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Ebook Localization in clinical neurology (7/E): Part 2

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Part 2 book “Localization in clinical neurology” has contents: Cranial nerves IX and X, the cerebellum, the localization of lesions affecting the hypothalamus and pituitary gland, the anatomic localization of lesions in the thalamus, the localization of lesions affecting the cerebral hemispheres, localization of lesions in the autonomic nervous system,… and other contents.

12 Cranial Nerves IX and X (The Glossopharyngeal and Vagus Nerves) Anatomy of Cranial Nerve IX (Glossopharyngeal Nerve) The glossopharyngeal nerve contains motor, sensory, and parasympathetic fibers The nerve emerges from the posterior lateral sulcus of the medulla oblongata dorsal to the inferior olive in close relation with cranial nerve X (the vagus nerve) and the bulbar fibers of cranial nerve XI (the spinal accessory nerve) (Fig 12-1 ) [11,45] These three nerves then travel together through the jugular foramen Within or distal to this foramen, the glossopharyngeal nerve widens at the superior and the petrous ganglia and then descends on the lateral side of the pharynx, passing between the internal carotid artery and the internal jugular vein The nerve winds around the lower border of the stylopharyngeus muscle (which it supplies) and then penetrates the pharyngeal constrictor muscles to reach the base of the tongue Figure 12-1 Ventral view of medulla and cranial nerves IX, X, and XI exiting together through the jugular foramen Dorsal roots of C1 through C6 in the The motor fibers originate from the rostral nucleus ambiguus and innervate upper cervical spinal cord are also shown (From Daube JR, Reagan TJ, the stylopharyngeus muscle (a pharyngeal elevator) and (with the vagus nerve) Sandok BA Medical Neurosciences: An Approach to Anatomy, Pathology, and the constrictor muscles of the pharynx Physiology by System and Levels 2nd ed Boston, MA: Little, Brown, 1986 By The sensory fibers carried in the glossopharyngeal nerve include taste permission of Mayo Foundation.) afferents, supplying the posterior third of the tongue and the pharynx, and general visceral afferents from the posterior third of the tongue, tonsillary region, posterior palatal arch, soft palate, nasopharynx, and tragus of the ear By way of the tympanic branch of the glossopharyngeal nerve (Jacobson nerve), sensation is supplied to the tympanic membrane, eustachian tube, and the mastoid region Taste afferents and general visceral afferent fibers have their cell bodies in the petrous ganglion and terminate mainly in the nucleus of the solitary tract (the rostral terminating fibers convey taste, and the caudal terminating fibers convey general visceral sensation); exteroceptive afferents have their cell bodies in the superior and petrous ganglia and terminate in the spinal nucleus of the trigeminal nerve The glossopharyngeal nerve also carries chemoreceptive and baroreceptive afferents from the carotid body (chemoreceptors) and carotid sinus (baroreceptors), respectively, by way of the carotid sinus nerve (nerve of Hering) The parasympathetic fibers carried in the glossopharyngeal nerve originate in the inferior salivatory nucleus, located in the periventricular gray matter of the rostral medulla, at the superior pole of the rostral nucleus of cranial nerve X These parasympathetic preganglionic fibers leave the glossopharyngeal nerve at the petrous ganglion and travel by way of the tympanic nerve or Jacobson nerve (coursing in the petrous bone) and the lesser superficial petrosal nerve to reach the otic ganglion (just below the foramen ovale), where they synapse The postganglionic fibers then travel by way of the auriculotemporal branch of the trigeminal nerve, carrying secretory and vasodilatory fibers to the parotid gland Figure: Ventral view of medulla and cranial nerves IX, X, and XI exiting together through the jugular foramen Dorsal roots of C1 through C6 in the upper cervical spinal cord are also shown (From Daube JR, Reagan TJ, Sandok BA Medical Neurosciences: An Approach to Anatomy, Pathology, and Physiology by System and Levels 2nd ed Boston, MA: Little, Brown, 1986 By permission of Mayo Foundation.) Clinical Evaluation of Cranial Nerve IX Motor Function Stylopharyngeal function is difficult to assess Motor paresis may be negligible with glossopharyngeal nerve lesions, although mild dysphagia may occur and the palatal arch may be somewhat lower at rest on the side of glossopharyngeal injury (However, the palate elevates symmetrically with vocalization.) Sensory Function The integrity of taste sensation may be tested over the posterior third of the tongue and is lost ipsilaterally with nerve lesions Sensation (pain, soft touch) is tested on the soft palate, posterior third of the tongue, tonsillary regions, and pharyngeal wall These areas may be ipsilaterally anesthetic with glossopharyngeal lesions Reflex Function The pharyngeal or gag reflex is tested by stimulating the posterior pharyngeal wall, tonsillar area, or base of the tongue The response is tongue retraction associated with elevation and constriction of the pharyngeal musculature The palatal reflex consists of elevation of the soft palate and ipsilateral deviation of the uvula with stimulation of the soft palate The afferent arcs of these reflexes