The acute paralysis in Guillain-Barr� syndrome is related to a Na+ channel blocking factor in the cerebrospinal fluid

Abstract

The effect of cerebrospinal fluid (CSF) from patients with severe polyradiculoneuritis (Guillain-Barré syndrome, GBS) on voltage-dependent Na+ channels of myoballs was studied. The transient Na+ currents, elicited by repetitive stimulation at 1 Hz, were inhibited by the CSF from most of the GBS patients to 10%-40% the control value. The inhibition was complete in about 5 s and was fully reversible. Such inhibition was never seen with control CSF. The blocking property of the CSF from GBS patients was lost after the number of cells and the protein content had been lowered by means of a clinical filtration technique for cerebrospinal fluid. The results demonstrate that in Guillain-Barré syndrome blocking factors of Na+ channels are present in the CSF, impairing neuron impulse conduction, and thereby causing muscular weakness and sensory disturbances in the affected patient.

Ca²⁺ Channel Currents Inhibited by Serum from Select Patients with Guillain-Barré Syndrome

“…Patch clamp studies show inhibition of transient sodium currents by cerebrospinal fluid from patients with GBS [5, 6]. Furthermore, serum from patients with GBS directly blocks the ion-conducting pore of the sodium channel [7].…”

“…Furthermore, neuromuscular junctions (NMJs), which are rich in gangliosides, lie outside the blood-nerve barrier, and are an important site of many antibody-mediated autoimmune diseases. Electrophysiological studies demonstrate the presence of factors which block sodium channels in the cerebrospinal fluid of patients with GBS, thus impairing the conduction of nerve impulses, resulting in muscular weakness and sensory disturbances in patients with GBS [5, 6]. Patch clamp studies further show direct inhibition of the ion-conducting pore of voltage-gated sodium channelsby exposure to serum from patients with GBS [7].…”

Multimodal Therapy in Life-Threatening Cerebral Lupus erythematosus: The Benefit of Cerebrospinal Fluid Pheresis

“…It is conceivable that, similar to Guillain-Barre syn drome [9,10,12,13] or Miller-Fisher syndrome [pers. unpubl.…”

“…This technique has been described elsewhere [9][10][11]. For CSF fil tration the patient is put in a sideward position, the spinal tap is carried out in the normal way, the needle is left in position and connected with the CSF filtration system.…”

CSF Filtration: Scientific and Clinical Update

“…Recently published data describing in vitro studies, animal experiments and clinical experience in Ulm, Germany (2-6) encouraged other centres to use this technique. We are actually facing an increase in clinical data on CSF filtration (1,(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) with enlargement of the range of indications as well as an understanding of the possible mechanism of CSF filtration in various neurological diseases (3)(4)(5)(18)(19)(20)(21)(22). Even though it is still an experimental approach more than 40 hospitals -predominantly situated in the German speaking countries -are presently using CSF filtration.…”