Monuments to Cajal in Madrid, Spain: Rejection of public tributes.

During Santiago Ramón y Cajal's lifetime, two monuments to him were erected in Madrid. However, Cajal excused himself from attending their inaugurations for reasons that have so far remained unexplained. The present report has therefore investigated the political context and personal circumstances that might account for his behavior. The first monument is a fountain in El Retiro Park, the work of sculptor Victorio Macho, inaugurated in 1926 during a major confrontation between progressive intellectuals and physicians against the dictatorship of Miguel Primo de Rivera. An official press release warned of a prison sentence for those who attempted a second (illegal) inauguration. The second monument appeared in 1931, barely a month after the establishment of the Second Spanish Republic. This full-body statue, standing>3m (around 10ft) high on a narrow pedestal, was financed by medical students and sculpted by Lorenzo Domínguez, a Chilean medical student. Its unlikely height and thinness earned it the nickname 'The Pencil'. At present, it flanks the entrance to Cajal's old classroom at the Madrid College of Physicians. Closer inspection reveals fractures as evidence of its having been broken into pieces at some point, presumably during bombings that took place in 1936. The calcareous Novelda stone used in its construction and its exposure to the elements may also partly explain its deterioration. A few metres away, a second sculpture, apparently a replica of the original, was inaugurated in 1998. Cajal's excuses for not attending the inaugurations of both his monuments may have different explanations. Regarding the fountain, it was probably a gesture of solidarity against those (many of whom were doctors) opposing the dictatorship whereas, when The Pencil was inaugurated, Cajal was 79 years old, and his physical limitations might have accounted for his inability to attend the ceremony. Thus, given the different political context in which each inauguration took place, Cajal's attitude was presumably in line with his politics, but also the result of his age-related infirmities.

Cajal's unbearable cephalalgias: The consequences of a misdiagnosis.

Around the age of 66 Cajal consulted neurologist Nicolas Achúcarro complaining of "unbearable cephalalgias". He had not ever suffered from headaches. The diagnosis of early arteriosclerosis that was considered at the time a physiological, irreversible ailment of ageing had a strong emotional impact on Cajal. Comorbid depression, insomnia and self-treatment with escalating doses of Veronal® (barbital), a short-acting barbiturate, presumably aggravated the situation. Exposure to warm environments and being involved in tense discussions were identified as triggering factors of the headaches. Achúcarro and Cajal were probably assuming scientific concepts at the time, such as cerebral congestion, increased temperature at the cerebral cortex during mental activity and vasoconstriction and vasodilatation phenomena. Up to his death aged 82, no evidence was discovered of any organic nervous system disorder. Cajal remained anxious up to the end of his life fearing an impending cerebral haemorrhage. The diagnosis was followed by profound lifestyle changes such as social isolation, which forced him to leave his laboratory. Cajal's cephalalgias marked in some way the end of the Spanish school of neurohistology.

Azañón's disease. A 19th century epidemic of neurolathyrism in Spain.

The cultivation and consumption of grasspea (Lathyrus sativus) in Spain probably dates back centuries, especially during times of famine when the neurotoxic potential of this legume was expressed in the form of a spastic paraparesis known as neurolathyrism. Little known outside the country, the epidemic of neurolathyrism in the years following the Spanish Civil War (1936-1939) came to affect more than a thousand people. In late 1872, during the Six Years Revolutionary Term, young Alejandro San Martín Satrústegui (1847-1908), then editor of the popular weekly El Siglo Médico, travelled to Azañón, a remote village in the province of Guadalajara, to clarify a so-far unknown disease. We analysed the original article published in 1873 by San Martin, as well as communications sent by El Siglo Médico readers reporting similar cases in many other Castilian provinces. San Martín's neurological findings in seven personally examined cases were astonishingly accurate; he concluded the subjects' neurological deficits resulted from injury to the lateral columns in the lower portion of the spinal cord. Description of the clinical findings provided both by San Martín, and by the readers of El Siglo Médico, leave no doubt as to the diagnosis of neurolathyrism. However, none suspected the patient's staple food was the determinant cause of the disease. San Martín proposed the eponym Azañón's disease for lack of a better name the same year (1873) in which Cantani in Italy introduced the term lathyrism. The epidemic of neurolathyrism that affected many Castilian towns represents one of the best-documented in Europe during the last third of the 19th century.

