Cinical and ENMG findings in patients with rare variants of Guillain Barré Syndrome

Abstract

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most recognized form of Guillain Barré Syndrome (GBS), while others variants such as Miller-Fisher syndrome (MFS) and pharyngo-cervico-brachial (PCB) variant are less recognized. To describe rare variants of GBS. Clinical and paraclinical data were reviewed. Our patients were respectively 8 and 38 years-old. The first patient presented for walk disturbance and the second one for swallowing disorders. At neurological examination, our first case presented ophtalmoplegia, right peripheral facial palsy, areflexia and proprioceptive ataxia. The other yielded areflexia, ataxia and palsy of the ninth, tenth and eleventh cranial nerves. Brain and spinal MRI showed radicular enhancement in the last root in our first case. Lumbar puncture revealed albumino-cytological dissociation in only one case. Electroneuromyography (ENMG) matched with the diagnosis of AIDP with mainly sensory axonal loss in the first case and motor demyelinating damage in the second case. The diagnosis was consistent with MFS in the first case and PCB variant of GBS in the second one. The PCB and MFS variant are rare forms of GBS. Ataxia commonly described in both of our cases may lead to overlapping syndrome and supports the view that PCB and MFS belongs to a continuous spectrum. Ataxia makes also difficult to differentiate these clinical entities from brainstem injury. However, areflexia and ENMG help to make the diagnosis. Further studies are needed to clarify whether these entities have specific patterns in electrophysiological studies.