[Non-surgical potentially reversible dementias]. / Demencias potencialmente reversibles no quirúrgicas.

AIMS: In this study, we review dementias that are potentially reversible. The paper summarises the causes that essentially require management by medical means, while causes of a surgical nature will be dealt with in a second article. These papers attempt to avoid mistaken diagnoses and labels in patients with a high potential to improve their cognitive disorder and to guide us towards a more suitable management. DEVELOPMENT: Dementia is a public health problem, mainly in countries with long life expectancy. It has an incidence of 3-11% in patients over the age of 65, and 20-50% in those over 85 years old. Most of them (50-70%) have Alzheimer-type dementia, followed by the vascular type (20%); there is a smaller percentage of cases of the so-called subcortical dementias and also those secondary to medical and/or surgical conditions that suggest potential reversibility. These latter cases are not easy to recognise and their incidence, depending on the series, ranges from 0 to 37%. Once they have been diagnosed, it is still difficult to state whether they will in fact turn out to be reversible. Their most common causes, such as deficiencies, metabolic disorders, chronic diseases, toxins, and so on, must be detected as early as possible, which can be done by means of clinical observation and use of the laboratory. CONCLUSIONS: Although the potential to improve in patients with a diagnosis of reversible dementia is still subject to discussion, this brief review guides us in the search for their causes and their management, since late detection and management are very likely to be the cause of a poor progression.

Demencias potencialmente reversibles no quirúrgicas / Non-surgical potentially reversible dementias

Objetivo. Revisamos las demencias que presentan un potencial de reversibilidad. El presente artículo resume las demencias de manejo básicamente médico, mientras que en una segunda entrega destacaremos las de índole quirúrgica. Estos artículos pretenden evitar los diagnósticos y etiquetas erróneas en pacientes que tienen un alto potencial de mejoría de su trastorno cognitivo y orientar hacia un manejo más adecuado. Desarrollo. La demencia es un problema de salud pública, principalmente en los países con expectativa de vida amplia. Su incidencia es de 3-11% en pacientes mayores de 65 años, y de 20-50% en mayores de 85 años. La mayoría (50-70%) presentan demencia de tipo Alzheimer, seguida del tipo vascular (20%), y quedan en menor porcentaje las denominadas demencias subcorticales y las secundarias a patologías médicas y/o quirúrgicas, potencialmente reversibles. Estas últimas no son fáciles de reconocer, y su incidencia en distintas series va del 0 al 37%. Una vez diagnosticadas, es todavía difícil poder asegurar si serán en realidad reversibles; por tanto, debemos detectar precozmente sus causas más comunes, como las carenciales, metabólicas, asociadas a enfermedades crónicas, tóxicas, etc., para lo que nos valdremos de la observación clínica y del laboratorio. Conclusiones. Aunque todavía se encuentra en disputa el potencial de mejoría de los pacientes con un diagnóstico de demencia reversible, esta revisión sucinta nos orienta a la búsqueda intencionada de sus causas y su manejo, ya que es altamente probable que su detección y manejo tardíos sean la causa de una pobre evolución

Aims. In this study, we review dementias that are potentially reversible. The paper summarises the causes that essentially require management by medical means, while causes of a surgical nature will be dealt with in a second article. These papers attempt to avoid mistaken diagnoses and labels in patients with a high potential to improve their cognitive disorder and to guide us towards a more suitable management. Development. Dementia is a public health problem, mainly in countries with long life expectancy. It has an incidence of 3-11% in patients over the age of 65, and 20-50% in those over 85 years old. Most of them (50-70%) have Alzheimer-type dementia, followed by the vascular type (20%); there is a smaller percentage of cases of the so-called subcortical dementias and also those secondary to medical and/or surgical conditions that suggest potential reversibility. These latter cases are not easy to recognise and their incidence, depending on the series, ranges from 0 to 37%. Once they have been diagnosed, it is still difficult to state whether they will in fact turn out to be reversible. Their most common causes, such as deficiencies, metabolic disorders, chronic diseases, toxins, and so on, must be detected as early as possible, which can be done by means of clinical observation and use of the laboratory. Conclusions. Although the potential to improve in patients with a diagnosis of reversible dementia is still subject to discussion, this brief review guides us in the search for their causes and their management, since late detection and management are very likely to be the cause of a poor progression

[Diabetic neuropathy: vascular disease?]. / Neuropatía diabética: una enferemedad vascular?

