Hereditary Melkersson-Rosenthal syndrome and multiple sclerosis.

UNLABELLED: The revision of MEDLINE from 1966 to 2003 did not report any association between multiple sclerosis (MS) and Melkersson-Rosenthal syndrome (MRS). This is a case report of a 51-year-old woman, with history of four recurrent Bell's palsies. In 1999 she developed a right facial paralysis due to a supranuclear pyramidal lesion with right monoparesis. The family history showed five relatives with recurrent Bell's paralysis and plicata tongue. PHYSICAL EXAMINATION: right Bell's paralysis, left supranuclear facial paralysis, furrowed tongue, right hemiparesis with pallor of the optic disks. Brain magnetic resonance imaging (MRI) demonstrated the typical lesions of MS and CSF oligoclonal bands. This is the first observation of a patient with hereditary MRS and MS. The link between both diseases is discussed.

[A severe episode in a patient with recurrent disseminated acute encephalitis due to vaccination against hepatitis B. For or against vaccination?]. / Brote grave en paciente con encefalitis aguda diseminada recurrente por vacuna contra LA hepatitis B: A favor o en contra de la Vacunación?

INTRODUCTION: Several reports of new cases of CNS demyelination or reactivation of MS after hepatitis B vaccination have raised the possibility of a causal relationship. Conversely, some authors have concluded that the risk of developing a demyelinating CNS event after a hepatitis B vaccination is unknown. PATIENTS AND METHODS: We have observed a 40 year old man, with diabetes mellitus (DM) type 1 and a previous history of recurrent acute demyelinating encephalomyelitis (rADEM). The patient had experienced three episodes of neurological dysfunction and he fulfilled the criteria for definite clinical MS but MRI showed demyelinating lesions in the pons and cerebellum without MRI criteria of MS. CSF analysis showed oligoclonal banding. The patient had been clinically stable during the last 6 years. Yearly MRI during this period had not shown any new disease activity. He was admitted in our MS Clinic due to dizziness, nausea, vomiting and diplopia, 6 weeks after the first of the two injections for hepatitis B vaccine after participating in the national programme of vaccination in DM type 1 patients. Clinical examination showed intranuclear ophtalmoplegia, visual loss in the left eye and worsening of the previous cerebellar and pyramidal signs. MRI showed an increase in the old lesions with high intensity signals on T2 weighted sequences with post gadolinium enhancement on T1 weighted sequences located in the brainstem and mesencephalon. The patient s diabetes mellitus deteriorated with ketoacidosis that needed increased doses of insulin. His condition worsened and he developed partial motor seizures. He improved 15 days later but he still had involvement of the cerebellar and pyramidal systems and occasional dizziness. CONCLUSIONS: As pointed out by some authors and in view of this observation, it would seem reasonable, as a precautionary measure, to avoid hepatitis B vaccination in patients with a personal or family history of symptoms suggestive of a demyelinating disease of the CNS.

Brote grave en paciente con encefalitis aguda diseminada recurrente por vacuna contra la hepatitis B.¿A favor o en contra de la vacunación? / A severe episode in a patient with recurrent disseminated acute encephalitis due to vaccination against hepatitis b. For or against vaccination?

Introducción. Varios estudios han informado de casos nuevos de desmielinización del SNC o de reactivación de la esclerosis múltiple (EM) después de una vacunación contra la hepatitis B (HB). A la inversa, algunos autores han llegado a la conclusión de que se desconoce el riesgo de desarrollar un fenómeno de desmielinización del SNC tras una vacunación HB. Pacientes y métodos. Hemos observado el caso de un hombre de 40 años de edad con diabetes mellitus (DM) del tipo I e historia previa de encefalomielitis con desmielinización aguda recurrente (EDA-r). Había experimentado tres episodios de disfunción neurológica y cumplido los criterios de EM clínicamente definida, pero la RM mostró lesiones en el tallo cerebral y en el cerebelo sin criterio de EM por RM. El análisis del LCR mostró bandas oligoclonales. Había permanecido estable clínicamente durante los últimos seis años, sin mostrar actividad alguna de la enfermedad. Ingresó en clínica por EM porque padecía mareos, náuseas, vómitos y diplopía, seis semanas después de las dos primeras inyecciones contra la hepatitis, dentro del programa nacional de vacunación de los pacientes de DM del tipo I. El examen clínico mostró oftalmoplejía intranuclear, pérdida de visión del ojo izquierdo y afectación de los signos cerebelares y piramidales previos. La RM mostró un aumento de las viejas lesiones, con señales de alta intensidad en las secuencias de imágenes en T2 y aumento de posgadolinio en los sitios de las secuencias de imágenes en T1 del tronco cerebral y el mesencéfalo. La DM se deterioró con cetoacidosis, por lo que se aumentaron las dosis de insulina. Empeoró y desarrolló ataques convulsivos motores parciales. A los 15 días mejoró, pero todavía sufría afectación de los sistemas cerebelares y piramidales, y mareos ocasionales. Conclusiones. Parece razonable, como medida de precaución, evitar la vacunación HB en pacientes con historia personal o familiar de síntomas sugestivos de una enfermedad desmielinizante del SNC (AU)