ABSTRACT
INTRODUCTION: The cerebellum has been traditionally associated with motor control learning and performance. However, since 1970 a growing body of clinical and experimental evidences has suggested that the cerebellum may be involved in nonmotor cognitive functions as well. OBJECTIVE: To explore the presence of eventual cognitive impairment in non-demented patients with isolated degenerative cerebellar diseases. PATIENTS AND METHODS: Twelve patients with the diagnosis of selective degenerative cerebellar disorders, either inherited or sporadic, were selected (mean age: 40.42 +/- 13.49 years; mean education level: 9.92 +/- 3.99 years; duration of illness: 12.13 +/- 11.27 years, MMSE: 26.75 +/- 1.5) and evaluated through a standardized neuropsychological tests battery. Normalized Z scores were estimated and compared against 0, employing the t test for one sample. RESULTS: Significant cognitive deficits were found in the following domains: executive, visuo-spatial, memory and attention functions. Performance on the Wisconsin test showed a significative number of perseverative errors. Memory deficits included verbal learning and free recall difficulties, with good recognition of the material presented. CONCLUSIONS: The findings of this study are consistent with the role of the cerebellum as modulator of mental functions. The cognitive deficits resulting from cerebellar pathology may be related with the disruption of cerebello-cortical connexions involving a complex network which includes the prefrontal region, suggesting that the cerebellum may process cortical information coming from different areas linked with the control of cognition.
Subject(s)
Cerebellar Diseases/physiopathology , Cerebellum/pathology , Neuropsychological Tests , Adolescent , Adult , Cerebellum/physiology , Cognition/physiology , Cognition Disorders/physiopathology , Female , Humans , Male , Memory Disorders/physiopathology , Middle AgedABSTRACT
We conducted a clinical and electromyographical study in patients with Chagas' disease in the indeterminate or chronic stages of the illness. Altogether 841 patients were examined. Only 511 were admitted within the protocol; the remainder patients were rejected because they showed other causes able to damage the nervous system. Fifty two (10.17%) out of the 511 patients showed signs and symptoms of peripheral nervous system involvement in the form of sensory impairment and diminished tendon jerks suggesting the presence of neuropathy. Forty five of them were submitted to a conventional electromyographical examination. Fifteen of them showed normal results, while the remainder 30 disclosed a reduced interference pattern, being most of the remaining motor unit potentials fragmented or poliphasic, reduced sensory and motor conduction velocities and diminished amplitude of the sensory action potential. The findings suggest that some chagasic patients in the indeterminate or chronic stages of the disease may develop a clinical mild sensory-motor peripheral neuropathy.
Subject(s)
Chagas Disease/physiopathology , Neural Conduction , Peripheral Nervous System/physiopathology , Adolescent , Adult , Chronic Disease , Electromyography , Electrophysiology , Female , Humans , Male , Middle AgedABSTRACT
Only 12 AIDS cases with hemichorea were reported in the literature. We report the first case of hemichorea associated with AIDS and cerebral toxoplasmosis in our country. A 26-year-old man had 3 episodes of focal seizures on the left side with subsequent loss of consciousness. A few weeks later, he noticed progressive left-sided weakness. Examination revealed a left hemiparesis. MRI of the head showed a round mass in the right frontal lobe and a smaller lesion in the left temporo-occipital area. Laboratory showed positive serum ELISA and Western Blot analysis for HIV antibodies. Serum tests for Toxoplasma showed elevated titers. He was treated with pyrimethamine and sulfadiazine. His weakness improved and he had no further seizures. Two weeks later, choreic movements appeared in the left foot, finally involving the entire left hemibody. A second MRI showed a new small lesion in the right cerebral peduncle. The patient completed 6 weeks of treatment, with further reduction in the size of the lesions. Nevertheless, the left hemichorea persisted. We believe that the hemichorea our patient had was caused by the contralateral peduncular lesion. Lesions involving the subthalamic nucleus or its connections may cause contralateral hemiballismus or hemichorea. In spite of the favorable response to antitoxoplasmic therapy, the hemichorea persisted. The present report illustrates an uncommon neurological complication in AIDS. We believe that a combination of a focal cerebral lesion and the HIV infection caused the movement disorder presented by the patient.
