[The influence of Spanish neuropsychiatry on the improbable medicine of Ramon Gomez de la Serna]. / Influencia de la neuropsiquiatria española en la medicina inverosimil de Ramon Gomez de la Serna.

INTRODUCTION: El doctor inverosimil (The Improbable Doctor) is a novel by Ramon Gomez de la Serna (1888-1963) in which the reader follows, by means of a series of different clinical cases, the adventures of Doctor Vivar. AIMS: To present and discuss, for the first time, the influence that Spanish neuropsychiatry had on this novel, taking the following short stories as study models: 'Casos cerebrales' ('Brain Cases') and 'La risita' ('The Giggles'). DEVELOPMENT: In these two tales, the improbable doctor embodies the three defining elements of Spanish neuropsychiatry. The repetitions of Alzheimer's disease (or in the way in which progressive general paralysis is referred to) hints at its Germanic orientation. The mixed neuropsychiatric component is exemplified in both his neurological ('La risita') and psychiatric skills ('Casos cerebrales'). Doctor Vivar's affinity to things histopathological is apparent in his wide and timely usage of neurohistological terminology, mainly from Cajal. CONCLUSIONS: It can be deduced that the Spanish neuropsychiatric school had a strong influence on Gomez de la Serna's El doctor inverosimil.

TITLE: Influencia de la neuropsiquiatria española en la medicina inverosimil de Ramon Gomez de la Serna.Introduccion. El doctor inverosimil es una novela de Ramon Gomez de la Serna (1888-1963) donde seguimos, a traves de diversos casos clinicos, las andanzas del doctor Vivar. Objetivo. Presentar y discutir, por primera vez, el influjo que tuvo la neuropsiquiatria española en esta novela, tomando como modelos de estudio los siguientes relatos: 'Casos cerebrales' y 'La risita'. Desarrollo. En este par de relatos, el doctor inverosimil encarna los tres elementos definitorios de la neuropsiquiatria española. En sus repeticiones de la enfermedad de Alzheimer (o en la forma en que se refiere a la paralisis general progresiva) insinua su orientacion germanica. El componente mixto neuropsiquiatrico esta ejemplificado en su destreza tanto neurologica ('La risita') como psiquiatrica ('Casos cerebrales'). La afinidad histopatologica del doctor Vivar es evidente en su amplio y oportuno uso de la terminologia neurohistologica, principalmente la cajaliana. Conclusiones. Es valido deducir que hay una influencia notoria de la escuela neuropsiquiatrica española en El doctor inverosimil de Gomez de la Serna.

[The two faces of veno-arteriolar reflex: cutaneous vasodilatation and vasoconstriction to raise and to lower the arm]. / Las dos caras del reflejo venoarteriolar: vasoconstriccion y vasodilatacion cutanea al bajar y subir el brazo.

INTRODUCTION: The veno-arteriolar reflex (VAR) is triggered by an increase in the transmural venous pressure on placing a part of the body in the same direction as the gravitational acceleration below the heart. AIM: To assess the VAR in healthy subjects on raising a part of the body above the level of the heart. SUBJECTS AND METHODS: VAR was studied in 16 healthy subjects (20-65 years old) by means of changes in the blood flow in the skin detected using a digital infrared photoplethysmograph attached to the fingertip under the following conditions: right arm at the height of the heart, right arm below the heart and right arm below the level of the heart. The variables measured were: amplitude of the blood flow in the skin with the arm raised to the height of the heart (baseline amplitude), percentage decrease of the blood flow in the skin with the arm below the heart and percentage increase in blood flow with the arm above the heart. RESULTS: The percentage of vasoconstriction with the right arm below the heart was 35%, and that of vasodilation, 50%. CONCLUSIONS: Evaluation of the VAR with the arm below the heart causes vasoconstriction, and elevation of the arm causes an important degree of vasodilation. Vasoconstriction and vasodilation are maintained while the limb is kept above or below the heart. This is an economical and potentially very useful way of studying the innervation of the microcirculation in a number of different peripheral neuropathies of thin and mixed fibres.

