Once having established that the patient has a disease of the peripheral nerves and having ascertained its clinical and electrophysiologic pattern and time course, one is usually able to determine its cause. This is accomplished most readily by allocating the case in question to one of the categories listed in Table 43-2, which classifies the peripheral nerve diseases syndromically according to their mode of evolution and clinical presentation. Our use of the terms acute, subacute, and chronic neuropathy must be explained. By acute, we mean evolution in terms of days, and by subacute, evolution in terms of weeks. Chronic is divided into two groups: one in which the neuropathy has progressed for a period of several months to a few years and another in which progression is over many years, most of which prove to have a genetic cause. It can be restated that these temporal properties are, with the topographic pattern, the main determinants in the categorization of neuropathy.
Diseases of the peripheral nerves are considered in a more comprehensive fashion in the two-volume Peripheral Neuropathy, edited by Dyck and colleagues and in the text by Amato and Russell cited in the references. Also recommended are more concise monographs by Schaumburg and associates and by Asbury and Thomas, and the atlas on the pathology of peripheral nerve by King.
A number of differences separate the polyneuropathies in this category: (1) acute inflammatory demyelinating or axonal polyneuropathy (GBS), (2) vasculitic polyneuropathies, (3) porphyria, (4) certain toxic polyneuropathies, and (5) acute sensory and autonomic polyneuropathies. Of these various acute polyneuropathic diseases, the Guillain-Barré demyelinative syndrome, because of its frequency and gravity, is most demanding of the physician’s attention.
Guillain-Barré Syndrome (Landry-Guillain-Barré-Strohl Syndrome, Acute Inflammatory Demyelinating Polyneuropathy, AIDP)
This is the most common cause of acute or subacute generalized paralysis in practice. (During certain past epochs it was exceeded in frequency by polio.) GBS occurs in all parts of the world and in all seasons, affecting children and adults of all ages and both sexes. A mild respiratory or gastrointestinal infection or immunization precedes the neuropathic symptoms by 1 to 3 weeks in approximately 60 percent of cases. Typical is a nondescript upper respiratory infection, but almost every known febrile infection and immunization has at one time or another been reported to precede GBS (some probably coincidentally). In recent years, it has been appreciated from serologic studies that the enteric organism Campylobacter jejuni is the most frequent identifiable antecedent infection, but it accounts for only a relatively limited proportion of cases. Other common antecedent events or associated illnesses include viral exanthems in children and numerous other viral illnesses in adults and children, particularly the large viruses of the herpes family (cytomegalovirus [CMV], Epstein-Barr virus [EBV], HIV), and less often, bacterial infections other than Campylobacter (Mycoplasma pneumoniae, Lyme disease). There are less certain associations with lymphoma (particularly Hodgkin disease) and with the systemic autoimmune diseases.
The earliest description of an afebrile generalized paralysis is probably that of Wardrop and Ollivier, in 1834. Important landmarks were Landry’s report (1859) of an acute, ascending, predominantly motor paralysis with respiratory failure leading to death among peasants on his land; Osler’s (1892) description of “febrile polyneuritis”; and the account by Guillain, Barré, and Strohl (1916) of a benign polyneuritis with albuminocytologic dissociation in the CSF (increase in protein without cells). The first comprehensive account of the pathology of GBS was that of