RESUMO
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.
Assuntos
Síndrome de Melkersson-Rosenthal/complicações , Síndrome de Melkersson-Rosenthal/genética , Esclerose Múltipla Recidivante-Remitente/complicações , Esclerose Múltipla Recidivante-Remitente/genética , Feminino , Humanos , Pessoa de Meia-Idade , LinhagemRESUMO
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.