A disease called by the French neurologists Guillain and Wagghe (1916) “the primary acute treatable polyradiculoneuritis with protein- cell dissociation.” It is due to the primary demyelination of the fibers of the peripheral nerves and roots. It is characterized by the acute development of symmetrical flaccid paralysis, covering both the distal and often the proximal limbs, the slight severity of sensory disorders and protein-cell dissociation in the cerebrospinal fluid.
The process spreads in an upward direction, sometimes ending in damage to the cranial nerves.
According to some clinicians, Guillain – Barré syndrome can be considered as the initial stage (as a less severe variant) of Landry’s ascending paralysis . The latter develops just as rapidly, but ends in the course of the ascending process with paralysis of the respiratory muscles and bulbar disorders1.
The disease is relatively common, accounting for about 1/6 of patients with polyneuritis. The preceding moments can be various infections, intoxications, metabolic and endocrine diseases, surgical interventions, and cooling. In a significant part of cases, no external causes for the development of Guillain – Barré syndrome can be identified. All this, according to some authors, indicates the polyetiological nature of the disease, and according to others, only about the multiplicity of antigenic and resolving factors. The histopathological picture for 3-4 days is limited to edema of the roots; later, myelin sheath degeneration is detected. Axons suffer relatively little, which determines the possibility of curing even severe paralysis. Clinical manifestations unfold against a background of normal or subfebrile temperature. Leukocytosis in the blood and a shift of the formula to the left are possible. Pain appears in the limbs, more in the joints, as well as paresthesia.
Then a typical picture of the disease develops rapidly. Against the background of continuing malaise, weakness of the limbs, especially the legs, appears. In cases where this weakness prevails not in the distal, but in the proximal sections, they talk about the pseudomyopathic type of polyneuritis. Weakness often reaches a degree of paralysis. Tendon reflexes decrease and disappear, muscles become flabby. Amyotrophy is negligible. Sometimes cranial nerves are also affected, most often facial. Prosoplegia (often bilateral) may be the first manifestation of the disease. Less commonly, the bulbar and oculomotor group of nerves are involved in the process. There are pains in the extremities and hyperesthesia, in some cases there is hypesthesia or mild hypalgesia in the distal sections, such as socks and gloves. Proprioception disorders are less commonly detected . Symptoms of tension are noted. Protein cell dissociation is found in the cerebrospinal fluid , the protein content is increased, and xanthochromia is also found .
After the progression phase, the process goes into a relatively stationary period, after which the disease regress begins, sometimes lasting six months. The possibility of a complete recovery of even common paralysis has given rise to call this type of polyradiculoneuropathy treatable, or benign. However, this is not always the case. Some patients remain of varying severity persistent motor defects. In some cases, the spread of the process to the respiratory and bulbar muscles requires the transfer of patients to mechanical ventilation. This last circumstance should always be kept in mind when planning treatment tactics in the first days of an illness. Even with the use of the entire arsenal of resuscitation measures, the most severe forms of Guillain – Barré syndrome can result in death. The protein content in the cerebrospinal fluid can increase for months, and sometimes it remains long-term elevated after recovery. Although the disease and named the island Polyradiculopathy ,. not so rarely (on average in 1/6 patients) it does not have an acute onset and the formation of paresis lasts for months and even years – a chronic form of Guillain-Barré syndrome ( Shtulman D.R., Golubeva V.V., 1978). In 5-6% of cases, the course is recurrent.
The diagnosis with a typical acute onset is made on the basis of the rapid development of paresis and paralysis with characteristic protein- cell dissociation, a relatively weak sensory component of polyneuropathy . Against acute myelitis (developing, in particular, as Landry’s ascending paralysis ), the absence of sphincter disturbances in the presence of even very gross paresis and paralysis of the legs speaks. The limited process in Guillain – Barré syndrome to the limits of the radicular- neuritic segment of the nervous system determines the rule that has almost no exceptions, according to which the clinical picture does not have any conduction symptoms and any other signs of damage to the substance of the brain and spinal cord. In a chronic course, the absence of the above-mentioned spinal disorders makes it possible to exclude a spinal cord tumor, which can be thought of in connection with the presence of protein- cell dissociation. On the other hand, protein- cell dissociation eliminates myopathy or spinal amyotrophy . Against the latter are the results of electrophysiological studies: electromyographic signs of damage to the peripheral nerve, and not the front horns, a decrease in the speed of conduction along the nerves. In all cases of Guillain – Barré syndrome , a thorough somatic examination is necessary to exclude visceral, hematological and other diseases.