TITLE: Las dos caras del reflejo venoarteriolar: vasoconstriccion y vasodilatacion cutanea al bajar y subir el brazo.Introduccion. El reflejo venoarteriolar (RVA) lo provoca un incremento en la presion venosa transmural al colocar una parte del cuerpo en el sentido de la aceleracion gravitatoria por debajo del corazon. Objetivo. Evaluar el RVA en sujetos sanos al levantar una parte del cuerpo por encima del corazon. Sujetos y metodos. En 16 sujetos sanos (20-65 años) se estudio el RVA mediante cambios en el flujo sanguineo de la piel con un fotopletismografo digital infrarrojo colocado en el pulpejo en sujetos sanos durante las siguientes condiciones: brazo derecho a la altura del corazon, brazo derecho 40 cm por debajo del corazon y brazo derecho 40 cm por encima del corazon. Las variables medidas fueron: amplitud del flujo sanguineo de la piel con el brazo a la altura del corazon (amplitud basal), porcentaje de disminucion del flujo sanguineo de la piel con el brazo por debajo del corazon y porcentaje de aumento del flujo sanguineo de la piel con el brazo por encima del corazon. Resultados. El porcentaje de vasoconstriccion con el brazo derecho por debajo del corazon fue del 35%, y el de vasodilatacion, del 50%. Conclusiones. La evaluacion del RVA con el brazo por debajo del corazon provoca vasoconstriccion, y la elevacion del brazo produce una importante vasodilatacion. La vasoconstriccion y la vasodilatacion se mantienen mientras la extremidad se mantenga por encima o por debajo del corazon. Este es un estudio potencialmente muy util y economico para estudiar la inervacion de la microcirculacion en diversas neuropatias perifericas de fibras delgadas y mixtas.

[Electroencephalographic normal variants or with uncertain significance]. / Variantes normales o de significado incierto en el electroencefalograma.

Electroencephalography is an important tool in the diagnosis of primary or secondary disorders of central nervous system, epilepsy is one of the most important. Sometimes normal electroencephalographic activity simulates epileptiform activity. This activity does not have pathological value and is considered a variant of normal brain activity. The main groups based on the classification proposed by Blum and Cervone are: rhythmic patterns; epileptiform patterns; lambda waves, and age dependent changes. These changes are frequently seen during drowsiness, sleep and during activation maneuvers and more common in children and adolescents. The aim of this review is to present the most important characteristics of each of these variants, in order to prevent them being confused with abnormal brain activity.

[Reversible dementias and treatable dementias]. / Demencias reversibles y demencias tratables.

INTRODUCTION: When studying patients with dementia the search of reversible or treatable entities has a primordial role. Many patients do not recover the previous cognitive level; however, that does not signify that the disease is not treatable with partial recovery or prevention of secondary progression. DEVELOPMENT: The prevalence of reversible dementias is around 15% in most series. Rutine studies include complete blood cell count, erythrocyte sedimentation rate, electrolytes and chemistry panel, liver function tests, vitamin B12 and folate serum levels, urianalysis and syphilis serology. Special tests such as thyroid function tests, protein electrophoresis, human immunodeficiency virus serology, heavy metals serum levels, electrocardiography, cerebrospinal fluid analysis, and chest roentnography should be performed according to the patient clinical profile. Neuroimaging should be done in all cases. Non-contrasted computed tomography must be performed first. Magnetic resonance imaging is most useful for searching for more subtle structure abnormalities. Electroencephalogram, positron emission tomography and single photon emission computed tomography are not recommended as routine studies. Brain biopsy is indicated in few cases. CONCLUSION: Most common causes of reversible and treatable dementias include: medications, obstructive hydrocephalus, infectious, vascular, immunological, psychiatric, toxic and metabolic disorders, among others.

