[Peripheral neuropathy in systemic lupus erythematosus]. / Neuropatía periférica en el lupus eritematoso sistémico.

INTRODUCTION: Systemic lupus erythematosus (SLE) is the connective tissue disorder in which neuropsychiatric complications are most often seen. The prevalence of peripheral neuropathy varies from 5 to 27% depending on the series of cases described, with subclinical forms being most frequent. OBJECTIVES: To determine the prevalence of subclinical peripheral neuropathy in a group of patients with SLE, define their characteristics, and investigate any possible associations with clinico analytical parameters of the disease. PATIENTS AND METHODS. 32 patients with SLE were diagnosed in an Internal Medicine Outpatient Clinic and followed up for at least a year. They were studied prospectively and consecutively by clinical examination, laboratory test and neurophysiological studies (electroneurogram). RESULTS: Neurophysiological study showed peripheral neuropathy to be present in 50% of the patients, who were mainly asymptomatic (75%) and found to be normal on neurological examination (81.2%). The most frequent type of neuropathy was polyneuropathy (37.5%) which was predominantly axonal symmetrical and sensomotor followed by isolated mono neuropathy (6.2%) and multiple mononeuritis (6.2%). No clinical or analytical parameter was significantly associated with the development of peripheral neuropathy. CONCLUSIONS: The considerable frequency of subclinical peripheral neuropathy in patients with SLE and the absence of associated clinical or analytical parameters makes it necessary to carry out neurophysiological studies in these patients to detect its presence and establish the precise extent of the disorder.

Neuropatía periférica en el lupus eritematoso sistémico / Peripheral neuropathy in systemic lupus erythematosus

El lupus eritematoso sistémico (LES) es la enfermedad del tejido conectivo que con mayor frecuencia presenta complicaciones neuropsiquiátricas. La prevalencia de neuropatía periférica varía de un 5 a un 27 por ciento según las series, siendo más frecuentes las formas subclínicas. Objetivos. Determinar la prevalencia de neuropatía periférica subclínica en un grupo de pacientes con LES, definir sus características e investigar las posibles asociaciones existentes con parámetros clinicoanalíticos de la enfermedad. Pacientes y métodos. Se han diagnosticado 32 pacientes con LES en una consulta externa del Servicio de Medicina Interna, con seguimiento en la misma durante al menos un año; fueron estudiados de forma prospectiva y consecutiva mediante examen clínico, test de laboratorio y estudio neurofisiológico (electroneurograma). Resultados. El estudio neurofisiológico demostró neuropatía periférica en un 50 por ciento de los pacientes, en su mayoría asintomáticos (75 por ciento) y con exploración neurológica normal (81,2 por ciento). El tipo de neuropatía más frecuente fue la polineuropatía (37,5 por ciento) de predominio sensitivomotor simétrica axonal, seguida de la mononeuropatía aislada (6,2 por ciento) y la mononeuritis múltiple (6,2 por ciento). Ningún parámetro clínico o analítico se asoció significativamente con el desarrollo de neuropatía periférica. Conclusiones. La elevada frecuencia de neuropatía periférica subclínica en los pacientes con LES y la ausencia de parámetros clínicos o analíticos asociados a la misma hace necesaria la práctica de un estudio neurofisiológico en estos pacientes para detectar su presencia, y establecer con exactitud la extensión de la enfermedad (AU)

[Exophthalmos and cranial neuropathy as a form of presentation of Wegener's granulomatosis]. / Exoftalmos y neuropatía craneal como forma de presentación de la granulomatosis de Wegener.

The cases of three patients with Wegener's granulomatosis presenting as exophthalmos and cranial neuropathy are reported. In the first patient's symptoms were secondary to the presence of a retro-orbital mass. In the second and third patients there was dissemination of neighboring granulomatous processes. Paranasal sinuses were involved in two patients, but clinical manifestations were evident in only one. Full remission was achieved in all three. Two patients had been received immunosuppressant therapy for inflammatory processes evaluated a posteriori in the context of Wegener's granulomatosis. The immunosuppressant treatment seems to have conditioned this unusual clinical presentation.

[Biopercular lesion with inverse dissociation]. / Lesión biopercular con disociación inversa.

Foix-Chavany-Marie Syndrome or anterior opercular syndrome constitutes the cortical form of pseudobulbar palsy. Its most common etiology is stroke in the region of either operculum. Clinically it can be characterized by severe dysarthria and facio-pharyngo-glosso-masticatory dysplasia with automatic-voluntary dissociation. An inversion of this classical dissociation can be seen in patients with emotional paralysis in the face, in whom asymmetry of emotional expression but conservation of voluntary movement can be seen. The lesional topography of this inverse dissociation corresponds to the supplementary motor area. both processes support the existence of an anatomical base which is different for emotional movements and voluntary types. We describe the clinical case of a 65 year old woman with a history of breast cancer who presented a subacute and progressive clinical picture of dysphagia and severe dysarthria. Upon neurological examination slight velo-palato-pharyngea was noticed; most relevant was paralysis of the facial muscles for either emotional or automatic movements while voluntary innervation was maintained. Neuroradiological studies show the presence of brain metastasis located in both Roland opercula. Although the neuroanatomic bases for automatic-voluntary dissociation remain unclear, our case lends weight to the evidence that impulses for voluntary and emotional movements originate in different cortical areas or else take a different route through the brain. We have been unable to find any cases of biopercular lesion with inverse dissociation described in the literature we reviewed.