[Paretic neurosyphilis: report of five patients]. / Parálisis general sifilítica: presentación de 5 casos.

We report five male patients, aged 35 to 63 years who suffered from paretic neurosyphilis. The clinical course was that of a subacute dementia with a frontal syndrome, with more apathy than euphoria. All were HIV negative and four were heterosexual. In all, the cerebrospinal fluid had a mononuclear pleocytosis and a positive VDRL. EEG was abnormal in the 3 cases in whom it was performed. One patient in whom a brain angiography was performed, had images of vasculitis. Treatment with 18-24 million units of penicillin per day during two weeks or more, was partially effective.

[Posterior cortical atrophy. Report of five cases]. / Atrofia cortical posterior.

Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuo spatial (Balint's and Gertsmann's syndromes) or visuo perceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55years and three females aged 50, 54 and 56 years, with posterior cortical atrophy. Ophthalmologic study was normal in all. Presenting signs and symptoms were visual ataxia, simultagnosia, agraphia, acalculia, spatial disorientation and unilateral neglect (Balint's and Gerstmann's syndromes). Apperceptive visual agnosia, aphasia, apraxia and alexia were also observed. One female had cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipitoparietal areas.

[Prevention and treatment of Alzheimer disease]. / Perspectivas en la prevención y tratamiento farmacológico de la enfermedad de Alzheimer.

The pharmacological interventions for Alzheimer disease should be based in its pathogenic mechanisms such as amyloidogenesis, tau hyperphosphorilation, disturbances in neurotransmission and changes in neuronal trophism. Other therapies derive from epidemiological observations, such as antioxidants and anti-inflammatory drugs, estrogens, statins and anti hypertensive drugs. Some life style interventions, such as changes in diet, exercise and brain stimulation could also be beneficial for the prevention of Alzheimer disease. Ongoing research on pathogenic mechanisms promises the discovery of more effective therapies. Healthy life style should always be recommended due to its benefit and lack of untoward effects.

[Frontotemporal dementia: Review of 63 cases]. / Demencia frontotemporal: Experiencia clínica.

BACKGROUND: Front temporal dementias (FTD) are neurodegenerative disorders characterized by alterations in behavior, affection and language, with relative sparing of episodic memory. There are three major forms of FTD: the frontal or behavioral form, progressive non-fluent aphasia and semantic dementia (that may begin as a fluent progressive aphasia). AIM: To report a retrospective clinical experience of patients with frontotemporal dementia. MATERIAL AND METHODS: Review of 3,700 records of neuropsychological assessments of patients with behavioral disturbances, studied between 1981 and 2008. Of these, 63 patients (59% females) complied with the criteria for frontotemporal dementia. RESULTS: There were 47 cases with the frontal variant, four with non-fluent progressive aphasia and six with fluent progressive aphasias (2 evolved to semantic dementia). The mean age of onset was 60+/-11 years. There were no familiar cases of FTD. CONCLUSIONS: It is clinically difficult to diagnose FTD, since evaluation of attitude or language is required. In addition to structural images, functional images were helpful in some cases, but the definitive diagnosis is anatomical.

Trastornos del lenguaje en el adulto / Language disorders in adults

El presente trabajo tiene por objetivo principal describir los trastornos del lenguaje en el adulto a consecuencia de un daño cerebral. Las alteraciones del lenguaje oral se abordarán considerando los siguientes tópicos: Algunos conceptos lingüísticos básicos para describir con exactitud las alteraciones del lenguaje; el sistema funcional del lenguaje en el que participan áreas persilvianas, hemisferio derecho y zonas prefrontales; las afasias que son un trastorno parcial o total del lenguaje; los pacientes afásicos considerando sus antecedentes clínicos, la actitud frente al defecto, el deterioro intelectual y otros defectos asociados; el tratamiento de las afasias que tiene por objetivo que el paciente logre una comunicación funcional y una adaptación psicosocial en relación a la alteración de lenguaje y por último, los trastornos del discurso que comprometen especialmente su contenido y su uso. En relación a las alteraciones del lenguaje lectoescrito se describirán los diferentes tipos de agrafias y alexias. Finalmente, cabe señalar dos aspectos, el primero, es que además de las afasias existen otros trastornos del lenguaje y el segundo, es que la evaluación del lenguaje es fundamental para el manejo y la rehabilitación del paciente.