The Madrid School of Neurology (1885-1939).

The emergence of neurology in Madrid between 1885 and 1939 had well-defined characteristics. On foundations laid by Cajal and Río-Hortega, pioneers combined clinical practice with cutting-edge neurohistology and neuropathology research. Luis Simarro, trained in Paris, taught many talented students including Gayarre, Achúcarro and Lafora. The untimely death of Nicolás Achúcarro curtailed his promising career, but he still completed the clinicopathological study of the first American case of Alzheimer's disease. On returning to Spain, he studied glial cells, including rod cells. Rodríguez Lafora described progressive myoclonus epilepsy and completed experimental studies of corpus callosum lesions and clinical and neuropathology studies of senile dementia. He fled to Mexico at the end of the Spanish Civil War (1936-1939). Sanchís Banús, a sterling clinical neurologist, described the first cluster of Huntington's disease in Spain, and he and Río-Hortega joined efforts to determine that pallidal degeneration underlies rigidity in advanced stages of the disease. Just after the war, Alberca Llorente eruditely described inflammatory diseases of the neuraxis. Manuel Peraita studied "the neurology of hunger" with data collected during the siege of Madrid. Dionisio Nieto, like many exiled intellectuals, settled in Mexico DF, where he taught neurohistological methods and neuropsychiatry in the tradition of the Madrid School of Neurology.

La Salpetrière antes de Charcot: una visita de Pedro González Velasco / La Salpetrière Hospital before Charcot: A visit described by Pedro Gonzalez Velasco

Introducci¨®n: Bajo su direcci¨®n, Charcot fue capaz de convertir La Salpetri¨¨re en centro puntero de la neurolog¨ªa mundial. Sin embargo, es escasa la informaci¨®n sobre las condiciones asistenciales que pudo encontrar a su llegada en 1862. Desarrollo: El art¨ªculo publicado en 1860 por el español Pedro Gonz¨¢lez Velasco tras visitar al famoso hospicio representa un valioso testimonio sobre los cuidados que recib¨ªan los pacientes poco antes de la llegada de Charcot. Aunque esencialmente encomi¨¢stico, el art¨ªculo describe tambi¨¦n la situaci¨®n, bastante menos satisfactoria, de pacientes con trastornos mentales graves, epilepsia y par¨¢lisis confinados en jaulas, con un simple lecho de paja sobre el suelo como ¨²nica cama y un desag¨¹e para recoger sus deyecciones. Velasco no era alienista, sino un cirujano de prestigio y defensor apasionado del positivismo. Como amigo personal, compañero de hospital y correligionario pol¨ªtico de Jos¨¦ Mar¨ªa Esquerdo, conoc¨ªa de primera mano la lucha de este por mejorar la asistencia neuropsiqui¨¢trica en Madrid. Conclusiones: El art¨ªculo de Velasco, en ¨²ltima instancia, sirve de pretexto para denunciar las deplorables condiciones asistenciales de esta clase de pacientes en el Hospital General de Madrid. Por otro lado, Charcot tendr¨¢ que afrontar no solo el desarrollo de la neurolog¨ªa, sino mejorar tambi¨¦n las condiciones de vida de los enfermos en La Salpetri¨¨re(AU)

