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As one can readily understand, several cranial nerves may be affected by a single disease process The rst clinical problem that arises is whether the lesion lies within or outside the brainstem Lesions lying on the surface of the brainstem or at the base of the skull are characterized by involvement of adjacent cranial nerves (often occurring in succession and sometimes painful) and by late and only slight, if any, involvement of the long sensory and motor pathways These are discussed below and listed in Table 47-1 by their eponymic designations The opposite is true of intramedullary, intrapontine, and intramesencephalic lesions; lesions within the brainstem that involve cranial nerves often produce a crossedsensory or motor paralysis (cranial nerve signs on one side of the body and tract signs on the opposite side) In this way, a number of distinctive brainstem syndromes, to which eponyms have been attached, are produced These are listed in Table 34-3 An extramedullary lesion is more likely to cause bone erosion or to be associated with encroachment on cranial nerves (seen radiographically) The special problems of multiple cranial nerve palsies of the ocular motor nerves are addressed in Chap 14 Involvement of multiple cranial nerves outside the brainstem may be the result of trauma (obviously sudden in onset); localized infections such as herpes zoster (less acute onset); Lyme disease as reported by Schmutzhard and colleagues; cytomegalovirus (CMV) infection in an AIDS patient; Wegener granulomatosis, sarcoidosis, or other types of granulomatous disease (subacute onset); or compression by tumors and saccular aneurysms (more chronic development with pain) The sequential painless affection of contiguous or noncontiguous nerves over several days or weeks is particularly characteristic of meningeal carcinomatosis or lymphomatosis The eighth nerve is commonly incorporated in neoplastic meningeal in ltrations Among the solid tumors that cause local compression of nerves, neuro bromas, schwannomas (acoustic neuroma), meningiomas, cholesteatomas, carcinomas, cordomas, and chondromas have all been observed Nasopharyngeal carcinoma (Schmincke tumor or lymphoepithelioma) may implicate several cranial nerves in succession by invading the base of the skull (mainly the fth and sixth but also higher nerves; Fig 47-5), as do basilar invagination and Chiari malformation Several lower cranial nerves may be involved on one side by a carotid artery dissection In France, a successive involvement of all cranial nerves on one side has been called the Garcin syndrome, or hemibasal
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Figure 47-5 Nasopharyngeal carcinoma invading the anterior base of the skull and causing third and fth nerve palsies Coronal CT scan with bone windows
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syndrome It has been reported in chondromas and chondrosarcomas of the clivus but may occur with nasopharyngeal carcinomas as well The main causes of multiple cranial nerve palsies of extramedullary origin are listed in Table 47-2 Multiple or single cranial nerve palsies of abrupt onset may precede or accompany infectious mononucleosis and sometimes other viral or mycoplasmal diseases DeSimone and Snyder have assembled a series of 20 such cases associated with mononucleosis; bilateral facial paralysis was the most common presentation, bilateral optic neuritis the next, and in three cases, three or four cranial nerves were involved The prognosis is excellent The question of Table 47-2 Causes of extramedullary multiple cranial nerve palsies Meningeal processes Carcinomatous and lymphomatous meningitis Infectious radiculitis (tuberculous, fungal, syphilitic, Lyme) Idiopathic pachymeningitis Lesions affecting nerves at the skull base Metastasis of solid tumor or lymphomatous in ltration Local spread from nasopharyngeal tumor, chordoma, sarcoma Trauma Vascular occlusion or dissection (carotid artery dissection, jugular vein thrombosis) Paget disease, basilar invagination, Arnold-Chiari and other bony disorders Processes within nerves Perineural invasion of spindle cell, basal cell, parotid, and squamous cell cancer Granulomas and infectious diseases (Listeria, sarcoid, Wegener granulomatosis, diphtheria, HIV, Lyme disease, CMV infection in AIDS, Sjogren syndrome, idiopathic) Herpes zoster and other viral and postinfectious in ammatory lesions (Guillain-Barre syndrome) Mixed connective tissue disease Idiopathic Tolosa-Hunt-like syndrome affecting nonorbital nerves Melkersson-Rosenthal syndrome
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