Transcranial Doppler as a useful tool in the detection of cervical steno-occlusive arteriopathy in infants.

OBJECTIVE: The early detection of cervical steno-occlusive arteriopathy is essential to rapidly initiate appropriate treatment and to potentially improve neurological outcome. To accurately confirm the diagnosis, cerebral imaging is the gold standard, but it cannot be performed if the patient is unstable or if the facility is unavailable. CASES: Here we report our experience of using transcranial Doppler (TCD) ultrasound as a readily available, easy-to-use bedside tool to guide the rapid screening and management of cervical steno-occlusive arteriopathy in infants. DISCUSSION AND CONCLUSION: Children with traumatic cervical steno-occlusive arteriopathy, TCD is a potentially useful tool for early diagnosis.

[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.