The main purpose of the current review is to describe the language disorders in adult as a consequence of brain damage. The oral language disorders will be approached regarding the following topics: some basic linguistic concepts to describe language disturbances accurately; the functional language system involving perisylvian areas, right hemisphere and prefrontal areas; aphasia that represent a total or partial language impairment; the patient with aphasia, regarding their clinical background, their attitude towards the alteration, the cognitive impairment and other associated disorders; the aphasia treatment which has for purposes to achieve a functional communication and psychological and social adaptation considering their language impairment and, at last, the discourse impairment which affects their use and content. Regarding the reading and writing impairment, agraphia and alexia types will be described. Finally, it is important to mention two aspects: first, besides aphasia there are other language disorders and, second, language assessment is essential to the management and rehabilitation of the patient.

[Charles Bonnet syndrome: report of three cases]. / Síndrome de Charles Bonnet: presentación de tres casos y revisión de la literatura.

Charles Bonnet syndrome is characterized by the presence of visual hallucinations associated with loss of vision. We report three patients aged 74, 84 and 80 years (two women) with a severe loss of vision due to ocular diseases and silent visual hallucinations, that they recognized as unreal. Two patients felt that the hallucinations were ominous. Years later, two suffered a probable Alzheimer's disease (AD). One of them had a mild cognitive impairment when the syndrome appeared, that evolved into an AD with psychosis. This syndrome is not uncommon in older patients with loss of vision and it is probably underdiagnosed. Its pathogenesis is probably a cortical deafferentation. The content of the visual hallucinations (faces, landscaped, and so on) coincides with the activation of different areas of the visual association cortices.

Parálisis general sifilítica: presentación de 5 casos / Paretic neurosyphilis: report of five patients

We report five male patients, aged 35 to 63 years who suffered from paretic neurosyphilis. The clinical course was that of a subacute dementia with a frontal syndrome, with more apathy than euphoria. All were HIV negative and four were heterosexual. In all, the cerebrospinal fluid had a mononuclear pleocytosis and a positive VDRL. EEG was abnormal in the 3 cases in whom it was performed. One patient in whom a brain angiography was performed, had images of vasculitis. Treatment with 18-24 million units of penicillin per day during two weeks or more, was partially effective.

[Clinical variants of Alzheimer disease]. / Variabilidad y variantesde la enfermedad de Alzheimer.

The heterogeneity and variants of Alzheimer disease (AD) are reviewed. There are cases with a slow or fast evolution and with early or late onset. Most cases are sporadic but there are also hereditary forms. About 50% of patients show neuropsychiatric disorders (depression and psychoses). Some cases have a greater deficit of right or left hemispheric functions. Among the variants, there are forms that start as pure aphasias, predominantly prefrontal cases and posterior cortical forms. Occasionally AD may simulate other disorders such as supranuclear palsy, corticobasal ganglionar degeneration and Jacob-Creutzfeldt disease. Finally, there are mixed forms, in which AD is associated with cerebrovascular disease (very commonly) and with other diseases such as dementia with Lewy bodies. We conclude that AD is a heterogeneous disorder and, therefore, clinical diagnosis may be insufficient. Biological markers and specific imaging studies are needed for a correct clinical diagnosis.

Atrofia cortical posterior / Posterior cortical atrophy. Report of five cases

Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuo spatial (Balint's and Gertsmann's syndromes) or visuo perceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55years and three females aged 50, 54 and 56 years, with posterior cortical atrophy. Ophthalmologic study was normal in all. Presenting signs and symptoms were visual ataxia, simultagnosia, agraphia, acalculia, spatial disorientation and unilateral neglect (Balint's and Gerstmann's syndromes). Apperceptive visual agnosia, aphasia, apraxia and alexia were also observed. One female had cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipitoparietal areas.

El espectro de las demencias frontotemporales / The spectrum of frontotemporal dementias

With the purpose of displaying the spectrum of frontotemporal dementias and associated disorders we present a series of patients with: frontotemporal dementia (frontal variant), progressive nonfluent aphasia, semantic dementia, apraxia of speech, and amyotrophic lateral sclerosis associated with frontotemporal dementia. In addition we present cases of corticobasal degeneration and progressive supranuclear palsy that may be considered in the frontotemporal dementia spectrum. The diagnostic difficulties, which require of semiologic expertise more than complementary exams, are underscored. On the other hand the necessity of neuropathologic confirmation is emphazised, since the clinical manifestations are not always in accordance with the autopsy features.