INTRODUCTION: The major neurologic complications of diabetes are: 1) Neuropathy, both peripheral and autonomic, with principal manifestations in the lower limbs; 2) Microvascular disease, mainly affecting the retina, resulting in blindness; and 3) Macrovascular disease, presenting with atherosclerosis in the cerebral arteries causing ischemic cerebrovascular disease and stroke. METHOD: The definition of diabetic neuropathy has changed over the last 50 years. Over the last 20 years there have been three main pathogenetic theories to explain diabetic neuropathy: the polyol pathway theory, the microvascular theory, and the glycosylation end product theory. It is apparent that several pathophysiologic factors probably operate simultaneously, and it may be too simplistic to attempt to explain the many clinical and pathologic findings of diabetic neuropathy through a single theory. Diabetic peripheral neuropathy is not caused by large vessel peripheral vascular disease, however, it does appear that small vessel disease plays a role. CONCLUSION: It seems likely, that microangiopathy on the one hand and changes of various metabolic pathways due to hyperglycemia on the other hand are much more related to each other than it was suggested previously.

Neuropatía diabética: ¿una enfermedad vascular? / Diabetic neuropathy: vascular disease?

Introducción. Las principales complicaciones neurológicas de la diabetes son: 1) Neuropatía periférica y autonómica, cuyas manifestaciones principales ocurren en las extremidades inferiores; 2) Enfermedad microvascular, que afecta principalmente a la retina y desencadena ceguera, y 3) Enfermedad macrovascular, presente con aterosclerosis en las arterias cerebrales y que causa enfermedad isquémica cerebrovascular. Desarrollo. La definición de la neuropatía diabética (ND) ha cambiado en los últimos 50 años; en los últimos veinte se han propuesto tres teorías patogénicas principales para explicar la ND: la teoría de la vía poliol, la teoría microvascular y la teoría del producto final de la glucosilación. Probablemente, varios factores patofisiológicos actúan simultáneamente, y puede parecer muy simplista explicar que muchas manifestaciones clínicas y hallazgos patológicos en la ND pueden ser resultado de un solo mecanismo. La ND periférica no la ocasiona la enfermedad vascular periférica de grandes vasos; sin embargo, parece que la enfermedad de pequeños vasos desempeña un papel importante. Conclusión. Es probable que la microangiopatía, por un lado, y las alteraciones de varias vías metabólicas por la hiperglucemia, por otro, se relacionen más entre sí que como se había descrito anteriormente (AU)

Introduction. The major neurologic complications of diabetes are: 1) Neuropathy, both peripheral and autonomic, with principal manifestations in the lower limbs; 2) Microvascular disease, mainly affecting the retina, resulting in blindness; and 3) Macrovascular disease, presenting with atherosclerosis in the cerebral arteries causing ischemic cerebrovascular disease and stroke. Method. The definition of diabetic neuropathy has changed over the last 50 years. Over the last 20 years there have been three main pathogenetic theories to explain diabetic neuropathy: the polyol pathway theory, the microvascular theory, and the glycosylation end product theory. It is apparent that several pathophysiologic factors probably operate simultaneously, and it may be too simplistic to attempt to explain the many clinical and pathologic findings of diabetic neuropathy through a single theory. Diabetic peripheral neuropathy is not caused by large-vessel peripheral vascular disease, however, it does appear that small-vessel disease plays a role. Conclusion. It seems likely, that microangiopathy on the one hand and changes of various metabolic pathways due to hyperglycemia on the other hand are much more related to each other than it was suggested previously (AU)

Is it healthy to be chaotic?

The concept of Chaos has proven to be one of the greatest scientific advances that have led to radical philosophical implications. It deals with dynamic systems whose determining factors are completely unknown to us. Sometimes it seems that these dynamic systems exhibit a stochastic behavior while others portray simpler or better known behaviors where determinism is obvious. When the physician faces chaotic, dynamic systems, he or she wonders if it is healthy for these biologic systems to be chaotic. When analyzing the variation in brain and heart rates mathematically, the conclusion is that these rates are chaotic, complicated and unpredictable. Because each organ regulates its own performance, the mathematical variations seem to be the result of the organ's determinism rather than fluctuation. This healthy variability is neither a random nor an uncontrolled fluctuation. It is a certain, well-harmonized chaos, that 'provides the body with the flexibility to respond to different stimuli'.

