A 65-year-old woman was evaluated for progressive numbness and tingling involving different body parts. Onset was with her right foot. Numbness and paresthesia progressed to her left hand and then left foot. Numbness and tingling of her right hand also developed. Progressive gait instability also developed, leading to frequent falls. She started using a cane to walk. She also reported severe dry eyes and mouth and noticed an inability to sweat in hot weather.
Her neurologic examination showed pseudoathetosis with eye closure and sensory gait ataxia. She had profound vibration and proprioceptive loss in all extremities (lower extremities greater than upper extremities). She also had asymmetric reduction in pinprick sensation in her hands and feet distally. She had minimal distal weakness involving her toes and upper extremities. Her deep tendon reflexes were reduced in the upper limbs and absent in the lower limbs. Toes were downgoing to plantar stimulation.
Nerve conduction studies demonstrated asymmetric, sensory-predominant, axonal peripheral neuropathy with absent left median and ulnar sensory responses and relatively preserved (reduced) right median and ulnar sensory responses. Bilateral sural sensory responses were absent. Motor responses were relatively preserved. Cerebrospinal fluid analysis showed mildly increased protein concentration, and 5 cerebrospinal fluid-restricted oligoclonal bands. Thermoregulatory sweat testing showed anhidrosis involving the proximal limbs and the trunk and hypohidrosis of the forehead and distal extremities. Serum laboratory investigations identified an increased erythrocyte sedimentation rate. Serologic testing was remarkable for positive antinuclear antibody, rheumatoid factor, and Sjögren syndrome-A antibody. Chest computed tomography showed a nonspecific solitary nodule in the right upper lobe but was otherwise normal.
The patient was diagnosed with Sjögren sensory ganglionopathy (or neuronopathy).
The patient received a 12-week course of intravenous methylprednisolone. She was also started on mycophenolate mofetil. Two weeks later, the mycophenolate mofetil dose was increased. At follow-up 4 months later, she reported improvement in neuropathic pain, but the sensory loss and ataxia continued to be treatment refractory.
The presence of sicca symptoms (dry eyes and mouth), polyarthralgias, morning stiffness, and anti-Ro antibody seropositivity were supportive of a Sjögren syndrome diagnosis. Sjögren syndrome has been associated with various neuropathic presentations. Sensory ganglionopathy in Sjögren syndrome usually presents with asymmetrical sensory loss, neuropathic pain, sensory ataxia, and sometimes pseudoathetosis, which were presenting features in this case patient.
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