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BACKGROUND: Early and progressive disabling visual impairment is a core feature for the diagnosis of posterior cortical atrophy (PCA). However, some individuals that fulfil criteria over time might initially present with an onset of prominent posterior dysfunction other than visuoperceptual. METHODS: The clinical profile of five patients with a predominantly 'non-visual' posterior presentation (PCA2) was investigated and compared with sixteen individuals with visually predominant PCA (PCA1) and eighteen with typical amnestic Alzheimer disease (tAD). RESULTS: PCA2 patients showed significantly better performance than PCA1 in one visuospatial task and were free of Balint's syndrome and visual agnosia. Compared to tAD, PCA2 showed trends towards significantly lower performance in visuoperceptual tasks, more severe apraxia and more symptoms of Gerstmann's syndrome. CONCLUSIONS: Our sample of PCA2 patients did not present with clinically prominent visual symptoms but did show visual dysfunction on formal neuropsychological assessment (less pronounced than in PCA1 but more than in tAD) in addition to other posterior deficits. Broadening the definition of PCA to encompass individuals presenting with prominent 'non-visual' posterior dysfunction should be potentially considered in clinical and research contexts.

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BACKGROUND AND PURPOSE: Decreased plasma progranulin levels are a very specific marker for the diagnosis of frontotemporal lobar degeneration (FTLD) caused by mutations in the progranulin gene (GRN). A frequent neuroimaging pattern in this type of dementia is asymmetric cortical atrophy. The aim of this study was to screen for GRN-linked FTLD in cases with different cortical dementia phenotypes and asymmetric perisylvian atrophy. METHODS: Progranulin plasma levels were analyzed in a variety of FTLD phenotypes (n = 71), dementia of the Alzheimer type (DAT) (n = 22) and probable Lewy body dementia (n = 8), both latter groups presented with asymmetric perisylvian atrophy. A group of elderly controls (n = 29) and DAT cases with symmetric atrophy (n = 33) were also analyzed. The GRN gene was sequenced in cases with lower plasma levels. RESULTS: Four cases with clinical FTLD phenotypes and plasma levels below 70 ng/ml were found to carry different GRN mutations: M1?, C139R, a point mutation in the splice donor site of intron 3 (A89VfsX41), and a deletion in exon 9 (A303AfsX57), this latter one being a new mutation. Thirteen cases with levels between 72 and 85 ng/ml did not show pathogenic changes in the GRN gene. None of the cases with asymmetric atrophy and clinical phenotypes other than FTLD had GRN mutations. CONCLUSIONS: Asymmetric perisylvian atrophy is not likely to predict progranulin-linked FTLD unless it is associated with a consistent FTLD clinical phenotype.

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Neuropathological events featuring early stages of Alzheimer's disease (AD) appear in the entorhinal cortex (EC), subiculum (SB) and cornu ammonis 1 (CA1) of hippocampus, which may account for associative memory deficits in non-demented people with mild cognitive impairment (MCI). To test this hypothesis in vivo, we investigated whether volume changes in these regions are related to failures in associative memory in MCI as compared to cognitively normal (CN) elderly subjects. Volume changes in EC and hippocampal subfields were determined by using deformation-based morphometry techniques applied to probabilistic cytoarchitectonic maps derived from post mortem human brains. CN subjects were distinguished from MCI patients by firstly identifying local volume differences in EC and hippocampus, and then evaluating the way in which these anatomical changes correlated with performance in a non-intentional face-location association task. MCI patients not only performed worse than CN elders in building new associations, but they were further unable to benefit from semantic encoding to improve episodic binding. According to our initial hypothesis, local volume reductions in both EC and hippocampal CA accounted for group differences in associative memory whereas atrophy in CA, but not in EC, accounted for semantic encoding of associations. Two main conclusions can be drawn from the present study: i) access to semantic information during encoding does not reduce the episodic deficit in MCI; and ii) EC and hippocampal CA, two regions early affected by AD neuropathology, are responsible, at least partially, for associative memory deficits observed in MCI patients.