[Chronic hydrocephaly in adults. A diagnostic and therapeutic challenge]. / Hidrocefalia crónica del adulto. Reto diagnóstico y terapéutico.

OBJECTIVE: To describe the main clinical features of the different conditions which present as chronic hydrocephaly, emphasising the diagnosis and treatment. DEVELOPMENT: In the study of any dementia syndrome, normotensive hydrocephaly (HN) is of particular interest since this condition may be treated and cured. However, prediction of which cases will benefit from surgery is one of the most important aspects of diagnosis. The development of paraclinical investigations of the dynamics of cerebrospinal fluid (CSF) has led to great advances in the management of these chronic forms of hydrocephaly, since they are the best parameters for prognosis when deciding whether to insert a by pass. The treatment of post traumatic hydrocephaly has also benefited from these studies, since the cases of simple atrophy may now be distinguished from those which follow a clinical course similar to HN. Recently, two types of hydrocephaly have been identified as being very different from the typical forms. These are external hydrocephaly, in which CSF accumulates in the convexity of the cerebral hemispheres, and long term ventriculomegaly, in which severe ventricular dilation is accompanied by cognitive alterations and features of acute hydrocephaly. CONCLUSIONS: Treatment of each of these conditions is a challenge. Therefore careful analysis of the clinical features, imaging studies and CSF dynamics is essential when deciding whether or not to use by pass treatment.

Hidrocefalia crónica del adulto. Reto diagnóstico y terapéutico / Chronic hydrocephaly in adults. A diagnostic and therapeutic challenge

Objetivo. Presentar las principales características clínicas de las diferentes entidades que se manifiestan como hidrocefalia crónica, con énfasis en su diagnóstico y tratamiento. Desarrollo. En el estudio de cualquier síndrome demencial, la hidrocefalia normotensa (HN) tiene un interés particular por la potencial mejoría y curación del cuadro, pero la capacidad para predecir los casos en que será benéfica la cirugía es uno de los puntos más importantes en el diagnóstico. El advenimiento de estudios paraclínicos sobre la dinámica del líquido cefalorraquídeo (LCR) ha revolucionado el manejo de estas formas crónicas de hidrocefalia, ya que constituyen el mejor parámetro pronóstico para decidir colocar un sistema de derivación (SD). El tratamiento de la hidrocefalia postraumática también se ha beneficiado con estos estudios, ya que es posible determinar los casos de atrofia simple de los que cursan con evolución similar a la HN. Recientemente se han identificado dos variedades de hidrocefalia muy diferentes a las formas típicas: la hidrocefalia externa, en la que el LCR se acumula en la convexidad de los hemisferios cerebrales, y la ventriculomegalia de larga evolución, en la que una dilatación ventricular extrema se acompaña de alteraciones cognitivas y datos de hidrocefalia aguda. Conclusiones. Cada una de estas entidades ofrece un reto para su tratamiento, por lo que un análisis cuidadoso de las manifestaciones clínicas, de los estudios de imagen y de la dinámica del LCR es esencial para ofrecer o no un tratamiento en base a SD (AU)

[Attention deficit syndrome. Basic aspects of its diagnosis and treatment]. / Síndrome de atención deficitaria. Aspectos básicos del diagnóstico y tratamiento.

INTRODUCTION: The attention deficit syndrome is one of the commonest behavior disorders of infancy, although it is considerably underestimated. DEVELOPMENT: The aetiology of attention deficit syndrome is multifactorial since genetic, environmental, nutritional and psychosocial factors have been found to be associated with its development. Diagnosis depends mainly on the identification of behavior changes especially of inattention, impulsiveness and hyperactivity: it should be made at home and at school and based on the exclusion of severe organic and neurological disorders which also present initially with similar characteristics. Electrophysiological studies may be useful since multiple electroencephalographic changes have been reported, as have prolonged latency of III and V waves of brain stem evoked potentials, The best management requires educational therapy combined with controlled medical treatment. Use of stimulants should be carefully considered and the tricyclic drugs are preferably the first pharmacological option. CONCLUSIONS: Current findings regarding the repercussions of attention deficit syndrome in adults shows the need for early diagnosis and treatment of the condition so as to avoid severe personality disorders including antisocial and criminal behavior. It is essential that the parents and the child himself understand the condition in order to achieve satisfactory results of long term treatment.

