COVID-19 in Parkinson's disease: what holds the key?

INTRODUCTION: Parkinson's disease (PD) is more frequent in the elderly and increases the risk of respiratory infections. Previous data on PD and SARS-CoV-2 are scarce, suggesting a poor prognosis in advanced disease and second-line therapies. METHODS: A retrospective case-control study comparing patients with PD and COVID-19 and patients with PD without COVID-19 was conducted during the pandemic period in Spain (March 1st-July 31st 2020) in a tertiary university hospital. RESULTS: Thirty-nine (COVID-19 +) and 172 (COVID-19-) PD patients were included. Fifty-nine percent were males in both groups, with similar age (75.9 ± 9.0 COVID-19 + , 73.9 ± 10.0 COVID-19-), disease duration (8.9 ± 6.2 COVID-19 + , 8.5 ± 5.6 COVID-19-) and PD treatments. COVID-19 was mild in 10 (26%), required admission in 21 (54%) and caused death in 8 (21%) patients. Dementia was the only comorbidity more frequent in COVID-19 + patients (36% vs. 14%, p = 0.0013). However, in a multivariate analysis, institutionalization was the only variable associated with COVID-19 + (OR 17.0, 95% CI 5.0-60.0, p < 0.001). When considering severe COVID-19 (admission or death) vs. mild or absent COVID-19, institutionalization, neoplasm, dementia and a lower frequency of dopamine agonists were associated with severe COVID-19. In multivariate analysis, only institutionalization [OR 5.17, 95% CI 1.57-17, p = 0.004] and neoplasm [OR 8.0, 95%CI 1.27-49.8, p = 0.027] remained significantly associated. CONCLUSION: In our experience, institutionalization and oncologic comorbidity, rather than PD-related variables, increased the risk of developing COVID-19, and impacted on its severity. These findings suggest that epidemiologic factors and frailty are key factors for COVID-19 morbidity/mortality in PD. Appropriate preventive strategies should be implemented in institutionalized patients to prevent infection and improve prognosis.

Afectación encefálica global y uso de L-arginina en un paciente con síndrome de MELAS / Global cerebral involvement and L-arginine use in a patient with MELAS síndrome

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Forty years of Tuberculous meningitis: The new face of an old enemy.

BACKGROUND: Tuberculous meningitis (TBM) occurs in 1-5% of cases of tuberculosis. Without early treatment, mortality and permanent disability rates are high. METHODS: A retrospective study performed at a tertiary hospital in Madrid (Spain) to describe clinical, diagnostic, and therapeutic aspects of TBM and analyze epidemiological trends over forty years, divided into two intervals (1979-1998 and 1999-2018). RESULTS: Overall, TBM was diagnosed in 65 patients (1.8% of new tuberculosis diagnoses), 48 in the first period and 17 in the second one. Median age at diagnosis increased from 38.5 to 77 years (p = 0.003). The proportion of non-HIV immunosuppressed patients increased (from 2.1% to 29.4%, p < 0.001), while the percentage of patients with a history of drug-abuse decreased (from 33.3% to 5.9%, p = 0.027). The median time between the onset of neurological symptoms and lumbar puncture increased from seven to 15 days (p = 0.040). The time between the onset of symptoms and the initiation of tuberculostatic treatment also increased from eleven to 18 days (p = 0.555). Results from image, biochemical, and microbiological tests showed no differences between both periods. A decreasing trend was observed in survival rates at 1-week (from 97.9% to 64.7%, p < 0.001), 1-month (from 91.7% to 58.8%, p = 0.002) and 1-year (from 85.4% to 47.1%, p = 0.002) after TBM diagnosis. CONCLUSIONS: The profile of patients diagnosed with TBM has changed from a young HIV-infected patient with a history of drug addiction to an elderly patient with non-HIV immunosuppression. Diagnosis and start of treatment both experienced a noticeable delay in the second period, which could help explain the increase in mortality observed across the two periods.

[Acute global amnesia as an exclusive presenting symptom of thalamic infarct: a diagnostic challenge]. / Amnesia global aguda como forma exclusiva de presentacion de infarto talamico: un reto diagnostico.