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AIMS: To describe the relation between the level of cognitive impairment in Alzheimer's disease and the use of cholinesterase inhibitors (ChEIs) in neurology, geriatric and psychiatric units, and to establish the clinical profile of these patients. PATIENTS AND METHODS: An epidemiological, multi-centre, cross-sectional study was conducted. Subjects included in the study were consecutive outpatients diagnosed with Alzheimer's disease, in accordance with the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders, and who had been treated with rivastigmine, donepezil or galantamine, either on its own or in association with memantine in the last six months. The recruitment period lasted three months. In a single visit, researchers determined the medication that was used, the dose, the mini-mental test, the overall clinical impression-overall improvement and the overall clinical impression-severity of the disease. A total of 1940 patients were selected from neurology, psychiatric and geriatric services all over the country. Possible differences in the habits of different specialists as regards prescribing were analysed, together with the relation between cognitive impairment and the type of medication employed. RESULTS: The mean age of the patients was 77 +/- 6.6 years, 62% of whom were females; the mean score on the mini-mental test was 17.4 +/- 5.5. The mini-mental score was similar in patients treated with rivastigmine (18.02 +/- 5.23), donepezil (17.08 +/- 5.54) or galantamine (17.34 +/- 5.38). In patients who were treated with memantine in association with a ChEI, the mini-mental score was significantly lower (11.44 +/- 5.68) (p < 0.0001). The doses of the different ChEIs used by the specialists were similar. A higher percentage of patients had maximum doses of donepezil (81%) than in the cases of rivastigmine (43%) and galantamine (67%). CONCLUSIONS: The different specialists involved (neurologists, geriatricians and psychiatrists) displayed similar habits regarding the utilisation of ChEIs to treat Alzheimer's disease. There was no relation between the degree of impairment and the drug chosen, except in the case of memantine.

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Describir la relación entre el nivel de deterioro cognitivo en la enfermedad de Alzheimer y el uso de inhibidores colinesterásicos (IChE) en las consultas de neurología, geriatría y psiquiatría, y caracterizar el perfil clínico de estos pacientes. Pacientes y métodos. Estudio epidemiológico, multicéntrico y transversal. Se incluyeron pacientes consecutivos de consultas externas con diagnóstico de enfermedad de Alzheimer según criterios del Manual diagnóstico y estadístico de lostrastornos mentales, cuarta edición, en tratamiento con rivastigmina, donepecilo o galantamina, solo o asociado a memantina, en los últimos seis meses. El período de reclutamiento duró tres meses. En una visita única se determinó el fármaco utilizado, dosis, test minimental, impresión clínica global-mejoría global e impresión clínica global-gravedad de la enfermedad.Se seleccionaron 1.940 pacientes de consultas de neurología, psiquiatría y geriatría de todo el país. Se analizaron posibles diferencias en los hábitos de prescripción entre los diferentes especialistas, y la relación entre deterioro cognitivo y tipo de fármacoutilizado. Resultados. La edad media de los pacientes era de 77 ± 6,6 años, un 62% mujeres, con una media en el test minimental de 17,4 ± 5,5. El minimental era similar en los pacientes tratados con rivastigmina (18,02 ± 5,23), donepecilo (17,08 ± 5,54) o galantamina (17,34 ± 5,38). En los pacientes tratados con memantina asociada a un IChE, el minimental era significativamente inferior (11,44 ± 5,68) (p < 0,0001). Las dosis de los diferentes IChE utilizadas por los especialistas fueronsimilares. Un mayor porcentaje de pacientes tenía dosis máximas de donepecilo (81%) frente a rivastigmina (43%) y galantamina (67%). Conclusiones. Los hábitos de utilización de IChE en la enfermedad de Alzheimer son similares entre los diferentes especialistas implicados (neurólogos, geriatras, psiquiatras). No había relación entre el grado de deterioro y el fármaco elegido, excepto en el caso de la memantina