Demencias reversibles y demencias tratables / Reversible dementias and treatable dementias

Introducción. La identificación de identidades reversibleso tratables en el síndrome demencial es importante. Por desgracia,las demencias no son reversibles en todos los casos, pues enmuchas ocasiones el paciente no recupera el nivel intelectual previo;sin embargo, ello no significa que la enfermedad no sea tratabley que no se pueda obtener una mejoría parcial o prevenir y detenerla progresión secundaria del déficit cognitivo. Desarrollo. Laprevalencia de las demencias reversibles es en promedio del 15%.Los estudios rutinarios se realizan para la búsqueda de entidadestratables e incluyen la realización de biometría hemática, velocidadde sedimentación globular, química sanguínea, pruebas de funciónhepática, determinación sérica de los niveles de vitamina B12,folatos y electrolitos séricos, examen general de orina y serologíapara sífilis. Pruebas especiales como perfil hormonal (en particular,pruebas de función tiroidea), electroforesis de proteínas e inmunoglobulinasséricas, serología para virus de inmunodeficienciahumana, búsqueda de metales pesados, electrocardiograma, examendel líquido cefalorraquídeo y rayos X de tórax se deben realizarde acuerdo al contexto clínico del enfermo. Se recomienda realizarde manera rutinaria estudios de neuroimagen; la tomografíaaxial computarizada sin contraste sería el estudio inicial. La resonanciamagnética es más útil para la búsqueda de alteraciones estructuralesmás sutiles. El electroencefalograma, la tomografía poremisión de positrones y la tomografía computarizada por emisiónde fotón único no se recomiendan como estudios de rutina. La biopsiacerebral está indicada sólo en algunos casos. Conclusión. Lascausas más frecuentes de demencias reversibles y tratables incluyenalteraciones toxicometabólicas, infecciones, uso de fármacos,la hidrocefalia obstructiva, enfermedades psiquiátricas, autoinmunesy vasculares, entre otras

Introduction. When studying patients with dementia the search of reversible or treatable entities has a primordialrole. Many patients do not recover the previous cognitive level; however, that does not signify that the disease is not treatablewith partial recovery or prevention of secondary progression. Development. The prevalence of reversible dementias is around15% in most series. Rutine studies include complete blood cell count, erythrocyte sedimentation rate, electrolytes andchemistry panel, liver function tests, vitamin B12 and folate serum levels, urianalysis and syphilis serology. Special tests suchas thyroid function tests, protein electrophoresis, human immunodeficiency virus serology, heavy metals serum levels,electrocardiography, cerebrospinal fluid analysis, and chest roentnography should be performed according to the patientclinical profile. Neuroimaging should be done in all cases. Non-contrasted computed tomography must be performed first.Magnetic resonance imaging is most useful for searching for more subtle structure abnormalities. Electroencephalogram,positron emission tomography and single photon emission computed tomography are not recommended as routine studies.Brain biopsy is indicated in few cases. Conclusion. Most common causes of reversible and treatable dementias include:medications, obstructive hydrocephalus, infectious, vascular, immunological, psychiatric, toxic and metabolic disorders,among others

[Painful neuropathies: their pathophysiology and treatment]. / Neuropatías dolorosas: fisiopatología y tratamiento.

OBJECTIVES: The purpose of this study is to review the different studies published in the literature concerning the different physiological mechanisms involved in the genesis of painful neuropathy, as well as the diagnostic options and the different pharmacological treatments currently available. DEVELOPMENT: Distinct pathologies usually condition painful neuropathy, one of the main ones being diabetes mellitus. The triggering phenomenon is often some kind of damage to the tissues that contain nervous pain receptors, which later gives rise to a release of proinflammatory molecules, and triggers a cascade of phenomena that result in disorders in the central and peripheral nervous system (peripheral and central sensitisation). These disorders usually produce clinical manifestations, such as allodynia, paresthesias, among others, and these are sometimes the sole manifestation of painful neuropathy. Diagnosis of this syndrome is at times complicated due to the involvement of thin fibres, which cannot be identified by the conventional methods used in neurophysiological studies. There is also a broad range of pharmaceuticals used in the treatment of painful neuropathy that range from tricyclic antidepressants, non-steroidal anti-inflammatory drugs, opioid analgesics, antiarrhythmics and even agents for topical use. CONCLUSIONS: Diagnosis of thin fibre neuropathy is usually performed by carrying out a Quantitative Sudomotor Axon Reflex Test, quantitative sensory tests and a skin biopsy. As regards the pharmacological treatment, the new generation of anticonvulsive drugs like gabapentin seems to have advantages over the traditional pharmaceuticals, although their widespread use is still largely restricted by their cost.

