Approach to the patient: Multiple Cranial Nerve Palsies

First determine whether the process is within the brainstem or outside it. Lesions on the surface of the brainstem tend to involve adjacent cranial nerves in succession with only late and slight involvement of long sensory and motor pathways. The opposite is true of processes within the brainstem. Involvement of multiple cranial nerves outside the brainstem may be due to trauma, localized infections including varicella zoster virus, infectious and noninfectious (especially carcinomatous) causes of meningitis; granulomatous diseases such as granulomatosis with polyangiitis, Behçet’s disease, vascular disorders including those associated with diabetes, enlarging aneurysms, or locally infiltrating tumors. A purely motor disorder without atrophy raises the question of myasthenia gravis. Facial diplegia is common in Guillain-Barré syndrome. Ophthalmoplegia may occur with Guillain-Barré syndrome (Fisher variant) or Wernicke’s encephalopathy.

The cavernous sinus syndrome ( Fig. 190-3 ) is frequently life threatening. It often presents as orbital or facial pain; orbital swelling and chemosis; fever; oculomotor neuropathy; and trigeminal neuropathy affecting the ophthalmic (V1) and occasionally maxillary (V2) divisions. Cavernous sinus thrombosis, often secondary to infection from orbital cellulitis, a cutaneous source on the face, or sinusitis, is the most frequent cause; other etiologies include aneurysm of the carotid artery, a carotid-cavernous fistula (orbital bruit may be present), meningioma, nasopharyngeal carcinoma, other tumors, or an idiopathic granulomatous disorder (Tolosa-Hunt syndrome). In infectious cases, prompt administration of broad-spectrum antibiotics, drainage of any abscess cavities, and identification of the offending organism are essential. Anticoagulant therapy may benefit cases of primary thrombosis. Repair or occlusion of the carotid artery may be required for treatment of fistulas or aneurysms. Tolosa-Hunt syndrome generally responds to glucocorticoids.

FIGURE 190-3
Anatomy of the cavernous sinus in coronal section, illustrating the location of the cranial nerves in relation to the vascular sinus, internal carotid artery (which loops anteriorly to the section), and surrounding structures.

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