[Pharyngeal-cervical-brachial syndrome: A rare form of Guillain-Barré syndrome with severe acute bulbar palsy]. / Le syndrome pharyngo-cervico-brachial: un tableau de syndrome de Guillain-Barré atypique avec paralysie bulbaire sévère.

The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is rare in children. It is characterized by oropharyngeal, neck, and upper limb muscle involvement, without ataxia and disturbed consciousness. Although associated with anti-GT1a antibodies, there is no single clinical or serological marker of PCB syndrome. We report on two cases in a 14-year-old and a 15-year-old females. The first symptom was acute dysphonia followed by severe bulbar palsy with deglutition disorders, associated with involvement of other cranial nerves and arm and leg weakness. One of the girls had normal deep tendon reflexes. Both had normal cerebral imaging and normal cerebrospinal fluid. No sign of neuropathy was found on nerve conduction studies. The diagnosis of PCB syndrome was established based on the presence of antiganglioside antibodies. Both adolescents had IgG anti-GT1a antibodies. Anti-GQ1b and anti-GT1b antibodies were associated in the first case, anti-GM1 and anti-GD1a in the second case. Clinical improvement was fast after treatment with intravenous immunoglobulin therapy. Recovery was complete. Only a few cases of children and adolescents with PCB syndrome have been reported. The main differential diagnoses were excluded with brain MRI. The neurophysiological findings in PCB syndrome are axonal neuropathy rather than demyelinating neuropathy, which might be normal in the early stages of the disease. Positivity of anti-GT1a IgG antibodies is very helpful for the diagnosis of PCB syndrome. In atypical cases of bulbar palsy with other cranial nerve involvement and normal brain MRI, diagnosis of PCB syndrome should be considered. Recognizing the atypical cases of Guillain-Barré syndrome enables anticipatory monitoring for disease complications and identifies therapeutic options. The short- and long-term outcome of the PCB syndrome after intravenous immunoglobulin treatment seems favorable.

[Pseudostatus epilepticus: a severe complication of psychogenic nonepileptic seizures (PNES) in children]. / Pseudostatus epilepticus : une complication grave des crises non épileptiques psychogènes (CNEP) chez l'enfant.

Psychogenic nonepileptic seizures are clinical events that mimic epileptic seizures but are not associated with electroencephalographic discharges. These seizures are seldom reported in children in the literature and could be misinterpreted as generalized tonicoclonic seizures. We report the case of a child, already treated for epilepsy, who presented at 8 years of age with several psychogenic seizures leading to pseudostatus epilepticus. After several hospitalizations, the diagnosis of pseudostatus was established on the basis of clinical semiology, lack of EEG abnormalities during the seizures, and a positive provocation maneuver, which elicited and blocked the manifestations. The clinical spectrum of psychogenic seizures is wide and it is particularly difficult to differentiate psychogenic seizures from epileptic seizures, especially when occurring in children, some of whom are already treated for epilepsy. Well-described clinical features can suggest the diagnosis of psychogenic seizure. It is important and necessary to make the diagnosis as soon as possible in order to rapidly begin appropriate treatment including psychotherapy. In fact, the long-term prognosis in children is better than in the adult population. Associated risk factors, such as anxiety as reported in the present case, have to be sought. Recognizing psychogenic seizures will thus avoid their fixation in the child's personality and the risk of inappropriate and escalating treatments leading to iatrogenic complications.