Acute sensory neuronopathy as the presenting symptom of Sjögren's syndrome.
Sensory neuronopathy associated with Sjögren's syndrome (SS) usually has a subacute or chronic onset. We report the case of a 37-year-old woman who presented with an unusual hyperacute form of SS ganglionopathy. She initially developed paresthesias of her fingertips and rapidly became severely ataxic. Nerve conduction studies revealed abnormal sensory but normal motor functions. Lip biopsy showed findings consistent with SS. Sural nerve biopsy showed severe axonal loss. The patient showed modest improvement with immunosuppressive therapies.
Souayah
Nizar
N
Neurology Department, New Jersey Medical School, Newark, NJ 07101, USA. souayani@umdnj.edu
Chong
Peter Siao Tick
PS
Cros
Didier
D
D000328:Adult; D001259:Ataxia / Q000209:etiology; D004576:Electromyography; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009431:Neural Conduction; D010292:Paresthesia / Q000209:etiology; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D012678:Sensation Disorders / Q000209:etiology* / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology
D007166:Immunosuppressive Agents