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Cerebellar Degeneration Associated with Sjögren's Syndrome

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Cerebellar Degeneration Associated with Sjögren's Syndrome CASE REPORT Print ISSN 1738-6586 / On-line ISSN 2005-5013 J Clin Neurol 2012;8:155-159 http://dx.doi.org/10.3988/jcn.2012.8.2.155 Open Access Cerebellar Degeneration Associated with Sjögren’s Syndrome Mi Jung Kim,a,b Myoung Chong Lee,b,c Jae-Hong Lee,b Sun Ju Chungb aDepartment of Health Screening and Promotion Center, Asan Medical Center, Seoul, Korea bDepartment of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea cDepartment of Neurology, Cheongshim International Medical Center, Gapyeong, Korea Received July 12, 2010 BackgroundzzNeurologic manifestations of primary Sjögren’s syndrome (PSS) have been re- Revised September 8, 2010 ported to vary from sensory polyneuropathy to encephalopathy or psychiatric problems. How- Accepted September 8, 2010 ever, marked cerebellar degeneration associated with PSS has rarely been reported. Correspondence Case ReportzzWe describe a patient with Sjögren’s syndrome who exhibited rapidly progres- Sun Ju Chung, MD, PhD sive cerebellar ataxia, nystagmus, cognitive decline, and psychiatric problems. Brain magnetic Department of Neurology, resonance imaging revealed marked atrophy of the cerebellum, and 18F-fluorodeoxyglucose Asan Medical Center, positron-emission tomography demonstrated glucose hypometabolism of the cerebellum. University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, ConclusionszzOur PSS patient exhibited a progressive course of cerebellar syndrome, as evi- Songpa-gu, Seoul 138-736, Korea denced by cerebellar atrophy on serial brain images. J Clin Neurol 2012;8:155-159 Tel +82-2-3010-3988 Fax +82-2-474-4691 Key WordszzSjögren’s syndrome, cerebellar degeneration, ataxia, cognitive impairment. E-mail [email protected] Introduction Case Report Sjögren’s syndrome is a chronic autoimmune disorder of un- A 46-year-old woman was admitted to our neurologic clinic known etiology and pathogenesis. It is characterized mainly due to progressive gait disturbance. Three months before ad- by mononuclear cell infiltration of the exocrine glands, which mission, she developed vitiligo in her right anterior neck area, leads to dryness of the eyes and mouth.1 Although the disor- and she noticed unsteadiness of her gait. There was no family der has a strong predilection toward women (females : males, history of neurologic disorders and the patient had no expo- 9 : 1), both sexes and all ages may be affected.2 Approximately sure to toxins such as alcohol or drugs. one-third of patients with primary Sjögren’s syndrome (PSS) A neurological examination revealed that she had cerebel- present with systemic manifestations,3 but the prevalence of lar dysfunction: dysarthria, nystagmus, and ataxia. Ataxia was neurologic manifestations in PSS remains controversial.4-6 Pe- observed in all four extremities, but was worse in the left limbs ripheral neuropathy is reportedly the most common neurolog- and trunk. The patient had marked gait disturbance due to ic complication of PSS, followed by cranial nerve neuropathy ataxia. Speech analysis revealed that her speech disturbance and focal or multifocal involvement of the central nervous was a mixed type of dysarthria with ataxic, hyperkinetic, and system (CNS).4 Although ataxia due to PSS has also been de- flaccid components. Gaze-evoked nystagmus of both eyes was scribed,7,8 marked cerebellar atrophy associated with PSS has found in all directions. Motor power and sensory examina- rarely been reported. tions were normal. She had no parkinsonian features, her We describe herein a patient with PSS who exhibited rapid- deep-tendon reflexes were normoactive, and her plantar re- ly progressive ataxia accompanied by cerebellar atrophy. sponses were flexor bilaterally. She had mild depression and persecutory delusions related to her husband. cc This is an Open Access article distributed under the terms of the Cre- ative Commons Attribution Non-Commercial License (http://creative- Laboratory tests were positive for speckled-type antinuclear commons.org/licenses/by-nc/3.0) which permits unrestricted non-com- antibody, anti-Ro/SSA (4.5 S/C ratio), and anti-La/SSB (1.0 S/ mercial use, distribution, and reproduction in any medium, provided the ori- C ratio). Her antinuclear antibody titer was elevated by 1 : 160. ginal work is properly cited. Copyright © 2012 Korean Neurological Association 155 Cerebellar Degeneration in Sjögren’s Syndrome The following parameters were all normal: complete blood nance imaging (MRI) were normal (Fig. 1A-D). counts, biochemical screening, erythrocyte segmentation rate, Her ataxia and gait disturbance progressively worsened over C-reactive protein, thyroid function test, anti-hepatitis C virus, the next 6 months. She had