Severe outcome of pharyngeal-cervical-brachial pure motor axonal neuropathy.

We present two further cases of the pharyngeal-cervical-brachial (PCB) form of GBS, with unfavourable outcome, showing dramatic dissociation between upper and lower body Symptoms. Both patients showed rapidly progressive motor denervation with disappearance of Compound Muscle Action Potentials (CMAPs) in upper limbs muscles. Sensory Nerve Action Potentials (SNAPs) were instead normal. Normal reflexes, F waves and action potentials were elicited in lower limbs. Despite i.v. Immunoglobulin treatment no recovery was observed and both patients died within a year from onset of symptoms.

[Unusual familial association of epilepsy, myoclonus and muscular atrophy. Case report]. / Una insolita associazione familiare di epilessia, mioclonia e atrofia muscolare. Contributo clinico.

Here is a rare association of spinal muscular atrophy, myoclonia and epilepsy in two brothers. Analogies and particular aspects are discussed in comparison with the only case known in literature up to now.

[Electrophysiologic findings in a case of Fisher syndrome]. / Rilievi elettrofisiologici in un caso di sindrome di Fisher.

A case of Fisher syndrome is presented, with an electrophysiological study including EMG, Conduction Velocity, Brain stem Auditory evoked potentials and Somatosensory Evoked Potentials. The results seem to confirm the peripheral origin of ophthalmoplegia, ataxia and areflexia.