To describe the relation between the level of cognitive impairment in Alzheimer’s disease and the use ofcholinesterase inhibitors (ChEIs) in neurology, geriatric and psychiatric units, and to establish the clinical profile of these patients. Patients and methods. An epidemiological, multi-centre, cross-sectional study was conducted. Subjects included inthe study were consecutive outpatients diagnosed with Alzheimer’s disease, in accordance with the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders, and who had been treated with rivastigmine, donepezil or galantamine,either on its own or in association with memantine in the last six months. The recruitment period lasted three months. In a single visit, researchers determined the medication that was used, the dose, the mini-mental test, the overall clinicalimpression-overall improvement and the overall clinical impression-severity of the disease. A total of 1940 patients were selected from neurology, psychiatric and geriatric services all over the country. Possible differences in the habits of differentspecialists as regards prescribing were analysed, together with the relation between cognitive impairment and the type of medication employed. Results. The mean age of the patients was 77 ± 6.6 years, 62% of whom were females; the mean score on the mini-mental test was 17.4 ± 5.5. The mini-mental score was similar in patients treated with rivastigmine (18.02 ± 5.23),donepezil (17.08 ± 5.54) or galantamine (17.34 ± 5.38). In patients who were treated with memantine in association with a ChEI, the mini-mental score was significantly lower (11.44 ± 5.68) (p < 0.0001). The doses of the different ChEIs used by thespecialists were similar. A higher percentage of patients had maximum doses of donepezil (81%) than in the cases of rivastigmine (43%) and galantamine (67%). Conclusions. The different specialists involved (neurologists, geriatricians andpsychiatrists) displayed similar habits regarding the utilisation of ChEIs to treat Alzheimer’s disease. There was no relation between the degree of impairment and the drug chosen, except in the case of memantine

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Introducci¨®n. Existe una fuerte asociaci¨®n entre el alelo¦Å4 de la apolipoprote¨ªna E (APOE) y enfermedad de Alzheimer(EA), constituyendo este alelo un factor de riesgopara padecer esta enfermedad. Su asociaci¨®n con la demenciacon cuerpos de Lewy (DCL) es objeto de controversia. Enla DCL la presencia de APOE4 se ha relacionado con unamayor carga de placas seniles y degeneraci¨®n neurofibrilar.M¨¦todo. Estudio de casos y controles en el que se determin¨®el genotipo APOE mediante la t¨¦cnica de reacci¨®nen cadena de la polimerasa (PCR) modificada de Wenhamen 306 pacientes diagnosticados de EA probable, criteriosNINCDS-ADRDA, 58 casos de DCL probable, criterios de consensode McKeith et al. (1996), todos ellos con SPECT con 123IFP-CIT patol¨®gico, y 80 controles normales (CN) de edad y distribuci¨®npor sexos similares.Resultados. La frecuencia de alelos fue la siguiente:DCL ¦Å4: 16%; ¦Å3: 75%; ¦Å2: 9%; EA ¦Å4: 32%; ¦Å3: 67%; ¦Å2:1%; CN ¦Å4: 12%; ¦Å3: 83%; ¦Å2: 5%. En los tres grupos la distribuci¨®nde alelos en ambos sexos fue similar.Conclusiones. En la DCL la frecuencia de ¦Å4 (16%) esmuy inferior a la de la EA (32%) y muy pr¨®xima a la cifra delos CN (12%). Teniendo en cuenta que la presencia de alteracionesmorfopatol¨®gicas tipo Alzheimer en la DCL, fundamentalmentedegeneraci¨®n neurofibrilar, se correlacionainversamente con la presencia de signos parkinsonianos, esposible que este grupo represente a las formas puras de laenfermedad, aunque la falta de comprobaci¨®n neuropatol¨®gicano permite confirmar esta hip¨®tesis (AU)