Neuropatías dolorosas: fisiopatología y tratamiento / Painful neuropathies: their pathophysiology and treatment

Objetivo. El presente trabajo pretende hacer una revisión de los diversos mecanismos fisiopatológicos involucrados en la génesis de la neuropatía dolorosa, las opciones diagnósticas, así como los diversos tratamientos farmacológicos disponibles en la actualidad, comunicados en diversos estudios. Desarrollo. Diversas patologías suelen condicionar neuropatía dolorosa, y una de las principales es la diabetes mellitus. El fenómeno desencadenante suele ser un daño a tejidos que albergan receptores nerviosos del dolor, que posteriormente producen una liberación de moléculas proinflamatorias y desencadenan una cascada de fenómenos que culminarán en alteraciones del sistema nervioso central y periférico (sensibilización periférica y central). Estas alteraciones suelen dar manifestaciones clínicas, como son alodinia y parestesias, entre otras, y que en ocasiones son la única manifestación de la neuropatía dolorosa. El diagnóstico de ésta, en ocasiones, es complicado, debido a la afección de fibras delgadas, que no se pueden identificar por los métodos convencionales de estudios neurofisiológicos. También existe una amplia variedad de fármacos utilizados para el tratamiento de la neuropatía dolorosa, que incluyen antidepresivos tricíclicos, antinflamatorios no esteroideos, analgésicos opioides, antiarrítmicos e incluso agentes de uso tópico. Conclusiones. El diagnóstico de la neuropatía de fibras delgadas se suele realizar por medio de una prueba cuantitativa axonal sudomotora, pruebas sensitivas cuantitativas y una biopsia de piel. En cuanto al tratamiento farmacológico, los anticonvulsionantes de nueva generación, como la gabapentina, parecen tener ventajas sobre los fármacos tradicionales, aunque su coste todavía suponga una limitación para su uso (AU)

Aims. The purpose of this study is to review the different studies published in the literature concerning the different physiological mechanisms involved in the genesis of painful neuropathy, as well as the diagnostic options and the pharmacological treatments currently available. Development. Distinct pathologies usually condition painful neuropathy, one of the main ones being diabetes mellitus. The triggering phenomenon is often some kind of damage to the tissues that contain nervous pain receptors, which later gives rise to a release of proinflammatory molecules, and triggers a cascade of phenomena that result in disorders in the central and peripheral nervous system (peripheral and central sensitisation). These disorders usually produce clinical manifestations, such as allodynia, paresthesias, among others, and these are sometimes the sole manifestation of painful neuropathy. Diagnosis of this syndrome is at times complicated due to the involvement of thin fibres, which cannot be identified by the conventional methods used in neurophysiological studies. There is also a broad range of pharmaceuticals used in the treatment of painful neuropathy that range from tricyclic antidepressants, non-steroidal anti-inflammatory drugs, opioid analgesics, antiarrhythmics and even agents for topical use. Conclusions. Diagnosis of thin fibre neuropathy is usually performed by carrying out a quantitative sudomotor axon reflex test, quantitative sensory tests and a skin biopsy. As regards the pharmacological treatment, the new generation of anticonvulsive drugs like gabapentin seems to have advantages over the traditional pharmaceuticals, although their widespread use is still largely restricted by their cost (AU)

[Monitoring dermatomal somatosensory evoked potentials at the ERB point, the cervical spinal cord and the cerebral cortex in the diagnosis of cervical radiculopathy]. / Registro de potenciales evocados somatosensoriales dermatómicos en punto de Erb, médula cervical y corteza cerebral en el diagnóstico de la radiculopatía cervical.

