For personal use. Only reproduce with permission from The Lancet. THE LANCET Neurology Vol 2 October 2003 http://neurology.thelancet.com 634 Case report A 66-year-old woman was admitted to hospital on November 12, 2001, because of 5 months of visual disturbances and slight gait unbalance. Symptoms had worsened during the last 3 weeks, with severe oscillopsia, unbalance, left-arm clumsiness, and painful muscular spasms in the thighs at night. She was diagnosed with autoimmune hypothyroidism in February 1995 and pernicious anaemia in April 2000. The medical history of her family was unremarkable. Neurological tests showed persistent gaze-evoked down-beat nystagmus, ataxic gait that was possible only with a stick, and dysmetria on left-side finger-to-nose test. The International Cooperative Ataxia Rating Scale (ICARS) score was 34. Cerebral MRI showed mild atrophy of the cerebellar vermis without gadolinium-enhancement. Nucleus, parietal-gastric cell, and IgA gliadin antibodies were detected. Celiac ataxia was ruled out by digiunal biopsies and HLA typing (DRB*11,*16; DQB1*03,*05). CSF examination showed 9 cells/mm 3 , protein concentration was 68 mg/dL (normal <45 mg/dL), and oligoclonal IgG bands. There were no antibodies against Hu, Yo, or Ri in either the serum or the CSF. Antibodies against glutamic acid decarboxylase (GAD; radiobinding assay with in vitro translated sulphur-35-methionine- labelled GAD 65 ) were detected. In both serum and CSF, GAD 65 -antibody titre was 531 000 U/L (normal <3 U/L) and GAD 67 antibodies were found (>100 U/L; normal <3 U/L). Tyrosine phosphatase (IA-2) antibodies were present in serum (2·3 U/L; normal <0·7 U/L) but absent from CSF. Oral glucose tolerance test was normal. Screening, including total-body PET, found no malignancies. 5 days after admission, the patient experienced a sudden worsening of symptoms with dysarthria, dysphagia, inability to stand and walk without assistance, pronounced trunk ataxia, all-limb dysmetria, leg spasticity, increased deep-tendon reflexes, and bilateral Babinski sign. ICARS score was 60. Cerebral MRI was unchanged. Soon after methylprednisolone was given, we observed a large improvement (figure). Dysarthria, dysphagia, and spasticity recovered within 2 days. Ataxia significantly decreased and Babinski sign disappeared in the next 6 days. In December 2002, ICARS score was 36; serum GAD 65 -antibody titre was 165 000 U/L, and GAD 67 -antibodies were still present. 2 months later, nocturnal painful muscular spasms in the thighs reappeared. ICARS score was 30. Focal stiff-man syndrome was ruled out by needle electromyography. Motor evoked potentials were normal. Screening for Excellent response to steroid treatment in anti-GAD cerebellar ataxia Giuseppe Lauria, Davide Pareyson, Maria Giuseppina Pitzolu, and Elena Bazzigaluppi GL is at the Department of Clinical Neurosciences, University of Brescia; DP and MGP are at the National Neurological Institute, “Carlo Besta”, Milan; and EB is at the Diabetes and Endocrinology Unit, S Raffaele Vita Salute University Hospital and Scientific Institute, Milan, Italy. Correspondence: Dr Giuseppe Lauria, Clinica Neurologica, Università di Brescia, P le Spedali Civili, 1, 25125–Brescia, Italy. Tel +39 030 3995 633; fax +39 030 3995 027; email g_lauria@libero.it 0 50 000 100 000 150 000 200 000 250 000 300 000 350 000 400 000 450 000 500 000 550 000 0 10 20 30 40 50 60 70 80 90 100 * Serum GAD 65 titre (U/L) Methylprednisolone (1000 mg/day for 5 days) Immunoglobulin (0·4 g/kg/day for 5 days) Prednisone (1 mg/kg/day) Methylprednisolone (750 mg/day for 5 days) Prednisone (1 mg/kg/day) Prednisone (5 mg) Cyclophosphamide (100 mg/day) ICARS score Nov 12, 2001 Nov 17, 2001 Dec 10, 2001 Feb 5, 2002 Feb 15, 2002 Mar 5, 2002 May 20, 2002 Jul 10, 2002 Oct 10, 2002 Nov 20, 2002 Nov 25, 2002 Dec 13, 2002 Jan 20, 2003 Feb 20, 2003 May 2, 2003 Serum GAD 65 -antibody titre (bars) and ICARS score (dotted line) changes in response to treatment in a patient with GAD-antibody cerebellar ataxia. At the last control (*), antibody titre was 98·2 U/L.