INTRODUCTION: Isolated acute amnesia is an exceptional presenting symptom of thalamic stroke. This study analyses the clinical profile, the diagnosis, the treatment and the prognosis of these patients. CASE REPORTS: We conducted a retrospective review of the cases of thalamic infarct that presented exclusively as acute amnesia in our university tertiary hospital (n = 3) and a review of similar cases in PubMed (n = 20). 48% presented at least one risk factor of stroke (arterial hypertension, dyslipidaemia, diabetes mellitus, atrial fibrillation or a previous stroke). Amnesia was anterograde in three cases (13%) and global in the remaining 20 (87%). The infarct was detected in neuroimaging studies carried out within the first 24 hours in one patient (4%) and later in all the others; the average time until a diagnosis was established was 11 days. The initial CT scan was normal in five patients (22%). Eight cases (35%) required magnetic resonance imaging to detect the infarct. Of these, four subjects were studied directly with MR imaging. Amnesia clearly improved in eight patients (35%), and three of them (13%) made a full recovery. Fifteen patients (65%) presented mnemonic sequelae that interfered with their functional capacity. The clinical picture lasted less than 24 hours in two patients (9%). None of the cases received revasculisation therapy in the acute phase. CONCLUSION: The diagnosis of thalamic infarcts that begin exclusively with amnesia is very difficult and this has negative repercussions on their treatment in the acute phase. These infarcts can produce a functionally disabling memory deficit in a high percentage of patients.

TITLE: Amnesia global aguda como forma exclusiva de presentacion de infarto talamico: un reto diagnostico.Introduccion. La amnesia aguda aislada es una forma excepcional de presentacion del ictus talamico. Se analizan el perfil clinico, el diagnostico, el tratamiento y el pronostico de estos pacientes. Casos clinicos. Revision retrospectiva de los casos de infarto talamico que se presentaron exclusivamente como amnesia aguda en nuestro hospital terciario universitario (n = 3) y revision de casos similares en PubMed (n = 20). El 48% presentaba al menos un factor de riesgo de ictus (hipertension arterial, dislipidemia, diabetes mellitus, fibrilacion auricular o ictus previo). La amnesia fue anterograda en tres casos (13%) y global en los otros 20 (87%). El infarto se detecto en estudio de neuroimagen en las primeras 24 horas en un paciente (4%) y posteriormente en los demas, y la media de dias hasta el diagnostico fue de 11. La tomografia computarizada inicial fue normal en cinco (22%) pacientes. Precisaron estudio por resonancia magnetica ocho (35%) casos para detectar el infarto. De estos, cuatro sujetos se estudiaron directamente con resonancia magnetica. La amnesia presento una mejoria clara en ocho (35%) pacientes, y la recuperacion fue completa en tres (13%). Las secuelas mnesicas que interferian la capacidad funcional se presentaron en 15 pacientes (65%). La clinica persistio menos de 24 horas en dos pacientes (9%). Ningun caso recibio tratamiento revascularizador en fase aguda. Conclusion. Los infartos talamicos que comienzan de forma exclusiva con amnesia presentan notables dificultades diagnosticas que repercuten negativamente en su tratamiento en la fase aguda. Estos infartos pueden producir un deficit mnesico funcionalmente discapacitante en un porcentaje elevado de pacientes.

[Occipital condyle syndrome as the first symptom of a metastatic hepatocellular carcinoma. Two case reports]. / Sindrome del condilo occipital como primera manifestacion de un carcinoma hepatocelular metastasico. Presentacion de dos casos.

INTRODUCTION: Occipital condyle syndrome consists of the presence of unilateral occipital headache exacerbated by moving the head and is accompanied by paralysis of the ipsilateral hypoglossal nerve. One of its causes is infiltration of the base of the skull by bone metastases, especially those affecting the hypoglossal nerve due to infiltration as it passes through the osseous canal. CASE REPORTS: We report two clinical cases of occipital condyle syndrome secondary to metastatic hepatocarcinoma. The first is that of a 52-year-old male with liver cirrhosis secondary to liver pathology caused by hepatitis C virus with occipital condyle syndrome as the presenting symptom in disseminated hepatocarcinoma. The second case is that of a 56-year-old male after recurrence of hepatocarcinoma following a liver transplant, despite not fulfilling the Milan criteria. CONCLUSION: Occipital condyle syndrome is an alarm symptom and requires a thorough study by means of imaging tests, since it may be the first symptom of an undetected hepatocarcinoma.