Demencias frontotemporales: tres casos de la variante frontal / Frontotemporal dementia: three cases of the frontal variant

The frontotemporal dementias are characterized by behavioral and language disorders, instead of the prominent memory loss that characterizes Alzheimer’s disease. Their loss of social intelligence, the severe apathy or des inhibition does differentiate them to the courtesy of most Alzheimer patients. Three selected cases are reported: the first one was characterized by apathy, the second by loss of the control of impulses, and the third one by complex stereotypic behavior. The diagnostic difficulty is remarked with the initial misdiagnosis in each case, and the clinical aspects are exposed.

En la clínica de las demencias frontotemporales, a diferencia de la enfermedad de Alzheimer, destacan las alteraciones de la conducta y del lenguaje y no las fallas de la memoria episódica. Todavía son poco reconocidas en nuestro medio a pesar de ser una de las 3 primeras causas de demencia degenerativa. Se presentan 3 casos seleccionados de una serie clínica: uno con apatía, otro con desinhibición y el tercero con una conducta estereotipada. Se señalan los aspectos clínicos y los errores diagnósticos cometidos en cada caso.

Lenguaje oral en demencias frontotemporales: experiencia personal y revisión del tema / Oral language in frontotemporal dementia: clinical experience and review

Las variantes de las demencias frontotemporales son la variante frontal (VF), la afasia progresiva no fluente (APnF) y la demencia semßntica (DS), que se inicia como afasia progresiva fluente (APF). En una serie de 40 pacientes con estudio clínico e imagenológico encontramos 31 casos VF, 6 casos APF y 3 APnF. El lenguaje de los casos VF sólo a veces fue normal; se caracterizaba por ser poco informativo y poco fluente, con reducción de las ideas atingentes y aparición de ideas no atingentes o perseverativas. Diecisiete eran afßsicosy2/3 tenían defectos de comprensión. Su actitud era inadecuada, con demencia severa, apatía o euforia. En los grupos APF y APnF el iiscurso era relativamente informativo; la demencia era menos severa y la actitud mßs comprensible (ansiedad) que en el grupo VF. La fluidez diferenciaba esos grupos. Sólo en 2 sujetos del grupo APF se comprobó un defecto semßntico no verbal que permitió diagnosticarlos como DS. Se plantea que en la VF, a diferencia de la enfermedad de Alzheimer, existe una pérdida del interés comunicativo y del darse cuenta de la actitud del otro.

Frontotemporal dementia has 3 variants: frontal or behavioral (VF), progressive non fluent aphasia (APnF) and semantic dementia (DS). This one frequently starts as a progressive fluent aphasia (APF). In a series of 40 patients with clinical and brain imaging we found 31 VF, 6 APF and only 3 APnF cases. The oral language in VF patients was usually abnormal, non fluent and non informative; the number of ideas was reduced and some of them were out of context. Seventeen out of 31 VF had aphasia, and the deficit of oral comprehension was frequent. Their attitude was unusual, with severe dementia, apathy or euphoria. In groups APnF and APF the speech was fairly informative, dementia was less severe and their attitude (anxiety) more understandable than in the group VF. Fluency differentiates these groups. Only 2 subjects in the group APF had a nonverbal semantic defect that led to the diagnosis ofSD. We conclude that in the VF, unlike the usual Alzheimer's disease, there is a loss of interest in the communication and in realize the attitude of the other.

Demencia frontotemporal: experiencia clínica / Frontotemporal dementia: review of 63 cases

Background: Front temporal dementias (FTD) are neurodegenerative disorders characterized by alterations in behavior, affection and language, with relative sparing of episodic memory. There are three major forms of FTD: the frontal or behavioral form, progressive non-fluent aphasia and semantic dementia (that may begin as a fluent progressive aphasia). Aim: To report a retrospective clinical experience of patients with frontotemporal dementia. Material and methods: Review of 3,700 records of neuropsychological assessments of patients with behavioral disturbances, studied between 1981 and 2008. Of these, 63 patients (59 percent females) complied with the criteria for frontotemporal dementia. Results: There were 47 cases with the frontal variant, four with non-fluent progressive aphasia and six with fluent progressive aphasias (2 evolved to semantic dementia). The mean age of onset was 60±11 years. There were no familiar cases of FTD. Conclusions: It is clinically difficult to diagnose FTD, since evaluation of attitude or language is required. In addition to structural images, functional images were helpful in some cases, but the definitive diagnosis is anatomical.

