Relation of Multiple Cranial Nerve Dysfunction to the Guillain-barr'e Syndrome.

Multiple cranial nerve dysfunction can occur with primary intracranial processes (neoplasm, basilarvertebral insufficiency, congenital defects, aneurysms, dural vein thrombosis, multiple sclerosis), myopathy or neuromuscular junction disorder (myasthenia gravis, botulism, orbital 'myositis', ocular myopathy), and as a manifestation of a more systemic disorder (sarcoidosis, endocrinopathy, connective tissue disease, diphtheria, heavy metal intoxication, nutritional deficiency, diabetes). However, when the clinical syndrome is acute in onset, self limited in course and unaccompanied by evidence of systemic disorder, most of these possibilities become untenable. Multiple sclerosis then becomes the main clinical consideration and this diagnosis can usually be ruled in or out by the clinical findings and a period of observation. In 1938, Van Bogaert and Maere reported three patients with acute cranial nerve dysfunction that they felt represented a form of the Guillain-Barre syndrome confined to the cranial nerves. Since then several additional case reports have appeared, mainly in the French literature. The existence of such a clinical entity, however, has not been well accepted in the English literature. The controversy over what is and what is not the Guillain-Barre syndrome and whether the eponym should be used at all continues unabated. The clinical aphorism that the Guillain-Barre syndrome is easy to diagnose but impossible to define is as true today as it has been in the past. The diagnosis thus is based on a preponderance of established clinical features (Haymaker and Kernohan, 1949; Guillain, 1953; Baker, 1943; Dempsey, Karnosh, and Gardner, 1947; Duvoisin, 1960), none of which alone is pathognomonic. It is the purpose of this paper to review the literature pertaining to the existence and significance of a 'cranial nerve form' of the Guillain-Barre syndrome and to present additional case illustrations.