A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome

Abstract

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.

Brainstem auditory evoked potentials in patients with primary Sjögren’s syndrome without central nervous system involvement

“…The most common peripheral manifestations of pSS are axonal sensorimotor polyneuropathy, distal sensory polyneuropathy, small fibreneuropathy, and cranial nerves. Central nervous system is spilled or focal lesions of hemispheres, brainstem, cerebellum, and spinal cord [10][11][12]. Among the psychiatric manifestations, cognitive, depressive, anxiety, and fatigue are most often observed [10,13].…”

“…Three potential pathogenic mechanisms are currently being discussed. The first assumes that neurological symptoms are a consequence of intracranial mononuclear infiltrates, the second concerns the direct, pathogenic effect of anti-neuronal and anti-Ro antibodies on the nervous system structure, and the third assumes the appearance of ischemic lesions in the course of inflammation of small vessels [11,14]. Neuropathy of the VIII nerve in the course of pSS is described primarily as an isolated lesion [15].…”

Movement disorders in systemic autoimmune diseases: Clinical spectrum, ancillary investigations, pathophysiological considerations

“…Epidemiology Median age at onset was 62.5 years (range, 40 -74 years); 90% were female. Parkinsonism preceded other PSS manifestations in 70% of cases [51,53,54,56,57].…”

“…Despite differing methodologies, all found PSS to be associated with increased risk of PD, with adjusted hazard ratios (aHR) ranging from 1.23 to 1.56 [48]. Despite this, reports of parkinsonism in patients with PSS are limitedonly ten met our criteria for analysis [51][52][53][54][55][56][57][58].…”

“…In 30% of cases, there was additional early and prominent gait impairment and cognitive dysfunction, resembling progressive supranuclear palsy-parkinsonism (PSP-P) [51,55,58]. One case (10%) presented with asymmetric parkinsonism and visual hallucinations [54], and one other (10%) with symmetric parkinsonism, tremor, balance impairment, psychosis (auditory and visual hallucinations) and sensory ganglionopathy [56].…”