIH alongside the comorbidities associated with obesity. However, more robust studies are needed.

Autonomic Impairment in Severe Traumatic Brain Injury: A Multimodal Neuromonitoring Study.

OBJECTIVES: Autonomic impairment after acute traumatic brain injury has been associated independently with both increased morbidity and mortality. Links between autonomic impairment and increased intracranial pressure or impaired cerebral autoregulation have been described as well. However, relationships between autonomic impairment, intracranial pressure, impaired cerebral autoregulation, and outcome remain poorly explored. Using continuous measurements of heart rate variability and baroreflex sensitivity we aimed to test whether autonomic markers are associated with functional outcome and mortality independently of intracranial variables. Further, we aimed to evaluate the relationships between autonomic functions, intracranial pressure, and cerebral autoregulation. DESIGN: Retrospective analysis of a prospective database. SETTING: Neurocritical care unit in a university hospital. SUBJECTS: Sedated patients with severe traumatic brain injury. MEASUREMENTS AND MAIN RESULTS: Waveforms of intracranial pressure and arterial blood pressure, baseline Glasgow Coma Scale and 6 months Glasgow Outcome Scale were recorded. Baroreflex sensitivity was assessed every 10 seconds using a modified cross-correlational method. Frequency domain analyses of heart rate variability were performed automatically every 10 seconds from a moving 300 seconds of the monitoring time window. Mean values of baroreflex sensitivity, heart rate variability, intracranial pressure, arterial blood pressure, cerebral perfusion pressure, and impaired cerebral autoregulation over the entire monitoring period were calculated for each patient. Two hundred and sixty-two patients with a median age of 36 years entered the analysis. The median admission Glasgow Coma Scale was 6, the median Glasgow Outcome Scale was 3, and the mortality at 6 months was 23%. Baroreflex sensitivity (adjusted odds ratio, 0.9; p = 0.02) and relative power of a high frequency band of heart rate variability (adjusted odds ratio, 1.05; p < 0.001) were individually associated with mortality, independently of age, admission Glasgow Coma Scale, intracranial pressure, pressure reactivity index, or cerebral perfusion pressure. Baroreflex sensitivity showed no correlation with intracranial pressure or cerebral perfusion pressure; the correlation with pressure reactivity index was strong in older patients (age, > 60 yr). The relative power of high frequency correlated significantly with intracranial pressure and cerebral perfusion pressure, but not with pressure reactivity index. The relative power of low frequency correlated significantly with pressure reactivity index. CONCLUSIONS: Autonomic impairment, as measured by heart rate variability and baroreflex sensitivity, is significantly associated with increased mortality after traumatic brain injury. These effects, though partially interlinked, seem to be independent of age, trauma severity, intracranial pressure, or autoregulatory status, and thus represent a discrete phenomenon in the pathophysiology of traumatic brain injury. Continuous measurements of heart rate variability and baroreflex sensitivity in the neuromonitoring setting of severe traumatic brain injury may carry novel pathophysiological and predictive information.

Fragile X-associated tremor/ataxia syndrome treated with multitarget deep brain stimulation.

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a progressive hereditary neurodegenerative disorder which causes intention tremor and cerebellar ataxia. It typically affects the ageing population. Deep brain stimulation (DBS) is widely accepted in the treatment of common movement disorders and has been trialled in treating rare and complex neurodegenerative disorders. We report a case of a man in his 40s with a long history of tremor affecting his hands. MRI brain revealed high T2 signal in the middle cerebellar peduncles. Genetic testing revealed FMR1 premutation confirming the diagnosis of FXTAS. Subsequently, he was treated with multitarget DBS of the ventralis intermediate nucleus and ventralis oralis posterior nuclei bilaterally, with excellent neurological function at 9 years follow-up. This case suggests multitarget DBS for FXTAS with neurophysiology-guided DBS programming can provide excellent long-term tremor suppression in selected patients.

Tophaceous spinal gout in a young man with complete spinal cord injury.

Gout is a common inflammatory arthritis, caused by monosodium urate crystal deposition in the joints. Gout typically affects peripheral joints but less commonly it affects the spine. We report a case of a man in his 30s with chronic gout who presented acutely with thoracic back pain and flaccid paralysis of his lower limbs. CT showed abnormal facet joints at T8/9 with lucency suggestive of septic arthritis and MRI showed signal changes suggestive of epidural collection. The patient underwent emergency spinal surgery to evacuate the collection, followed by broad-spectrum antibiotic treatment. Cultures were negative; however, histopathology was suggestive of gout tophi. The patient was subsequently treated for spinal gout with prednisolone, allopurinol and colchicine and saw significant improvement in symptoms though he sustained a complete spinal cord injury. This case demonstrates that spinal gout is an important differential to consider in patients with chronic gout, presenting with acute back pain.

Multimodality imaging in transcatheter aortic valve implantation and post-procedural aortic regurgitation: comparison among cardiovascular magnetic resonance, cardiac computed tomography, and echocardiography.

OBJECTIVES: The purpose of this study was to determine imaging predictors of aortic regurgitation (AR) after transcatheter aortic valve implantation (TAVI) and the agreement and reproducibility of cardiovascular magnetic resonance (CMR), cardiac computed tomography (CCT), and transthoracic echocardiography (TTE) in aortic root assessment. BACKGROUND: The optimal imaging strategy for planning TAVI is unclear with a paucity of comparative multimodality imaging data. The association between aortic root morphology and outcomes after TAVI also remains incompletely understood. METHODS: A total of 202 consecutive patients assessed by CMR, CCT, and TTE for TAVI were studied. Agreement and variability among and within imaging modalities was assessed by Bland-Altman analysis. Postoperative AR was assessed by TTE. RESULTS: Of the 202 patients undergoing TAVI assessment with both CMR and TTE, 133 also underwent CCT. Close agreement was observed between CMR and CCT in dimensions of the aortic annulus (bias, -0.4 mm; 95% limits of agreement: -5.7 to 5.0 mm), and similarly for sinus of Valsalva, sinotubular junction, and ascending aortic measures. Agreement between TTE-derived measures and either CMR or CCT was less precise. Intraobserver and interobserver variability were lowest with CMR. The presence and severity of AR after TAVI were associated with larger aortic valve annulus measurements by both CMR (p = 0.03) and CCT (p = 0.04) but not TTE-derived measures (p = 0.10). Neither CCT nor CMR measures of annulus eccentricity, however, predicted AR after TAVI (p = 0.33 and p = 0.78, respectively). CONCLUSIONS: In patients undergoing imaging assessment for TAVI, the presence and severity of AR after TAVI were associated with larger aortic annulus measurements by both CMR and CCT, but not TTE. Both CMR and CCT provide highly reproducible information in the assessment of patients undergoing TAVI.