Multiple cranial neuropathies

K K Jain MD (Dr. Jain is a consultant in neurology and has no relevant financial relationships to disclose.)
Originally released August 25, 1998; last updated August 8, 2017; expires August 8, 2020

This article includes discussion of multiple cranial neuropathies, cranial polyneuropathy, and Garcin syndrome, hemibase syndrome, and polyneuritis cranialis. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

There are several syndromes of multiple cranial nerve involvement. One syndrome described by Garcin was a step-by-step ipsilateral involvement of all 12 cranial nerves unilaterally. This term is now used for multiple cranial nerve palsies that do not fit into any of the other named syndromes. This article describes the various pathologies associated with multiple cranial nerve palsies and their differential diagnoses. Neurologic diagnosis is helpful in the early detection of the lesions and their management by the appropriate specialists. Cancer detected early may be amenable to surgery and radiotherapy.

Key points

 

• Multiple cranial nerve palsies are a part of several neurologic syndromes.

 

• The original Garcin syndrome was described as unilateral involvement of all the cranial nerves, usually due to malignancy at the base of skull.

 

• A large number of diseases besides cancer, such as benign tumors, infections, traumatic brain injury, and vascular lesions, can cause multiple cranial nerve palsies and should be considered in the differential diagnosis.

 

• Treatment and prognosis of multiple cranial nerve palsies depends on the cause.

Historical note and terminology

Galen (131 AD to 201 AD) was the first to classify cranial nerves but counted only 7 pairs of nerves (Major 1961). This classification was employed until the 17th century. Galen demonstrated the existence of 11 cranial nerves of the 12 cranial nerves (he regarded the olfactory nerve as an extension of the brain) but combined several and arrived at a total of 7. Although Vesalius followed the classification of Galen, his excellent dissections of the brain show 9 pairs of cranial nerves (Vesalius 1543). Willis described the arteries at the base of the brain and showed 10 pairs of cranial nerves (Willis 1664). This was the first reclassification of cranial nerves since the time of Galen, and it provided the correct numbering up to cranial nerve X. He described the accessory nerve but did not give it the number it has now: XI. Complete classification of all 12 cranial nerves did not take place until a century later in Germany (Soemmerring 1788). Cranial nerve palsies were not described adequately for still another 100 years. The best description of cranial nerve disorders in the 19th century was in Gower's manual of diseases of the nervous system. This manual was considered to be the bible of neurology at that time, as it devoted 150 pages to cranial nerve disorders (Gowers 1886). Several of the syndromes of multiple cranial nerve palsies were described toward the end of the 19th century and at the beginning of the 20th century. These include the syndromes of Jackson (Jackson 1872), Avellis (Avellis 1891), Tapia (Tapia 1905), Collet (Collet 1915), Villaret (Villaret 1916), and Sicard (Sicard 1917).

Knowledge of cranial nerve lesions is a cornerstone of neurology, and numerous names have been attached to syndromes of various combinations of cranial nerve palsies. One syndrome described by Garcin was a step-by-step ipsilateral involvement of all 12 cranial nerves unilaterally, without the rise of intracranial pressure and long-tract signs (Garcin 1927). Most of the original patients had radiologically demonstrated infiltration of the base of the skull by malignant disease and, in some cases, inflammatory process. Unilateral involvement of all 12 cranial nerves is rare; all of the original cases did not have this. The syndrome has since been modified to include cases with involvement of 7 cranial nerves ipsilaterally as well as a few contralaterally (Roger et al 1969). In Europe, many cases of multiple cranial nerve palsies (even less than 7) that do not fit into any of the other named syndromes are described as Garcin syndrome. Another term used for multiple cranial nerve palsies due to lesions of the skull base is “hemibase syndrome” (Schiffer 1951). Some reported cases have other symptoms such as headaches and signs such as hypopituitarism (Abe et al 1988). One case has been reported with papilledema (Agnoli and Prencipe 1968). Another term used to describe multiple cranial nerve palsies is “polyneuritis cranialis.”

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