Validation of the Pascual Graphomotor Test in Cuban school children.

INTRODUCTION: The ability to draw is a complex perception and cognition function, which is acquired in infancy and is not usually investigated in the neuropaediatric clinic. OBJECTIVE: To validate the Pascual graphomotor test (PGT) in 5 to 11 year-old Cuban school children. PATIENTS AND METHODS: The PGT was performed on a total of 172 children from the city of Havana Círculo Infantil del Municipio Plaza nursery school and from the 1st to 5th year of a primary school in the same area. The sample was systematic. The test was repeated the following day. All the drawings were scored blind by a neurologist and neurology resident. RESULTS: For the validation of the test the differentiation with age and school year was taken as a validation criterion. A high correlation was obtained between the ages of the children and the scores obtained. The Spearman coefficient was -0.78 (P=0.01), and a there was a similar inverse correlation between the school year and the test scores (Spearman coefficient=-0.79, P=0.01). The test was very reliable, with an intraclass correlation coefficient (ICC) of 0.99 for inter-observer agreement and 0.97 for the test-retest. CONCLUSIONS: The test was valid according to the criterion employed, differentiation with age and school year. The PGT demonstrated good temporal and inter-observer stability. We believe that it is a very useful tool in the neurological examination of Cuban school children.

[Myasthenia gravis in infancy. A report of 12 cases]. / Miastenia grave en la infancia. Presentación de 12 casos.

INTRODUCTION: Myasthenia gravis (MG) shows specific clinical features in children. It is essential to know this and also the use of diagnostic techniques used in infancy and childhood for correct diagnosis. OBJECTIVES: To analyze the clinical behaviour of this disorder and the use of complementary tests in the diagnosis of paediatric patients. PATIENTS AND METHODS: We studied a group of 12 children diagnosed as having MG, who were admitted to the Instituto de Neurolog a y Neurocirug a de Ciudad de La Habana (Cuba) between March 1997 and June 2001. Data were obtained from the clinical histories regarding the clinical picture, anticholinesterase test, repetitive stimulation test (RST), simple fibre test (SFT), computerized axial tomography (CAT) of the mediastinum and the treatment given in each case. RESULTS: Juvenile myasthenia gravis (JMG) presented in 91% of the patients studied. The average age of onset of JMG was 7.45 years, with no difference in presentation in the two sexes. The extrinsic muscles of the eye were most affected and the form with generalized clinical involvement predominated at the time of admission. The RST was positive in four of the nine patients in whom it was done (44%) and the SFT was positive in the six cases in which it was done. No changes were found in the mediastinum on CAT scanning. Mestinon and prednisone were the most commonly used drugs. CONCLUSION: In our group JMG was the most frequent form seen. Neurophysiological studies were very useful diagnostic tools.

[Mondini dysplasia: recurrent bacterial meningitis in adolescence]. / Displasia de Mondini: meningitis bacteriana recurrente en la adolescencia.

INTRODUCTION: Episodes of recurrent bacterial meningitis can occur in patients due to either congenital or acquired disorders. Congenital deformity of the bony labyrinth can be linked to a fistulous tract communicating it with the intracranial subarachnoid space. Mondini deformity is a frequent malformation in congenitally deaf patients. CASE REPORT: We report the case of an adolescent with a history of being unable to hear in one ear who, from the age of 10 years, began to suffer repeated bacterial meningoencephalitis with microbiological recovery of Streptococcus pneumoniae on three occasions. The type of germ recovered in the cerebrospinal fluid (CSF) and the history of congenital deafness that was detected when the patient was 3 years old were the diagnostic clues to the possible anomaly of the inner ear with a CSF fistula. The clinically proven CSF rhinorrhea contributed to the diagnosis of an ear anomaly with a fistula. Computerised axial tomography and magnetic resonance studies of the petrous portion of the temporal bone revealed the malformation that was later found and closed during the surgical intervention on the affected ear. The clinical absence of rhinorrhea, a year's progression without new infections after operating on the patient and post-surgery imaging studies were all proof that the fistula had closed. CONCLUSIONS: Mondini dysplasia with CSF fistula must be included as a possible diagnosis when faced with a patient with recurrent bacterial meningoencephalitis. Imaging studies, especially magnetic resonance, enable the clinician to check the diagnosis and the CSF fistula can be closed with ear surgery.