Introduction: Under Charcot's leadership, La Salpetri¨¨re was transformed into one of the world's top neurology centres. However, there is little information regarding the patient care facilities which Charcot would have encountered upon his arrival in 1862. Development: A paper published in 1860 by Spanish physician Pedro Gonz¨¢lez Velasco following a visit to that famous hospital is a valuable testimony to the quality of patient care just prior to Charcot¡äs arrival. Although it essentially praises the institution, the article also describes the largely unsatisfactory conditions endured by patients with severe mental disorders, epilepsy and paralysis, who were locked inside cages with simple straw pallets on the floor for beds and open holes for toilets. Rather than an alienist, Velasco was a well-known surgeon and passionate advocate of positivism. As a personal friend and hospital fellow of Jose Maria Esquerdo's, with similar political affiliations, he had first-hand knowledge of the struggle to improve neuropsychiatric care in Madrid. Conclusions: Publishing his paper ultimately provided Velasco with a pretext for denouncing the deplorable care conditions endured by similar patients in Hospital General de Madrid. Meanwhile, Charcot would go on to improve the living conditions of inpatients at La Salpetri¨¨re and found the specialty of neurology(AU)

La Salpêtrière Hospital before Charcot: a visit described by Pedro González Velasco.

INTRODUCTION: Under Charcot's leadership, La Salpêtrière was transformed into one of the world's top neurology centres. However, there is little information regarding the patient care facilities which Charcot would have encountered upon his arrival in 1862. DEVELOPMENT: A paper published in 1860 by Spanish physician Pedro González Velasco following a visit to that famous hospital is a valuable testimony to the quality of patient care just prior to Charcot's arrival. Although it essentially praises the institution, the article also describes the largely unsatisfactory conditions endured by patients with severe mental disorders, epilepsy and paralysis, who were locked inside cages with simple straw pallets on the floor for beds and open holes for toilets. Rather than an alienist, Velasco was a well-known surgeon and passionate advocate of positivism. As a personal friend and hospital fellow of Jose Maria Esquerdo's, with similar political affiliations, he had first-hand knowledge of the struggle to improve neuropsychiatric care in Madrid. CONCLUSIONS: Publishing his paper ultimately provided Velasco with a pretext for denouncing the deplorable care conditions endured by similar patients in Hospital General de Madrid. Meanwhile, Charcot would go on to improve the living conditions of inpatients at La Salpêtrière and found the specialty of neurology.

Quince años de historia de la enfermedad de Parkinson en España (1963-1977). Un recuerdo personal / Fifteen years History of Parkinson´s disease in Spain (1963-1977). A personal recollection

La enfermedad de Parkinson (EP) y los trastornos del movimiento, actualmente una de las principales subespecialidades entre los neurólogos españoles, era un tema de interés limitado en la década de los años sesenta. Queda bien reflejado en Revista de Neurología, la primera revista española dedicada exclusivamente a la neurología. En un período de cinco años (1973-1977), apenas un 3% de los artículos publicados se relacionaba con este tema. La función de los ganglios basales era poco conocida. La EP se entendía como un trastorno del tomo muscular en el que la rigidez explicaba la mayoría o todos los signos parkinsonianos. Se consideraba ‘parkinsonismo arteriosclerótico’ cuando el paciente tenía más de 60 años; la mayoría eran tratados por médicos generales, mientras que los pacientes más jóvenes eran canalizados hacia la cirugía estereotáxica. La experiencia inicial con levodopa en España tuvo lugar en torno a 1970, por lo general en pacientes muy incapacitados tras largos años de enfermedad. Por ello, y seguramente por administrarse entonces dosis relativamente elevadas, era frecuente observar discinesias coreicas de considerable gravedad. También inesperados fueron los efectos conductuales relacionados con el nuevo tratamiento, como estados maníacos o hipersexualidad, iniciando así el interés por los aspectos no motores de la enfermedad, apenas definidos hasta entonces. El cambio de opinión entre los médicos al considerar la EP un trastorno común en la población al margen de la edad, la capacidad de identificar los signos precoces y, sobre todo, la posibilidad de disponer de un tratamiento eficaz constituyeron importantes acicates para despertar el interés de los neurólogos españoles hacia los parkinsonismos (AU)

