A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome
A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and re...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Neurological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2017/5696512 |
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| author | João Peres Simão Cruz Rita Oliveira Luís Santos Ana Valverde |
| author_facet | João Peres Simão Cruz Rita Oliveira Luís Santos Ana Valverde |
| author_sort | João Peres |
| collection | DOAJ |
| description | A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. A simultaneous improvement of the cognitive abilities allowed for the detection of sensory ataxia of the lower limbs. Sensory ganglionopathy was then detected with electrophysiological studies. A diagnosis of Sjögren syndrome was suspected and confirmed by salivary gland scintigraphy, Schirmer’s test, and submaxillary gland biopsy. We report a case of Sjögren syndrome associated with central and peripheral nervous system involvement, without sicca symptoms preceding the neurological clinical picture. The coexistence of ganglionopathy and a favourable response to immunosuppression are key features that can lead to the correct diagnosis in cases with atypical CNS symptoms, mimicking a rapidly progressive dementia. |
| format | Article |
| id | doaj-art-c6cf2cf55fb549f38745f276eb29ce1c |
| institution | OA Journals |
| issn | 2090-6668 2090-6676 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Neurological Medicine |
| spelling | doaj-art-c6cf2cf55fb549f38745f276eb29ce1c2025-08-20T02:19:41ZengWileyCase Reports in Neurological Medicine2090-66682090-66762017-01-01201710.1155/2017/56965125696512A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren SyndromeJoão Peres0Simão Cruz1Rita Oliveira2Luís Santos3Ana Valverde4Neurology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, PortugalNeurology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, PortugalAnatomic Pathology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, PortugalNeurology Department, Hospital de Egas Moniz-Centro Hospitalar Lisboa Oeste, Lisbon, PortugalNeurology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, PortugalA 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. A simultaneous improvement of the cognitive abilities allowed for the detection of sensory ataxia of the lower limbs. Sensory ganglionopathy was then detected with electrophysiological studies. A diagnosis of Sjögren syndrome was suspected and confirmed by salivary gland scintigraphy, Schirmer’s test, and submaxillary gland biopsy. We report a case of Sjögren syndrome associated with central and peripheral nervous system involvement, without sicca symptoms preceding the neurological clinical picture. The coexistence of ganglionopathy and a favourable response to immunosuppression are key features that can lead to the correct diagnosis in cases with atypical CNS symptoms, mimicking a rapidly progressive dementia.http://dx.doi.org/10.1155/2017/5696512 |
| spellingShingle | João Peres Simão Cruz Rita Oliveira Luís Santos Ana Valverde A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome Case Reports in Neurological Medicine |
| title | A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome |
| title_full | A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome |
| title_fullStr | A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome |
| title_full_unstemmed | A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome |
| title_short | A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome |
| title_sort | case report of nonvasculitic autoimmune inflammatory meningoencephalitis with sensory ganglionopathy a rare presentation of sjogren syndrome |
| url | http://dx.doi.org/10.1155/2017/5696512 |
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