Treatment Treatment of the Lyme neuropathic syndromes is with intravenous antibiotics, preferably ceftriaxone 2 g daily for 1 month. More prolonged or intravenous treatment has not been shown to be superior. Corticosteroids have an uncertain role in the painful radicular syndromes, but we have used them in low doses and they relieved pain. In most series, there is recovery or virtually complete resolution of radicular symptoms in approximately 90 percent of patients, although this may take months. Facial palsies also tend to improve, but with a lower rate of complete resolution. It has been stated that many of the peripheral and cranial neuropathies improve even without treatment, but this has not been studied systematically.
This is a chronic, slowly progressive autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, particularly the parotid and lacrimal glands, that results in keratoconjunctivitis sicca and xerostomia (dry eyes and mouth). These core features may be combined with arthritis or with a wide range of other abnormalities, notably lymphoma, vasculitis, IgM paraproteinemia, renal tubular defects (renal tubular acidosis), and, quite often, 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 Sjögren disease. The sicca symptoms are often mild and reported only upon specific inquiry. A symmetrical sensory polyneuropathy or a sensory ganglionopathy are the most common patterns. 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 asymmetrical sensory loss, mostly of position sense and involving the upper limbs predominantly, in association with tonic pupils and trigeminal anesthesia that is probably a variant of the ganglionopathy.
The sensory polyneuropathies of the Sjögren syndrome are of particular interest to neurologists, as they will encounter most cases before other physicians (Griffin et al). More than 80 percent of affected patients are older women. The polyneuropathic syndrome often begins with paresthesias of the feet, usually mild in degree. The main clinical features are subacute and widespread sensory loss that may include the trunk and sometimes, profoundly diminished kinesthetic sense, giving rise to sensory ataxia of the limbs and of gait that reflect a ganglionitis. Loss of pain and temperature sensation is variable; tendon reflexes are abolished. A nondescript largeor small-fiber distal sensory neuropathy is also known, for which reason testing for Sjögren-related serum antibodies is included in the general evaluation of polyneuropathies in older patients, and the heterogeneity of sensory neuropathy presentation has been emphasized by several authors. In time, some 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.