Introduction. There is a strong association betweenthe ¦Å4 allele of apolipoprotein E (APOE) and Alzheimer¡¯sdisease (AD). This converts this allele into a risk factorfor the development of AD. The association between APOE4and dementia with Lewy bodies (DLB) is under discussion.In DLB, the presence of APOE4 has been related with a greateramount of senile plaques and neurofibrillary tangles.Method. This is a case-control study in which the APOEgenotype was determined using the modified PCR techniqueof Wenham in 306 patients with diagnosis of probably AD,NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeithet al. consensus criteria (1996), all of them with SPECT withpathological 123I-FP-CIT and 80 normal controls (NC) havingsimilar age and gender distribution.Results. The frequency of alleles was: DLB group ¦Å4:16%; ¦Å3: 75%; ¦Å2: 9%; AD: ¦Å4: 32%; ¦Å3: 67%; ¦Å2: 1%;and in the normal control group: ¦Å4: 12%; ¦Å3: 83%; ¦Å2:5%. The percentage of alleles in both genders was similarin the three groups.Conclusions. APOE4 percentage in DLB group (16%)was lower than in AD group (32 %), and similar to thecontrol group (12 %). Considering that the presence ofmorphopathological Alzheimer type alterations in DBL,essentially neurofibrillary tangles, is inversely correlatedwith the presence of Parkinsonian signs, this group mayrepresent pure forms of the disease, although the lack ofneuropathological demonstration does not make it possibleto confirm this hypothesis (AU)

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INTRODUCTION: There is a strong association between the e4 allele of apolipoprotein E (APOE) and Alzheimer's disease (AD). This converts this allele into a risk factor for the development of AD. The association between APOE4 and dementia with Lewy bodies (DLB) is under discussion. In DLB, the presence of APOE4 has been related with a greater amount of senile plaques and neurofibrillary tangles. METHOD: This is a case-control study in which the APOE genotype was determined using the modified PCR technique of Wenham in 306 patients with diagnosis of probably AD, NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeith et al. consensus criteria (1996), all of them with SPECT with pathological 123I-FP-CIT and 80 normal controls (NC) having similar age and gender distribution. RESULTS: The frequency of alleles was: DLB group epsilon4: 16%; epsilon3: 75%; epsilon2: 9%; AD: epsilon4: 32%; epsilon3: 67%; epsilon2: 1%; and in the normal control group: epsilon4: 12%; epsilon3: 83%; epsilon2: 5%. The percentage of alleles in both genders was similar in the three groups. CONCLUSIONS: APOE4 percentage in DLB group (16%) was lower than in AD group (32%), and similar to the control group (12%). Considering that the presence of morphopathological Alzheimer type alterations in DBL, essentially neurofibrillary tangles, is inversely correlated with the presence of Parkinsonian signs, this group may represent pure forms of the disease, although the lack of neuropathological demonstration does not make it possible to confirm this hypothesis.

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INTRODUCTION: Topographic disorientation is defined as the difficulty to find one s way in familiar surroundings. It can be due to an amnesic or agnostic like defect, which is known as topographic amnesia or agnosia. This disorder can give rise to disability in the life of the patient and may well go undetected unless a suitable neuropsychological study is conducted. CASE REPORTS: We report the cases of two patients with infarction in the territory of the right posterior cerebral artery who began with hemianopsia and topographic disorientation. One of the cases was due to a disorder affecting spatial memory and the other was caused by errors in visuospatial perception. The battery of tests for studying visuospatial perception VOSP was administered. CONCLUSIONS: Occipital lesions are often associated with topographic disorientation, especially when it is the right hemisphere that is injured. It is important to detect this alteration, which makes the patient totally dependent on others even in his or her own home. The appearance of an amnesic or agnostic type disorientation can be related with a disorder affecting one of the two systems that play a part in the processing of visual data. A guided neuropsychological study can provide us with a great deal of information about the type of disorder presented by the patient.

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Introducción. La desorientación topográfica se define como la dificultad para encontrar el camino en un entorno familiar. Puede deberse a un defecto de tipo amnésico o agnósico, que se conoce como amnesia o agnosia topográfica. Este trastorno puede producir invalidez en la vida del paciente y pasar desapercibido si no se realiza un estudio neuropsicológico apropiado. Casos clínicos. Se presentan dos pacientes con infartos en el territorio de la arteria cerebral posterior derecha que iniciaron con hemianopsia y desorientación topográfica; uno de los casos se debía a una afectación de la memoria espacial, y el otro, a fallos en la percepción visuoespacial. Se aplica la batería de tests para el estudio de la percepción visuoespacial VOSP. Conclusiones. Las lesiones occipitales se asocian con frecuencia a la desorientación topográfica, especialmente cuando el hemisferio lesionado es el derecho. Es importante detectar esta alteración, que hace al paciente completamente dependiente para desenvolverse incluso en su propia casa. La aparición de una desorientación de tipo amnésico o agnósico puede relacionarse con la afectación de uno de los dos sistemas que participan en el procesamiento de la información visual. Un estudio neuropsicológico orientado puede aportar mucha información acerca del tipo de alteración que presenta el paciente (AU)