Subject(s)
Acquired Immunodeficiency Syndrome/complications , Chorea/complications , Toxoplasmosis, Cerebral/complications , Adult , Humans , Magnetic Resonance Imaging , Male , Toxoplasmosis, Cerebral/pathologyABSTRACT
Se observan complicaciones neurologicas en 40 por ciento de enfermos con SIDA. De estos, en 10 por ciento puede ser la manifestación inicial de la enfermedad. En otro 11 por ciento pueden aparecer trastornos del movimimiento. Comunicamos el primer caso de hemicorea asociada a toxoplasmose cerebral y SIDA en nuestro pais. Hombre de 26 anos, con diagnóstico de SIDA y toxoplasmosis cerebral. Habia comenzado con crisis motoras simples de hemicuerpo izquierdo, con generalización secundaria y luego perdida de fuerza progresiva en dicho hemicuerpo. La RMN de cerebro mostro una lesión frontal derecha y otra temporo-occiptal izquierda, con gran edema perilesional y efecto de masa. Las serologias para HIV y toxoplasmosis fueron positivas. Comenzo tratamiento con sulfadiazina y pirimetamina. Al duodecimo dia aparecieron movimientos involuntários del pie izquierdo, coreicos, que se extendieron mas tarde a todo ese miembro inferior y luego al hemicuerpo. Nueva RMN de cerebro mostro disminución del edema y efecto de masa de las lesiones. Sin embargo, se observó una nueva lesion a nivel peduncular derecho. Movimientos involuntarios en pacientes con toxoplasmosis cerebral la literatura mundial y el primero en nuestro pais de hemicorea asoicada a toxplasmosis y SIDA
Subject(s)
Humans , Male , Adult , Chorea/complications , Acquired Immunodeficiency Syndrome/complications , Toxoplasmosis, Cerebral/complications , Magnetic Resonance Spectroscopy , Toxoplasmosis, Cerebral/diagnosis , Toxoplasmosis, Cerebral/therapyABSTRACT
UNLABELLED: We proposed to investigate subclinical cognitive impairment secondary to chronic Chagas' disease (CCD). No similar study was previously done. The neuropsychological performance of 45 chronic Chagasic patients and 26 matched controls (age, education place and years of residency in endemic area) was compared using the Mini Mental State Exam (MMSE), Weschler Memory Scale (WMS) and the Weschler Adult Intelligent Scale (WAIS). Non-parametric tests and Chi2 were used to compare group means and multivariate statistics in two way frequency tables for measures of independence and association of categorical variables with the disease. RESULTS: Chagasic patients showed lower MMSE scores (p < .004), poor orientation (p < .004), and attention (p < .007). Lower WMS MQ were associated with CCD (Chi2 5.9; p < .01; Fisher test p < .02). Lower WAIS IQ were associated with CCD (Chi2 6.3, p < .01; Fisher test p < .01) being the digit symbol (p < .03), picture completion (p < .03), picture arrangement (p < .01) and object assembly (p < .03) subtests the most affected. The impairment in non-verbal reasoning, speed of information processing, problem solving, learning and sequencing observed in chronic Chagas disease patients resembles the cognitive dysfunction associated with white matter disease.
Subject(s)
Chagas Disease/complications , Cognition Disorders/etiology , Adult , Chagas Disease/psychology , Chronic Disease , Educational Status , Female , Frontal Lobe/physiopathology , Humans , Male , Multivariate Analysis , Wechsler ScalesABSTRACT
El objetivo de esta investigación fue determinar compromiso cognitivo en pacientes con enfermedad de Chagas en estadio cronico. Se estudio el perfil cognitivo de 45 pacientes chagasicos cronicos (CC) y 26 controles apareados por edad, educación, lugar y tiempo de residencia en area endemica. El Minimental State (MMSE), la escala de memoria de Weschlelr (WMS) y el test de Inteligencia de Weschler (WAIS) han dio utilizados para evaluar ambos grupos. Para el estudio estadistico de los datos se utilizaron pruebas no parametricas, Chi2 y estadistica multivariada en tabla de 2 x 2 para medir la association o independencia de variables categoriales con la presencia de enfermedad. Los resultados mostraron que los pacientes alcanzaban score menor que los controles en el MMSE (p < 0.004) debido basicamente a una mas pobre orientacion (P < 0.004) y atencion (p < 0.007). Cocientes bajos de memoria en el WMS se asociaron a la presencia de enfermedad (Chi25.9, p < 0.01; test de Fisher p < 0.02). Cocientes bajos de inteligencia en el WAIS se asociaron con la presencia de enfermedad (Chi26.3, p < 0.01; test de Fisher p < 0.01). Los subtests simbolos digitos (p < 0.03), completamientos de figuras (p < 0.03), ordenamiento de laminas (p < 0.01) y rompecabezas (p < 0.03) mostraron mayor compromiso. Estos resultados sugieren disfuncion del razonamiento no verbal, disminucion de la velocidad de procesado de la informacion y dificultad en la resolucion de problemas nuevos, en la habilidad de secuenciacion y en el aprendizaje. Este conjunto de hallazgos sugiere posible compromiso de la sustancia blanca subcortical en estos enfermos