INTRODUCTION: Recording at various levels of the somatosensory pathway is often used in somatosensory evoked potentials to mixed nerve stimulation (SEP), but not in dermatomal somatosensory evoked potentials (DSEP) in which only the cortical potential is usually recorded. The aim of our study was to compare the recordings of upper limb DSEP at Erb point, cervical cord, and subcortical and cortical levels with SEP recordings in healthy subjects and patients with cervical radiculopathy. PATIENTS AND METHODS: 17 patients with clinical history, MRI and electromyography consistent with cervical radiculopathy and 17 healthy subjects were included. Median and ulnar nerves were stimulated at the wrist; and C6, C7 and C8 dermatomes at the 1st, 3rd and 5th fingers respectively. All the potentials obtained with SEP and DSEP were compared between controls and patients by t test for independent samples. We also used Pearson s correlation for height/latencies, weight/amplitude and age/peripheral nerve conduction velocity (PNCV). RESULTS: DSEP potentials were of similar morphology of those observed in SEP but had longer latencies and smaller amplitudes. We found a positive correlation between height and latencies, and a negative association of weight with amplitude of peripheral potential, and age/PNCV. No difference between controls and the neurological intact segments of patients was found. 13 patient had DSEP altered while only 5 of them had altered SEP recorded. The most common finding was prolongation of the conduction time of the segment N9 N13 on DSEP recordings. CONCLUSION: We found that it is possible to record and to identify all the potentials in DSEP as observed in the SEP. On cervical radiculopathy, DSEP with the present technique increase the sensitivity and give some additional and useful information regarding the extension and localization of the pathology. Besides, DSEP recording is a non invasive technique, non traumatic and well tolerated for our patients.

Registro de potenciales evocados somatosensoriales dermatómicos en punto de Erb, médula cervical y corteza cerebral en el diagnóstico de la radiculopatía cervical / Monitoring dermatomal somatosensory evoked potentials at the Erb point, the cervical spinal cord and the cerebral cortex in the diagnosis of cervical radiculopathy

Introducción. El registro de potenciales evocados somatosensoriales en puntos intermedios de la vía somatosensorial, como el punto de Erb y la médula cervical, se utiliza sólo con estimulación de nervio mixto (PESS), y no en potenciales evocados somatosensoriales con estímulo dermatómico (PESSD). El objetivo del estudio fue comparar el registro del PESSD de miembros superiores en punto de Erb, médula cervical y regiones subcorticales y corticales, con el registro del PESS convencional en sujetos sanos y pacientes con radiculopatía cervical (RC). Pacientes y métodos. Participaron 17 sujetos sanos y 17 pacientes con historia clínica consistente con RC, confirmada por electromiografía y RM. Se estimularon los nervios mediano y cubital en la muñeca, y los dermatomas C6, C7 y C8 en el 1.º, 3.º y 5.º dedos. Se compararon los componentes de los PESS con los PESSD, en controles y pacientes, por medio de la prueba t de Student para muestras independientes y se realizaron correlaciones de Pearson entre talla/latencias, peso/amplitud y edad/velocidad de conducción periférica (VCNP) para determinar los factores que correlacionaban en el estudio. Resultados. Los PESSD presentaron similar morfología, pero latencias más largas y menor amplitud, que los PESS. Se encontró una correlación directa entre talla y latencias e inversa entre peso y amplitud del potencial periférico así como entre edad y la VCNP. No hubo diferencias entre el grupo control y el lado no afectado del grupo de pacientes. En 13 pacientes se detectó alteración en los PESSD, mientras sólo se detectó en cinco en los PESS. La alteración más común fue la prolongación en la conducción del segmento N9-N13. Conclusiones. Con el presente estudio se confirma que es posible registrar e identificar adecuadamente todos los componentes de los PESSD a lo largo de la vía somato sensorial, para utilizarlos en la clínica al igual que en los PESS convencionales. En la RC, los PESSD con captaciones intermedias de la vía aumentan la sensibilidad diagnóstica y brindan información adicional referente a la extensión y localización de la lesión; ofrecen, además, la ventaja de ser un estudio no invasivo, no traumático y mejor tolerado por los pacientes (AU)

[Peripheral neuropathy during a second cycle of treatment with high doses of interferon-alpha in a patient with hepatitis C, and a review of the literature]. / Neuropatía periférica durante un segundo ciclo de tratamiento con dosis altas de interferón alfa en un paciente con hepatitis C y revisión de la bibliografía.

