Síndrome de Miller Fisher, un desafío diagnóstico de oftalmoplejía: reporte de un caso / Miller Fisher syndrome, a diagnostic challenge of ophthalmoplegia: a case report

El síndrome de Guillain-Barré (SGB), y sus derivados, entre ellos el síndrome de Miller Fisher (SMF); junto a otras patologías de origen neurológico como la Polineuropatía desmielinizante inflamatoria crónica (CIDP), las polineuropatías de causa metabólica, miastenia gravis, esclerosis lateral amiotrófica (ELA), síndrome de Lambert-Eaton, encefalopatía de Wernicke entre otras; presentan signos y síntomas neurológicos de presentación común. De este modo, la importancia del examen neurológico acabado; y los exámenes de apoyo diagnóstico como: laboratorio -destacando el líquido cefalorraquídeo (LCR)-, electromiografía, y toma de imágenes, son cruciales para esclarecer el diagnóstico. Así, es posible ofrecer un tratamiento de forma precoz, basado en la evidencia, y con el objetivo de disminuir la letalidad de la enfermedad. En el presente texto se plasma un subgrupo de patología de SGB, el SMF, el cual posee una incidencia significativamente baja, una clínica característica, y un pronóstico bastante ominoso sin un tratamiento adecuado. En el presente texto se plasma el reporte de un caso abordado en el Hospital San Pablo de Coquimbo, Chile.

Guillain-Barré syndrome (GBS) and its derivatives, including Miller Fisher syndrome (MFS), along others pathologies of neurological origin such as chronic inflammatory demyelinating polyneuropathy (CIDP), metabolic polyneuropathies, myasthenia gravis, amyotrophic lateral sclerosis (ALS), Lambert-Eaton syndrome, Wernicke's encephalopathy and well as others, have common neurological signs and symptoms. In this way, the importance of a thorough neurological examination, and supporting diagnostic tests such as: laboratory, -cerebrospinal fluid (CSF)-electromyography, and imaging, are crucial to clarify the diagnosis. Thus, it is possible to offer early, evidence-based treatment with an aim of reducing the disease's lethality. In the text below we present a subgroup of GBS pathology, MFS, which has a significantly low incidence, a characteristic clinical picture, and a rather ominous prognosis without adequate treatment. In the following text/paper is shown the report of a case approached in San Pablo Hospital, from Coquimbo, Chile.

Sindrome de Guillain-Barre variante axonal en pediatria: reporte de caso / Guillain-Barre Syndrome axonal variant in pediatrics: case report

RESUMEN El síndrome de Guillain-Barré es una polirradiculoneuropatía inflamatoria aguda, caracterizada por debilidad simétrica de carácter progresivo, de inicio distal asociado con arreflexia y síntomas sensitivos leves. La variante NMAA es una entidad poco frecuente en América Latina. Se reporta el caso de un paciente de sexo masculino, de seis años, previamente sano, con diagnóstico de síndrome de Guillain-Barré variante axonal, secundario a cuadro de vías aéreas superiores. La importancia del reporte radica en informar al pediatra y a otros profesionales de la salud acerca de la existencia de esta entidad y así aumentar la sospecha diagnóstica, considerando su baja incidencia. Se destaca también la posibilidad de encontrar variabilidad en las formas clínicas típicas de presentación, como lo fue el caso presentado, ya que es un subtipo de la enfermedad que está clásicamente caracterizada como grave, de evolución tórpida y con frecuentes secuelas.

SUMMARY Guillain-Barre syndrome is an acute inflammatory polyradiculoneuropathy characterized by progressive symmetric weakness of distal onset associated with areflexia and mild sensory symptoms. The AMAN variant is a rare entity in Latin America. The case of a 6-year-old male patient, previously healthy, with a diagnosis of Guillain-Barre Syndrome, axonal variant, secondary to upper airway symptoms, is reported. The importance of the report lies in informing the pediatrician and other health professionals about the existence of this entity and thus increasing the diagnostic suspicion considering its low incidence. We also highlight the possibility of finding variability in the typical clinical forms of presentation, as was the case presented, since it is a subtype of the disease that is classically characterized as severe and with torpid evolution. It is also associated with frequent sequelae.

Guillain-Barré Syndrome in a Patient with Sickle Cell Anemia.

Description A 24-year-old African American male with a history of sickle cell anemia (Hb S/S) presented to an outside hospital with acute colitis, acute renal failure and sickle cell crisis and was treated with supportive measures. On day 3 of hospitalization, he developed bilateral ascending paralysis with sacral numbness. Magnetic resonance imaging (MRI) demonstrated epidural lipomatosis, which was attributed as the cause of his paralysis. He was transferred to our facility for neurosurgery evaluation. Based on the physical examination, Guillain-Barré Syndrome (GBS) was suspected. This conclusion lead to a lumbar puncture with cerebrospinal fluid (CSF) analysis that confirmed the diagnosis. He was then treated with intravenous immunoglobulin (IVIg), which resolved his symptoms. We present this case to highlight the importance of a physical exam rather than relying heavily on imaging studies. Physical exam findings lead to a diagnosis, which was then confirmed with appropriate testing.

Epidemiology of Guillain-Barré syndrome in Finland 2004-2014.

At total mean incidence of 0.84-1.1/100,000 the occurrence of Guillain-Barré syndrome (GBS) is reported to be low in Finland compared to other Caucasian populations. However, a recent study from Southwestern Finland reported an incidence of 1.82/100,000 which is comparable to other Caucasian populations. We analyzed discharge data covering the years 2004 through 2014 on all neurological admissions in all Finnish university and central hospitals with a primary diagnosis of GBS. A total of 989 admissions due to GBS (917 individuals) were identified. The standardized (European population) annual incidence rate was 1.70/100,000 person-years (95% confidence interval 1.60-1.81). GBS incidence had an increasing trend with age. The likelihood of GBS was higher among girls and adolescent women than boys and men of same age (male:female incidence rate ratio [IRR] 0.56), while in the older age groups (>19 years) the occurrence of GBS was higher among males than females (male:female IRR 1.59). The incidence of GBS remained stable during the study period. There was no seasonal variation in GBS admission frequencies (p = 0.28). No significant effect of the 2009-2010 H1N1 influenza or vaccination against it for GBS occurrence was observed. We suggest that GBS is as common, and has similar age-distribution in Finland as in other European countries. Sex-associated susceptibility for GBS appears to be different in children-adolescents and adults.