ESCLEROSIS LATERAL AMIOTRÓFICA
La imagen de Stephen Hawking postrado en un silla de ruedas es la primera que se viene a la cabeza cuando se piensa en la esclerosis lateral amiotrófica (ELA), un trastorno neurodegenerativo que provoca que los músculos se atrofien.
No ocurre lo mismo con la inteligencia. De ahí que Hawking sea famoso por su contribución a la astrofísica. De hecho, su perfil público como conferenciante es ampliamente conocido. Habla por medio de un sintetizador de voz. Y es que la ELA “afecta a la musculatura periférica, esofágica y respiratoria”, explica Emilia Barrot, jefa del Servicio de Neumología del Hospital Virgen del Rocío, de Sevilla.
A Hawking le diagnosticaron esta enfermedad con 21 años, en 1963, cuando ante la aparición de los primeros síntomas de torpeza motora e instado por su madre acudió a la consulta. Por aquel entonces le dieron dos años de vida. De cumplirse el pronóstico no podría acabar su doctorado en Oxford. Por suerte, esto no sucedió. Ahora bien, su caso se complicaría en 1985, fecha en la que encontrándose en el CERN (el gran acelerador de partículas de Ginebra), Hawking tendría que ingresar de urgencia al sufrir una neumonía.
Debilidad gradual
En la ELA, las células nerviosas se desgastan o mueren y dejan de enviar mensajes a los músculos, lo que conduce a un debilitamiento muscular progresivo, fasciculaciones y dificultades para mover los brazos, las piernas y el cuerpo.
Los mensajes de las neuronas motoras cerebrales (las llamadas superiores) son transmitidos a las neuronas motoras de la médula espinal (las inferiores) y de allí a cada músculo. La alteración de este vínculo es el causante de esta incapacidad para controlar el movimiento voluntario. “La primera señal de alarma es un déficit motor”, subraya en este punto Barrot.
En adelante, “la sospecha se confirma con una electromiograma”, continúa. La enfermedad empeora gradualmente y cuando fallan los músculos del diafragama y del área torácica respirar por uno mismo se torna difícil, por lo que los pacientes se ven obligados a recurrir a un respirador artificial. Y he aquí la principal amenaza para la superviviencia. La mayoría de las personas con ELA fallecen por un fallo respiratorio entre los primeros tres y cinco años desde que ésta se desencadena, aunque cerca del 10 por ciento, entre quienes se incluye Hawking, sobreviven diez años o más.
Dependencia y fatalidad
En cualquier caso, ya sea a corto o medio plazo, “el resultado es la parálisis completa y el curso es fatal”, señala Barrot. Poco a poco “la persona se convierte en un ser absolutamente dependiente para realizar cualquiera de sus actividades cotidianas y, además de un ventilador para oxigenarse, necesitará una sonda para comer y algún tipo de vehículo para desplazarse”.
En este sentido, “existen dos cartas de presentación típicas. La primera impacta contra el sistema motor y la segunda forma clínica sería la bulbar. En las formas completas, además de afectación muscular, el paciente presenta problemas de inervación facial, con manifiestas dificultades para la masticación y la deglución, así como para expresarse. Hawking resuelve este aspecto con una aplicación informática conectada a su silla, que incluye un catálogo de palabras que el científico selecciona por medio de dos botones que acciona con su mano izquierda y a través del movimiento de la cabeza o los ojos.
Otro de los signos más visibles es la sonrisa sardónica, una facie descrita como una cara de terror sonriente. Esta curiosa expresión facial, que acompaña la fisionomía de Hawking, se debe a una contracción de los maseteros que produce lo que se denomina trismus e impide abir la boca. Silbar o besar son otras tareas casi imposibles.
Así las cosas, ¿qué puede hacerse frente a esta evolución cuesta arriba? “Existe una forma hereditaria pero es minoritaria, por lo que la mayoría de casos cursan de forma esporádica sin causa conocida. Se ha estudiado su asociación con el exceso de ejercicio y con ciertos tóxicos infecciosos; sin embargo, no se ha constatado asociación alguna, al menos no directa, motivo por el que no disponemos de tratamiento curativo”. Para paliar el daño neuronal está el riluzole, que frena la liberación de glutamato.
MANIFESTACIÓN CLÍNICA
La ELA, que se da en entre dos y cuatro personas por cada 100.000 habitantes, trae consigo una pérdida de la fuerza muscular y la coordinación que lleva a dificultades para levantar objetos, caminar, alimentarse, hablar y hasta para respirar. La cabeza tiende a mantenerse baja y el patrón de discurso, de haberlo, suele ser pausado, mientras que la voz se torna ronca. El paciente puede sufrir de náuseas, ahogarse con facilidad, babear y presentar contracturas y espamos.
CON VISOS DE FUTURO
“Que ningún enfermo sea tratado fuera de un equipo multidisciplinar”. Este es el principal reto al que se enfrenta la ELA. “Y, sobre todo, que dentro de estas unidades comienza a incluirse el apoyo psicológico”, destaca Barroto. Y es que, dado que la función intelectual no resulta perjudicada, el apoyo emocional es vital para encarar este trastorno. Por otra parte, “habría que incidir en la necesidad de un diagnóstico precoz y avanzar en el terreno del tratamiento. Existen actualmente ensayos clínicos a nivel nacional con fármacos como el olesoxime, un modulador del poro mitocondrial.
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