6th Edition of Neurology World Conference 2026

Abstract

Background: - The causes of sporadic amyotrophic lateral sclerosis (SALS) and other types of motor neuron disease (MND) remain largely unknown (1). Sjogrens syndrome may present as peripheral sensory neuropathy or, more rarely, as mononeuritis multiplex, but MND is rare. We here present two cases that presented to us with clinical features suggestive of Motor Neuron Disease that ultimately proved out to be secondary to Sjogrens Syndrome.
Materials and Methods: We describe two interesting cases that were ALS but later turned out to be secondary Sjogrens syndrome. 
Case 1 -year-old female presented with progressive bulbar symptoms and proximal and distal weakness of both upper and lower limb and clinically detected to have fasciculation in tongue along with increased reflexes B/T/K (3+)  and S/A ( 2+). Plantar: B/L flexor. No sensory or cerebellar signs. On evaluation, electrophysiologically, definite ALS, Anti-Ro (+), ANA 2(+), speckled, lip biopsy S/o Sjogrens syndrome. His PET scan and salivary scintigraphy were normal. Her other work-up for motor neuron disease was negative
 CASE 2: 
Discussion: - Sjogrens syndrome is a multisystem disorder that predominantly presents with dry eyes and dry mouth.   Sjogrens syndrome is complicated by neurological complications in as many as 20% of patients [2, 3]. In approximately 25–92% of this group, these symptoms may precede dry eyes and mouths. [4-5].  MND is a rare presentation of Sjogrens syndrome, mentioned in only a very few case reports.  UMN signs are more predominant than LMN signs and occur early in Sjogrens syndrome patients with MND.  There are possibly two mechanisms underlie the involvement of the involvement of the upper motor neuron in patients with Sjogrens syndrome [6]. One possible mechanism could be attributed to autoimmune origin of UMN involvement, as in such patients the presence of various anti-neuronal antibodies is reported in the literary works. Subclinical Sjogrens syndrome plays an additive role in leading to another possibility for the progression of upper motor neuron dysfunction.    
Conclusions: Motor neuron disease is a relentlessly progressive disorder, with median survival from the time of diagnosis of three to five years.  So correct diagnosis is utmost importance before committing on motor neuron disease. This case also talks about the importance of proper history-taking and complete evaluation in neurology to come to proper diagnosis.  Neurological symptoms occur commonly in Sjogrens syndrome, although MND is rare. Immunosuppressant’s should be tried in such cases