This report describes an unusual fungal infection of an intrathecal baclofen pump which, to our knowledge, has not been reported previously. We describe a 39-year-old man with severe lower limb spasticity due to secondary progressive multiple sclerosis that was managed with insertion of an intrathecal baclofen pump. He subsequently presented with distinct neurological decline secondary to an intrathecal baclofen pump infection with Aspergillus terreus.

The preoperative evaluation of patients with intracranial aneurysms typically includes a contrast-enhanced vascular study, such as computed tomography angiography (CTA), magnetic resonance angiography (MRA), or digital subtraction angiography. However, there are numerous absolute and relative contraindications to the administration of imaging contrast agents, including pregnancy, severe contrast allergy, and renal insufficiency. Evaluation of patients with contrast contraindications thus presents a unique challenge. We identified three patients with absolute contrast contraindications who presented with intracranial aneurysms. One patient was pregnant, while the other two had previous severe anaphylactic reactions to iodinated contrast. Because of these contraindications to intravenous contrast, we performed non-contrast time-of-flight MRA with 3D reconstruction (TOF MRA with 3DR) with maximum intensity projections and volume renderings as part of the preoperative evaluation prior to successful open surgical clipping of the aneurysms. In the case of one paraclinoid aneurysm, a high-resolution non-contrast CT scan was also performed to assess the relationship of the aneurysm to the anterior clinoid process. TOF MRA with 3DR successfully identified the intracranial aneurysms and adequately depicted the surrounding microanatomy. Intraoperative findings were as predicted by the preoperative imaging studies. The aneurysms were successfully clip-obliterated, and the patients had uneventful post-operative courses. These cases demonstrate that non-contrast imaging is a viable modality to assess intracranial aneurysms as part of the surgical planning process in patients with contrast contraindications. TOF MRA with 3DR, in conjunction with high-resolution non-contrast CT when indicated, provides adequate visualization of the microanatomy of the aneurysm and surrounding structures.

The clinical pattern and angiographic distribution of cerebral atherosclerosis varies among different ethnic groups. This study was designed to identify the clinical background for intracranial and extracranial atherosclerotic stenosis in older eastern Chinese patients using digital subtraction angiography. We retrospectively reviewed the data collected from the Nanjing Stroke Registry Program, from January 2004 to March 2011. The analysis focused on the intracranial or extracranial location of stenosis in the anterior and posterior circulations. In total, records of 1041 patients were included in the study. Of these patients, 19.88% had intracranial carotid stenosis, 18.73% had stenosis in the extracranial vessels, and 33.33% had concurrent stenoses. A total of 2002 stenotic sites were detected in 749 patients. Among those patients with stenosis, a single stenosis was found in 170 (16.33%) and multiple stenoses were found in 579 (55.62%). The prevalence of intracranial stenosis in the single-stenosis group was 54.12%, while in the multiple-stenosis group it was 47.87% (p=0.127). A higher incidence of severe stenosis (70-99% blockage) and occlusion was found in the intracranial vessels than in the extracranial vessels (p=0.018). Older Chinese patients with atherosclerotic stenosis tend to have more intracranial stenoses.

Left atrial (LA) enlargement is a predictor of cardiovascular outcomes such as atrial fibrillation (AF), stroke, and death. The aim of this study was to explore the relationship between LA size and cognitive function in elderly patients without any signs of clinical dementia, AF or previous stroke. We assessed the cognitive status and LA volume (LAV) of 108 consecutive patients (27 males; mean age, 74.8±6.9years) with sinus rhythm. Cognitive status was assessed by the Mini Mental State Examination (MMSE). Patients with a MMSE score of ⩽25 were considered to have cognitive impairment. LAV was measured with two-dimensional echocardiography. LAV index (LAVI) was obtained by indexing LAV to body surface area. Thirty-five patients (32.4%) had cognitive impairment. The patients with cognitive impairment had significantly larger left atria than the patients with normal cognitive function. Receiver operating characteristic analysis revealed a cut-off point of ⩾34mL/m(2) for LAVI to predict patients with cognitive impairment (sensitivity, 97.1%; specificity, 52.1%; positive predictive value, 49.3%; negative predictive value, 97.4%). LAVI⩾34mL/m(2) was significantly associated with cognitive impairment (p=0.001, odds ratio=36.91, 95% confidence interval=4.8-284.2). Logistic regression analysis revealed that LAVI⩾34mL/m(2) and age were independently associated with cognitive impairment. Increased LAVI is associated with cognitive impairment. Assessment of cognitive function may be recommended in elderly patients with enlarged left atria.

Salivary gland tumors comprise a group of 24 tumor subtypes with a wide range of clinical behaviors and propensities for metastasis. Several prognostic factors have been identified that help predict the development of systemic metastases, most commonly to the lung, liver, or bone. Metastases to the brain are rare. To better understand the behavior of salivary gland tumors that metastasise to the brain, we performed a retrospective cohort analysis on a series of patients to highlight features of their medical and surgical management. From 2007 to 2011, a database of 4117 elective craniotomies were queried at a single institution to identify patients surgically treated for salivary gland metastases to the brain. Three patients were identified. Histologic subtypes included salivary duct carcinoma, poorly differentiated carcinoma, and papillary mucinous adenocarcinoma. They had all undergone previous treatment for their primary malignancy. The mean time to intracranial metastasis was 48months from initial diagnosis (range, 14-91months). Treatment for intracranial metastases included surgical resection, whole brain radiation, stereotactic radiosurgery, and chemotherapy. Intracranial metastases from salivary gland tumors are rare, present years after diagnosis of the primary tumor, and are treatable with multimodality therapy.

