Abstract: Schilder's disease, or myelinoclastic diffuse sclerosis, is a rare disorder characterised by an inflammatory white matter plaque of demyelination. Clinical signs and symptoms might be atypical for early multiple sclerosis and at imaging the lesion is easily taken for a brain tumour. Regardless of the use of Poser's criteria for clinical diagnosis of Schilder's disease proposed in 1986, diagnostic difficulties are still present, as evidenced by the many reported cases in the English literature revised (Pubmed indexed, period 1998-2008). It clearly emerges that neuroradiological features, observable in additional magnetic resonance sequences are crucial, besides the consideration of Poser's criteria, in differentiating between demyelinating lesions and brain tumours. A 29-year-old female patient is presented, where a careful evaluation of both the clinical and radiological features, which might have been at a first glance misleadingly suggestive for a brain tumour, allowed non-invasive diagnosis of Schilder's disease.

Abstract: The aim of this review was to evaluate the imaging tools used in diagnosis and perioperative assessment of moyamoya disease, with particular attention to the last decade.

Abstract: The aim of this study is to report the authors' initial clinical experience of a 320-detector row computed tomography (CT) scanner in cerebrovascular disorders. Volumetric CT using the full 160-mm width of the 320 detector rows enables full brain coverage in a single rotation that allows for combined time-resolved whole-brain perfusion and four-dimensional CT angiography (CTA). The protocol for the combined dynamic CTA and CT perfusion (CTP) is presented, and its potential applications in stroke, stenoocclusive disease, arteriovenous malformations and dural shunts are reviewed based on clinical examples. The combined CTA/CTP data can provide visualization of dynamic flow and perfusion as well as motion of an entire volume at very short time intervals which is of importance in a variety of pathologies with altered cerebral hemodynamics. The broad coverage enabled by 320 detector rows offers z-axis coverage allowing for whole-brain perfusion and subtracted dynamic angiography of the entire intracranial circulation.

Abstract: OBJECTIVE: The influence of cerebrovascular disease (CVD) on the short- and long-term results of surgery was evaluated in a series of consecutive patients with idiopathic normal-pressure hydrocephalus (iNPH). METHODS: Patients with suspected iNPH admitted to our department between June 1996 and June 2003 were evaluated with four clinical and handicap scales. CVD and risk factors for vascular disease were rated. All patients underwent intracranial pressure monitoring via a spinal catheter. Sixty-six patients received a ventriculoperitoneal shunt with a programmable valve. Prospective assessments were programmed at 2 weeks and 3 months after surgery (short-term follow-up). Long-term follow-up evaluations were arranged in June 2004 with patients and/or relatives and health/home care assistants. RESULTS: At the short-term follow-up examination, a significant clinical improvement was globally present in 89% of the patients (P < 0.05). CVD, such as leucoaraiosis or previous strokes, were present in 71% of the patients. Patients both with and without CVD and/or risk factors for vascular disease presented a significant improvement (P < 0.05) after shunting; 85 and 100% of the patients with and without CVD, respectively. At the long-term follow-up examination (mean, 52 +/- 24.8 mo), 24% of the patients were dead and 8% had experienced stroke. Globally, 60% of the patients were still improved (P < 0.05); 52 and 79% of the patients with and without CVD, respectively. CONCLUSION: A high success rate in treatment of iNPH is possible in patients with and without CVD. Despite poorer short- and long-term treatment outcome of iNPH patients with CVD, a long-lasting improvement in their quality of life favors surgery.

Abstract: Though a rare cause of stroke in the general population, in almost one quarter of young patients affected by stroke cervical artery dissection (CAD) is the underlying cause. Among these cases "spontaneous" dissections, intended as non-traumatic, represent about 34% of posterior circulation arterial dissection in patients aged less than 18 years. We here describe the case of a seven-year-old boy who developed a spontaneous vertebral artery dissection (VAD) leading to occipito-mesial, thalamo-capsular and cerebellar infarction. Once a traumatic origin was excluded, clinical history and laboratory findings were further analysed: fever associated with tonsillitis during the previous week, raised inflammatory indices, a throat culture positive for beta haemolysing Streptococcus group A and high titres of streptococcal antibodies were found. This case suggests that patients with CAD referred as spontaneous deserve extensive analysis. Subjects presenting with a dissection and an underlying infection are likely to have a hyperinflammatory response (although further experience is needed). In these patients immediate start of antibiotic therapy, treatment with anti-inflammatory drugs and further a lifelong prophylaxis with antibiotics before any invasive procedure are strongly recommended.

Abstract: A 62-year-old woman developed dysphasia, signs of intracranial hypertension and seizures. An MRI scan evidenced extra-axial contrast enhancement in the absence of mass lesions. CSF analysis was negative for malignant cytology and viral or bacterial microbiology. In the absence of other evidence, considering the acute clinical onset and MRI picture, a viral encephalitis was suspected and antiviral therapy was started, however, with no effect. Death occurred three weeks after presentation because of acute brain swelling. Autopsy revealed leptomeningeal gliomatosis and diffuse parenchymal infiltration at the cerebellar and left temporal lobe. Primary leptomeningeal gliomatosis (PLMG) is a rare event. The reported case and the reviewed literature evidence that clinical signs at presentation are non-specific, CSF negative findings are common, the diagnosis is always delayed and the prognosis dismal.

Abstract: The surgical treatment of intracerebral hemorrhage (ICH) is one of the most controversial areas of neurosurgery. Randomized trials are inconclusive due to the small number of patients enrolled or because performed in pre CT era. We analyze the 232 patients admitted to the Niguarda Hospital of Milano, Italy, for ICH during the period January 2001-December 2002. Sixty patients were operated: 29 had secondary haemorrhage, and 30 a primary one. The strategy followed in these patients is presented. The series suggests that indication to surgery is a complex process including several factors that have to be all considered in each patient.