Frontotemporal dementia

Neurological Sciences - Frontotemporal dementia is a clinicopathological syndrome caused by progressive degeneration of the frontal lobes, anterior temporal lobes or both. A wide spectrum of...     

Cholinergic balance in dementia with Lewy bodies: reversible worsening of Parkinsonism at rivastigmine dosage modulation

We describe a patient with probable dementia with Lewy bodies (DLB) whose Parkinsonism worsened after administration of rivastigmine within the therapeutic dose range. Some extrapyramidal signs (EPS) then reversed to pre-treatment level after rivastigmine dose reduction. We draw attention to the need of EPS monitoring during titration of cholinesterase inhibitors in patients with DLB. This is the first report to our knowledge of iatrogenic worsening of Parkinsonism which was successfully managed by dose reduction.

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Sommario Si descrive il caso di un paziente affetto da Demenza a corpi di Lewy (Dementia with Lewy Bodies, DLB) probabile, in cui si è assistito ad un peggioramento del parkinsonismo dopo somministrazione di rivastigmina a dosi terapeutiche. Alcuni segni extrapiramidali sono regrediti al livello pre-trattamento con una riduzione posologica di rivastigmina. Si sottolinea la necessità di un monitoraggio dei segni extrapiramidali durante la titolazione della terapia con inibitori dell’acetilcolinesterasi cerebrale in pazienti con DLB. Questo è il primo caso descritto, a nostra conoscenza, di un peggioramento iatrogeno di parkinsonismo efficacemente gestito con una riduzione posologica della terapia con rivastigmina.

     

Observations on dementias with possibly reversible symptoms

While the evaluation of patients with dementia must also address the possibility of treatable illnesses, recent studies found that reversible diseases are detected in only about 1% of dementia cases. Data on the frequency and evolution of potentially reversible dementias (PRD) in defined clinical settings can be useful in order to optimize diagnostic protocols, thus reducing over-investigation and waste of resources. We reviewed a series of 513 patients (mean age 69.3 +/- 4.2 years, mean education level 7.4 +/- 4.5 years, sex ratio M/F 217/296) referred to a memory clinic by their general practitioner, in order to identify PRD. All the subjects had undergone neurological and neuropsychological examination, and laboratory tests. Patients considered to be demented also underwent CT brain scan. 362 patients (70.6%) met the criteria for dementia. We identified 26 PRD cases (7.2% of dementia cases, 5.1% of the entire sample). In 13 patients (3.6% of dementia cases), a complete clinical and neuropsychological reversal of dementia was seen after treatment. In 5 of them (1.4%), a partial regression was obtained, while 8 showed no improvement. We conclude that potential and actual reversibility do not coincide, and that "true" reversibility is rare (even if not negligible) in clinical practice. Careful clinical history and examination appeared the most useful part of the evaluation to identify PRD. Standard blood tests and vitamin B12 assay were also useful, while CT scan detected PRD causes only in patients with evidence of neurological signs.     

Researching a differential impairment of frontal functions and explicit memory in early Parkinson's disease.

An impairment at tasks sensitive to frontal lobe damage has been repeatedly reported in Parkinson's disease, but the exact nature of these deficits has not yet been clarified. Similarly, deficits of visuo-spatial functions have been frequently observed, but it is still debated whether verbal and visuo-spatial memory can be differentially affected. In this study we have compared the performance of 20 mild Parkinson's disease patients (I-II Hoehn and Yahr stage) and 18 matched normal controls, at tasks assessing frontal functions and explicit memory. We detected a selective deficit in set shifting and maintaining, without impairment in categorization and set formation. The lack of a selective increase in perseverative errors might indicate that perseverations either measure something different from set shifting or that they do not represent an index sensitive enough to set shifting impairment. Parkinson's disease patients were also significantly impaired at Raven's Progressive Matrices, a task assessing both frontal and visuo-spatial aspects. However, they did not show any differential impairment of visuo-spatial memory. Indeed, despite a trend of lower performance in visuo-spatial learning, memory performance of Parkinson's disease patients was significantly different from that of controls only at a free recall test which involved both verbal and visuo-spatial memory. We suggest the exploration of set shifting and maintaining to detect 'frontal' deficits in mild Parkinson's disease. We argue that Raven's Progressive Matrices is a valuable task for detecting subclinical cognitive deficits in Parkinson's disease, even if it does not show a specific profile of impairment in these patients. According to our results, a differential evaluation of verbal vs. visuo-spatial memory is not necessary in clinical practice, whilst free recall confirms its usefulness to detect subclinical impairments of memory functions.     

Free and cued selective reminding test: an Italian normative study

The presence of episodic memory impairment is required for the diagnosis of Alzheimer??s dementia by all current diagnostic criteria. The new research criteria proposed by Dubois et al. (Lancet Neurol 6:734?C746, 2007) require that the impairment should not improve significantly with cueing, recognition testing nor after the control of effective encoding. This is considered to be the core deficit of ??prodromal Alzheimer??s disease??. The Free and Cued Selective Reminding Test (FCSRT) is a memory test that allows in assessing these specific features of memory impairment. Here, we report normative data for an Italian version of the FCSRT. The test is based on the 12 pictorial stimuli, 6 belonging to the living domain, and 6 to the non-living domain. Six scores were derived from the performance of 227 healthy Italian adults, with age, sex and education homogenously distributed across subgroups: immediate free recall (IFR), immediate total recall (ITR), delayed-free recall (DFR), delayed total recall (DTR), Index of Sensitivity of Cueing (ISC), number of intrusions. In multiple regression analyses, age emerged as an influencing factor for both IFR and DFR, with older people obtaining lower scores. Education and gender appear to influence only IFR, with better performance by more educated subjects and females. Adjusted scores were used to determine inferential cutoff scores and to compute equivalent scores.     

