Brain-stem auditory evoked potentials and brain death.

BAEP records were obtained from 30 brain-dead patients. Three BAEP patterns were observed: (1) no identifiable waves (73.34%), (2) an isolated bilateral wave I (16.66%), and (3) an isolated unilateral wave I (10%). When wave I was present, it was always significantly delayed. Significant augmentation of wave I amplitude was present bilaterally in one case and unilaterally in another. On the other hand, in serial records from 3 cases wave I latency tended to increase progressively until this component disappeared. During the same period, wave I amplitude fluctuations were observed. A significant negative correlation was found for wave I latency with heart rate and body temperature in 1 case. Two facts might explain the progressive delay and disappearance of wave I in brain-dead patients: a progressive hypoxic-ischaemic dysfunction of the cochlea and the eighth nerve plus hypothermia, often present in brain-dead patients. Then the incidence of wave I preservation reported by different authors in single BAEP records from brain-dead patients might depend on the moment at which the evoked potential study was done in relation to the onset of the clinical state. It is suggested that, although BAEPs provide an objective electrophysiological assessment of brain-stem function, essential for BD diagnosis, this technique could be of no value for this purpose when used in isolation.

Diagnostico y tratamiento de la enfermedad de Friedreich. / Diagnosis and treatment of Friedreich's disease

Se analiza el cuadro clinico de la enfermedad de Friedreich, la anatomia patologica, la patogenia, las investigaciones complementarias de diagnostico, el diagnostico diferencial y algunas consideraciones sobre el tratamiento. Se hace una clasificacion de las enfermedades heredodegenerativas espinocerebelosas

Diagnostico y tratamiento de la corea de Huntington. / Diagnosis and treatment of Huntington's chorea

Se analiza el cuadro clinico de la corea de Huntington, las investigaciones complementarias de diagnostico, el diagnostico diferencial, y se actualiza el tratamiento desde un ponto de vista neurobioquimico

Diagnostico y tratamiento de la corea de Huntington. / Diagnosis and treatent of Huntington's chorea

Se analiza el cuadro clinico de la corea de Huntington, las investigaciones complementarias de diagnostico, el diagnostico diferencial, y se actualiza el tratamiento desde un ponto de vista neurobioquimico

Degeneración hapatolenticular (enfermedad de Wilson). Estudio de 7 casos / Hepatolenticular degeneration (Wilson's disease). A report of seven cases

Se realiza un estudio de siete pacientes con el diagnóstico de degeneración hepatolenticular (enfermedad de Wilson). La edad de comienzo de los síntomas en todos los casos siempre fue por debajo de los 30 años. En 5 de los pacientes se recogen antecedentes familiares de enfermedad similar y en dos de ellos había consanguinidad entre los padres. El síntoma inicial más frecuente fue el temblor, en tres casos. Se encontraron alteraciones del sistema extrapiramidal en todos los casos y el patognomónico anillo corneal de Kayser-Fleischer en 6 casos. En tres casos se detectan signos y síntomas de hepatopatía. La inteligencia estaba disminuida en tre casos. Los valores de la ceruloplasmina estuvieron por debajo de los 10 mg en 5 casos(AU)

[Primary amebic meningoencephalitis. Report of a suspicious case and review of the medical literature]. / Meningoencefalitis amebiana primaria. Estudio de un caso sospechoso y revisión de la literatura médica.

The case of a 24-year old patient who, in July 1976, submerged into a canal of Guanimar river and, five days later, had a febrile meningoencephalitic clinical picture, is presented; he was in a very severe condition and recovered without sequelae with a treatment based on amphotericin B. The negative cultures for bacteria, fungi and viruses, the characteristics of cerebrospinal fluid and the observation of free-life amebae in the water of the wash of rhinopharynx and pharingeal cavity allowed to establish, with great accuracy, the presuntive diagnosis of primary amebic meningoencephalitis.