Efficacy of high dose methylprednisolone in a patient with cervical dystonia and blepharospasm and Sjögren's syndrome.

Abstract

Dear Editor, Dystonia is a term indicating a heterogeneous group of hyperkinetic movement disorders characterised by involuntary muscle contractions, resulting in twisting and repetitive movements and abnormal postures. Sjogren’s syndrome (SS) is an autoimmune disorder characterised by lymphocytic infiltration and destruction of the salivary and lacrymal glands leading to xerostomia and xerophthalmia. Neurological complications have been reported in approximately 1.5–25 % of patients with SS, however, movement disorders like parkinsonism, chorea and dystonia are infrequent [1]. We herein report a patient with blepharospasm and cervical dystonia associated to primary SS. A 67-year-old woman came to our observation complaining of difficulty in maintaining head posture, with anterior flexion of the neck, blinking and spasming of the eyelids since 3 months. Five years previously she had been diagnosed with SS class III according to the Chisholm-Mason scoring system on the basis of xerophthalmia, xerostomia, positivity of rheumatoid factor (31 IU/ml) and a positive salivary gland biopsy. She had a copy of HLA-DRw52. Anti-Ro and antiLa antibodies were negative. She had been treated with pilocarpine chlorhydrate for 4 years which was then interrupted because of nausea. She also had hypothyroidism controlled with replacement therapy. At entry, neurological examination disclosed blepharospasm and spasmodic anterocollis. Haematological investigations revealed increased title of myeloperoxidasespecific antineutrophil cytoplasmic antibodies (MPOANCA) 7 IU/ml, normal rheumatoid factor levels and absence of other autoantibodies (ENA, ANA, antithyroid, crioaglutinin, anticardiolipin, antiphospholipid). Brain and cervical magnetic resonance imaging showed no structural lesion, electroneurography and electromyography studies were normal. The patient was initially treated with clonazepam (1 mg/ day for 20 days) without benefit, so intravenous steroid treatment (500 mg methylprednisolone daily for 3 days) was introduced with a rapid and significant improvement of cervical dystonia whereas blepharospasm persisted. Because of a previously reported poor tolerance to chronic steroids treatment, the patient was not treated chronically with prednisone, but we performed monthly endovenous methylprednisolone administrations; moreover, botulin toxin (20 UI of Botox) was administrated to treat blepharospasm. Presently, a year after onset, the patient is almost symptoms free. A variety of movement disorders have been described in patients with SS but its have been reported very rarely [1]. Only six cases of dystonia associated with SS have been reported in the literature (Table 1). The first report was in 1999, in a patient with SS and dystonia of the left hand and foot [2]; in the same year three other patients with SS who developed intermittent tonic/dystonic limb spasms were reported [3]; more recently, a case of orofacial dystonia [4] and a case of right arm paroxysmal dystonia [5] were described. In three of these patients treatment with steroids led to clinical improvement [2–4]. A case of cervical dystonia in association with SS had not been described before. Early recognition of the immunological pathogenesis of dystonia is important, as treatment, if initiated immediately V. Mantero (&) R. Balgera A. Rigamonti F. Basso A. Salmaggi Neurological Department, A. Manzoni Hospital, Via dell’Eremo 9/11, 23900 Lecco, Italy e-mail: vittorio.mantero@hotmail.com