[Multiple mononeuritis and eosinophilic fasciitis in a patient with idiopathic hypereosinophilic syndrome]. / Mononeuritis múltiple y fascitis eosinofílica en una paciente con síndrome hipereosinofílico idiopático.

INTRODUCTION: Hypereosinophilic syndrome is produced by what is usually a multiple infiltration of eosinophils into tissues, and may be secondary or idiopathic, depending on whether it is related to a specific aetiology or not. It is not uncommon for it to include nerve disease, but it is unusual for it to do so in the form of multineuritis. Exceptionally, pathogenesis into multiple mononeuritis appears to be related with neurotoxicity due to products derived from eosinophils rather than with infiltrating or inflammatory phenomena. This study describes the case of a female patient with hypereosinophilic syndrome with no verifiable cause, multineuritis and eosinophilic fasciitis. CASE REPORT: A 30-year-old female with no relevant history who visited because of some painless inguinal nodules that had appeared several weeks before. At almost the same time, she presented painful sensitive symptoms in her legs with a significant functional incapacity. An important degree of hypereosinophilia, eosinophilic fasciitis and non-neoplastic eosinophilic infiltration of the bone marrow was found, together with multiple mononeuritis. Treatment with oral corticoids improved the dermatological and haematological clinical features, and associating it with gabapentin improved the neuropathic symptoms. CONCLUSIONS: The patient, in accordance with current criteria, presented idiopathic hypereosinophilic syndrome with an undetermined subtype. To our knowledge, the association with eosinophilic fasciitis and multineuritis has not been reported to date. There is no proven infiltrating mechanism in multiple mononeuritis, which corroborates the poor control of the neuropathic clinical symptoms with oral corticoid therapy. Association with gabapentin, which stabilises the axonal membrane, also backs up the neurotoxic pathogenetic hypothesis.

Mononeuritis múltiple en un paciente con síndrome de Churg-Strauss. Pseudobloqueos como expresión electroclínica temprana / Multiple mononeuritis in a patient with Churg-Strauss syndrome. Pseudo conduction block as an early electroclinical expression

Introducción. La mononeuritis múltiple es una de las manifestaciones más frecuentes del síndrome de Churg-Strauss. Caso clínico. En un varón de 53 años con síndrome de Churg-Strauss, se describe una mononeuritis múltiple aguda sensitivomotora de las cuatro extremidades. Mediante electrodiagnóstico en los primeros siete días se demostraron bloqueos de conducción en varios nervios. Los estudios posteriores objetivaron una polineuropatía axonal sensitivomotora difusa y bilateral con asimetrías, pero sin bloqueos. La electrofisiología y la histología, así como los estudios experimentales, indicaban que se trataba de una axonopatía secundaria a isquemia aguda. Conclusiones. Sólo en pocos casos, como en éste, y en estudios muy incipientes se han descrito bloqueos de conducción que, por su relación habitual con desmielinización, provocan confusión diagnóstica y pronóstica. La evolución suele demostrar una axonopatía por degeneración walleriana. Por tanto, en estos casos parece recomendable extremar la cautela y realizar estudios neurofisiológicos en las siguientes semanas para evitar inadecuados juicios diagnósticos y pronósticos (AU)

Introduction. Multiple mononeuritis is one of the most frequent manifestations of Churg-Strauss syndrome. Case report. We describe a case of acute sensory-motor multiple mononeuritis in all four limbs of a 53-year-old male with Churg-Strauss syndrome. Electrodiagnosis performed within the first seven days revealed conduction blocks in several nerves. Follow-up studies showed diffuse bilateral sensory-motor axonal polyneuropathy with symmetries, but without blocks. Electrophysiological and histological analyses, together with experimental studies, showed it to be an axonopathy secondary to acute ischaemia. Conclusions. In only a few cases, such as this one and in a small number of incipient studies, have there been reports of conduction blocks that, owing to the fact that they are commonly related with demyelination, give rise to confusion when it comes to diagnoses and prognoses. The condition usually displays axonopathy due to Wallerian degeneration. It therefore seems recommendable in these cases to proceed with caution and to perform neurophysiological studies in the following weeks in order to prevent inappropriate diagnostic and prognostic judgements from being formed (AU)

[Multiple mononeuritis in a patient with Churg-Strauss syndrome. Pseudo conduction block as an early electroclinical expression]. / Mononeuritis múltiple en un paciente con síndrome de Churg-Strauss. Pseudobloqueos como expresión electroclínica temprana.

INTRODUCTION: Multiple mononeuritis is one of the most frequent manifestations of Churg-Strauss syndrome. CASE REPORT: We describe a case of acute sensory-motor multiple mononeuritis in all four limbs of a 53-year-old male with Churg-Strauss syndrome. Electrodiagnosis performed within the first seven days revealed conduction blocks in several nerves. Follow-up studies showed diffuse bilateral sensory-motor axonal polyneuropathy with symmetries, but without blocks. Electrophysiological and histological analyses, together with experimental studies, showed it to be an axonopathy secondary to acute ischaemia. CONCLUSIONS: In only a few cases, such as this one and in a small number of incipient studies, have there been reports of conduction blocks that, owing to the fact that they are commonly related with demyelination, give rise to confusion when it comes to diagnoses and prognoses. The condition usually displays axonopathy due to Wallerian degeneration. It therefore seems recommendable in these cases to proceed with caution and to perform neurophysiological studies in the following weeks in order to prevent inappropriate diagnostic and prognostic judgements from being formed.

Varón de 48 años con inflamación subcutánea y eosinofilia / No disponible

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