The aim of this study was to analyze survival, causes of death and cardiologic predictors of sudden death in a large cohort of patients with myotonic dystrophy type 1 (DM1). The study was comprised of 171 adult DM1 patients hospitalized at the Neurology Clinic in a 20-year period. Severe electrocardiographic (ECG) abnormality included at least one of the following: rhythm other than sinus, PR interval of ⩾240ms, QRS complex duration of 120ms or more, and second-degree or third-degree atrioventricular (AV) block. Survival data were analyzed by the Kaplan-Meier test, log-rank test and Cox regression analysis. During the mean follow-up period of 9.4±5.4years, a pacemaker was implanted in 5.8% of DM1 patients and 14% of patients died. The mean age at death was 55.6±12.5years. The most common causes of death in our cohort were sudden death (41.7%) and respiratory failure (29.2%). The presence of palpitations (hazard ratio [HR]=4.7, p<0.05) and increased systolic blood pressure (HR=9.8, p<0.05) were significant predictors of sudden death. Among ECG parameters, severe ECG abnormality (HR=4.7, p<0.05), right bundle branch block (RBBB; HR=3.9, p<0.05) and bifascicular block (HR=5.8, p<0.05) were significant predictors of sudden death.

Neuromyelitis optica (NMO) is an autoimmune disorder diagnosed by an elongated spinal cord lesion associated with unilateral or bilateral optic neuritis and anti-aquaporin 4 (AQP4) antibodies in the serum. It is triggered by or associated with several autoimmune diseases, but not with sarcoidosis. It responds to immunomodulators better than to steroid treatment. Sarcoidosis is an autoimmune disorder which manifests as non-caseating granulomas, usually in the lung parenchyma, but also in other tissues, including the brain. The involvement of the central nervous system in neurosarcoidosis differs considerably from that in neuromyelitis optica and the association of these two diseases concurrently in the same patient is unusual.

Subaxial cervical facet cysts are uncommon. We report two patients with intraspinal, extradural, subaxial cervical spinal facet cysts, and review the literature to describe the epidemiology, clinical presentation, imaging findings, and treatment options for these lesions. Intraspinal, extradural, cervical spinal cysts should be considered as part of the differential diagnosis in patients presenting with clinical signs of cervical radiculopathy or myelopathy.

Altered microRNA-205 (miR-205) expression has been found in glioma tissue samples and cell lines; however, the clinical significance of this is unclear. The aim of this study was to confirm the miR-205 expression pattern in human glioma and to investigate its clinical relevance. Quantitative reverse-transcription polymerase chain reaction assays showed that miR-205 expression was significantly lower in glioma tissues than in non-neoplastic brain tissues (P<0.001). Statistical analysis revealed a significant correlation between low miR-205 expression and both high grade glioma (World Health Organization [WHO] criteria, P=0.008) and a low Karnofsky performance status score (P=0.02). Survival analysis demonstrated that the cumulative 5-year overall survival rate of patients with glioma in the high miR-205 expression group was significantly higher than that in the low miR-205 expression group (P<0.001). Multivariate Cox regression analysis further indicated that miR-205 expression (P=0.01) and WHO grade (P=0.01) were independent prognostic indicators of the overall survival of patients with glioma. Moreover, subgroup analyses revealed that the cumulative 5-year overall survival rate of patients with high grade (III-IV) glioma was significantly worse for the low miR-205 expression group than for the high miR-205 expression group (P<0.001), but no significant difference was found for patients with low grade (I-II) glioma (P=0.09). In conclusion, down-regulation of miR-205 was associated with glioma progression. Our data are the first to suggest that miR-205 holds potential as a prognostic factor for glioma, especially for patients with advanced disease.

This study aimed to examine outcome in low risk transient ischaemic attack (TIA) patients presenting to emergency departments (ED) in a regional Australian setting discharged on antiplatelet therapy with expedited neurology review. All patients presenting to Gosford or Wyong Hospital ED with TIA, for whom faxed referrals to the neurology department were received between October 2008 and July 2010, were included in this prospective cohort study. Classification of low risk was based on an age, blood pressure, clinical features, duration of symptoms and diabetes (ABCD(2)) score <4 and the absence of high risk features, including known carotid disease, crescendo TIA, or atrial fibrillation. Patients with ABCD(2) scores ⩾4 or with high risk features were discussed with the neurologist on call (a decision regarding discharge or admission was then made at the neurologist's discretion). Patients were investigated with a brain CT scan and/or CT angiography, routine pathology, and an electrocardiogram. All discharged patients were commenced on antiplatelet therapy and asked to follow up with their local medical officer within 7days. The patients were contacted by the neurology department to arrange follow-up. Our primary outcome was the number of subsequent strokes occurring within 90days. Of 200 discharged patients for whom referrals were received, three patients had a stroke within 90days. None of these would have been prevented through hospitalisation. In conclusion, medical assessment, expedited investigation with immediate commencement of secondary prevention and outpatient neurology review may be a reasonable alternative to admission for low risk patients presenting to the ED with TIA.