Paediatric and adult vascular intracranial complications of sickle-cell disease.

BACKGROUND: To enumerate possible intracranial vascular sequelae of sickle-cell disease, to identify risk factors and outline management strategies. METHOD: Retrospective review of a single unit experience managing vascular intracranial complications of sickle-cell disease from 1995 until 2005. Information such as homozygosity/heterozygosity, duration of disease, disease control as indicated by haematology follow-up, concurrent sickle-cell disease (SCD)-related health problems and neurosurgical management was recorded. The pattern of vascular disease was analysed to reveal possible contributory/risk factors towards development of vascular intracranial complications. SUBJECTS: All patients presenting with vascular intracranial complications of sickle-cell disease from 1995 to 2005 were evaluated. OUTCOME MEASURES: Classification of vascular intracranial complications into one or more of the following categories: aneurysmal subarachnoid haemorrhage, non-aneurysmal subarachnoid/intraventricular haemorrhage and vasculitis. FINDINGS: There were ten patients in the study. All symptomatic vascular intracranial complications of SCD requiring neurosurgical intervention were homozygous for SCD. Aneurysms were likely to be multiple. Ruptured aneursyms in SCD were small (average 4 mm). There was a propensity for aneurysms to occur in the posterior circulation, in particular the posterior cerebral artery was frequently involved. Patients with aneurysms and Moyamoya-type vasculitis were likely to have occlusive disease of the internal carotid arteries. CONCLUSIONS: The vascular intracranial complications of sickle-cell disease have an aggressive natural history. Tight control of SCD may reduce the possibility of complications. Complications that arise should be managed in the context of the disease entity rather than in isolation. Consideration should be given to bypass procedures, parent vessel ligations and revascularization techniques. Transcranial Doppler may be used to identify SCD patients with cerebrovascular occlusive disease, who may have increased risk of aneurysmal rupture.

Scedosporium apiospermum brain abscess treated with surgery and voriconazole. Case report.

Scedosporium apiospermum is a fungus found in the soil and in contaminated water. Common cutaneous manifestations include Madura foot, a painless swelling on the sole of the foot. Invasive infection is usually associated with immunosuppression. The authors present a 16-month-old immunocompetent boy who had a near-drowning event. Following this, he was severely disabled with spastic quadriparesis. Early computerized tomography scans revealed diffuse hypoxic injury. Magnetic resonance images obtained 3 months after the initial event demonstrated multiple ring-enhancing lesions in the brain. Aspiration of the lesion was performed. Scedosporium apiospermum, the asexual form of Pseudallescheria boydii, was cultured. Conventional antifungal agents were commenced, with minimal effect. The child was subsequently treated with a new antifungal agent, voriconazole, a broad-spectrum triazole antifungal agent with good penetration into the cerebrospinal fluid. The patient improved, with a good radiological outcome and a moderate clinical outcome. The authors review the use of voriconazole for central nervous system infections and review the literature on S. apiospermum associated with near drowning.

Prefrontal cortex dysfunction and 'Jumping to Conclusions': bias or deficit?

The 'beads task' is used to measure the cognitive basis of delusions, namely the 'Jumping to Conclusions' (JTC) reasoning bias. However, it is not clear whether the task merely taps executive dysfunction - known to be impaired in patients with schizophrenia - such as planning and resistance to impulse. To study this, 19 individuals with neurosurgical excisions to the prefrontal cortex, 21 unmedicated adults with Attention Deficit Hyperactivity Disorder (ADHD), and 25 healthy controls completed two conditions of the beads task, in addition to tests of memory and executive function as well as control tests of probabilistic reasoning ability. The results indicated that the prefrontal lobe group (in particular, those with left-sided lesions) demonstrated a JTC bias relative to the ADHD and control groups. Further exploratory analyses indicated that JTC on the beads task was associated with poorer performance in certain executive domains. The results are discussed in terms of the executive demands of the beads task and possible implications for the model of psychotic delusions based on the JTC bias.