Síndrome de atención deficitaria.Aspectos básicos del diagnóstico y tratamiento / Attention deficit syndrome. Basic aspects of diagnosis and treatment

Introducción. El síndrome de atención deficitaria (SAD) es uno de los trastornos conductuales más comunes de la infancia, a pesar de lo cual es altamente subestimado. Desarrollo. La etiología del SAD es multifactorial pues se identifica con factores genéticos, ambientales, nutricionales y psicosociales en su desarrollo. El diagnóstico se basa primordialmente en la identificación de alteraciones conductuales en las que predominan la desatención, impulsividad e hiperactividad; debe realizarse en casa y en la escuela, y basarse en la exclusión de trastornos orgánicos y neurológicos severos que cursan también en períodos iniciales con manifestaciones semejantes. Los estudios de electrofisiología pueden ser útiles puesto que se han comunicado alteraciones electroencefalográficas múltiples, así como prolongaciones en las latencias de las ondas III y V de los potenciales evocados de tallo. El empleo de una terapia educativa combinada con un tratamiento médico controlado es la medida que ofrece mejores resultados en el manejo; asimismo, debe valorarse de forma muy cuidadosa la administración de estimulantes, con preferencia del uso de tricíclicos como primera medida farmacológica. Conclusiones. Los hallazgos actuales sobre la repercusión del SAD en la vida adulta ponen de manifiesto la necesidad de realizar un diagnóstico y tratamiento temprano de la entidad, para evitar graves trastornos de personalidad e incluso conductas antisociales y criminales. La comprensión del cuadro por parte de los padres y del niño afectado es también de vital importancia para lograr un tratamiento a largo plazo con resultados satisfactorios (AU)

Conceptos actuales sobre cerebelo y cognición / Current concepts regarding the cerebellum and cognition

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[Current concepts regarding the cerebellum and cognition]. / Conceptos actuales sobre cerebelo y cognición.

OBJECTIVE: To review the main cognitive processes regulated by the cerebellum and the anatomical circuits involved in their clinical correlation. DEVELOPMENT: The cerebellum is generally regarded as a regulator of motor function with a key role in movement coordination. Clinical evidence of the relation of the cerebellum to neural functions typically considered as cortical, is supplied by several neuropsychological alterations detected in both degenerative disorders and acute insult to the region such as vascular event and surgery. More anatomical circuits between the cerebellum and several cortical areas and limbic system, with the ventral pons region as main afference and efference relay of all these pathways. Cerebellar structures that are phylogenetically older such as the floculonodullar lobe, vermix, fastigial nuclei and globus nuclei have a tight relationship to emotional control and autonomic manifestations. More complex circuits are founded in the regulation of learning, motor planning and language. Functional imaging studies have helped to confirm the relationship between the cerebellum and memory processes, finding a selective activation of lateral regions during to cerebellar damage, such a frontal like syndromes, memory deficits and aphasia and even though dysmetria with incoordination between mental process velocity and its motor execution. CONCLUSIONS: All the data from clinical and functional studies indicate that the cerebellum has a central co-ordinating function not only of movement but also regulating thought. The cerebellum should, therefore, be reconsidered as a complex neurone system at much the same level as the more advanced cortical structures.

Juvenile familial amyotrophic lateral sclerosis: four cases with long survival.

Four cases are reported of juvenile familial amyotrophic lateral sclerosis (JFALS) with exceptionally long survival (mean=27 years), and consequent development of dementia. Subjects' mean age at onset was 15.7 years. Their clinical features and electrophysiological findings support the diagnosis. One subject's MRI scan showed severe atrophy to the cortex and brain stem; wallerian degeneration in the pyramidal pathway, as reported in other studies, could not be found. JFALS is characterized by the involvement of other neuronal systems not present in the adult form and by long survival after disease onset.

Frequency of motor neuron diseases in a Mexico City referral center.