Introduction. Topographic disorientation is defined as the difficulty to find one’s way in familiar surroundings. It can be due to an amnesic or agnostic-like defect, which is known as topographic amnesia or agnosia. This disorder can give rise to disability in the life of the patient and may well go undetected unless a suitable neuropsychological study is conducted. Case reports. We report the cases of two patients with infarction in the territory of the right posterior cerebral artery who began with hemianopsia and topographic disorientation. One of the cases was due to a disorder affecting spatial memory and the other was caused by errors in visuospatial perception. The battery of tests for studying visuospatial perception VOSP was administered. Conclusions. Occipital lesions are often associated with topographic disorientation, especially when it is the right hemisphere that is injured. It is important to detect this alteration, which makes the patient totally dependent on others even in his or her own home. The appearance of an amnesic or agnostic type disorientation can be related with a disorder affecting one of the two systems that play a part in the processing of visual data. A guided neuropsychological study can provide us with a great deal of information about the type of disorder presented by the patient (AU)

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Transient topographical disorientation (TTD) is a short-lasting inability to find one's way in a familiar environment, while the patient remains conscious and is able to recall what happened. We report the study of 10 patients with episodes of TTD, studied on the days following the last episode. The episodes of TTD could be separated into two types: the patients either reported difficulties in spatial orientation with preserved abilities to recognize landmarks and objects, or the difficulties appeared with the recognition of landmarks. Tests exploring spatial orientation, as well as higher visuoperceptive capacities were altered in most of the patients and brain SPECT showed hypoperfusion of the right hemisphere in all patients, which could also be demonstrated 2 years later in some cases. Altogether, our findings suggest that TTD is frequently associated with a more persistent right hemisphere dysfunction of unknown cause. This chronic alteration could represent either a sequel of the acute episode or a preexisting right hemisphere deficit, which inclined the acute insult to be manifested as TTD.

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INTRODUCTION AND DEVELOPMENT: This work reviews the relation between Alzheimer s disease (AD) and women, a very interesting issue both for its socio economic, and etiopathogenic and therapeutic aspects. Much of the prevalent research conducted in this field shows that a higher proportion of suffers from this disease are women, and in the work on incidence there is at least a tendency toward the same conclusion, especially at a very advanced age. In fact, the risk of suffering from AD is greater among women and most of the patients we attend are females, which is to a large extent associated with the fact that women live longer. However, it is possible that there are other biological factors involved and for this reason the action of estrogens on the brain and the consequences of women s being deprived of them during menopause is of special interest. CONCLUSIONS: Different studies have shown that the administration of hormone replacement therapy (HRT) lowers the risk of suffering from this disease, although design defects make it necessary to wait for the conclusions from other research work currently being conducted. There are also data that supports the idea that HRT can be beneficial in AD if it is administered in suitable doses. Obviously gender can influence or modulate other risk factors (RF). Genetic factors are not easily modified and for this reason research is currently aimed at factors in which a strong environmental component is involved. Another very controversial possible RF is lack of schooling, but some data support the notion that its influence can be especially harmful among females. This is a very important hypothesis because women make up the greater part of the illiterate population in Spain. Finally, women are also prevalent among caregivers and, therefore, suffer AD from both angles: they must care and be cared for. The reaction to this situation seems to be gender specific, which means that women in particular suffer the consequences of the lack of reciprocity brought about by AD something that does not happen in other equally devastating chronic processes, but which affect the physical sphere.