TITLE: Sindrome del condilo occipital como primera manifestacion de un carcinoma hepatocelular metastasico. Presentacion de dos casos.Introduccion. El sindrome del condilo occipital consiste en la presencia de cefalea occipital unilateral que empeora con los movimientos cefalicos y se acompaña de paralisis del XII par ipsilateral. La infiltracion de la base del craneo por metastasis oseas se encuentra entre sus etiologias, especialmente las que afectan por infiltracion al nervio hipogloso en su paso a traves del canal oseo. Casos clinicos. Se presentan dos casos clinicos de sindrome del condilo occipital secundario a un hepatocarcinoma metastasico. El primero, un varon de 52 años con cirrosis hepatica secundaria a hepatopatia por virus de la hepatitis C, con sindrome del condilo occipital como sintoma inicial en un hepatocarcinoma diseminado; y el segundo, un varon de 56 años, tras recidiva de un hepatocarcinoma despues de un trasplante hepatico, a pesar de no cumplir los criterios de Milan. Conclusion. El sindrome del condilo occipital es un sintoma de alarma y requiere realizar un estudio completo mediante pruebas de imagen, puesto que puede ser la primera manifestacion de un hepatocarcinoma oculto.

[Tarantism in Spain in the eighteen century: latrodectism and suggestion]. / El tarantismo en España en el siglo XVIII: latrodectismo y sugestion.

INTRODUCTION: Tarantism is the disease caused by the bite of the tarantula, in which the music tarantella triggers an involuntary dance. It is known in Italy since the sixteenth century. AIM: To analyze the tarantism reported in Spain at the end of the eighteenth century, with special attention to its neurological aspects, and to propose its medical and psychopathological explanation. DEVELOPMENT: An epidemic of people affected by the tarantula bite occurred in Spain in 1782. Spanish doctors described appropriately the clinical effects, identical to those produced by the bite of the spider black widow (Latrodectus tredecimguttatus), which was at that time identified as a tarantula. The cases reported by Francisco Xavier Cid cured with the involuntary dance triggered by the tarantella, as was described in Italy since the sixteenth century. Our interpretation is that this curative effect of dance in Spain was induced by suggestion. In Spanish patients there were no behavioral disturbances, periodic recurrences or collective involvement as those reported by Italian authors, which suggest an hysterical phenomenon, probably a continuation of the dancing mania of the Middle Age. CONCLUSIONS: Tarantism reported in Spain in the eighteenth century includes two different phenomena: the systemic symptoms produced by the tarantula bite, which is actually latrodectism, and the curative effect of the tarantella, explained by suggestion. The psychiatric disturbances, with a hysterical nature, falsely associated to the tarantula bite, observed in Italy, were not present among the Spanish cases of tarantism in the eighteenth century.

TITLE: El tarantismo en España en el siglo XVIII: latrodectismo y sugestion.Introduccion. El tarantismo es la enfermedad producida por la picadura de la tarantula, en la que la musica de la tarantela desencadena un baile involuntario. Se conoce en Italia desde el siglo XVI. Objetivo. Analizar el tarantismo descrito en España a finales del siglo XVIII, atendiendo especialmente a sus aspectos neurologicos, y proponer su explicacion medica y psicopatologica. Desarrollo. En 1782 hubo una epidemia de afectados por picadura de tarantula en España. Medicos españoles describieron correctamente los efectos clinicos, identicos a los provocados por la picadura de la araña viuda negra (Latrodectus tredecimguttatus), identificada en la epoca como tarantula. Los casos descritos por Francisco Xavier Cid curaban con el baile involuntario provocado por la tarantela, como se describia en Italia desde el siglo XVI. Interpretamos el efecto curativo de este baile en España como un fenomeno de sugestion. En los pacientes españoles no se producian los trastornos del comportamiento, las recidivas periodicas ni la afectacion colectiva descritos por autores italianos, y que sugieren un fenomeno histerico, probablemente continuacion de la mania danzante de la Edad Media. Conclusiones. El tarantismo descrito en España en el siglo XVIII incluye dos fenomenos distintos: los sintomas sistemicos producidos de la mordedura de la tarantula, que es en realidad un latrodectismo, y el efecto curativo de la tarantela, lo cual se explica por un fenomeno de sugestion. Los trastornos psiquicos falsamente asociados a la picadura de la tarantula observados en Italia, de origen histerico, no estuvieron presentes en los casos españoles de tarantismo del siglo XVIII.