Subject(s)
Adult , Humans , Male , Female , Cognition Disorders/etiology , Chagas Disease/complications , Chagas Disease/psychology , Educational Status , Frontal Lobe/physiopathology , Multivariate Analysis , Wechsler ScalesABSTRACT
The possible involvement of spinal alpha motor neurons, dorsal root ganglia and sensory fibers in human chronic Chagas' disease was previously demonstrated. More recently neuropsychological and sensory evoked potentials studies suggest the existence of central nervous system abnormalities in these patients. We assessed the state of central motor pathways in 46 patients with chronic Chagas' disease and 30 healthy volunteers by means of percutaneous cortical and spinal electrical stimulation. No significative slowness in pyramidal tracts (PT) conduction was found when comparing both groups. Neither any individual patient exhibited abnormally delayed PT conduction values beyond the upper normal limit of the healthy volunteers. These results suggest that, in contrast with other neural systems, the large myelinated PT fibers are usually spared in human chronic Chagas' disease.
Subject(s)
Chagas Disease/physiopathology , Evoked Potentials, Somatosensory/physiology , Adolescent , Adult , Chagas Disease/complications , Chronic Disease , Female , Humans , Male , Middle Aged , Neural Conduction/physiology , Reaction TimeABSTRACT
Estudios previos han demostrado que la enfermedad de Chagas cronica (ECHC) humana puede comprometer las motoneuronas espinales alfa, el ganglio de la raiz dorsal y las fibras sensitivas perifericas. Mas recientemente otras investigaciones han senalado que algunos de estos pacientes presentan anomalias en pruebas cognitivas y de potenciales evocados sensoriales, sugiriendo tambien la existencia de compromiso central en 46 pacientes con ECHC en 30 voluntarios sanos. Para ello se emplearon tecnicas de estimulación electrica cortical transcraneana y espinal percutanea. No se encontro un enlentecimiento significativo en la conducción piramidal del grupo con ECHC comparado con los voluntarios sanos. Considerados individualmente, en ningun paciente con ECHC la conducción corticoespinal presento valores que excedieran el limite superior normal del grupo control. Estos resultados sugieren que a diferencia de lo que ocurre con otros sistemas neurales, las fibras piramidales mielinizadas de grueso calibre se encuentran usualmente indemnes en pacientes con ECHC
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Cognition Disorders/etiology , Chagas Disease/physiopathology , Evoked Potentials, Somatosensory/physiology , Chronic Disease , Neural Conduction/physiology , Chagas Disease/complications , Reaction TimeABSTRACT
Un estudio piloto sobre el estado de las funciones cerebrales superiores fue llevado a cabo en 20 pacientes adultos con enfermedad de Chagas crónica. Sus resultados fueron comparados con un grupo control integrado por 10 sujetos normales agrupados por sexo y edad. Se empleó la bateria de pruebas neuropsicológicas de Luria. Los rendimientos de los pacientes chagásicos fueron en general inferiores a los de los controles, presentando diferencias estadísticamente significativas en las pruebas de memoria, definiciones(vocabulario), relaciones lógicas y analogías. De éstas, solo una definición, no estuvo contaminada por el nivel de educación. Los resultados sugieren que la enfermedad de Chagas, en su estadio crónico, puede ser responsable de la baja performance vista en algunas de las pruebas cognitivas. Sin embargo, es necesario efectuar estudios más extensos observando con mayor cuidado la selección de los pacientes y el nivel de educación antes de aceptar definitivamente esta presunción
Subject(s)
Cognition Disorders/etiology , Chagas Disease/complications , Chronic Disease , Cognition Disorders/classification , Cognition Disorders/diagnosis , Neuropsychological Tests , Memory Disorders/diagnosis , Memory Disorders/psychology , Concept FormationABSTRACT
Con la finalidad de investigar la existencia de alteraciones clínicas en el examen neurológico de pacientes que solo mostraban positividad serológica para la enfermedad de Chagas se examinaron a 249 individuos. El examen clínico neurológico mostró que el 11,24% (n:28) tenía alguna anormalidad, observándose como alteración más frecuente hiporreflexia en el 82%, seguida por las alteraciones objetivas en el 49,50%, y subjetivas en el 24,50% de la sensibilidad, configurando en la mayoría de los pacientes un síndrome neuropático. Estos hallazgos señalan que la enfermedad de Chagas, en su estadio crónico, puede ser la causa de manifestaciones clínicas que indican compromiso del sistema nervioso periférico
