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Clinical Pathways in Neuro-ophthalmology : An ... - E-Lib FK UWKS

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446 <strong>Cl<strong>in</strong>ical</strong> <strong>Pathways</strong> <strong>in</strong> <strong>Neuro</strong>-Ophthalmology, second edition<br />

bra<strong>in</strong>stem and lateral column of the sp<strong>in</strong>al cord to exit at the cervical (C8) and thoracic<br />

(T1-T2) levels (ciliosp<strong>in</strong>al center of Budge) of the sp<strong>in</strong>al cord as a second-order neuron.<br />

This second-order (<strong>in</strong>termediate) preganglionic neuron exits the ventral root and arches<br />

over the apex of the lung to ascend <strong>in</strong> the cervical sympathetic cha<strong>in</strong>. The second-order<br />

neurons synapse <strong>in</strong> the superior cervical ganglion and exit as a third-order neuron. The<br />

neural fibers for sweat<strong>in</strong>g of the face travel with the external carotid artery. The thirdorder<br />

postganglionic neuron travels with the carotid artery <strong>in</strong>to the cavernous s<strong>in</strong>us.<br />

With<strong>in</strong> the cavernous s<strong>in</strong>us, the sympathetic fibers jo<strong>in</strong> the abducens nerve for a short<br />

course and then travel with the ophthalmic division of the trigem<strong>in</strong>al nerve and jo<strong>in</strong> the<br />

nasociliary branch of the trigem<strong>in</strong>al nerve. The fibers pass through the ciliary ganglion<br />

and to the eye as the long and short ciliary nerves (Burde, 1992; Miller, 1985).<br />

The evaluation of HS <strong>in</strong>cludes two stages (Burde, 1992; Miller, 1985): (1) recognition of<br />

the cl<strong>in</strong>ical syndrome, and (2) confirmation and localization by pharmacologic test<strong>in</strong>g.<br />

Is the HS Isolated?<br />

Nonisolated HS should undergo imag<strong>in</strong>g with attention to the topographic localization<br />

of the cl<strong>in</strong>ical f<strong>in</strong>d<strong>in</strong>gs.<br />

Is a Central HS Present?<br />

Patients with a central HS can usually be identified by the presence of associated<br />

hypothalamic or bra<strong>in</strong>stem signs or symptoms (e.g., contralateral fourth nerve palsy,<br />

diabetes <strong>in</strong>sipidus, disturbed temperature or sleep regulation, men<strong>in</strong>geal signs, vertigo,<br />

sensory deficits, anhidrosis of the body, etc.). The etiologies of central HS are listed <strong>in</strong><br />

Table 20–9.<br />

Is a Preganglionic (Intermediate) HS Present?<br />

The preganglionic (<strong>in</strong>termediate) HS patient may have neck or arm pa<strong>in</strong>, anhidrosis<br />

<strong>in</strong>volv<strong>in</strong>g the face and neck, brachial plexopathy, vocal cord paralysis, or phrenic nerve<br />

palsy (Burde, 1992). The etiologies of preganglionic <strong>in</strong>termediate HS are listed <strong>in</strong><br />

Table 20–10.<br />

Is a Postganglionic HS Present?<br />

The postganglionic HS patient may have ipsilateral pa<strong>in</strong> and other symptoms suggestive<br />

of cluster or migra<strong>in</strong>e headaches (e.g., tear<strong>in</strong>g, facial flush<strong>in</strong>g, rh<strong>in</strong>orrhea)<br />

(DeMar<strong>in</strong>is, 1994; Manzoni, 1991). <strong>An</strong>hidrosis <strong>in</strong> postganglionic HS is often absent<br />

(Thompson, 1977b). Sweat glands of the forehead are supplied by the term<strong>in</strong>al branches<br />

of sympathetics to the <strong>in</strong>ternal carotid, and <strong>in</strong>volvement of these fibers after they have<br />

separated from the rema<strong>in</strong><strong>in</strong>g facial sweat fibers may expla<strong>in</strong> the occurrence of<br />

anhidrosis of the forehead with spar<strong>in</strong>g of the rest of the face <strong>in</strong> these patients.<br />

Postganglionic HS due to cavernous s<strong>in</strong>us lesions (e.g., thrombosis, <strong>in</strong>fection, neoplasm)

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