Nicola Gilberti

Is Frontotemporal Lobar Degeneration a Rare Disorder? Evidence from a Preliminary Study in Brescia County, Italy

Frontotemporal Lobar Degeneration (FTLD) has always been considered a rare disorder, but only a few epidemiologic studies are available. The aim of the present work was to ascertain all FTLD patients in a Northern Italy area from January 2001 to December 2008, and to estimate the disease prevalence. On the census day, 213 FTD patients were still alive, resulting in an overall prevalence of 17.6 per 100,000 inhabitants. The prevalence of FTLD in patients aged 45-65 years was 22 per 100,000 inhabitants (95% CI=17-27). The prevalence of FTLD was the highest in patients aged 66-75 (78 per 100,000 inhabitants, 95% CI=56-100), and it was still high over 75 years (54 per 100,000 inhabitants, 95% CI=36-69). FTLD is a more common form of dementia than previously recognized. Our results claimed that FTLD is not only an early-onset disorder, but it is frequent in advanced age as well.

Parkinson's disease and dementia

Parkinson’s disease (PD) is one of the most common neurodegenerative disorders, affecting about 1% of the population over the age of 60. In addition to motor abnormalities, there are several non-motor signs and symptoms that may create a considerable burden for patients and care-givers. Dementia is common and affects approximately 40% of PD patients during the course of the disease, the risk for the development of dementia being 6 times higher than in non-PD age-matched controls. In most cases, PD patients with dementia (PDD) display a dysexecutive syndrome and visuospatial deficits, while memory is relatively unaffected. The overlap between PDD and dementia with Lewy bodies suggests that they likely share similar underlying neuropathological processes.

Primitive reflex evaluation in the clinical assessment of extrapyramidal syndromes

The aim of the present study was to evaluate the role of primitive reflexes (PRs) as additional alert sign in routine clinical practice in patients with extrapyramidal syndrome. We considered glabellar, snout, palmomental and grasp reflexes in patients with mild stage of Lewy body dementia (LBD), corticobasal degeneration, progressive supranuclear palsy or Parkinson disease (PD). We also enrolled mild Alzheimer disease (AD) patients, and healthy subjects, as controls. LBD patients showed the highest prevalence of PRs compared with the other groups. The odds ratio of the risk of LBD in PRs ≥ 2 was 27.9 (95% CI 2.9–269.0) compared with control group, 14.6 (95% CI 2.7–79.6) compared with mild AD, and 19.7 (95% CI 3.7–104.3) compared with PD. These data suggest that the occurrence of combination of PRs might be an useful additional warning sign of possible diffuse Lewy body pathology more than other causes of extrapyramidal syndrome.

The Usefulness of Biological and Neuroimaging Markers for the Diagnosis of Early-Onset Alzheimer's Disease

The recent proposed criteria for Alzheimers Disease (AD) have strongly claimed the usefulness of biological and neuroimaging markers for early identification AD. Cerebrospinal fluid (CSF) Tau/Abeta ratio, hippocampal atrophy, posterior cingulate, and neocortical associative area hypometabolism, or amyloid burden evaluated by PiB compound, held the premises to increase diagnostic accuracy in the preclinical disease stages. Despite many efforts to identify subjects at risk of developing AD, less attention has been paid to presenile AD diagnosis. A few data are already available in early onset AD, mainly obtained in cases of monogenic disorder. In this paper, we discuss the current literature on the role of biological and neuroimaging markers in presenile AD.

“Head banging” causing subdural hemorrhage and internal carotid artery dissection

A 16-year-old caucasian male presented at the emergency department with sudden onset of severe diffuse headache. He denied head or neck trauma, but reported ‘‘head banging’’ during a rock concert the evening before the onset of headache. Clinical examination at the emergency department revealed only mild drowsiness without focal signs. Brain computed tomography (CT) scan and magnetic resonance imaging (MRI), including MR Angiography (MRA), revealed a right-sided subdural hematoma (SDH) with slight shift of the midline structures. No underlying brain lesions were detected. MRA showed a mild narrowing of the supraclinoid segment of the right carotid siphon and of the proximal M1-MCA segment. Digital subtraction angiography (DSA) confirmed the MRA findings and also revealed a perfusion delay in the right MCA territory, therefore suggesting a dissection of the right carotid siphon and proximal M1-MCA (Fig. 1). Headache gradually regressed over a period of 1 week and the patient was then asymptomatic. One month follow-up brain CT and MRI showed partial resorption of SDH and the appearance of a right-sided cerebral infarct in the temporal and parietal lobes (Fig. 1); patient did not experience new symptoms and neurological examination was normal. DSA was repeated showing normal size of the right ICA and MCA. At the 90 days follow-up the patient was asymptomatic and brain CT scan revealed gradual resorption of SDH and chronic right-sided cerebral infarct.

Percheron artery occlusion: an uncommon cause of decreased arousal

Percheron artery (PA) is a rare anatomical variant, in which both the medial thalami are supplied by a single paramedian artery (1). We report a young patient presenting with sudden coma due to bilateral thalamic stroke deriving from PA occlusion. A 41-year-old woman was found unresponsive in bed. Vital signs, general examination and routine blood tests were unremarkable. The Glasgow Coma Scale score was 8 (E1M5V2) with pupils equal and reactive to light. Fever and signs of meningeal irritation were absent. Brain CT scan was normal. Electroencephalografic (EEG) study disclosed the presence of symmetrical widespread theta activity, transiently interrupted by anterior delta activity (Fig. 1). Brain magnetic resonance imaging showed bilateral paramedian thalamic infarction (Fig. 2). Magnetic resonance angiography (MRA) was unremarkable, therefore bilateral thalamic infarction was attributed to occlusion of PA. PA is an uncommon anatomic variant of the paramedian artery, in which a single common trunk, originating from the P1 tract of the posterior cerebral artery, supplies both medial thalami with variable contribution to the rostral midbrain (2). Prevalence of this anatomical variant is unknown as it is very difficult to visualize on neuroimaging. Occlusion of this artery results in bilateral infarction of the paramedian thalami, with or without Correspondence: Andrea Morotti*, Clinica Neurologica, Università degli Studi di Brescia, Piazzale Spedali Civili 1, 25125 Brescia, Italia. E-mail: a.morotti@ymail.com