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The patient sought medical attention for a 6-month history of numbness in the hands, mainly on the left side, associated with clumsiness.</p><p id="par0015" class="elsevierStylePara elsevierViewall">She had also been experiencing numbness in both feet for 4 months, with unsteady walking and frequent stumbling and accidental falls. The patient denied other associated neurological symptoms. The rest of the history-taking by systems and organs only reported moderate xerophthalmia and xerostomia that had been present for years, with no recent changes.</p><p id="par0020" class="elsevierStylePara elsevierViewall">On neurological examination, the patient showed generalised areflexia, hypoesthesia in glove and stocking, as well as a moderate decrease in deep sensitivity, both vibratory and arthrokinetic, mainly in the lower limbs, without associated cerebellar abnormalities; gait with an increase in the base of support and heel strike, performing the tandem with difficulty and the Romberg’s being doubtful. The rest of the physical examination showed no relevant abnormalities.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient was admitted for study of sensory ataxia. A lumbar puncture was performed to study the cerebrospinal fluid, with normal cytobiochemistry and negative microbiological cultures. Normal thyroid profile. Normal levels of folic acid and vitamin B12. Negative serologies, including lues, HIV and hepatotropic viruses. Electromyogram with signs of universal mixed polyneuropathy, mainly sensory and axonal, of severe intensity and somewhat more pronounced in the lower limbs. Brain MRI with signs of early-stage cortico-subcortical atrophy. Cervical MRI showed discrete degenerative changes between C3 and C6, with no signs of secondary spinal cord involvement. Autoimmunity study with positive antinuclear antibodies at 1/320 titre with cytoplasmic pattern and positive anti-Ro/SS-A antibodies (71 IU/mL), with negative anti-La/SS-B, SM, RNP and DNA; normal C3 complement and mild C4 hypocomplementemia (13 mg/dl). Given the suspicion of primary Sjögren’s syndrome, a Schirmer test was performed as well as a salivary gland scintigraphy, confirming the diagnosis. In this context, the clinical features were compatible with the diagnosis of ataxic sensory neuronopathy, or posterior ganglionopathy, secondary to primary Sjögren’s syndrome. During admission, the patient was treated with intravenous immunoglobulins and corticosteroids, continuing after discharge with a tapering regimen of steroids and azathioprine 50 mg every 12 h. Subsequent examinations showed a partial progressive improvement of both ataxia and sensory symptoms, but an unstable gait persisted as sequelae, for which the patient needed support for walking, as well as moderate hypoesthesia in glove and stocking, mainly in the lower limbs.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The interest of the clinical case described lies in the importance of considering the autoimmune origin in the differential diagnosis of certain neurological manifestations, which may be the first manifestation of the autoimmune disease or, on occasions, even precede the onset of systemic symptoms by years.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Sánchez Berná I, Santiago Díaz C. Neuronopatía sensitiva atáxica: una manifestación infrecuente del síndrome de Sjögren. Med Clin (Barc). 2022;158:440.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Sjögren sensory neuronopathy (Sjögren ganglionopathy): long-term outcome and treatment response in a series of 13 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "P.R. Pereira" 1 => "K. Viala" 2 => "T. Maisonobe" 3 => "J. Haroche" 4 => "A. Mathian" 5 => "M. 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Journal Information
Vol. 158. Issue 9.
Pages 440 (May 2022)
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Vol. 158. Issue 9.
Pages 440 (May 2022)
Letter to the Editor
Sensory neuronopathy: A rare manifestation of Sjögren’s syndrome
Neuronopatía sensitiva atáxica: una manifestación infrecuente del síndrome de Sjögren
Isabel Sánchez Berná
, Carlos Santiago Díaz
Corresponding author
Servicio de Medicina Interna, Hospital Universitario Rey Juan Carlos, Móstoles, Madrid, Spain
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