Background: Current diagnostic criteria for behavioral variant of frontotemporal dementia (bvFTD) and typical Alzheimer's disease (AD) include a differential pattern of neuropsychological impairments (episodic memory deficit in typical AD and dysexecutive syndrome in bvFTD). There is, however, large evidence of a frequent overlap in neuropsychological features, making the differential diagnosis extremely difficult. Objectives: In this retrospective study, we evaluated the diagnostic value of different cognitive and neurobehavioral markers in bvFTD and AD patient groups. Methods: We included 95 dementia patients with a clinical and biomarker evidence of bvFTD (n = 48) or typical AD (n = 47) pathology. A clinical 2-year follow-up confirmed clinical classification. Performances at basic cognitive tasks (memory, executive functions, visuo-spatial, language) as well as social cognition skills and neurobehavioral profiles have been recorded. A stepwise logistic regression model compared the neuropsychological profiles between groups and assessed the accuracy of cognitive and neurobehavioral markers in discriminating bvFTD from AD. Results: Statistical comparison between patient groups proved social cognition and episodic memory impairments as main cognitive signatures of bvFTD and AD neuropsychological profiles, respectively. Only half of bvFTD patients showed attentive/executive deficits, questioning their role as cognitive marker of bvFTD. Notably, the large majority of bvFTD sample (i.e., 70%) poorly performed at delayed recall tasks. Logistic regression analysis identified social cognition performances, Frontal Behavioral Inventory and Mini-Mental State Examination scores as the best combination in distinguishing bvFTD from AD. Conclusion: Social cognition tasks and socio-behavioral questionnaires are recommended in clinical settings to improve the accuracy of early diagnosis of bvFTD.

BACKGROUND:Although only a few frontotemporal lobar degeneration (FTLD) patients develop frank amyotrophic lateral sclerosis (ALS), motor neuron dysfunctions (MNDys) occur in a larger proportion of patients. The aim of this study is to evaluate MNDys and ALS in a sample of consecutively enrolled sporadic FTLD patients.METHODS:Clinical and neurophysiological evaluations (i.e. needle electromyography) assessed lower (LMN) and upper (UMN) motor neuron function at the baseline in 70 probable FTLD patients (i.e., 26 behavioural variant-bvFTD, 20 primary progressive aphasias-PPAs and 24 corticobasal syndrome-CBS). To obtain a more accurate estimation, quantitative scales were also applied (i.e. ALSFRS-r and UMN scale). Patients were screened for MAPT, GRN and C9orf72 mutations. A mean clinical follow-up of 27.8±22.4 months assessed MNDys progression and the clinical presentation of ALS.RESULTS:Five genetic cases were identified. Within the sample of sporadic patients, a relative low rate of FTLD patients was diagnosed as probable ALS (5%), while a higher proportion of patients (17%) showed clinical and neurophysiological MNDys. Thirteen patients (20%) presented with isolated clinical signs of LMN and/or UMN dysfunction, and 8 patients (12%) showed neurogenic changes at the electromyography. No differences in FTLD phenotype and disease duration were found between MNDys positive and negative patients. Clinical MNDys were highly associated with positive electromyographic findings. At follow-up, no MNDys positive patient developed ALS.CONCLUSION:Neurophysiological and clinical examinations revealed mild MNDys in FTLD patients not fulfilling criteria for ALS. This condition did not evolve at a mean follow-up of two years. These results, indicating a subclinical degeneration of corticospinal tracts and lower motor neurons, suggest that FTLD patients may be more at risk of MNDys than the general population.

Objective: To evaluate the extent and distribution of clinical and neurophysiological signs of motor neuron dysfunction (MNDys) in frontotemporal lobar degeneration (FTLD). Background: Motor neuron disease (MND) has been reported in a proportion of FTLD patients (Lomen-Hoert et al., 2002; Burrell et al., 2011). Nevertheless, only a few develop MND at follow-up. The incidence, severity, extent and functional significance of MNDys in FTLD is still largely unexplored. Methods: 71 FTLD (i.e., 26 bvFTD, 21 PPA and 24 CBD) patients were consecutively enrolled. Clinical signs of lower (i.e., focal muscular atrophy, fasciculations and weakness) and upper (i.e., pathologic hyperreflexia, spasticity and Babinski sign) MNDys, as well as EMG-ENG data, were recorded at baseline. Restrictive neurophysiological criteria were applied (de Carvalho et al., 2008). Patients were screened for MAPT , GRN and C9orf72 mutations. The mean follow-up was 26.1±22.1 months. Results: Overall prevalence of MNDys signs at EMG was 15.4% (i.e., 11 out 71 patients). Four patients (2 bvFTD and 2 PNFA; 5.6%) fulfilled El Escorial criteria for MND; the other seven showed active denervation combined to collateral reinnervation confined to either cervical or lumbar district. Two of them showed associated clinical signs of lower MNDys (fasciculations) in the same district. Seven patients showed denervation without clinical evidence of MND and twelve isolated chronic denervation. No statistical differences in FTLD phenotype, disease duration or clinical severity were found between EMG positive and negative patients. No patient with clinical or EMG positive findings developed MND at follow-up. Conclusion: A subgroup of FTLD patients with mild neurophysiological signs of MNDys not fulfilling criteria for MND can be found at the time of first diagnosis. This condition did not evolve to MND at a 2-year follow-up. Subclinical secondary degeneration of corticospinal tracts and lower motor neurons is present in the FTLD spectrum. Disclosure: Dr. Cerami has nothing to disclose. Dr. Marcone has nothing to disclose. Dr. Marangoni has nothing to disclose. Dr. Crespi has nothing to disclose. Dr. Dodich has nothing to disclose. Dr. Canessa has nothing to disclose. Dr. Iannaccone has nothing to disclose. Dr. Michele has nothing to disclose. Dr. Golzi has nothing to disclose. Dr. Giusti has nothing to disclose. Dr. Cappa has received personal compensation in an editorial capacity for Behavioral Neurology.