Asociación inversa entre enfermedad de Alzheimer y cáncer / Inverse relationship between Alzheimer's disease and cancer

The increase in life expectancy is accompanied by a concomitant increase in the prevalence of age related disorders, including neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, and various types of cancers. We here report epidemiologic findings suggesting thatthe development of the Alzheimer and a history of cancer are inversely related. The inverse relationship was found when all cancer types were analyzed and also when skin cancers (bothmelanomas and those with good prognosis, that constitute 50% of all cancers) were analyzed separately. In addition, in a population study we found that this inverse relationship was found with dementia of the Alzheimer type and mixed dementia, but not with vascular dementia, suggesting that the association is only with degenerative diseases. We discuss possible explanations for this inverse relationship, among them, the possibility that a common biological mechanism might be regulated in opposite directions in neurodegenerative diseases and cancer.

Anartria o apraxia del habla progresiva / Progressive anarthria or speech apraxia

Two cases of progressive anarthria are reported; we remark their close but distinct relation with speech apraxia. Both of them were older female, with a progressive loss of speech, bilateral paresis of lower face, tongue and palatal muscles. They also had mild pyramidal signs and a fronto-subcortical cognitive deterioration. Brain TC and MRI were within normal limits. One of them had a possible progressive supranuclear palsy, the other one a possible corticobasal degeneration. The analysis of similar cases reports let us to conclude than there are several pathologies that can cause a progressive pseudobulbar palsy. The final diagnosis must be by postmortem examination of the brain.

Se presentan dos casos de anartria progresiva, discutiendo la relación o el diagnóstico diferencial con la apraxia del habla progresiva. En ambos casos se trataba de mujeres mayores de 65 años con un cuadro de pérdida progresiva del lenguaje oral, con diparesia facial, lingual y velar, deterioro cognitivo de tipo frontal y discretos signos piramidales. Las imágenes cerebrales estructurales fueron normales. Uno de ellos pudo corresponder a una parálisis supranuclear progresiva, la otra a una degeneración corticobasal. Se analiza la literatura, llegando a la conclusión de que existen una serie de cuadros que pueden presentarse con un síndrome pseudobulbar progresivo. El diagnóstico definitivo debiera ser patológico.

Demencia semántica: presentación de dos casos y revisión de la literatura / Semantic dementia: report of two cases

Frontotemporal dementia is a neurodegenerative condition that presents with a number of distinct behavioral phenotypes. One of them is semantic dementia (SD), where exists a profound impairment for semantic knowledge related to atrophy of temporal poles. Pathologically, in most cases positive intraneuronal ubiquitin and tau negative inclusions are observed. SD is characterized by fluent, effortless, grammatical speech which lacks informational content, with limited and repetitive content, as well as semantic paraphasias. Also, patients may present with associative visual agnosia, surface dyslexia or dysgraphia, behavioral alterations. Both episodic and autobiographical memory are close to normality. Two female patients with fluent progressive aphasia are reported; they failed in a simple test of semantic association (to point to one of four objects with lesser relation to others). Autobiographical memory was fair. SD can be wrongly diagnosed as left-sided variant of Alzheimer's disease; absence of episodic amnesia and parietal defects may be useful for clinical diagnosis.

La demencia semántica es una variante de las atrofias lobares frontotemporales que se caracteriza por la degradación del conocimiento semántico, de los conceptos. En ella existe una atrofia predominante de los polos temporales, a veces asimétrica; la patología generalmente muestra neuronas con inclusiones ubiquitina (+) y tau (-). Se expresa como una afasia progresiva fluente, sin disartria ni agramatismo, con anomia y parafasias verbales o semánticas, poco informativa. Pueden existir agnosia visual asociativa y alteraciones conductuales; a veces existen alexia y/o agrafía de superficie. Hay respeto relativo de la memoria episódica y autobiográfica. Se presentan dos mujeres con afasia progresiva fluente, alteraciones conductuales y falla en una tarea semántica (4º excluido). A pesar de estos defectos la memoria autobiográfica y el desempeño en sus actividades cotidianas estaban dentro de lo normal. Se concluye que estos cuadros deben diferenciarse de los casos de enfermedad de Alzheimer de predominio izquierdo; el respeto de la memoria episódica y la falta de compromiso parietal descartarían clínicamente esta última patología.

