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INTRODUCTION: Vulpian-Bernhardt syndrome (VBS) is an atypical rare clinical phenotype of amyotrophic lateral sclerosis (ALS) that causes a significant delay in diagnosis, and thus it is important to recognise its clinical and electrophysiological features. MATERIALS AND METHODS: Retrospective cross-sectional study. We reviewed the clinical records of patients diagnosed with ALS in the period from January to December 2019. Those meeting criteria for VBS were included so as to describe their frequency as well as their clinical and electrophysiological features. RESULTS: Twenty patients (15.8%) met criteria for VBS; 55% were female; age at onset of symptoms was 46.6 ± 12.9 years; 40% were smokers; median delay in diagnosis was 24 (12-96) months; median time to involvement of the second body segment was 24 (12-132) months, which was lumbosacral in 65%; mean Revised Amyotrophic Lateral Sclerosis Functional Rating Scale score was 27 ± 7 points; 45% met the El Escorial criteria for ALS defined at diagnosis and 58.8% met the Awaji criteria. There were 19 nerve conduction studies and 17 electromyograms, and an abductor digiti minimi-abductor pollicis brevis (ADM/APB) ratio < 0.6 was found in 63% (split hand). CONCLUSIONS: There is a significant delay in the diagnosis of motor neuron diseases in general and more particularly in VBS. Calculating the ADM/APB ratio and applying the Awaji criteria in the electrophysiology study can be a valuable aid to increase diagnostic certainty in this clinical entity.
TITLE: Síndrome de Vulpian-Bernhardt. Frecuencia, características clínicas y electrofisiológicas en un centro de atención de tercer nivel en México.Introducción. El síndrome de Vulpian-Bernhardt (SVB) es un fenotipo clínico atípico e infrecuente de la esclerosis lateral amiotrófica (ELA) que condiciona un importante retraso diagnóstico, por lo que reconocer sus características clínicas y electrofisiológicas tiene relevancia. Materiales y métodos. Estudio retrospectivo y transversal. Se revisaron los expedientes clínicos de pacientes con diagnóstico de ELA en el período de enero de 2017 a diciembre de 2019. Se incluyeron los que cumplían criterios para SVB para describir su frecuencia, características clínicas y electrofisiológicas. Resultados. Veinte pacientes (15,8%) cumplieron los criterios para el SVB; el 55% eran mujeres; la edad de inicio de los síntomas era de 46,6 ± 12,9 años; presentaba tabaquismo el 40%; la mediana de retraso del diagnóstico fue de 24 (12-96) meses; la mediana en afectarse un segundo segmento corporal fue de 24 (12-132) meses, que fue el lumbosacro en el 65%; el promedio en la escala Revised Amyotrophic Lateral Sclerosis Functional Rating Scale fue de 27 ± 7 puntos; el 45% cumplía los criterios de El Escorial para ELA definida en el momento del diagnóstico y el 58,8%, los de Awaji. Se contó con 19 estudios de neuroconducción y 17 electromiogramas, y se encontró una razón abductor digiti minimi-abductor pollicis brevis (APB/ADM) menos de 0,6 en el 63% (mano dividida). Conclusiones. Existe un retraso importante en el diagnóstico de enfermedades de la motoneurona en general y de SVB en particular. Calcular la razón APB/ADM y aplicar los criterios de Awaji en el estudio de electrofisiología puede ser de gran ayuda para aumentar la certeza diagnóstica en esta entidad clínica.
Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/fisiopatologia , Adulto , Estudos Transversais , Fenômenos Eletrofisiológicos , Feminino , Humanos , Masculino , México , Pessoa de Meia-Idade , Estudos Retrospectivos , Centros de Atenção TerciáriaRESUMO
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.
Assuntos
Literatura Moderna/história , Medicina na Literatura/história , Neuropsiquiatria/história , História do Século XIX , História do Século XX , Transtornos Mentais/história , Transtornos Mentais/psicologia , Modelos Neurológicos , Modelos Psicológicos , Neuropsiquiatria/tendências , Médicos , EspanhaRESUMO
A 40-year-old woman was found to have bilateral Adie's pupils and generalized muscle stretch areflexia. She did not have orthostatic hypotension but, in an ECG strip in the office, she appeared to have an almost fixed heart rate. We thus studied the heart rate variability (HRV) and the systolic blood pressure variability (SBPV) in supine and standing position and also during rhythmic breathing. We found a decreased HRV in the time domain with very low standard deviation in supine and standing position and during rhythmic breathing. HRV in the frequency domain was low with a decrease in the absolute power of HF and LF and a decrease in the sympathovagal balance in supine and standing positions. SBPV in the time and frequency domains was found to be normal. This patient with Holmes-Adie syndrome had an asymptomatic severe loss of HRV and a preserved SBPV. The global decrease in the HRV in the time and frequency domains indicated that she had both vagal and sympathetic cardiac denervation, whereas the preserved SBPV suggested normal innervation of the blood vessels.
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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.
Assuntos
Braço/fisiologia , Reflexo , Vasoconstrição , Vasodilatação , Adulto , Idoso , Humanos , Pessoa de Meia-Idade , Fluxo Sanguíneo Regional , Pele , Adulto JovemRESUMO
All reports written in Cuba about toxoplasmosis (1913-58) are listed in chronological order, both those that have appeared in a publication and those which have been submitted to at a scientific meeting though they may still remain unpublished. Brief explanatory notes are commented upon each one and the place to find them are listed.