INTRODUCTION:The evidence for the clinical effectiveness of cognitive rehabilitation in patients with Alzheimer's Disease (AD) is debated. Therefore it is important to collect more evidence about the outcome of non-pharmacological therapy of dementia.MATERIAL AND METHODS:We report data concerning the rehabilitation of 50 patients with probable AD admitted during a 17-month period in a specialized unit. Participants were affected by dementia ranging from mild to severe. The patients were treated with the Reality Orientation Therapy (ROT), integrated, when needed, with individualised cognitive approaches. The results concern: the cognitive status, evaluated by means of the Mini Mental State Examination (MMSE), the functional status, evaluated with the Activity of Daily Living (ADL) scale, the assessment of psychological and behavioural disorders measured with the Neuropsychiatry Inventory (NPI). The cognitive, functional, and psychopathological assessments were administered at admission and discharge.RESULTS:The mean MMSE scores at admission and discharge were respectively 16.06 and 17.54 (Wilcoxon Ranks Test: p=0.005). Mean ADL scores were 4.86 at admission and 5.02 at discharge (p=0.011). Mean NPI scores were respectively 21.46 and 12.26 (p<0.001).CONCLUSIONS:This survey of the 17-month experience suggests that a comprehensive treatment program may have beneficial effects on cognitive, functional, and in particular neuropsychiatric outcomes. The results should be verified with a randomised clinical trial.

We report an [18F]fluordeoxyglucose (FDG)-PET study performed in an 11-year-old girl with a 5-month history of epilepsia partialis continua (epc). Visual inspection of PET images showed a hypermetabolic focus in the right central cortex and in the ipsilateral thalamus, which was confirmed by the absolute values of regional cerebral glucose metabolism (rCMRGlu). The thalamic hypermetabolism provides evidence for an involvement of thalamic nuclei in this ictally epileptic process.The scalp EEG revealed a theta-delta and sharp wave focus in the right Rolandic cortex at the same location as the hypermetabolic zone seen in PET. Simultaneously recorded EMG of the left tibialis anterior muscle showed regular jerks, time-locked to the sharp waves at the right central region, and myoclonic ‘storms’ during focal motor seizures. The results of the brain biopsy and the child's clinical course led us to a diagnosis of ‘chronic encephalitis’ of Rasmussen.

Brain magnetic resonance imaging (MRI) was studied in patients with mild-to-moderate temporal lobe epilepsy (TLE), well controlled by pharmacotherapy, and with normal computed tomographic (CT) scans. Magnetic resonance imaging abnormalities were found in 19 patients; of these, nine had abnormalities in temporomesial regions and four in temporobasal regions. Six patients had white matter MRI lesions of nonspecific significance. The temporomesial MRI lesions were compatible with sclerosis of Ammonis cornu. Patients with this MRI finding had more severe and longer lasting TLE than those without MRI abnormalities. The temporobasal lesions were interpreted as potentially developing brain lesions. Correlation between EEG and MRI findings was good. We conclude that MRI is more useful than CT for diagnosis of patients with mild-to-moderate TLE.

The brainstem auditory evoked potentials of 84 epileptic patients in chronic monotherapy with carbamazepine (n = 36), phenobarbital (n = 19), Na-valproate (n = 20) or progabide (n = 9) were studied. The mean values of I-III and I-V interpeak latencies (IPLs) were respectively 2.16 +/- 0.11 and 4.03 +/- 0.17 in the control group, 2.31 +/- 0.09 and 4.17 +/- 0.16 in the valproic group, 2.30 +/- 0.17 and 4.19 +/- 0.20 in the carbamazepine group, 2.17 +/- 0.16 and 4.10 +/- 0.18 in the phenobarbital group and 2.16 +/- 0.11 and 4.12 +/- 0.17 in the progabide group. The prolongation of I-III and I-V IPLs was statistically significant only for the valproic acid and carbamazepine groups. Neither duration of the epilepsy and treatment and frequency of seizures nor the serum drug levels were correlated with I-V IPL values.

Platelet function and thrombin activity were investigated in 12 hospitalized patients (7 men and 5 women, mean age 53 years) who had had transient cerebral ischemic attacks in the previous 2-12 weeks. Each patient was given an extensive clinical and instrumental evaluation, including Doppler sonography of the cervical and lower limb vessels, cerebral angiography, and head computed tomography scan, after which relevant atherosclerotic disease was excluded. The controls consisted of 12 subjects hospitalized for nonvascular neurologic problems and matched for age, sex, and risk factors to the transient ischemic attack patients. Collagen-induced platelet thromboxane B2 production, plasma beta-thromboglobulin, and fibrinopeptide A were significantly higher in the patients than the controls. Platelet aggregability by collagen was the same in the 2 groups. Platelet hyperfunction and enhanced thrombin activity are present in patients some weeks after the acute episode, suggesting that the hemostatic system has a primary pathogenetic role.

In a patient with transient global amnesia, computed tomography demonstrated a left temporal haemorrhage sparing the hippocampal region.

Cardiac abnormalities are a known complication of subarachnoid haemorrhage. The case is reported of a woman who experienced recurrent ventricular fibrillation after subarachnoid haemorrhage. Lidocaine exerted a dose-related effect on the prevention of life-threatening arrhythmia.