Toxicidad cerebelosa por citarabina: serie de 4 casos / Cerebellar toxicity due to cytarabine: A series of 4 cases

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Gustavo Pittaluga y la expedición para el estudio de la enfermedad del sueño en los territorios españoles del golfo de Guinea (1909) / Gustavo Pittaluga and the expedition to study sleeping sickness in the Spanish territories of the Gulf of Guinea (1909

Introducción. La enfermedad del sueño, o tripanosomiasis africana, produjo a principios del siglo x x una gran mortalidad. Por ello, las potencias coloniales organizaron diversas expediciones científicas, que favorecieron el conocimiento de la enfermedad. Objetivo. Estudiar la primera investigación realizada en España sobre tripanosomiasis africana y en el campo de la medicina tropical, mediante una expedición científica organizada por Cajal a los territorios españoles del golfo de Guinea en el año 1909. Desarrollo. Dirigió la expedición el parasitólogo Gustavo Pittaluga, que sería una de las figuras más importantes en la medicina y la salud pública en España durante el primer tercio del siglo x x. Le acompañaron Luis Rodríguez Illera y Jorge Ramón Fañanás, hijo de Cajal. Recorrieron durante cuatro meses los territorios de Guinea, recogiendo información clínica y epidemiológica sobre la enfermedad del sueño y otras enfermedades, examinando a numerosos pacientes, a los que se les realizaba análisis hematológicos y parasitológicos. En la descripción clínica de los 14 pacientes con tripanosomiasis, encontramos la primera descripción de un síndrome de opsoclono-mioclono. En un caso se realizó estudio patológico cerebral. Se hicieron también importantes estudios entomológicos y estudios experimentales sobre la tripanosomiasis. Conclusiones. Esta expedición se enmarca dentro del impulso de renovación de la ciencia española, que Cajal encabezó a través de la Junta de Ampliación de Estudios recién creada. En las investigaciones realizadas en Guinea, Pittaluga mostró un alto nivel científico, tanto en clínica e higiene como en parasitología y entomología, equiparable a otros estudios europeos coetáneos (AU)

Introduction. Sleeping sickness, or human African trypanosomiasis, caused an important mortality at the beginnings of the twentieth century. For this reason the European colonial countries organized several scientific expeditions which contributed decisively to the knowledge of the disease. Aim. To study the first investigation performed in Spain on African trypanosomiasis and in the field of tropical medicine, which was accomplished by a scientific expedition to the Spanish territories in the Gulf of Guinea organized by Cajal in 1909. Development. The parasitologist Gustavo Pittaluga, who became one of the most outstanding figures in Spanish medicine and public health during the first third of the twentieth century, commanded the expedition. Other members were Luis Rodríguez Illera and Jorge Ramón Fañanás, Cajal’s son. Along four months they travelled through the Spanish territories of Guinea, collecting clinical and epidemiological information on sleeping sickness and other diseases, and examining a great number of patients, who had hematological and parasitological studies performed. In the clinical description of the 14 cases of trypanosomiasis studied we have found the first description of the opsoclonus-myoclonus syndrome. A pathological study of the brain was performed in one case. In addition, important entomological studies and experimental investigations on trypanosomiasis were also performed. Conclusions. This expedition took place in the context of the impulse of renovation of Spanish science headed by Cajal through the Junta de Ampliación de Estudios, recently created. In the investigations performed in Guinea, Pittaluga demonstrated a high scientific standard in the fields of clinical medicine, hygiene, parasitology and entomology, comparable with other contemporary European studies (AU)

[Central pontine and extrapontine myelinolysis: clinical case, brain magnetic resonance and evolution in 13 patients]. / Mielinolisis central pontina y extrapontina: cuadro clínico, resonancia magnética cerebral y evolución en 13 pacientes.