Síndrome de Charles Bonnet: presentación de tres casos y revisión de la literatura / Charles Bonnet syndrome: Report of three cases

Charles Bonnet syndrome is characterized by the presence of visual hallucinations associated with loss of vision. We report three patients aged 74, 84 and 80 years (two women) with a severe loss of vision due to ocular diseases and silent visual hallucinations, that they recognized as unreal. Two patients felt that the hallucinations were ominous. Years later, two suffered a probable Alzheimer's disease (AD). One of them had a mild cognitive impairment when the syndrome appeared, that evolved into an AD with psychosis. This syndrome is not uncommon in older patients with loss of vision and it is probably underdiagnosed. Its pathogenesis is probably a cortical deafferentation. The content of the visual hallucinations (faces, landscaped, and so on) coincides with the activation of different areas of the visual association cortices).

Afasia progresiva fluente: ¿una forma de presentación inicial de demencia semántica? / Fluent progressive aphasia: a form of inicial presentation of semantic dementia?

Se presenta el caso de un hombre de 57 años que presenta un cuadro degenerativo con afasia fluente y moderadas alteraciones de conducta, sin defecto semántico significativo, agnosia visual ni agrafia lexical y su estudio imagenológico cerebral. Se discuten los conceptos de afasia progresiva fluente y demencia semántica, concluyendo que la primera puede evolucionar hacia esta última. Nuestro caso se encontraría en esta situación. Se señala que la patología de las afasias progresivas es variable, siendo lo más frecuente una degeneración frontotemporal (con o sin inclusiones de proteína tau); con menor frecuencia corresponde a una enfermedad de Alzheimer o una degeneración corticobasal.

We report a 57-year-old man presenting with progressive fluent aphasia and behaviour disorder, with no visual semantic loss, visual agnosia, lexical agraphia, nor alexia. We include in this report the brain images, and we discuss the borders between fluent progressive aphasia and semantic dementia. We conclude that fluent progressive aphasia may be the first stage of semantic dementia, noting that the neuropathology of progressive fluent aphasia usually corresponds to frontotemporal lobar degeneration, with or without Tau protein inclusions, as in Alzheimer's Disease or corticobasal degeneration.

Variabilidad y variantes de la enfermedad de Alzheimer / Clinical variants of Alzheimer disease

The heterogeneity and variants of Alzheimer disease (AD) are reviewed. There are cases with a slow or fast evolution and with early or late onset. Most cases are sporadic but there are also hereditary forms. About 50 percent of patients show neuropsychiatric disorders (depression and psychoses). Some cases have a greater deficit of right or left hemispheric functions. Among the variants, there are forms that start as pure aphasias, predominantly prefrontal cases and posterior cortical forms. Occasionally AD may simulate other disorders such as supranuclear palsy, corticobasal ganglionar degeneration and Jacob-Creutzfeldt disease. Finally, there are mixed forms, in which AD is associated with cerebrovascular disease (very commonly) and with other diseases such as dementia with Lewy bodies. We conclude that AD is a heterogeneous disorder and, therefore, clinical diagnosis may be insufficient. Biological markers and specific imaging studies are needed for a correct clinical diagnosis.

Somatoparafrenia: presentación de 3 casos / Somatoparaphrenia: report of three cases

Se presentan 3 casos de somatoparafrenia reunidos en el curso de 30 años. Se trata de adultos con lesiones vasculares extensas del hemisferio derecho, con hemiplejia, heminanestesia y heminanopsia. Todos tuvieron la convicción delirante de que su brazo paralizado pertenecía a un familiar próximo. Se señala la variabilidad de los síntomas (somatoparafrenia, personificación, misoplejia), la relación de estos con la anosodiaforia más que con la anosognosia y con lesiones del hemisferio derecho. Se concluye que, a semejanza de los problemas en el reconocimiento de rostros o del entorno, es necesario distinguir entre el nivel de la percepción y el nivel de la interpretación como propio o como familiar.