Sensory ganglionopathies result from the degeneration of dorsal root ganglia in large fiber sensory nerves and manifest as non-length-dependent sensory symptoms with preserved motor function. Autoimmune and paraneoplastic processes are known causes of this condition. Although this is a purely sensory pathology, deafferentation weakness is a potential consequence. We report a case of sensory ganglionopathy that progressed to severe dysphagia and quadriparesis.

A 51-year-old female, active smoker, with chronic obstructive pulmonary disease and history of unprovoked coagulopathy presented with subacute generalized weakness, numbness and paresthesias, as well as progressive dysphagia. By admission, she had been bedbound for one month. Her physical exam was notable for significant weakness in all extremities that mildly improved with concentration and visual input. She also demonstrated diffuse loss of sensation to light touch, vibration, and joint proprioception associated with pseudoathetosis in her extremities, and absent reflexes. She also exhibited severe dysphagia that ultimately required placement of a percutaneous endoscopic gastrostomy (PEG) tube.

Extensive evaluative tests, including a lumbar puncture and multiple brain and whole spine magnetic resonance imaging studies, were unremarkable. Nerve conduction tests and electromyography revealed normal motor responses with diffuse absent sensory responses, consistent with severe sensory ganglionopathy. Paraneoplastic and autoimmune laboratory results were negative. She was treated for idiopathic sensory ganglionopathy with intravenous immunoglobulin (IVIG) which resulted in gradual recovery. Her PEG tube was removed after one course of IVIG. Two Position Emission Tomography (PET) Scans, twelve months apart, showed no malignancy. IVIG was stopped after one year due to steady improvement in her symptoms.

Sensory ganglionopathy is rare, and our case report emphasizes that this diagnosis can progress to profound deafferentation weakness and dysphagia due to the integral role of sensory feedback in swallowing mechanics and coordinating muscle movements.