OBJECTIVE: To analyze the incidence and clinical characteristics of MND (Motor Neuron Diseases) in a Mexican institution. MATERIAL AND METHODS: It was a retrospective study, of 274 definitive MND patients seen in a neurological reference hospital of Mexico City during the period of 1965-1995 (248 as Amyotrophic Lateral Sclerosis, 15 as Progressive Bulbar Palsy, 8 as Primary Lateral Sclerosis and 3 as Progressive Spinal Atrophy). RESULTS: The frequency of MND increased gradually in our institution in the 31 years revised. The mean age of onset in our series was approximately 48 years in contrast to a higher age found in other series. The clinical features are similar to those found elsewhere. CONCLUSIONS: Our study showed that the frequency of MND is increasing in Mexico in a similar fashion to that observed in the rest of the world. This makes conceivable that the incidence of MND in Mexico may also resemble the figures reported worldwide. Prospective population studies are required to establish the incidence of MND in Mexico.

Free-GABA levels in the cerebrospinal fluid of patients suffering from several neurological diseases Its potential use for the diagnosis of diseases which course with inflammation and tissular necrosis.

Free GABA levels were measured in the cerebrospinal fluid (CSF) of 74 neurological patients suffering from cerebral cysticercosis (n = 9), Parkinson's disease (n = 5), multiple sclerosis (n = 6), epilepsy (n = 24), meningeal tuberculosis (n = 6), viral encephalitis (n = 3), cerebrovascular disease (n = 8) and several kinds of dystonia (n = 5). A statistical significant four-fold elevation in free GABA levels was found in patients with cerebral cysticercosis. A non statistical significant two-fold increase in free GABA levels was also encountered in the CSF of patients affected by cerebrovascular disease and viral encephalitis. No changes in CSF free GABA levels were found in patients suffering from any of the other disorders. It is suggested that free GABA levels may be elevated in the CSF of patients suffering from neurological diseases which course with inflammation and tissular necrosis such as cerebral cysticercosis. Much work is needed however to establishd whether CSF free GABA levels can be used as a diagnostic tool in at least some type of these patients.

[Electroencephalographic alterations in patients with migraine with and without aura]. / Alteraciones electroencefalográficas en pacientes con migraña con y sin aura.

We studied 14 patients with migraine with aura (group 1) and 16 patients with migraine without aura (group 2), 92.8% had EEG alterations in ictal and 57% in the interictal phase in group 1. 37.5% had EEG alterations in ictal and 18.7% in the interictal phase in group 2. There was a statistical difference between the two groups and between both phases. We think that patients with EEG alterations during both phase, may have a lower ischemic threshold.

Reflejo palmomentoniano.Su significado clinico en enfermedad neurologica. / Palmo-mental reflex. The clinical significance in neurological disease

Se realizo estudio prospectivo, ciego, para establecer la relacion del reflejo palmomentoniano (PM) y enfermedad neurologica con anormalidad en la tomografia axial computada (TAC). Cien pacientes no seleccionados, asistentes a la consulta externa del INNN fueron estudiados mediante investigacion del PM, antecedente de trauma craneoencefalico (TCE) y TAC.Los pacientes se agruparon por diagnostico, las edades fluctuaron entre 3 y 82 anos, el 46% fueron del sexo masculino. Se realizo analisis de correlacion multivariada y el Stepwise entre las diferentes variables. Los resultados del analisis fueron: 1) El PM y TAC anormal fue mas frecuente en neoplasias intracraneales, traumatismos del sistema nervioso y enfermedades heredo-degenerativas y desmielinizantes; 2) El PM resulto mas frecuente en pacientes con antecedentes de TCE y en quienes presentaban TAC anormal; 3) El PM unilateral predomino en pacientes menores de 29 anos, principalmente en menores de 10 anos; 4) EL MP unilateral se asocio frecuentemente con TAC normal; (P < 0.01). Consideramos que existe relacion entre la presencia del PM y anormalidad estructural en la TAC. El PM unilateral se relaciono con posible retardo en la maduracion cerebral. Concluimos que este reflejo es un signo de valor clinico en enfermedad neurologica

Sindrome de Fisher. Presentacion de 4 casos y revision de la literatura. / Fisher's syndrome. Report of 4 cases and review of the literature

El Sindrome de Fisher (oftalmoplejia, ataxia y arreflexia) es considerado variante del Sindrome de Guillain-Barre-Strohl, por las siguientes similaridades: historia de infeccion viral inespecifica, ausencia de reglejos miotaticos, curso clinico benigno y disociacion albuminocitologica en el LCR Se reportan 4 casos con este sindrome (tres del Hospital Universitario y uno del Instituto Nacional de Neurologia y Neurocirugia de Mexico), realizando revision de la literatura. Estos casos representan el segundo reporte de Sindrome de Fisher en nuestro pais