INTRODUCTION: Central pontine myelinolysis (CPM) is a disease characterized by the destruction of the myelin in the brainstem, generally associated with alcoholism, rapid correction of hyponatremia and other electrolytic alterations. The clinical symptoms, etiopathogenic factors, neuroimaging and evolution of the series of patients diagnosed of central pontine/extrapontine myelinolysis (CPEM) are described. METHODS: Review of all the clinical histories with diagnoses of CPM made in our hospital since 1989. All the cases were reviewed, ruling out those having a magnetic resonance or clinical picture not clearly consistent with the diagnosis. Age, symptoms, comorbidity, associated metabolite alterations and clinical evolution were analyzed. RESULTS: 13 cases whose ages ranged from 28 to 81 years were identified. Hyponatremia was identified during the clinical course in six patients, with neurological worsening associated to its correction in 3 of them. No sodium disorders were identified in 7 patients. Seven of the patients had associated alcoholism. Hyperintense lesions were found in all the cases in T2 sequences and FLAIR in the brainstem consistent with the typical pattern of the osmotic demyelinization syndrome. The severity of the clinical picture identified varied from a symptomatic patient to coma in 9 cases. In regards to the clinical course, four patients completely recovered, eight had residual symptoms with different severity and one patient died. CONCLUSIONS: The series is representative of the clinical and etiopathogenic spectrum of the osmotic demyelinization syndrome. Most of the clinically symptomatic patients improve if the secondary complications are controlled.

Mielinolisis central pontina y extrapontina: cuadro clínico, resonancia magnéticacerebral y evolución en 13 pacientes / Central pontine and extrapontine myelinolysis: clinical case, brain magnetic resonance and evolution in 13 patients

Introducción. La mielinolisis central pontina (MCP) es una entidadcaracterizada por la destrucción de mielina en la base de la protuberancia,generalmente asociada con enolismo, corrección rápidade la hiponatremia y otras alteraciones electrolíticas. Se describe laclínica, factores etiopatogénicos, neuroimagen y evolución de unaserie de pacientes con diagnóstico de mielinolisis central pontina/extrapontina(MCP/E).Métodos. Revisión de todas las historias clínicas con diagnósticode MCP realizados en nuestro hospital desde el año 1989. Se revisarontodos los casos descartando aquellos con resonancia magnéticao cuadros clínicos no claramente compatibles con el diagnóstico.Se analizó la edad, clínica, comorbilidad, alteraciones metabólicasasociadas y evolución clínica.Resultados. Se identificaron 13 casos con edades comprendidasentre los 28 y 81 años. Se identificó hiponatremia durante el cursoclínico en 6 pacientes, produciéndose en 3 de ellos un empeoramientoneurológico asociado a la corrección de la misma. En 7 pacientesno se identificaron trastornos del sodio. Siete de los pacientes presentabanenolismo asociado. En todos los casos se reconocieron lesioneshiperintensas en secuencias T2 y FLAIR en la protuberanciacompatibles con el patrón típico del síndrome de desmielinizaciónosmótica. La gravedad del cuadro clínico identificado varió desde unpaciente asintomático hasta el coma en 9 casos. En cuanto a la evoluciónclínica, 4 pacientes se recuperaron completamente, 8 presentaronsintomatología residual de distinta severidad y 1 paciente falleció.Conclusiones. La serie es representativa del espectro clínico yetiopatogénico del síndrome de desmielinización osmótica. La mayoríade los pacientes clínicamente sintomáticos mejoran si las complicacionessecundarias son controladas (AU)