Esclerodermia lineal en golpe de sable y epilepsia. A propósito de un caso infantil / Linear scleroderma en coup de sabre and epilepsy: Presentation of a case in a child

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Validación del test grafomotor de Pascual en niños escolares cubanos / Validation of the Pascual Graphomotor Test in Cuban school children

Introducción: La capacidad para dibujar constituye una función practognósica compleja, que se adquiere en la infancia y que no suele explorarse asiduamente en la consulta neuropediátrica. Objetivo:Validar el test grafomotor (TGM) de Pascual en escolares cubanos de 5 a 11 años. Pacientes y métodos: Realizaron el TGM un total de 172 niños pertenecientes al preescolar de un Círculo Infantil del Municipio Plaza de Ciudad de la Habana y los que cursaban del primer al quinto grado en una escuela primaria de esta misma área. El muestreo fue sistemático. El test se repitió al siguiente día. Todos los dibujos fueron puntuados a ciegas por un neurólogo y una residente de neurología. Resultados: Para la validación del test se tomó como criterio de validación la diferenciación con la edad y el curso escolar. Se obtuvo una alta correlación entre las edades de los niños y las puntuaciones obtenidas (coeficiente de Spearman=−0,78; p<0,01) y similar correlación inversa entre el curso escolar y las calificaciones del test (coeficiente de Spearman=−0,79; p<0,01). El test resultó muy fiable, siendo el coeficiente de correlación intraclase (CCI) para la concordancia interobservador de 0,99 y para el test-retest de 0,97.Conclusiones: El test fue válido de acuerdo al criterio empleado de diferenciación con la edad y el curso escolar. El TGM demostró una gran estabilidad temporal e interobservador. Consideramos que es un instrumento muy útil en la exploración neurológica del niño en edad escolar en Cuba (AU)

Introduction: The ability to draw is a complex perception and cognition function, which is acquired in infancy and is not usually investigated in the neuropaediatric clinic. Objective: To validate the Pascual graphomotor test (PGT) in 5 to 11 year-old Cuban schoolchildren. Patients and methods: The PGT was performed on a total of 172 children from the city ofHavana Círculo Infantil del Municipio Plaza nursery school and from the 1st to 5th year ofa primary school in the same area. The sample was systematic. The test was repeated the following day. All the drawings were scored blind by a neurologist and neurology resident. Results: For the validation of the test the differentiation with age and school year was takenas a validation criterion. A high correlation was obtained between the ages of the children andthe scores obtained. The Spearman coefficient was −0.78 (P = 0.01), and a there was a similar inverse correlation between the school year and the test scores (Spearman coefficient =−0.79, P = 0.01). The test was very reliable, with an intraclass correlation coefficient (ICC) of 0.99 for inter-observer agreement and 0.97 for the test-retest. Conclusions: The test was valid according to the criterion employed, differentiation with ageand school year. The PGT demonstrated good temporal and inter-observer stability. We believethat it is a very useful tool in the neurological examination of Cuban school children (AU)

Displasia de Mondini: meningitis bacteriana recurrente en la adolescencia / Mondini dysplasia: recurrent bacterial meningitis in adolescence

Episodes of recurrent bacterial meningitis can occur in patients due to either congenital or acquired disorders. Congenital deformity of the bony labyrinth can be linked to a fistulous tract communicating it with the intracranial subarachnoid space. Mondini deformity is a frequent malformation in congenitally deaf patients. CASE . We report the case of an adolescent with a history of being unable to hear in one ear who, from the age of 10 years, began to suffer repeated bacterial meningoencephalitis with microbiological recovery of Streptococcus pneumoniae on three occasions. The type of germ recovered in the cerebrospinal fluid (CSF) and the history of congenital deafness that was detected when the patient was 3 years old were the diagnostic clues to the possible anomaly of the inner ear with a CSF fistula. The clinically proven CSF rhinorrhea contributed to the diagnosis of an ear anomaly with a fistula. Computerised axial tomography and magnetic resonance studies of the petrous portion of the temporal bone revealed the malformation that was later found and closed during the surgical intervention on the affected ear. The clinical absence of rhinorrhea, a year's progression without new infections after operating on the patient and post-surgery imaging studies were all proof that the fistula had closed...(AU)