INTRODUCTION: Alpha interferons (IFN a) have been shown to be effective in patients with chronic active hepatitis C. IFN a treatment may be associated with neurologic complications, including peripheral neuropathy. CASE REPORT: We describe a patient with active hepatitis C treated with IFN a, who developed peripheral neuropathy after a second cicle of treatment with interferon. He received a first cicle of treatment with IFN a during 22 weeks (6 MU/day 3 times a week). Afther that he did not received treatment during one year and then he received the second cycle (6 MU/day 3 times a week). After 3 months of therapy the patient complained about paresthesias of both legs. CONCLUSIONS: IFN a related neuropathy is probably rare; however, some cases may be misdiagnosed. Diagnosis of IFN a related neuropathy should be considered by physicians, particularly in patients given longterm treatment and high IFN a dosage. In this case we think that the patient had an acumulative efectt of interferon though he did not received treatment with interferon during one year.

Neuropatía periférica durante un segundo ciclo de tratamientocon dosis altas de interferón alfa en un paciente con hepatitis Cy revisión de la bibliografía / Peripheral neuropathy during a second cycle of treatment with high doses of interferon-alpha in a patient with hepatitis C, and a review of the literature

Introducción. La utilización de interferón alfa (IFN) ha mostrado su efectividad en el tratamiento de la infección por el virus de la hepatitis C. Se han descrito efectos secundarios del uso de interferón en el sistema nervioso, entre ellos la neuropatía. Caso clínico. En este artículo describimos un caso de neuropatía periférica asociada al uso de IFN-alfa en un paciente con hepatitis C, quien recibió un primer ciclo de tratamiento durante 22 semanas en dosis de 6 millones UI tres veces a la semana. Tras permanecer un año sin recibir ninguna terapia, recibió un segundo ciclo de IFN-alfa en dosis de 6 millones UI tres veces por semana. Después de tres meses de tratamiento el paciente desarrolló datos de neuropatía periférica. Los estudios de electrofisiología mostraron una polineuropatía axonal. Conclusiones. La neuropatía asociada al uso de IFN-alfa es una manifestación rara; sin embargo, hay que considerar que puede estar subdiagnosticada, ya que en la mayoría de los pacientes no se sospecha esta patología. El diagnóstico debe considerarse en pacientes que reciben tratamiento a largo plazo y en los que reciben dosis altas. Pensamos que en nuestro caso pudo haber un efecto acumulativo de la dosis de IFN-alfa , aunque el paciente había estado un año sin recibir IFN-alfa (AU)

Pruebas diagnósticas en miastenia gravis. Conformación de un estándar diagnóstico mediante un método de consenso / Diagnostic test in myasthenia gravis. Creation of a standard for diagnosis using a method of consensus

Introducción. En la actualidad la mejor prueba para el diagnóstico de miastenia gravis (MG) es la electromiografía de fibra única (EFU). Debido al coste de la prueba, pocos hospitales en el mundo la pueden tener para uso rutinario para confirmar el diagnóstico de MG. Objetivo. Identificar un estándar de oro aceptable para hospitales que no cuenten de manera habitual con EFU mediante la utilización de una metodología de consenso realizado en tres etapas. Sujetos y métodos. Las dos primeras etapas del estudio se realizaron mediante encuestas prolectivas y la tercera etapa mediante una metodología Delfos. En la primera etapa se aplicó un instrumento a 55 neurólogos generales donde se preguntaba cuál era la mejor combinación de pruebas y la mejor prueba aislada con las que habitualmente realizaban el diagnóstico de MG. En la segunda etapa se aplicó un segundo instrumento de recolección a 15 expertos de unidad motora (EUM), quienes analizaron cuatro situaciones clínicas frecuentes en la práctica (cuadro clínico clásico de MG generalizada, paciente con cuadro de miastenia ocular, cuadro de MG generalizada de mediana probabilidad y cuadro clínico de baja probabilidad), identificando la mejor prueba aislada y el mejor conjunto de pruebas para el diagnóstico de miastenia gravis con y sin el uso de EFU. Finalmente, se crearon cuatro flujogramas de las cuatro situaciones clínicas analizadas que fueron consensuados mediante una metodología Delfos con ocho neurólogos EUM. Resultados. Hemos identificado un estándar de oro aceptable para hospitales que no cuenten de manera general con EFU mediante una metodología de consenso. También hemos consensuado cuatro flujogramas diagnósticos de las situaciones clínicas más frecuentes a las que nos enfrentamos en la práctica clínica habitual. Conclusión. Hemos encontrado una serie de resultados con una utilidad importante para el diagnóstico de MG aplicables a médicos generales, médicos internistas y neurólogos que no cuenten con todas las pruebas disponibles para el diagnóstico de esta enfermedad (AU)

[Diagnostic tests in myasthenia gravis. Creation of a standard for diagnosis using a method of consensus]. / Pruebas diagnósticas en miastenia gravis. Conformación de un estándar diagnóstico mediante un método de consenso.