Introduction. Central pontine myelinolysis (CPM) is a diseasecharacterized by the destruction of the myelin in the brainstem,generally associated with alcoholism, rapid correction ofhyponatremia and other electrolytic alterations. The clinicalsymptoms, etiopathogenic factors, neuroimaging and evolutionof the series of patients diagnosed of central pontine/extrapontinemyelinolysis (CPEM) are described.Methods. Review of all the clinical histories with diagnosesof CPM made in our hospital since 1989. All the cases were reviewed,ruling out those having a magnetic resonance or clinicalpicture not clearly consistent with the diagnosis. Age, symptoms,comorbidity, associated metabolite alterations and clinical evolutionwere analyzed.Results. 13 cases whose ages ranged from 28 to 81 yearswere identified. Hyponatremia was identified during the clinicalcourse in six patients, with neurological worsening associated toits correction in 3 of them. No sodium disorders were identifiedin 7 patients. Seven of the patients had associated alcoholism.Hyperintense lesions were found in all the cases in T2 sequencesand FLAIR in the brainstem consistent with the typical pattern ofthe osmotic demyelinization syndrome. The severity of the clinicalpicture identified varied from a symptomatic patient to comain 9 cases. In regards to the clinical course, four patients completelyrecovered, eight had residual symptoms with different severityand one patient died.Conclusions. The series is representative of the clinical andetiopathogenic spectrum of the osmotic demyelinization syndrome.Most of the clinically symptomatic patients improve if thesecondary complications are controlled (AU)

[Tethered cord syndrome in the adult]. / Síndrome de médula anclada en el adulto.

INTRODUCTION: The tethered cord syndrome (TCS) is a congenital malformation with a pathologic fixation of the spinal cord in the spinal canal. It presents clinically as musculoskeletal, cutaneous, urological and neurological manifestations. The diagnosis is based on the clinical manifestations and on the MRI (Magnetic Resonance Imaging) of the lumbar spine. It is usually diagnosed in childhood, but the symptoms can appear in adult life. METHOD: We reviewed all the cases of TCS in the adult diagnosed in our hospital between 1998 and 2005. The following parameters were evaluated: mean age at onset, initial symptoms, signs, MRI findings and outcome. RESULTS: Four 22 to 72 year old patients were diagnosed. The age at onset varied from 16 to 52 years old and the diagnosis took between 2 and 20 years to be established. The most frequent initial symptoms were the muscular atrophy and the motor weakness in the lower extremities. Two patients exhibited cutaneous stigmata (one had hypertrichosis and the other one a lipoma in the sacrum area) and one a partial agenesis of the sacrum. The most frequent MRI finding was a low lying cord with a lipoma in the sacrum area. In three patients the cord was detethered surgically, but only two of them improved. CONCLUSIONS: The TCS is an uncommon disease in adult, which is usually diagnosed very late in the adult. Because of its insidious and non specific symptomatology, and of its potential surgical treatment, it should be considered in the differential diagnosis of medullar syndromes and polyneuropathies.

Síndrome de médula anclada en el adulto / Tethered cord syndrome in the adult

Introducción. El síndrome de médula anclada (SMA) es una malformación congénita en la que se produce una fijación de la médula dentro del canal espinal. Clínicamente se caracteriza por afectación musculoesquelética, cutánea, neurológica y urológica. El diagnóstico se basa en la clínica y en la resonancia magnética (RM) lumbar. Suele diagnosticarse en la infancia, aunque algunos pacientes inician los síntomas en la edad adulta. Método. Hemos revisado todos los casos de SMA en el adulto diagnosticados en nuestro hospital entre 1998 y 2005. Estudiamos la edad media de presentación, los síntomas iniciales, la exploración física, los hallazgos radiológicos y la evolución. Resultados. Cuatro pacientes, entre 22 y 72 años, fueron diagnosticados de SMA. La edad de inicio de los síntomas variaba entre 16 y 52 años y el diagnóstico se demoró entre 2 y 20 años. Las manifestaciones iniciales más frecuentes fueron la atrofia muscular y la pérdida de fuerza en miembros inferiores. Dos pacientes asociaban estigmas cutáneos (uno hipertricosis sacra y otro lipoma sacro) y uno agenesia atípica del sacro. En la RM el hallazgo más frecuente fue la presencia de un cono medular descendido y de un lipoma en el canal sacro. Tres pacientes fueron intervenidos, con mejoría posterior en dos de ellos. Conclusiones. El SMA es una enfermedad poco frecuente y de diagnóstico muy tardío en el adulto. Por su sintomatología insidiosa e inespecífica hay que considerarlo en el diagnóstico diferencial de los síndromes medulares y de las polineuropatías dada su potencial reparación quirúrgica