mild cognitive impairment and Venereal Disease Research Laboratory test, serum C3, C4, and scored 25 on the Korean version of the Mini Mental Status CH50, anti-ds-DNA, lupus anticoagulant, anticardiolipin anti- Examination. Her Montreal Cognitive Assessment9 score was body, cytoplasmic antineutrophil cytoplasmic antibodies, peri- 14. The patient exhibited impairment in frontal executive func- nuclear antineutrophil cytoplasmic antibodies, serum folate, tion, visuospatial function, attention, calculation, and delayed vitamin B12, anti-human immunodeficiency virus (HIV) anti- recall on bedside examination. Oculomotor examination re- body, HIV antigen, and paraneoplastic antibodies (anti-Hu, vealed mild saccadic pursuit eye movements and saccadic anti-Ri, anti-Yo, and anti-CRMP5). The following malignancy dysmetria. Positional nystagmus of both eyes was purely hor- screening procedures produced unremarkable results: whole- izontal, and was triggered by an apogeotropic head position, body 18F-fluorodeoxyglucose positron-emission tomography which was not associated with vertigo. The nystagmus was (18F-FDG PET), tumor markers (alpha fetoprotein, carcinoem- not improved by repetitive canalith-repositioning maneuvers. bryonic antigen, CA-125, CA-19-9, and CA 15-3), cerebrospi- She suffered from moderate xerostomia and xerophthalmia. nal fluid cytology, mammography, and abdomen-pelvic com- Follow-up brain MRI revealed marked cerebellar atrophy puted tomography scan. The findings of brain magnetic reso- with an enlarged fourth ventricle and cisterna magna (Fig. A B C D E F G H I Fig. 1. Brain MRI and 18F-FDG PET results for the patient. A-D: Initial brain MRI was unremarkable. E-H: Follow-up brain MRI, performed 6 months later, revealed marked cerebellar atrophy and an enlarged fourth ventricle and cisterna magna without cerebral cortical atrophy. I: 18F-FDG PET, performed at the same time as the follow-up MRI, revealed decreased glucose metabolism in the bilateral cerebellum. 18F- FDG PET: 18F-fluorodeoxyglucose positron-emission tomography. 10 min ANT RT.LAT RAO 20 min Post IV 40 min A B Fig. 2. Salivary scan and biopsy of the salivary gland. A: Salivary scan showing no uptake of the parotid and submandibular glands. B: Bi- opsy of a minor salivary gland showing focal lymphocytic infiltration. ANT: anterior, RAO: right anterior oblique, Rt. LAT: right lateral. 156 J Clin Neurol 2012;8:155-159 Kim MJ et al. 1E-H). Brain 18F-FDG PET revealed decreased glucose me- with PSS. We evaluated possible causes of primary cerebellar tabolism in the bilateral cerebellum (Fig. 1I). Mutation analy- degeneration in our patient, including alcohol, drug abuse, nu- sis for dentatorubral-pallidoluysian atrophy and spinocerebel- tritional deficiency, genetic causes of cerebellar ataxia, parane- lar ataxia types 1, 2, 3, 6, and 7 were negative. A salivary scan oplastic marker, autoimmune disorders, and a rare manifesta- revealed no uptake of the parotid and submandibular glands tion of HIV infection.11 Diagnosis by exclusion ultimately led (Fig. 2A). Biopsy of a minor salivary gland revealed focal to the belief that her cerebellar degeneration was related to lymphocytic infiltration involving exocrine glands (Fig. 2B). PSS. The result of Schirmer’s test was strongly positive. The patient Our patient with established PSS exhibited rapidly progres- was diagnosed with PSS according to American and European sive cerebellar ataxia and subacute development of cerebellar criteria.10 atrophy. In contrast to the uniform features of the peripheral Intravenous methylprednisolone (500 mg/day) was admin- nervous system complications of PSS, CNS abnormalities are istered for 3 days and then maintenance therapy with oral associated with a much wider spectrum of manifestations.5 methylprednisolone (1 mg/kg/day) was continued for 1 month. Our patient had severe pancerebellar dysfunction that did not Hydroxychloroquine (200 mg, t.i.d.), quetiapine (12.5 mg/ respond to medical treatment. day), escitalopram (5 mg/day), and buspirone (10 mg, t.i.d.) The pathophysiologic mechanisms underlying the CNS in- were also administered. However, there was no improvement volvement in PSS remain unclear. Previous studies have sug- in her neurological and psychiatric abnormalities. Unfortu- gested that ischemia plays a role because CNS lesions in PSS nately, her gait disturbance and dysarthria progressed further, were found to be associated with mononuclear inflammation with persistent psychiatric symptoms during the 18-month fol- and cerebral ischemic vasculopathy, resulting in micro or low-up period. macro infarcts.6,12,13 Other studies found that abnormalities in humoral and cellular immunity, including antineuronal anti- Discussion body, might be involved in the pathogenesis of the CNS man- ifestations of Sjögren’s syndrome.8,13 In addition,
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