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or more often by an opportunistic CMV infection in a patient with AIDS Diagnosis This is both aided and at times confused by serologic testing (see Chap 32) The enzyme-linked immunosorbent assay (ELISA) is not altogether satisfactory because it frequently yields false-positive and, occasionally, false-negative results Western blot testing is more speci c Knowledge that the patient has lived in or visited an endemic area is useful but far more important is evidence of a tick bite followed by the characteristic rash or a wellde ned history of nonneurologic manifestations of Lyme disease (cardiac, arthritic) Indeed, one should be cautious in making this diagnosis unless a primary Lyme infectious syndrome had been present Bifacial palsy in any of these clinical contexts also favors the diagnosis Treatment Treatment of the neuropathic disease is with intravenous antibiotics, preferably ceftriaxone 2 g daily for 1 month Corticosteroids have an uncertain role in the painful radicular syndromes but we have used them in low doses and they apparently relieved the pain Sjogren-Sicca Syndrome This is a chronic, slowly progressive autoimmune disease characterized by lymphocytic in ltration of the exocrine glands, particularly the parotid and lacrimal glands resulting in keratoconjunctivitis sicca and xerostomia (dry eyes and mouth) The latter features may be combined with arthritis or with a wide range of other abnormalities, notably lymphoma, vasculitis, renal tubular defects, and quite often by a predominantly sensory polyneuropathy (see review by Kaplan et al) In the series collected by Grant and colleagues, the neuropathy was the presenting problem in 87 percent of 54 patients with the sicca complex The sicca symptoms are usually mild and often reported only upon speci c inquiry A symmetrical sensory polyneuropathy or sensory ganglionopathy is the most common pattern Sensorimotor polyneuropathy, polyradiculoneuropathy, autonomic neuropathy, or mononeuropathy (most often of the trigeminal nerve, as described by Kaltrieder and Talal) are less common We have observed yet another neuropathic syndrome taking the form of an asymmetrical sensory loss, mostly of position sense and involving the upper limbs predominantly, in association with tonic pupils and trigeminal anesthesia The sensory polyneuropathy of the Sjogren syndrome more of an in ammatory ganglionitis or sensory neuronopathy (Grif n et al) is of particular interest to neurologists, since most cases will rst be seen by them More than 90 percent of the patients are older women The sensorimotor polyneuropathic syndrome begins with paresthesias of the feet and is usually mild in degree The main clinical features are a subacute and widespread sensory loss that may include the trunk and a profoundly diminished kinesthetic sense, giving rise to sensory ataxia of the limb and of gait Loss of pain and temperature sensation is variable; tendon re exes are abolished In time, many of these patients develop autonomic abnormalities such as bowel atony, urinary retention, loss of sweating, and pupillary changes There is usually little or no pain but there have been exceptions Diagnosis Sjogren syndrome should be suspected in an older woman with sensory neuropathy or neuronopathy, particularly if sicca symptoms are present The evaluation of suspected Sjogren disease is aided by the Schirmer or Rose Bengal tests (page 461), which can demonstrate a reduction of tearing Even without this con rmatory test, we have found it advisable to perform a biopsy of the lip (at the epithelial-mucosal juncture) to detect in ammatory.

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