Multisystem neuronal involvement and sicca complex: broadening the spectrum of complications

Neurological complications of primary Sjögren's syndrome

“…Alexander and coworkers briefly mentioned lower motor neuron syndrome as a possible complication of PSS but they gave no information about clinical picture, course and investigations [1,2]. Recently, two cases of motor neuron syndrome associated to sicca complex have been reported [27].The clinical picture was not characteristic of amyotrophic lateral sclerosis (ALS) since besides motor involvement, paralytic ileus and flaccid bladder were present in one case and prominent sensory impairment associated to a tonic pupil and hearing loss were present in the other. Autopsy was performed in one patient and disclosed a severe loss of motor neurons with gliosis in the spinal cord.…”

Connective Tissue Diseases, Vasculitis, and the Nervous System

“…49 Mild autonomic neuropathy, with manifestations such as Adie's pupil, orthostatic hypotension, impaired sweating, constipation or diarrhea, and abnormal cardiac refl exes, often accompanies the other neuropathies of Sjögren's syndrome. 50 Predominantly motor neuropathy is less common in patients with Sjögren's syndrome; motor neuron disease is linked to Sjögren's syndrome by case reports. 44 Mononeuritis multiplex or multiple cranial neuropathies may occur in patients with Sjögren's syndrome.…”

“…In an occasional patient, more severe autonomic dysfunction is the predominant neurological problem. 50 Carpal tunnel syndrome occurs in some patients with Sjögren's syndrome. In contrast to trigeminal sensory neuropathy, the multiple cranial neuropathies also affect motor function and can include nerves III, V, VI, VII, IX, X, and XII.…”

Neuromuscular complications of connective tissue diseases

“…Estimates of the incidence of peripheral nervous system disease in patients with Sjögren's syndrome range from nearly 50% to less than 10% 5, 15, 77, 87. More common associations include trigeminal sensory neuropathy, symmetric distal sensory or sensorimotor neuropathy, sensory neuronopathy, autonomic neuropathy, and carpal tunnel syndrome; other entrapment neuropathies, including mononeuritis multiplex, chronic inflammatory demyelinating polyneuropathy, and motor neuropathy, are less common 10, 89, 174. For example, among 33 patients with Sjögren's syndrome and peripheral neuropathy, 70% had sensorimotor neuropathy and 30% had a distal pure sensory pattern 119.…”

“…The syndrome has been described based on cases with purely sensory involvement, but dysfunction of the autonomic nervous system has appeared clinically in a few patients with features such as orthostatic hypotension, anhydrosis, or Adie's pupils, and has proven to be detectable in many patients with more sophisticated autonomic testing 183. Occasionally patients also have disease of motor nerves 89. Sensory nerve conduction studies have shown low‐amplitude or absent sensory nerve action potentials in involved extremities.…”