ABSTRACT
OBJECTIVE: Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), and Bickerstaff brainstem encephalitis (BBE) are usually monophasic, but some patients experience recurrences after long asymptomatic intervals. We aimed to investigate clinical features of recurrent GBS, MFS, and BBE at a single hospital. METHODS: Records from 97 consecutive patients with GBS, MFS or BBE who were admitted to a tertiary hospital between 2001 and 2013 were reviewed. Clinical and laboratory features of patients with recurrent GBS, MFS, or BBE were investigated. RESULTS: Patients included 55 (32 males) with GBS, 34 (22 males) with MFS, and 8 (6 males) with BBE. Recurrent cases occurred in 2 (4%) of the 55 patients with GBS, 4 (12%) of the 34 patients with MFS, and 2 (25%) of the 8 patients with BBE. Patients with recurrent MFS had a tendency to be younger at the first episode than patients with non-recurrent MFS (median, 22 versus 37years old). Symptoms and signs were less severe during relapses than during the initial episode in recurrent patients. CONCLUSIONS: Recurrences occurred more frequently in patients with MFS or BBE compared with those with GBS. Patients with recurrent MFS might be younger than those with non-recurrent MFS.
Subject(s)
Brain Stem/pathology , Classical Lissencephalies and Subcortical Band Heterotopias , Encephalitis , Guillain-Barre Syndrome , Adult , Antibodies/blood , Antigens, CD/immunology , Classical Lissencephalies and Subcortical Band Heterotopias/epidemiology , Classical Lissencephalies and Subcortical Band Heterotopias/metabolism , Classical Lissencephalies and Subcortical Band Heterotopias/physiopathology , Encephalitis/epidemiology , Encephalitis/metabolism , Encephalitis/physiopathology , Female , Gangliosides/immunology , Guillain-Barre Syndrome/epidemiology , Guillain-Barre Syndrome/metabolism , Guillain-Barre Syndrome/physiopathology , Humans , Male , Middle Aged , Recurrence , Retrospective Studies , Young AdultSubject(s)
Classical Lissencephalies and Subcortical Band Heterotopias/genetics , Classical Lissencephalies and Subcortical Band Heterotopias/physiopathology , Lissencephaly/genetics , Lissencephaly/physiopathology , Agenesis of Corpus Callosum , Cerebellum/abnormalities , Chromosome Deletion , Chromosomes, Human, Pair 17/genetics , Classical Lissencephalies and Subcortical Band Heterotopias/epidemiology , Corpus Callosum/physiopathology , Female , Gastroesophageal Reflux/epidemiology , Gene Expression , Humans , Lissencephaly/epidemiology , Male , Point Mutation/geneticsABSTRACT
We describe a child with epilepsy associated with double-cortex syndrome in whom vagus nerve stimulation (VNS) generated parkinsonian symptoms. A 13-year-old girl presented with refractory secondary generalized epilepsy from the age of 6 years and mental retardation. Her electroencephalography (EEG) showed diffuse polyspike and wave discharges. Magnetic resonance imaging (MRI) showed double-cortex syndrome. She was submitted to extended callosal section at the age of 10 years, which yielded 50% seizure frequency reduction. She was submitted to VNS by the age of 12 years. As stimulation intensity was increased, there was appearance of extrapyramidal symptoms: She developed bilateral tremor and rigidity, and gait and postural disturbance. All symptoms disappeared 7-10 days after VNS was turned off. Several attempts to reactivate VNS led to the same results. During the periods when VNS was on she presented with marked seizure frequency reduction. This is the first report of a clinically evident direct effect of VNS on the basal ganglia.