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Introducción y desarrollo. Este trabajo revisa la relación entre la enfermedad de Alzheimer (EA) y la mujer, un capítulo muy interesante, tanto por sus aspectos socioeconómicos, como por los etiopatogénicos y terapéuticos. En efecto, gran parte de los trabajos de prevalencia muestran un predominio femenino en esta enfermedad, y en los trabajos de incidencia hay al menos una tendencia al predominio femenino, especialmente en edad muy avanzada. De hecho, el riesgo de padecer una EA es superior en el sexo femenino y la mayor parte de los enfermos que asistimos son mujeres, lo que se relaciona en gran medida con la mayor supervivencia de la mujer. No obstante, es posible que intervengan otros factores biológicos y, en este sentido, la acción de los estrógenos sobre el cerebro y las consecuencias de su deprivación durante la menopausia son muy interesantes. Conclusiones. Diversos estudios han mostrado que la administración de terapéutica hormonal sustitutiva (THS) disminuye el riesgo de padecer la enfermedad, aunque defectos de diseño hacen necesario esperar las conclusiones de otros trabajos en marcha. También existen datos que apoyan que la THS puede conseguir beneficios en la EA si se administra a dosis adecuadas. Por supuesto, el sexo puede influir o modular otros factores de riesgo (FR). Los factores genéticos se modifican poco y, por ello, se prefiere de momento dirigir la atención a los factores de fuerte componente ambiental. En este sentido, la falta de escolarización es un posible FR muy discutido, pero ciertos datos apoyan que su influencia puede ser especialmente perniciosa en el sexo femenino; este es un dato importante, porque precisamente entre los analfabetos de nuestro país predominan las mujeres. Finalmente, en lo que se refiere a los cuidadores, también aquí predominan las mujeres, que, por tanto, sufren la EA en ambas vertientes: recibir y administrar cuidados. La reacción a esta situación parece ser genero específica, de forma que la mujer sufre especialmente las consecuencias de la falta de reciprocidad que ocasiona la EA, algo que no ocurre en otros procesos crónicos igualmente devastadores, pero que afectan la esfera física (AU)

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INTRODUCTION: Calcium embolism is an uncommon cause of stroke which may not be diagnosed in cases which do not involve cardiac surgery or catheterization. The emboli may come from cardiac valves or calcified atheroma of the aortic or carotid arteries. CASE REPORTS: Two patients with cerebral infarcts secondary to spontaneous calcium embolism confirmed by neuro imaging. In both cases on CT scans there were dense points corresponding to calcified material within the middle cerebral artery or one of its branches. In the first case migration of the calcified point following the course of the artery was observed. CONCLUSIONS: Cranial CAT scans are essential for diagnosis of calcium embolism. Migration of the calcified point confirms the diagnosis. It is still not clear whether valve replacement is necessary in these patients and treatment with antiaggregants and/or anticoagulants is controversial.

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Introducción. La embolia cálcica es una causa infrecuente de ictus que puede pasar desapercibida en los casos que no se asocian a cirugía o cateterismo cardíaco. Los émbolos pueden originarse en válvulas cardíacas o en ateromas calcificados de aorta o carótida. Casos clínicos. Dos pacientes con infarto cerebral secundario a embolismo de calcio espontáneo confirmado por neuroimagen. En ambos se apreciaron en la TAC puntos densos correspondientes a material cálcico dentro de la arteria cerebral media o alguna de sus ramas. En el primero además se observó la migración del punto cálcico siguiendo el trayecto de la arteria. Conclusiones. La TAC craneal es fundamental para el diagnóstico de embolismo cálcico. La migración del punto cálcico confirma el diagnóstico. La necesidad de recambio valvular en estos pacientes no se ha establecido suficientemente y el empleo de tratamiento antiagregante o anticoagulante es controvertido (AU)