probably involve the glossopharyngeal nerve, whereas the efferent arcs involve both the glossopharyngeal and vagus nerves Unilateral absence of these reflexes is seen with glossopharyngeal nerve lesions Autonomic Function Salivary secretion (from the parotid gland) may be decreased, absent, or occasionally increased with glossopharyngeal lesions, but these changes are difficult to demonstrate without specialized quantitative studies Localization of Lesions Affecting the Glossopharyngeal Nerve Lesions affecting the glossopharyngeal nerve also usually involve the vagus and therefore syndromes affecting both nerves are much more common than nerve lesions occurring in relative isolation Supranuclear Lesions Supranuclear lesions, if unilateral, do not result in any neurologic deficit because of bilateral corticobulbar input to the nucleus ambiguus However, bilateral corticobulbar lesions (pseudo-bulbar palsy) result in severe dysphagia [23] along with other pseudo-bulbar signs (e.g., pathologic laughter and crying, spastic tongue, explosive spastic dysarthria) With stimulation, the gag reflex may be depressed or markedly exaggerated, resulting in severe retching and even vomiting Nuclear and Intramedullary Lesions These lesions include syringobulbia, demyelinating disease, vascular disease, motor neuron disease, and malignancy Such lesions commonly involve other cranial nerves, especially the vagus, and other brainstem structures (e.g., Wallenberg syndrome) and are therefore localized by “the company they keep.” Extramedullary Lesions Cerebellopontine Angle Syndrome The glossopharyngeal nerve may be injured by lesions, especially acoustic tumors, occurring in the cerebellopontine angle Here there may be glossopharyngeal involvement associated with tinnitus, deafness, and vertigo (cranial nerve VIII), facial sensory abnormalities (cranial nerve V), and occasionally other cranial nerve or cerebellar involvement Jugular Foramen Syndrome (Vernet Syndrome) Lesions at the jugular foramen, especially glomus jugulare tumors and basal skull fractures, injure cranial nerves IX, X, and XI, which travel through this foramen [7,51] Other etiologies include neuroma, metastasis to the skull base, cholesteatoma, meningioma, infection, and giant cell arteritis [19] Vernet syndrome consists of the following: Ipsilateral trapezius and sternocleidomastoid paresis and atrophy (cranial nerve XI) Dysphonia, dysphagia, depressed gag reflex, and palatal droop on the affected side associated with homolateral vocal cord paralysis, loss of taste on the posterior third of the tongue on the involved side, and anesthesia of the ipsilateral posterior third of the tongue, soft palate, uvula, pharynx, and larynx (cranial nerves IX and X) Often dull, unilateral aching pain localized behind the ear Occipital condylar fracture may cause paralysis of cranial nerves IX and X [53] Lehn et al describe a man with occipital condylar fracture complicated by bilateral palsies of IX and X nerves associated with debilitating postural hypotension, dysphagia, severe gastrointestinal dysmotility, issues with airway protection as well as airway obstruction, increased oropharyngeal secretions, and variable respiratory control [33] Lesions within the Retropharyngeal and Retroparotid Space The glossopharyngeal nerve may be injured in the retropharyngeal or retroparotid space by neoplasms (e.g., nasopharyngeal carcinoma, paragangliomas [7,51,55]), abscesses, adenopathy, aneurysms [52], trauma (e.g., birth injury [20]), or surgical procedures (e.g., carotid endarterectomy) Resulting syndromes include the Collet–Sicard syndrome (affecting cranial nerves IX, X, XI, and XII) and Villaret syndrome (affecting cranial nerves IX, X, XI, and XII, the sympathetic chain, and occasionally cranial nerve VII) Villaret syndrome has been described due to a carotid-artery dissection and an associated aneurysm [13] The glossopharyngeal nerve may rarely be damaged in isolation by retropharyngeal or retroparotid space lesions resulting in a “pure” glossopharyngeal syndrome (mild dysphagia, depressed gag reflex, mild palatal droop, loss of taste on the posterior third of the tongue, glossopharyngeal distribution anesthesia) For example, traumatic internal maxillary artery dissection and pseudoaneurysm may present with isolated glossopharyngeal nerve palsy [1] Glossopharyngeal (Vagoglossopharyngeal) Neuralgia Glossopharyngeal neuralgia [9,10,14,17,46,50] refers to a unilateral pain (usually stabbing, sharp, and paroxysmal) located in the field of sensory distribution of the glossopharyngeal or vagus nerves Patients usually describe an abrupt, severe pain in the throat or ear that lasts seconds to minutes and is often triggered by chewing, coughing, talking, yawning, swallowing, and eating certain foods (e.g., highly spiced foods) The pain may occasionally be more persistent and have a dull aching or burning quality Other areas (e.g., larynx, tongue, tonsils, face, jaw) may also be affected According to the International Headache Society (IHS) Classification of Headache Disorders (ICHD-3 beta), the diagnostic criteria for glossopharyngeal neuralgia are as follows [9]: A Paroxysmal 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a dopamine–secreting glomus vagale tumor Neurology 20 11;76:1 021 –1 022 Bickerstaff ER, Howell JS... 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