INTRODUCTION: Nowadays the best test for the diagnosis of myasthenia gravis (MG) is the single fiber electromyography (SFEMG). Due to the cost of the test it has not become routine in most part of the hospitals to confirm the diagnosis of MG. OBJECTIVE: To identify an acceptable gold standard for hospitals which do not have access to SFEMG, but have access to some other classical test through the use of a consensus methodology. SUBJECTS AND METHODS: The study was realized in three stages. The first two stages were done through a prolective survey and the third stage through a Delfos methodology. During the first stage 59 general neurologist were interviewed, applying an instrument in which they were asked which were the best test and the best group of tests to diagnose MG without using the SFEMG. During a second stage, a second collection instrument was applied to 15 experts in motoneuron diseases to identify the best isolated test and the best test scheme with and without using the SFEMG in four frequent clinical situations in clinical practice (typical clinical case of generalized MG, patient with ocular myasthenia, patient with generalized MG of medium and low probability). Finally four flowcharts were created of the four analyzed clinical situations and were approved through a Delfos methodology with 8 neurologist motoneuron disease. RESULTS: We identified an acceptable gold standard for hospitals which do not have access to SFEMG through the use of a consensus methodology. We have also completed four diagnostic flowcharts about the most frequent clinical situations that we have faced in the ordinary practice. CONCLUSIONS: We have found a series of important results for the diagnosis of MG available to general physicians, internists and neurologist that do not have all the test for the diagnosis of MG.

[Significance of intracranial hypertension management in cryptococcal meningitis in patients with acquired immunodeficiency syndrome. Report of 2 cases]. / Importancia del manejo de la hipertensión endocraneana en meningitis criptocócica en pacientes con síndrome de inmunodeficiencia adquirida. Informe de dos casos.

Two cases of cryptococcal meningitis and increased intracranial pressure in patients with acquired immunodeficiency are described. Both patients presented high intracranial pressure that persisted despite optimal antifungal treatment (amphotericin B, 5-flucytosine initially, and fluconazole posteriorly). The elevated intracranial pressure produced headache, seizures, and reduced visual and auditory acuity. CAT scan demonstrated absence of ventricular dilatation or focal lesions. Both cases were treated with adequate antifungal therapy, as well as with repeated lumbar punctures and placement of a lumboperitoneal shunt due to the persistence of elevated intracranial pressure. One patient presented with unilateral loss of vision due to optic nerve atrophy. After one year of follow-up, one patient died due to progression of his disease, while the other is still alive and without evidence of neurological disease. Intracranial hypertension is a frequent clinical manifestation of cryptococcal meningitis in patients with acquired immunodeficiency syndrome (AIDS) that requires adequate diagnosis and management. Treatment should be directed towards the reduction of intracranial pressure though repeated lumbar punctures and, in some cases, with lumboperitoneal or ventricular-peritoneal shunts.

[A case of Hallervorden-Spatz disease with magnetic resonance imaging data]. / Un caso de enfermedad de Hallervorden-Spatz con hallazgos de resonancia magnética.

Hallervorden-Spatz disease (HSD) is an uncommon disorder, progressive and degenerative of the basal ganglia. It begins in the first or second decade of life and is characterized by a dominant extrapiramidal signs, dystonia and progressive dementia. It is autosomic recessive, although sporadic cases are seen in 15%. There is no biological marker for the disease. The post-mortem findings include iron deposits in the globus pallidum and pars reticulata of the substantia nigra. Magnetic resonance imaging (MRI) in T2 shows symmetric hypointense lesions in both globus pallidum with a hyperintense center: giving the "tiger's eye" sign. This is the first case reported in Mexico of sporadic HSD with typical clinical and MRI findings.