Fluorescence in situ hybridization (FISH) for evaluating residual disease in Philadelphia chromosome-positive chronic myeloid leukemia during interferon alpha therapy

Fluorescence in situ hybridization (FISH) is a quantitative technique which allows, by means of specific probes, to detect the t(9;22) translocation typical of chronic myeloid leukemia (CML) hematopoietic cells. We have evaluated FISH on interphase nuclei as a method for assessment of residual disease in bone marrow samples from 5 Philadelphia chromosome positive (Ph(+)) CML patients after 12 months therapy with interferon alpha; results were compared with findings obtained by conventional cytogenetics and by polymerase chain reaction (PCR). Our data indicate that FISH is more sensitive than cytogenetics for evaluation of residual disease, being positive in 1 out of 2 cases scored as Ph negative by cytogenetics, but is less sensitive than PCR which turned out to be positive in all patients. As additional advantage over conventional cytogenetics, FISH on interphase nuclei can be performed also on samples lacking metaphases or having poor chromosome spreading or unsatisfactory chromosome banding.     

Normative data for two neuropsychological tests sensitive to frontal dysfunction

Interest in frontal functions has progressively increased over recent years; however, despite this, there are only a few frontal tasks for which Italian normative data are available. The objective of this study was to obtain reference values for two frontal tests from a random sample of normal adults: the first (the Test of Classification and Recall of Pictures) has been shown to reveal selective impairment in patients with unilateral frontal excision; the second (the Odd-Man-Out Test) derives from tasks which have been used to evaluate frontal functions in animals and detect frontal impairment in Parkinson patients. We tested 100 normal subjects, aged 22 to 79 years, stratified by education according to the Italian school system. The correction grids for both tests are also presented. The usefulness of these tests is represented not only by their sensitivity to frontal impairment, but also by the possibility of dissociating the various cognitive aspects of frontal lobe dysfunction.

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Sommario L'interesse nei confronti delle funzioni frontali è progressivamente aumentato negli ultimi anni. Nonostante ciò, solo per pochi test frontali sono disponibili dati normativi italiani. Lo scopo di questo studio era ottenere valori normativi per due test frontali utilizzando un campione di soggetti normali selezionati in maniera casuale. Il primo test, ossia il Test di Classificazione e Rievocazione di Figure, risulta selettivamente compromesso in pazienti sottoposti a escissione frontale unilaterale; il secondo test, ossia il Test della Percora Nera, deriva da compiti utilizzati per rilevare la compromissione frontale negli animali ed è stato usato per individuare una disfunzione di tipo frontale nei parkinsoniani. Sono stati testati 100 soggetti normali, di età compresa fra i 22 e i 79 anni, stratificati per scolarità secondo il modello scolastico italiano. Vengono presentate le griglie di correzione per entrambi i test. La loro utilità consiste non solo nella loro sensibilità al danno frontale, ma anche nella possibilità di dissociare aspetti cognitivi differenti della disfunzione dei lobi frontali.

     

Allodynia in different forms of migraine

Neurological Sciences - About 60% of patients complain of cutaneous allodynia during migraine episodes, often in the periorbitary region of the pain side. Pre-clinical studies have shown that the...     

Changes in erythropoiesis, iron metabolism and oxidative stress after half-marathon

OBJECTIVE: In marathon runners changes in red blood cell count, haematocrit and haemoglobin in relation to haemodilution have been reported. Moreover, it has been hypothesized that strenuous exercise induces oxidant stress through several different mechanisms. This study investigated the haematological variables, iron status and oxidative indices before, immediately and 48 h after a race in 8 healthy trained males aged 33-44 years running a 21-km marathon in 79 +/- 3 min. METHODS: The haematological parameters were determined by standard procedures. Erythropoietin and soluble-transferrin receptor were evaluated immunoenzymatically. Nontransferrin-bound iron (NTBI) was assayed by high-performance liquid chromatography after nitrilotriacetic acid chelation. Malonyldialdehyde (MDA) concentration was assayed colorimetrically. RESULTS: The total number of reticulocytes rose significantly after the run with a significant increase in the high-RNA-content fraction (14 +/- 5, p < 0.0006). Erythropoietin rose by 26% (15.0 +/- 2.8 mU/ml, p < 0.004) and by 25% (14.9 +/- 2.13 mU/ml, p < 0.02) immediately and 48 h after the race, respectively. Serum iron and serum ferritin remained unchanged but NTBI and serum MDA increased significantly immediately after running (1.16 +/- 0.40 mmol/l, p < 0.0008; 0.76 +/- 0.16 mmol/l, p < 0.0001). Significant positive correlations at any time between MDA and polymorphonuclear neutrophils (p = 0.0005), MDA and NTBI (p = 0.0018), polymorphonuclear neutrophils and NTBI (p = 0.0008) and between lactate dehydrogenase and NTBI (p = 0.0212) were observed. CONCLUSIONS: The erythropoietic changes observed in marathon runners are the results of several interacting mechanisms that involve either the haemopoietic system per se or erythrocyte haemolysis and oxidative stress.