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Chagas Disease/complications , Chagas Disease/diagnosis , Reflex, Abnormal , Peripheral Nervous System Diseases/etiology , Neurologic Manifestations , Chronic DiseaseABSTRACT
Un estudio piloto sobre el estado de las funciones cerebrales superiores fue llevado a cabo en 20 pacientes adultos con enfermedad de Chagas crónica. Sus resultados fueron comparados con un grupo control integrado por 10 sujetos normales agrupados por sexo y edad. Se empleó la bateria de pruebas neuropsicológicas de Luria. Los rendimientos de los pacientes chagásicos fueron en general inferiores a los de los controles, presentando diferencias estadísticamente significativas en las pruebas de memoria, definiciones(vocabulario), relaciones lógicas y analogías. De éstas, solo una definición, no estuvo contaminada por el nivel de educación. Los resultados sugieren que la enfermedad de Chagas, en su estadio crónico, puede ser responsable de la baja performance vista en algunas de las pruebas cognitivas. Sin embargo, es necesario efectuar estudios más extensos observando con mayor cuidado la selección de los pacientes y el nivel de educación antes de aceptar definitivamente esta presunción
Subject(s)
Chagas Disease/complications , Cognition Disorders/etiology , Cognition Disorders/classification , Cognition Disorders/diagnosis , Memory Disorders/diagnosis , Memory Disorders/psychology , Chronic Disease , Concept Formation , Neuropsychological TestsABSTRACT
Con la finalidad de investigar la existencia de alteraciones clínicas en el examen neurológico de pacientes que solo mostraban positividad serológica para la enfermedad de Chagas se examinaron a 249 individuos. El examen clínico neurológico mostró que el 11,24% (n:28) tenía alguna anormalidad, observándose como alteración más frecuente hiporreflexia en el 82%, seguida por las alteraciones objetivas en el 49,50%, y subjetivas en el 24,50% de la sensibilidad, configurando en la mayoría de los pacientes un síndrome neuropático. Estos hallazgos señalan que la enfermedad de Chagas, en su estadio crónico, puede ser la causa de manifestaciones clínicas que indican compromiso del sistema nervioso periférico
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Chagas Disease/complications , Reflex, Abnormal , Chagas Disease/diagnosis , Peripheral Nervous System Diseases/etiology , Chronic Disease , Neurologic ManifestationsABSTRACT
A comprehensive investigation of nerve motor conduction velocity was carried out in the ulnar nerve of 70 patients in the chronic stage of Chagas disease. It was found that 2.6% of them, had slowness of the large motor axons at distal segments and 14% at proximal segments. Twenty seven percent of them had also slowness of conduction at the smallest motor fibers. These observations signal that some patients in the chronic stage of the infection may develop a motor neuropathy.
Subject(s)
Chagas Disease/diagnosis , Neural Conduction/physiology , Peripheral Nervous System Diseases/physiopathology , Ulnar Nerve/physiopathology , Adolescent , Adult , Chagas Disease/complications , Chagas Disease/physiopathology , Child , Chronic Disease , Electrophysiology , Female , Humans , Male , Middle Aged , Nerve Fibers/physiologyABSTRACT
Foi feita investigaçäo da velocidade de conduçäo motora no nervo ulnar de 70 pacientes em fase crônica da doença de Chagas. Foi verificado que 2,6% deles apresentavam lentificaçäo em grandes axônios motores em segmentos distais e 14% em segmentos proximais: 27% deles apresentavam também conduçäo lentificada em pequenas fibras motoras. Essas observaçöes indicam que alguns pacientes na fase crônica da doença podem desenvolver uma neuropatia motora
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Chagas Disease/diagnosis , Neural Conduction/physiology , Peripheral Nervous System Diseases/physiopathology , Ulnar Nerve/physiopathology , Chagas Disease/complications , Chagas Disease/physiopathology , Chronic Disease , Electrophysiology , Nerve Fibers/physiologyABSTRACT
Eleven patients with chronic lead intoxication were submitted to somatosensory evoked potential (SEP) studies. All patients demonstrated increased lead blood levels and reduced ALA D activity in red blood cells. Three patients showed delayed spinal arrival (N13 wave), four delayed cortical arrival (N20 wave), and three prolonged central conduction time (time elapsing between N13 and N20 waves) (see table 1). No relationship was found between the abnormal findings and the levels of lead or ALA D. Time of intoxication was not related to the altered electrophysiological features either. The findings reported suggest that, beside the well known peripheral involvement in chronic lead intoxication, some patients may develop central nervous system impairment perhaps related to myelin involvement as suggested by the prolonged SEP central conduction time.
