The use of corticosteroids in cryptogenic palsy of the third cranial nerves. A case report
Introduction. In spite of the rarity of isolated paralysis of the third cranial nerves in infancy, we present an acquired cryptogenic case which had an excellent, rapid response to treatment with corticosteroids started three months after onset of the condition. Clinical case. A girl aged three and a half years, with no significant past clinical history, presented with a third cranial nerve syndrome of sudden onset, with divergent squint and right ptosis, but no changes in pupil reactivity. All the complementary tests done were normal. After three months of observation, since the condition persisted, treatment was started with oral prednisone 2 mg/kg/day for three months. The ptosis disappeared in two weeks, and two years after treatment was stopped has not reappeared. The absence of pain would suggest that this was not a Tolosa-Hunt syndrome, although pain-free episodes have been described. The absence of alteration in pupil reactions is against, although does not exclude, compression of the third cranial nerve. This finding, together with the spectacular response to corticosteroids would support the diagnosis of inflammation limited to the motor fibres, probably due to a viral infection. Conclusion. We conclude that corticosteroids may be considered in the treatment of cryptogenic cranial neuropathy, once other possible aetiologies have been excluded, with the object of modulation of the theoretical immune mechanisms involved.
Caso clínico Se trata de una niña de tres años y tres meses de edad sin antecedentes de interés, que de forma súbita presentó un síndrome de III par craneal parcial, con estrabismo divergente y ptosis derecha, sin alteraciones de la pupilomotricidad. Todas las pruebas complementarias realizadas fueron normales. Tras cinco meses de conducta expectante, y ante la persistencia de la clínica, se instaura tratamiento con prednisona oral 2 mg/kg/día (tres meses), con desaparición total de la ptosis en dos semanas, que no ha reaparecido tras dos años desde la retirada del tratamiento. La ausencia de dolor no apoya un síndrome de TolosaHunt, aunque se ha descrito la existencia de episodios sin dolor. La ausencia de alteraciones de la motilidad pupilar va en contra, aunque no excluye, un III par por compresión. Este dato, junto a la espectacular respuesta corticoidea, apoya un problema inflamatorio limitado a las fibras motoras, probablemente en relación con una infección viral.
Conclusión Concluimos que la corticoterapia podría considerarse como posibilidad terapéutica ante neuropatías craneales criptogénicas, una vez excluidas otras etiologías posibles, con objeto de modular un hipotético mecanismo inmune implicado