Parkinson’s disease (PD) and movement disorders, currently one of the leading subspecialties among Spanish neurologists, raised only modest interest during the seventies. This is well reflected in Revista de Neurología, the first journal entirely devoted to Neurology in Spain. Along five years (1973-1977) only 3% out of papers published were devoted to this subject. The functions of the basal ganglia were poorly known. PD was regarded as a disorder of muscular tone, rigidity playing a pivotal role to explain most, if not all, parkinsonian signs. ‘Arteriosclerotic parkinsonism’, whatever the symptoms might be, was applied to any form of parkinsonism occurring after the age of sixty. Such cases were usually treated by general practitioners, while younger patients were as a rule channeled for stereotactic surgery. Initial experience on levodopa therapyin Spain began around 1970, the drug being initially administered to severely disabled patients after long years of disease course. This, and probably because of the use of somewhat liberal doses of the new drug as well, severe chorei form dyskinesias were particularly common. Unexpectedly, neurologists were newly confronted to unusual, levodopa-related behavioral effects, such as manic behavior and overt hypersexuality, arising interest on non-motor aspects of PD, which had been mostly neglected at the time. Generalized awareness among physicians of PD as a common disorder in a wide age range in the general population, recognition of early signs and, most important, availability of an effective therapy, prompted widespread interest of Spanish neurologists on parkinsonism (AU)

[Fifteen years history of Parkinson's disease in Spain (1963-1977). A personal recollection]. / Quince años de historia de la enfermedad de Parkinson en España (1963-1977). Un recuerdo personal.

Parkinson's disease (PD) and movement disorders, currently one of the leading subspecialties among Spanish neurologists, raised only modest interest during the seventies. This is well reflected in Revista de Neurologia, the first journal entirely devoted to Neurology in Spain. Along five years (1973-1977) only 3% out of papers published were devoted to this subject. The functions of the basal ganglia were poorly known. PD was regarded as a disorder of muscular tone, rigidity playing a pivotal role to explain most, if not all, parkinsonian signs. 'Arteriosclerotic parkinsonism', whatever the symptoms might be, was applied to any form of parkinsonism occurring after the age of sixty. Such cases were usually treated by general practitioners, while younger patients were as a rule channeled for stereotactic surgery. Initial experience on levodopa therapy in Spain began around 1970, the drug being initially administered to severely disabled patients after long years of disease course. This, and probably because of the use of somewhat liberal doses of the new drug as well, severe choreiform dyskinesias were particularly common. Unexpectedly, neurologists were newly confronted to unusual, levodopa-related behavioral effects, such as manic behavior and overt hypersexuality, arising interest on non-motor aspects of PD, which had been mostly neglected at the time. Generalized awareness among physicians of PD as a common disorder in a wide age range in the general population, recognition of early signs and, most important, availability of an effective therapy, prompted widespread interest of Spanish neurologists on parkinsonism.

Punción lumbar, más indicaciones / Further indications for lumbar puncture

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Nummular headache secondary to an intracranial mass lesion.

Nummular headache is a coin-shaped, chronic cephalalgia usually considered to stem from epicranial tissues. We describe a patient complaining of circumscribed pain in the head as the only symptom of a subtentorial meningioma. This observation underlines the need to revise the concept of circumscribed, referred pains in the head arising from pain-sensitive intracranial structures.

Reduced reciprocal inhibition is seen only in spastic limbs in patients with neurolathyrism.

If reduced reciprocal inhibition plays a causal role in the pathophysiology of spasticity as has been suggested in several studies, the inhibition is expected to be impaired in spastic, but not in normal muscles. Patients with neurolathyrism offer a possibility of testing this prediction since the spastic symptoms in these patients are restricted to the lower extremities only. Three patients with neurolathyrism were tested. Their data were compared with 15 age-matched healthy subjects. All patients showed signs of spasticity in the legs. Two patients had normal voluntary muscle force in the lower extremities and one had decreased force. No clinical abnormalities were found in the upper extremities. Reciprocal inhibition between ankle dorsiflexor and plantarflexor muscles was absent in all patients, whereas the inhibition between wrist extensor and flexor muscles was present and of normal size and latency. These findings are consistent with the hypothesis that reduced reciprocal inhibition plays a causal role in the pathophysiology of spasticity.