[100 years of the Babinski sign]. / Los cien años del signo de Babinski.

In 1896 Joseph Francois Felix Babinski described for the first time the phenomenon of the toes. In his first paper he simply described extension of all toes with noxious stimulation of the sole of the foot. It was not until 1898 that he specifically described the extension of the hallux with stimulation of the lateral border of the sole. Babinski was probably not aware at the time that E. Remak, a German physician, had previously described the sign. In his third paper of 1903 Babinski concludes that if other authors had described the abnormal reflex before him, they found it fortuitously and did not realize its semiologic value. Babinski probably discovered it by a combination of chance, careful observation and intuition. He also had in mind practical applications of the sign particularly in the differential diagnosis with hysteria and in medico-legal areas. Several of his observations and the physiopathological mechanism proposed by him are still valid today. He realized since 1896 that the Babinski reflex was part of the flexor reflex synergy. He observed that several patients during the first hours of an acute cerebral or spinal insult had absent extensor reflexes. He realized that most patients with the abnormal reflex had weakness of the toes and ankles. He found a lack of correlation between hyperactive myotatic reflexes and the presence of an upgoing hallux. He discovered that not all patients with hemiplegia or paraplegia had the sign. He thought erroneously that some normal subjects could have an upgoing toe. His dream of a practical application of the sign has been fully achieved. The motto of Babinski was Observatio summa lex. Perhaps there is no better dictum in clinical neurology.

[Evidence of genetic and environmental factors in manual laterality. Study of 120 Mexican families]. / Evidencia de componentes genéticos y ambientales en la lateralización manual. Estudio en 120 familias mexicanas.

Environmental factors as well as different modes of inheritance has been suggested to explain the etiology of left-handedness. In order to improve knowledge of this problem, manual skill (MS) of parents (n = 234), siblings (n = 506) and children (n = 126) of 60 right-handed (RHI) and 60 left-handed (LHI) index cases (IC), born in Mexico City, were studied. Parents and siblings of both IC had similar frequencies of left-handedness. Quite the contrary, 36.7% of children of LHI were left-handed, while 7.3% children of RHI happen to be left-handed (P < 0.00025). No differences were found in the appearance of perinatal environmental factors. These findings are explained in part according to the pressure exerted by parents and/or teachers for dextrality. The impact of this influence modifies the effect of several unknown genes (multifactorial inheritance). The understanding of the above mechanisms in the etiology of MS is relevant not only for academic purposes, but for the educational sphere as well.

Changes in cardiac repolarization and ventricular conduction in a case of acute hematomyelia. Report of a case.

A twenty year old man presented an acute hematomyelia at T2-T3 level and had electrocardiographic changes suggesting subendocardial and subepicardial ischemia; he also had precordial pain and elevation of the MB fraction of creatine phosphokinase. Neurons providing heart inervation are located at the T2-T3 spinal level. The electrocardiographic changes observed were considered neurogenic in origin and were transient. Although there are experimental reports showing electrocardiographic changes associated with compression of the upper part of thoracic spinal cord, this is the first report to our knowledge, in which an acute spinal injury is shown to be associated with neurogenic changes in ventricular repolarization simulating acute myocardial ischemia.

[Plasma cells in the cerebrospinal fluid of patients with cerebral cysticercosis]. / Células plasmáticas en el líquido cefalorraquídeo en pacientes con cisticercosis cerebral.

We studied in twenty patients with proven cerebral cysticercosis the presence of plasma cells in the cerebrospinal fluid (CSF). We found plasma cells in 50 per cent of the patients. All of them had subarachnoid cysticercosis or basal arachnoiditis. The presence of plasma cells was significantly associated to higher protein levels, greater number of inflammatory cells, and higher levels of IgG in the CSF. None of the patients had received medical treatment or steroids prior to the test. These findings suggest a role of plasma cells in the pathogenesis of the cysticercotic disease. The presence of plasma cells in the CSF is part of the immune response against the parasite. We also found that plasma cells are more frequently encountered in the CSF (50 per cent) than eosinophils (20 per cent) in patients with cerebral cysticercosis.