Clinical History

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Patients with dysfunction of structures innervated by CN XI or XII or their supranuclear input may present to the physician with various complaints. The more common complaints when CN XI is involved are difficulty rotating the head, complaint that the head "droops" forward, or complaint that the shoulders seem asymmetrical or misshapen. The more common complaints when CN XII is involved are slurred speech, garbled speech, speaking with "marbles in my mouth," difficulty chewing, complaints that "my tongue gets in the way," or difficulty swallowing. Occasionally, a patient notices that the tongue is asymmetrical, appearing small and wrinkled on one side with protrusion. These complaints may be associated with neurological signs, including weakness of the sternocleidomastoid

(SCM) and trapezius muscles (with or without atrophy), winging of the scapula, deviation and weakness of the tongue (with or without atrophy), dysarthria, and dysphagia.^ '111

Patients may present with complaints of acute, subacute, and chronic onset. Acute presentations, characterized by the rapid onset of symptoms and signs, include the medial medullary syndrome (i.e., an acute cerebrovascular event involving the posterior circulation), basal skull fracture, shoulder trauma, and dislocation of upper cervical vertebra. Subacute presentations include lesions due to multiple sclerosis, brain stem abscesses, and tuberculous meningitis. Chronic presentations are typically seen in motor neuron disease, acoustic neuroma, meningioma, and other tumors of the central and peripheral nervous system. '10]

Dysfunction of the eleventh and twelfth cranial nerves may be the result of various precipitating events or underlying etiologies, and several of these were alluded to earlier. These include trauma to the head, neck, or shoulder region (basal skull fracture, stab wound, or gunshot wound of the neck; dislocation of upper cervical vertebra; shoulder trauma), various infectious etiologies including bacterial (tuberculosis, brain abscess, syphilis) and viral (acute poliomyelitis), inflammatory or granulomatous processes (multiple sclerosis, Guillain-Barre syndrome, neurosarcoidosis), metabolic disorders (Paget's disease), structural disorders (platybasia, syringobulbia), exogenous toxins (botulism), acute cerebrovascular events (medial medullary syndrome), neoplasms (glomus cell tumor, neurofibroma, meningioma, acoustic neuroma, metastatic carcinoma), and iatrogenic events (radiation therapy involving the neck, neck surgery). U , '121

Lesions of these cranial nerves may be accompanied by any of a large number of associated diseases, symptoms, or signs, including the several systemic illnesses (systemic sarcoidosis, tuberculosis, carcinoma, syphilis), other neurological disorders (multiple sclerosis, Guillain-Barre syndrome, motor neuron disease), and other associated symptoms and signs referable to involvement of regional anatomy, particularly long tracts passing through the brain stem (resulting in weakness with spasticity, dysautonomia, sensory disturbances, gait instability, and incoordination) and other cranial nerves (resulting in facial numbness, nystagmus, dysphagia). '10' ,

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