Introducción ambulatoria de apomorfina en infusión subcutánea continua en pacientes con enfermedad de Parkinson avanzada: experiencia y recomendaciones / Shifting to subcutaneous infusion of apomorphine in advanced Parkinson's disease patients on an out-patient basis: experience and recommendations

Introducción. El cambio de medicación antiparkinsoniana oral a infusión subcutánea continua (ISC) de apomorfina en la enfermedad de Parkinson (EP) puede ser complejo. No está claro si es necesaria la hospitalización. Objetivo. Comparar de manera prospectiva la eficacia y efectos adversos de apomorfina en ISC en pacientes parkinsonianos con fluctuaciones on-off incontrolables manejados ambulatoriamente. Material y métodos. Se inició ISC de apomorfina en siete pacientes con EP en lista de espera quirúrgica. Dos pacientes fueron ingresados, siendo el resto manejados ambulatoriamente en un medio hospitalario. Todos fueron evaluados a diario (media: 6,1 días), programándose después revisiones a los 3 y 6 meses. Se ofreció la posibilidad de consultas telefónicas, visitas no programadas y asistencia neurológica de urgencia las 24 h. Se intentó suprimir toda la medicación oral paralelamente al ajuste en la dosis de apomorfina. Se estimó el tiempo off en vigilia mediante un diario de fluctuaciones y la función motora mediante la subescala UPDRS y una escala de discinesias. Se valoraron el estado anímico, cambios neuropsiquiátricos y calidad de vida. Resultados. Todos los pacientes requirieron consultas telefónicas (media: 4,4), pero fueron escasas las visitas no programadas (media: 1,5). Globalmente el tiempo off se redujo un 68,1 %. Fue posible suprimir toda la medicación antiparkinsoniana excepto la levodopa. Conclusión. El inicio de apomorfina ISC en régimen ambulatorio es eficaz y seguro, a condición de realizarlo en un medio hospitalario con ajustes diarios de medicación durante la primera semana y asesoramiento telefónico posteriormente

Introduction. Shifting from oral medication to continuous subcutaneous infusion of apomorphine (CSIA) in Parkinson's disease (PD) may be complicated. It is unclear whether hospitalization is necessary Objective. To observe efficacy and side effects in advanced PD patients shifting to CSIA managed as out-patients with hospital-based support. Material and methods. CSIA was started on 7 PD patients with uncontrollable on-off fluctuations on a waiting list for surgery. Two patients were initially admitted to hospital, all others being managed as out-patients. Awake off time was assessed with daily charts and motor function with UPDRS subscale and a scale for dyskinesias severity. Appropriate scales were applied for mood changes, neuropsychiatric complications and quality of life measures. Apomorphine dosage and oral antiparkinsonian medication were adjusted daily (mean: 6.1 days) according to motor responses. Follow-up visits were scheduled at 3 and 6 months. Patients were offered phone calls, unprogrammed visits, and 24 hours neurological care if required. Results. All patients required phone calls (mean, 4.4), but unprogrammed visits were rare (1.5). Off-time was reduced in the overall group by a 68.1 %. Co-medication with levodopa was necessary in all patients, but all other antiparkinsonian medications could be discontinued. Conclusion. Shifting from oral dopaminergic therapy to CSIA is safe and effective in advanced PD managed on an out-patient basis in a hospital-based setting providing that daily in-person evaluation is available during the first week and follow-up by phone calls thereafter

[Shifting to subcutaneous infusion of apomorphine in advanced Parkinson's disease patients on an out-patient basis: experience and recommendations]. / Introducción ambulatoria de apomorfina en infusión subcutánea continua en pacientes con enfermedad de Parkinson avanzada: experiencia y recomendaciones.