Subject(s)
Evoked Potentials, Somatosensory , Lead Poisoning/physiopathology , Median Nerve/physiology , Neural Conduction , Adult , Aged , Aminolevulinic Acid/blood , Female , Humans , Lead Poisoning/blood , Male , Middle AgedABSTRACT
Once pacientes con cifras elevadas de plombemia y disminuidas de delta aminolevulínico dehidrasa (ALA D), fueron estudiados mediante la realización de potenciales evocados somatosensitivos obtenidos por estimulación del nervio mediano, midiendo el ingreso medular, el arribo cortical y el tiempo de conducción central (TCC). Los datos fueron comparados con controles. Se correlacionaron con las determinaciones bioquímicas y con el tiempo de exposición al tóxico. Por encima del limite superior control, se encontró el ingreso medular en tres pacientes, el arribo cortical en quatro y el TCC en tres. Los valores de N13,N20 y TCC no mostraron correlación con los parámetros bioquímicos ni con el tiempo de exposición al tóxico. Estos hallazgos sugieren que, si bien la neuropatía periférica condiciona el retraso del ingreso medular y del arribo cortical, es probable que exista también compromiso de la mielina central en algunos de los pacientes crónicamente expuestos
Subject(s)
Adult , Middle Aged , Humans , Male , Female , Evoked Potentials, Somatosensory , Lead Poisoning/physiopathology , Neural Conduction , Aminolevulinic Acid/blood , Lead/blood , Median Nerve/physiologyABSTRACT
An electrophysiological investigation of the state of the neuromuscular transmission (nmt) was carried out in 58 patients with the diagnosis of chronic Chagas' disease. On repetitive supramaximal nerve stimulation it was found that some patients did not show abnormalities, others had decremental muscle responses, others developed enhancement of the muscle evoked potential amplitudes, while some other patients combined both types of pathological responses. The findings suggest that some patients with chronic Chagas' disease develop impairment of nmt, though data obtained in this study do not give information about neither the type of impairment nor the localization (pre or postsynaptic, or both) of the damage.
Subject(s)
Chagas Disease/physiopathology , Neuromuscular Junction/physiopathology , Peripheral Nerves/physiopathology , Synaptic Transmission , Adult , Electric Stimulation , Electrophysiology , Evoked Potentials , Female , Humans , Male , Middle AgedABSTRACT
Thirty five patients with acute Chagas' disease who demonstrated parasitaemia at the time of the investigation were submitted to a detailed electromyographical study. With their muscles at rest, 12 patients showed fibrillation potentials and/or positive sharp waves. On volitional contraction, 7 had short duration motor unit potentials (MUPs) and low polyphasic MUPs. On motor and sensory nerve fibers conduction studies, 20 disclosed values below the lower control limit within one or more nerves. Finally, 12 patients produced a muscle decremental response on nerve supramaximal repetitive stimulation. The findings signal that primary muscle involvement, neuropathy and impairement of the neuromuscular transmission, either isolated or combined, may be found in the acute stage of human Chagas' disease.
Subject(s)
Chagas Disease/physiopathology , Motor Neurons/physiology , Peripheral Nerves/physiopathology , Acute Disease , Adolescent , Adult , Child , Child, Preschool , Electromyography , Female , Humans , Male , Neuromuscular Junction/physiopathology , Synaptic TransmissionABSTRACT
Seventy five patients with the diagnosis of chronic Chagas' disease were studied by employing EPs techniques. Two of them had delayed arrival of the signal to the Erb's point and one to the spinal cord when looking at SEPs. Two patients had increment of the time interval between waves Ist and IIIrd, when studying PEATs. These findings were interpreted as due to peripheral nerve fibers damage, a feature described in previous papers. The most striking finding was the prolonged time interval between waves N13 and N20 (SEPs) found in two patients and between waves IIIrd and Vth (PEAT) seen in 7 affected subjects. These observations suggested the development of some sort of CNS involvement, perhaps related to myelin damage, in patients who reached the chronic state of the infection.