Table 2

- Diagnostic test and outcome.

Patient numberHemoglobinCSF protein, control <400 mg/LCT/ MRI brain & spineTreatmentOutcome
16-12 g/dl1326 mg/LEncephalomalacia, cortical and cerebellar atrophy, cervical cord lesions from brainstem up to C6IVIg, corticosteroids, plasma exchangeDied at the age of 14
211 g/dl-NormalIVIgWalking with canes with high steppage gait
37-11 g/dl-Bilateral thalami, basal ganglia, occipital lobesIVIg, corticosteroidsDied at the age of 10
411-13 g/dl685 mg/LBilateral thalami, internal capsule, corpus callosum lesionsIVIg, corticosteroidsStanding but not walking, aphasia, intellectual impairment
58-11 g/dl-NormalIVIgFlaccid paralysis of lower limbs, just sitting
67-11 g/dl872 mg/LExtensive demyelination supra and infratentorial structures including brainstemIVIg, corticosteroids, eculizumabPersistent vegetative state
77.5-10 g/dl-Right middles artery stroke, left hemispheric atrophyIVIgPersistent vegetative state on ventilator support
87.5-9.9 g/dl914 mg/LNormalIVIg, plasma exchangeFlaccid lower limbs