Miastenia grave en la infancia. Presentación de 12 casos / Myasthenia gravis in infancy. A report of 12 cases

Introducción. La miastenia grave (MG) presenta particularidades clínicas en los niños. El conocimiento de éstas, además de la utilización de métodos diagnósticos en la infancia y la niñez, es de suma importancia para su identificación. Objetivos. Analizar el comportamiento clínico de la enfermedad y la utilidad de estudios complementarios para el diagnóstico en pacientes pediátricos. Pacientes y métodos. Estudiamos un grupo de 12 niños con diagnóstico de MG, ingresados en el Instituto de Neurología y Neurocirugía de Ciudad de La Habana (Cuba), entre marzo de 1997 y junio del 2001. Se recopilaron datos de las historias clínicas relacionados con: cuadro clínico, prueba con anticolinesterásicos, test de estimulación repetitiva (TER), test de fibra única (TFU), tomografía axial computarizada (TAC) de mediastino y tratamiento usado en cada caso. Resultados. La miastenia grave juvenil (MGJ) se presentó en el 91 por ciento de los pacientes estudiados. La edad promedio de inicio para la MGJ fue de 7,45 años, sin diferencia en su presentación en relación con el sexo. La musculatura extraocular resultó la más afectada y predominó la forma clínica generalizada en el momento del ingreso. El TER fue positivo en cuatro de los nueve pacientes en que se realizó (44 por ciento) y el TFU fue positivo en los seis casos realizados. No se comprobaron alteraciones en la TAC de mediastino. El mestinón y la prednisona fueron los medicamentos más usados. Conclusión. En nuestro grupo, la MGJ resultó la forma más frecuente, y los estudios neurofisiológicos constituyeron instrumentos diagnósticos de gran utilidad (AU)

Displasia de Mondini: meningitis bacteriana recurrente en la adolescencia / Mondini dysplasia: recurrent bacterial meningitis in adolescence

Introducción. Los episodios recurrentes de meningitis bacteriana pueden presentarse en pacientes por alteraciones congénitas o adquiridas. La deformidad congénita del laberinto óseo puede asociarse con un trayecto fistuloso entre éste y el espacio subaracnoideo intracraneal. La deformidad de Mondini es una malformación frecuente en pacientes con sordera congénita. Caso clínico. Adolescente con antecedente de no oír por un oído, que a partir de los 10 años de edad comenzó a presentar meningoencefalitis bacterianas de repetición, con aislamiento microbiológico en tres ocasiones de Streptococcus pneumoniae. El tipo de germen aislado en el líquido cefalorraquídeo (LCR) y el antecedente de sordera congénita que se detectó a los 3 años de edad fueron las pistas diagnósticas de la posible anomalía del oído interno con fístula de LCR. La rinorrea de LCR que se demostró clínicamente contribuyó al diagnóstico de anomalía del oído con fístula. Los estudios de tomografía axial computarizada y resonancia magnética del peñasco evidenciaron la malformación, que luego, en el acto operatorio del oído afectado, fue posible comprobar y cerrar. La ausencia clínica de rinorrea, la evolución sin nuevas infecciones un año después de operar a la paciente y los estudios posquirúrgicos con imágenes aportaron evidencias del cierre de la fístula. Conclusión. La displasia de Mondini con fístula de LCR debe ser una posibilidad diagnóstica ante un paciente con meningoencefalitis bacteriana de repetición. Los estudios de imágenes, sobre todo la resonancia magnética, permiten comprobar el diagnóstico, y la cirugía del oído permite el cierre de la fístula de LCR (AU)