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Bibliografias como Assunto , Toxoplasmose , Cuba , HumanosRESUMO
Fifty patients with manic-depressive psychosis, 120 neurotics and 100 healthy individuals were studied. They underwent the toxoplasmin intradermal test. The highest percentage of reactors was found among patients with manic-depressive psychosis (66,0%). Patients with depressive neurosis accounted for the highest number among neurotics (55,6%). The intensity of reaction was higher among patients with manic-depressive psychosis. Neurotic patients were compared to schizophrenic patients from a previous study conducted by one out of the authors. It is concluded that the percentage of reactors is higher among patients with depressive mental disorders, and also that this percentage increases with mental deterioration in patients.
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Transtorno Bipolar/imunologia , Transtornos Neuróticos/parasitologia , Toxoplasma/imunologia , Antígenos , Transtorno Bipolar/parasitologia , Humanos , Testes Intradérmicos , Transtornos Neuróticos/imunologia , Toxoplasmose/imunologiaRESUMO
A hundred schizophrenic patients admitted to the "10 de Octubre" Clinical-Surgical Teaching Hospital and 158 schizophrenic patients admitted to the Psychiatric Hospital, both in Havana, and 100 control healthy individuals who did not consume psychopharmacons were studied. They underwent intradermal tests with toxoplasmin. Differences in reactor percentages between patients and healthy individuals were statistically significative as it has been reported in the foreign medical literature. The highest percentage of reactors in the group admitted to the Psychiatric Hospital where most advanced schizophreniacs are found leads to the conclusion that the more severe the patient state the higher the frequency and intensity of test reactivity among patients. Other factors as the dwelling conditions, age and sex are analyzed.
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Esquizofrenia/imunologia , Toxoplasma/imunologia , Adolescente , Adulto , Idoso , Antígenos , Cuba , Humanos , Testes Intradérmicos , Pessoa de Meia-IdadeRESUMO
One hundred mentally handicapped patients admitted to the 10 de octubre clinical-surgical teaching hospital in the Havana city, 100 patients admitted to the day hospital of such institution and 100 normal subjects were studied. The first two groups had different psychiatric diseases. They underwent toxoplasmin tests. The percentage of reactors increased from 30% among healthy subjects to 45,9% among the those attending the day hospital and to 60% among those admitted to wards; this is statistically significative. It is concluded that the higher the severity of the psychiatric disease, the poorer the hygienic habits and the higher the contact with the parasite phocuses.
Assuntos
Transtornos Mentais/parasitologia , Toxoplasmose/parasitologia , Antígenos , Cuba , Feminino , Hospitalização , Humanos , Masculino , Transtornos Mentais/complicações , Transtornos Mentais/epidemiologia , Transtornos Mentais/imunologia , Testes Cutâneos , Toxoplasmose/complicações , Toxoplasmose/epidemiologia , Toxoplasmose/imunologiaRESUMO
Intradermal tests using toxoplasmin were performed among three hundred mentally handicapped patients admitted to the "10 de Octubre" Clinical-Surgical Teaching Hospital in Havana between 1976--1977 and 100 healthy subjects. A statistically significant difference between reactors among patients (54,7%) and reactors among healthy subjects (30,0%) was found. Results are compared to those from a similar paper performed in the Psychiatric Hospital of Havana in 1967. It is concluded that the higher the severity of affections the higher the percentage of reactors in the surveyed series.
Assuntos
Antígenos , Testes Intradérmicos , Testes Cutâneos , Toxoplasmose/imunologia , Cuba , Feminino , Hospitais Psiquiátricos , Humanos , Testes Intradérmicos/métodos , Masculino , Transtornos Mentais/epidemiologia , Transtornos Mentais/imunologia , Testes Cutâneos/métodos , Toxoplasmose/epidemiologiaRESUMO
Sixty five patients with an ophthalmoscopic diagnosis of chorioretinitis who, underwent complement fixation tests and intradermoreaction using Toxoplasma antigens were studied. 95,4% of them disclosed antibody titers with the former and 58,5% reactors were found with the latter. Sixty five individuals with neither backgrounds nor clinical symptoms of toxoplasmosis were used as control, and 27,7% of reactors in both tests were likewise found. The differences among results are highly statistically significant; this points out toxoplasmosis as an important cause of chorioretinitis in our patients. Complement fixation tests were more sensitive than intradermoreactions as well as specific as the latter. Results were correlated to age, sex and animal contacts.
Assuntos
Coriorretinite/etiologia , Toxoplasmose Ocular/complicações , Adolescente , Adulto , Fatores Etários , Criança , Coriorretinite/imunologia , Testes de Fixação de Complemento , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores Sexuais , Testes CutâneosRESUMO
Three hundred and ninety patients with suspected toxoplasmosis due to their contact with animales--they owned them, or work with them--are studied. The great significance of this way of acquiring the disease is stated. Every patient had a complement fixation test and an intradermal reaction test with toxoplasmine. An 85.2% positiveness to complement fixation, and a 64.1% to intradermal test were found among those patients who informed animal contact; a 70,6% positiveness to complement fixation, and a 56,2% to intradermal reaction was found in the patients who denied having any contact with animals. This showed both the importance of animal contact as well as other forms of transmission. The contacts were also studied, and the animals were classified according to J. Jira, the researcher: maximal, high, minimal and unreceptiveness to toxoplasma. The possibility of acquiring toxoplasmosis from other sources besides the close contact with animals must be taken into consideration.