Introduction. The tethered cord syndrome (TCS) is a congenital malformation with a pathologic fixation of the spinal cord in the spinal canal. It presents clinically as musculoskeletal, cutaneous, urological and neurological manifestations. The diagnosis is based on the clinical manifestations and on the MRI (Magnetic Resonance Imaging) of the lumbar spine. It is usually diagnosed in childhood, but the symptoms can appear in adult life. Method. We reviewed all the cases of TCS in the adult diagnosed in our hospital between 1998 and 2005. The following parameters were evaluated: mean age at onset, initial symptoms, signs, MRI findings and outcome. Results. Four 22 to 72 year old patients were diagnosed. The age at onset varied from 16 to 52 years old and the diagnosis took between 2 and 20 years to be established. The most frequent initial symptoms were the muscular atrophy and the motor weakness in the lower extremities. Two patients exhibited cutaneous stigmata (one had hypertrichosis and the other one a lipoma in the sacrum area) and one a partial agenesis of the sacrum. The most frequent MRI finding was a low lying cord with a lipoma in the sacrum area. In three patients the cord was detethered surgically, but only two of them improved. Conclusions. The TCS is an uncommon disease in adult, which is usually diagnosed very late in the adult. Because of its insidious and non specific symptomatology, and of its potential surgical treatment, it should be considered in the differential diagnosis of medullar syndromes and polyneuropathies

[Post-encephalitic syndromes in the Spanish medical literature]. / Síndromes postencefalíticos en la literatura médica española.

AIM: A large number of patients with encephalitis lethargica developed different post-encephalitic syndromes (PES), which have an important medical and social impact. We studied the clinical and historical aspects of PES in Spain by reviewing the medical literature published in this country between 1918 and 1936. DEVELOPMENT: There are no statistical data concerning PES in Spain, although Spanish physicians drew attention to their high rate of prevalence and their repercussions on community health. Most of the 140 patients that were reviewed (74%) presented predominant Parkinsonism, but some features of Parkinsonism were observed in nearly all cases. Other movement disorders (focal dystonias, chorea, myoclonus, oculogyric crises, abnormalities affecting breathing rate) were described, as well as sleep, endocrine and vegetative disorders. Psychiatric disorders were often reported, the most frequent being bradyphrenia associated to Parkinsonism, but a hypomanic picture with impulsive behaviour was very characteristic among young people. PES was diagnosed on average two years after the episode of acute encephalitis lethargica, although it often appeared immediately afterwards. Many studies discuss the contribution made by PES to further our knowledge of the pathophysiology of extrapyramidal diseases and about the involvement of the basal ganglia in psychiatric and behavioural disorders. CONCLUSIONS: Despite the absence of statistical data, Spanish authors highlighted the important repercussions the PES had on community health, as well as the role they played in extending our knowledge of the pathophysiology of the basal ganglia. Cases of Parkinsonism were predominant, although all kinds of post-encephalitic manifestations were reported.

Síndromes postencefalíticos en la literatura médica española / Post-encephalitic syndromes in the spanish medical literature

