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Interruption of postganglionic sympathetic bers at any point along the internal carotid arteries or a lesion of the superior cervical ganglion results in miosis, drooping of the eyelid, and abolition of sweating over one side of the face; this constellation is the Horner, or more properly, Bernard-Horner syndrome (see also page 242) The same syndrome in less obvious form may be caused by interruption of the preganglionic bers at any point between their origin in the intermediolateral cell column of the C8-T2 spinal segments and the superior cervical ganglion or by interruption of the descending, uncrossed hypothalamospinal pathway in the tegmentum of the brainstem or cervical cord The common causes are neoplastic or in ammatory involvement of the cervical lymph nodes or proximal part of the brachial plexus, surgical and other types of trauma to cervical structures (eg, jugular venous catheters), carotid artery dissection, syringomyelic or traumatic lesions of the rst and second thoracic spinal segments, and infarcts or other lesions of the lateral part of the medulla (Wallenberg syndrome) There is also an idiopathic variety that is in some cases hereditary If a Horner syndrome develops early in life, the iris on the affected side fails to become pigmented and remains blue or mottled gray-brown (heterochromia iridis) A lesion of the stellate ganglion, eg, compression by a tumor arising from the superior sulcus of the lung, produces the interesting combination of a Horner syndrome and paralysis of sympathetic re exes in the limb (the hand and arm are dry and warm) With preganglionic lesions, facial ushing may develop on the side of the sympathetic disorder; this is brought on in some instances by exercise (harlequin effect) Keane has provided data as to the relative frequency of the lesions causing oculosympathetic (Horner) paralysis In 100 suc-

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Autonomic Neuropathy in Infants and Children (Riley-Day Syndrome) and Other Inherited Dysautonomias

6:

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This is a familial disease of children, inherited as an autosomal recessive trait The main symptoms are postural hypotension and lability of blood pressure, faulty regulation of temperature, diminished hearing, hyperhidrosis, blotchiness of the skin, insensitivity to pain, emotional lability, and cyclic vomiting The tendon re exes are hypoactive and mild slowing of motor nerve conduction velocities is common There is denervation sensitivity of the pupils and other structures The main pathologic feature is a de ciency of neurons in the superior cervical ganglia and in the lateral horns of the spinal cord Also, the number of unmyelinated nerve bers in the sural nerve is greatly decreased according to Aguayo and to Dyck and their colleagues (see also page 1159) It is likely that this disease represents a failure of embryologic migration or formation of the rst- and second-order sympathetic neurons It is now known that this defect is the result of a mutation in the gene coding for a protein that is associated with the transcription regulator, Ikappa B (Anderson et al) Autonomic symptoms may also be a prominent feature of the small- ber neuropathy of Fabry disease ( -galactosidase de ciency) as a result of the accumulation of ceramide in hypothalamic and intermediolateral column neurons (see pages 839 and 1159) Another inherited form of peripheral dysautonomia is characterized by severe pain in the feet on exercise and an autosomal

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cessive cases, 63 were of central type due to brainstem strokes, 21 were preganglionic from trauma or tumors of the neck, 13 were postganglionic due to miscellaneous causes, and in 3 cases the localization could not be determined (see Chap 14 for further discussion) The pupillary disturbances associated with oculomotor nerve lesions, the Adie pupil, and other parasympathetic and sympathetic abnormalities of pupillary function are considered fully in Chap 14 The combination of segmental anhidrosis and an Adie pupil is sometimes referred to as the Ross syndrome; it may be abrupt in onset and idiopathic or may follow a viral infection

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tactual stimuli of the limbs and abdomen, and pressure on the bladder An exaggerated vasopressor reaction also occurs in response to injected NE In such attacks, the patient experiences paresthesias of the neck, shoulders, and arms; tightness in the chest and dyspnea; pupillary dilatation; pallor followed by ushing of the face; sensation of fullness in the head and ears; and a throbbing headache Plasma NE and dopamine rise slowly during the autonomic discharge When such an attack is severe and prolonged, myocardial infarction, seizures, and visual defects have been observed Clonidine, up to 02 mg three times daily, has been useful in preventing the hypertensive crises

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