INTRODUCTION: Calcium embolism is an uncommon cause of stroke which may not be diagnosed in cases which do not involve cardiac surgery or catheterization. The emboli may come from cardiac valves or calcified atheroma of the aortic or carotid arteries. CASE REPORTS: Two patients with cerebral infarcts secondary to spontaneous calcium embolism confirmed by neuro imaging. In both cases on CT scans there were dense points corresponding to calcified material within the middle cerebral artery or one of its branches. In the first case migration of the calcified point following the course of the artery was observed. CONCLUSIONS: Cranial CAT scans are essential for diagnosis of calcium embolism. Migration of the calcified point confirms the diagnosis. It is still not clear whether valve replacement is necessary in these patients and treatment with antiaggregants and/or anticoagulants is controversial (AU)

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We report a family with hereditary neuropathy with liability to pressure palsies (HNPP) and chromosome 17p11.2 deletion. This family exhibits a peculiar phenotype consisting in recurrent brachial plexopathy episodes. This phenotype has to be distinguished from hereditary neuralgic amyotrophy on clinical grounds. Although the incidence of brachial plexopathy on HNPP is relatively high it is unusual as the sole symptom of the disease. It is noteworthy that in the six published families with this peculiar phenotype most of the acute episodes became evident after sleep. A greater liability of the plexus and a greater vulnerability to mechanical factors during sleep hours are the suggested mechanisms to explain this rare clinical onset. Recurrent painless brachial plexopathy when associated to generalized conduction abnormalities should suggest a HNPP.

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Presentamos una familia con neuropatía hereditaria con susceptibilidad a la presión (NHSP), portadora de la deleción 17p11.2, con un fenotipo clínico peculiar consistente en parálisis recurrentes del plexo braquial. Este fenotipo de NHSP debe distinguirse clínicamente de la amiotrofia neurálgica hereditaria. Aunque la incidencia de plexopatía braquial en la NSHP es relativamente alta, es bastante inusual que constituya la única manifestación clínica de la enfermedad. En las seis familias hasta ahora descritas con este fenotipo es de destacar que el factor desencadenante más frecuente de los episodios lo constituye el sueño. Una mayor susceptibilidad del plexo braquial y una mayor vulnerabilidad del mismo a factores mecánicos durante las horas de sueño son propuestas para explicar esta presentación clínica inusual. La plexopatía braquial recurrente no dolorosa asociada a anomalías difusas de conducción debe hacer sospechar una NSHP (AU)

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BACKGROUND: Psychotic symptoms appear during the course of Alzheimer's disease, but their frequency and intensity vary according to different studies and their nature remains unsettled. OBJECTIVES: To study the frequency and intensity of psychotic symptoms in two transversal series of patients with Alzheimer's disease and analyze its relationship with the duration of the disease and severity of cognitive impairment. PATIENTS AND METHODS: This study has been carried out in patients suffering from probable Alzheimer's disease (NINDS-ADRDA criteria). The stage of the disease was determined according to FAST, and the intensity of cognitive impairment in Mini Mental State Examination was classified as mild, moderate or severe. Frequency and intensity of psychotic symptoms (delusions, hallucinations and misidentifications) were determined by means of semistructured interviews (BEHAVE-AD 78 patients and CUSPAD 69 patients). The results obtained in these three groups of patients were compared through ANOVA variance analysis and mean contrast. Variance and covariance analysis were done to determine the relationship between psychotic symptoms and other variables (degree of cognitive impairment, length of evolution and stage of the disease). For this purpose, the patients with Alzheimer's disease but without psychotic symptoms were considered as control and compared to patients with psychotic symptoms. RESULTS: Nearly half the patients had psychotic symptoms. Delusions appeared earlier and were more frequent than hallucinations and misinterpretations. The more severe was the cognitive impairment, the more frequent and intense were psychotic symptoms, but the difference was significant only in cases with severe cognitive impairment. Hallucinations appeared mainly in patients with advanced dementia and were related firstly with the intensity of functional and cognitive impairment and secondly with the duration of the disease. CONCLUSIONS: Mild psychotic symptoms, especially delusions, appear early during the course of Alzheimer's disease. The frequency and intensity of these symptoms increase in parallel with the functional and cognitive impairments caused by the disease. Hallucinations, which appear mainly when the dementia is severe, can be considered as an evolutive marker of the process. Psychotic symptoms differ from those occurring in other disorders, either neurologic or psychiatric in nature.

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