INTRODUCTION: Shifting from oral medication to continuous subcutaneous infusion of apomorphine (CSIA) in Parkinson's disease (PD) may be complicated. It is unclear whether hospitalization is necessary. OBJECTIVE: To observe efficacy and side effects in advanced PD patients shifting to CSIA managed as out-patients with hospital-based support. MATERIAL AND METHODS: CSIA was started on 7 PD patients with uncontrollable on-off fluctuations on a waiting list for surgery. Two patients were initially admitted to hospital, all others being managed as out-patients. Awake off time was assessed with daily charts and motor function with UPDRS subscale and a scale for dyskinesias severity. Appropriate scales were applied for mood changes, neuropsychiatric complications and quality of life measures. Apomorphine dosage and oral antiparkinsonian medication were adjusted daily (mean: 6.1 days) according to motor responses. Follow-up visits were scheduled at 3 and 6 months. Patients were offered phone calls, unprogrammed visits, and 24 hours neurological care if required. RESULTS: All patients required phone calls (mean, 4.4), but unprogrammed visits were rare (1.5). Off-time was reduced in the overall group by a 68.1 %. Co-medication with levodopa was necessary in all patients, but all other antiparkinsonian medications could be discontinued. CONCLUSION: Shifting from oral dopaminergic therapy to CSIA is safe and effective in advanced PD managed on an out-patient basis in a hospital-based setting providing that daily in-person evaluation is available during the first week and follow-up by phone calls thereafter.

Trastornos del movimiento de la encefalopatía de Hashimoto / Movement disorders in Hashimoto's encephalopathy

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[Long-term risk of relapses in Tolosa-Hunt syndrome]. / Riesgo de recaídas a largo plazo en el síndrome de Tolosa-Hunt.

INTRODUCTION: The reviewed diagnostic criteria of Tolosa- Hunt síndrome (THS) by the International Classification of Headache Disorders (ICHD-II, 2004) includes demonstration of inflammatory changes by magnetic resonance imaging (MRI) in the cavernous sinus region as an alternative to biopsy. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia. It remains unclear for how long such close observation should be extended to assure the diagnosis nor whether THS represents a self-limited condition in the long-term. CASE REPORT: An observational study along 13 years of a patient fulfilling current ICHD-II criteria for THS to clarify the natural history of the disorder. RESULTS: We witnessed three episodes, the presenting one causing severe left orbital pain, ipsilateral abducens palsy , and hypoesthesia in the territory of the ophthalmic branch of the trigeminal nerve. Gadolinium MRI showed enhancement of tissue infiltrating the lateral wall of the carotid sinus. A relapse 21 months later caused involvement of left oculomotor and abducens nerves in addition to orbital pain which responded rapidly to steroids. Ensuing remission maintained for up to 11 years concluded in a new relapse again characterized by severe ipsilateral orbital pain and frontal numbness, but no ophthalmoplegia. A gadolinium-MRI did not show enhancement on this occasion despite prompt response to steroids. CONCLUSION: The risk of relapses in THS and its response to steroids may be maintained for as long as 13 years.

Epidemiological assessment of levodopa use in Cuba: 1993-1998.

PURPOSE: The purpose of this study is to describe and evaluate the use of levodopa in Cuba in order to provide a basis for intervention aimed at improving pharmacological treatment of individuals presenting with Parkinson's disease (PD). METHODS: We studied the amount of levodopa, both plain and combined, distributed by the central laboratory to hospital and community pharmacies in Cuba in the period 1993-1998. An internationally established drug-classification system and a reported method for epidemiological assessment of levodopa sales were applied. Sweden in 1994 served as the reference population. RESULTS: National crude rates of levodopa use basically remained stable since 1994, and in 1998 stood at 0.11 defined daily dose (DDD) per 1000 inhabitants/day, approximately 15 times lower than the corresponding figure for the reference population. Annual provincial use of levodopa showed considerable geographical variation, with the lowest rates in the eastern provinces and the highest rates in Havana City (Ciudad de La Habana). Adjustment for age reduced such differences by approximately 50%. CONCLUSIONS: Levodopa use in Cuba is low and consistent with the reported low prevalence of the diagnosis of PD. Results suggest that the diagnosis and treatment of PD can be improved, with emphasis on better detection of PD.