Objetivo. Un gran número de pacientes de encefalitis letárgica desarrollaba diferentes síndromes postencefalíticos (SPE), de importante impacto medicosocial. Hemos estudiado los aspectos clínicos e históricos de los SPE en España, mediante una revisión de la literatura médica publicada en este país en el período 1918-1936. Desarrollo. No existen datos estadísticos sobre los SPE en España, aunque médicos españoles llamaron la atención sobre su alta prevalencia y su repercusión sociosanitaria. La mayoría de los 140 pacientes revisados (74%) presentaron parkinsonismo predominante, pero en casi todos se apreciaba algún rasgo parkinsoniano. Se describieron otros trastornos del movimiento (distonías focales, corea, mioclonías, crisis oculógiras, anomalías del ritmo respiratorio), así como trastornos del sueño, endocrinos y vegetativos. A menudo se comunicaron alteraciones psiquiátricas: la más frecuente era la bradifrenia asociada a parkinsonismo, pero fue muy característico un cuadro hipomaníaco con conducta impulsiva en jóvenes. El diagnóstico del SPE se realizó una media de dos años después del episodio de encefalitis letárgica aguda, aunque con frecuencia apareció inmediatamente después. En muchos trabajos se discute sobre la contribución de los SPE al desarrollo del conocimiento de la fisiopatología de las enfermedades extrapiramidales y sobre la implicación de los ganglios basales en los trastornos psiquiátricos y de conducta. Conclusiones. En ausencia de datos estadísticos, los autores españoles reflejaron la importante repercusión sociosanitaria de los SPE, así como su papel en el conocimiento de la fisiopatología de los ganglios basales. Predominaron los parkinsonismos, aunque se describieron todo tipo de manifestaciones postencefalíticas

Aim. A large number of patients with encephalitis lethargica developed different post-encephalitic syndromes (PES), which have an important medical and social impact. We studied the clinical and historical aspects of PES in Spain by reviewing the medical literature published in this country between 1918 and 1936. Development. There are no statistical data concerning PES in Spain, although Spanish physicians drew attention to their high rate of prevalence and their repercussions on community health. Most of the 140 patients that were reviewed (74%) presented predominant Parkinsonism, but some features of Parkinsonism were observed in nearly all cases. Other movement disorders (focal dystonias, chorea, myoclonus, oculogyric crises, abnormalities affecting breathing rate) were described, as well as sleep, endocrine and vegetative disorders. Psychiatric disorders were often reported, the most frequent being bradyphrenia associated to Parkinsonism, but a hypomanic picture with impulsive behaviour was very characteristic among young people. PES was diagnosed on average two years after the episode of acute encephalitis lethargica, although it often appeared immediately afterwards. Many studies discuss the contribution made by PES to further our knowledge of the pathophysiology of extrapyramidal diseases and about the involvement of the basal ganglia in psychiatric and behavioural disorders. Conclusions. Despite the absence of statistical data, Spanish authors highlighted the important repercussions the PES had on community health, as well as the role they played in extending our knowledge of the pathophysiology of the basal ganglia. Cases of Parkinsonism were predominant, although all kinds of post-encephalitic manifestations were reported

[How did encephalitis lethargica affect Spain? An analysis of the cases reported between 1918 and 1936]. / Cómo fue la encefalitis letárgica en España? Análisis de los casos publicados entre 1918 y 1936.

AIM: Encephalitis lethargica spread as a pandemic over the period 1918-1921. Clinically, it displayed an important degree of polymorphism and the clinical presentation varied from one outbreak to the next and from one region to another. Few studies have been carried out on the encephalitis lethargica epidemic in Spain. To determine the characteristics of its clinical and developmental features, we reviewed the cases reported in the Spanish medical literature over the period 1918-1936. DEVELOPMENT: A total of 120 cases were analysed. Most of them (75%) occurred during the winter of 1919-1920. The mean age was 27 years (range: 0-59 years). Most of them presented feverish syndromes (82%). The most frequent neurological symptoms were disorders affecting the cranial nerves, especially the oculomotor nerves, sleep rhythm disorders, altered mental or conscious status, pupillary abnormalities and movement disorders. Important variations were observed in the clinical presentation and in the developmental course. Although most cases did not present pure clinical forms, the predominant forms were somnolent-ophthalmoplegic (46%) and hyperkinetic (17%), which were associated with delusions with chorea or myoclonias. The most uncommon were amyostatic forms (Parkinsonian, 4%). The mortality rate was 23%. Most of the survivors (69%) were cured in the acute phase with no significant sequelae. CONCLUSIONS: The maximum incidence of encephalitis lethargica in Spain was registered during the winter of 1919-1920. It presented an important degree of clinical polymorphism, with predominance of an association between lethargy and ophthalmoplegia, and was linked to a high mortality rate.