A document by Sushmita Bhattarai. Click on the document to view its contents.

Hypokalemic Quadriparesis as the Initial Presentation of Primary Sjögren’s Syndrome with Distal Renal Tubular Acidosis: A Case ReportRunning TitleHypokalemia in Sjögren’s SyndromeAuthors and AffiliationsDr. Sagun Baral, MD (Corresponding Author) Department of Internal Medicine, Rapti Academy of Health Sciences, Dang, NepalEmail: sagunbaral01@gmail.comORCID: 0009-0005-5980-9335Dr. Sushmita Bhattarai, MD Department of Internal Medicine, Rapti Academy of Health Sciences, Dang, Nepal ORCID: 0009-0001-4450-4791Dr. Ajaya Raj Gautam, MD Department of Internal Medicine, Rapti Academy of Health Sciences, Dang, NepalDr. Prabesh Raghubanshi, MD Department of Internal Medicine, Rapti Academy of Health Sciences, Dang, NepalDr. Krishna KC, MD Department of Internal Medicine, Rapti Academy of Health Sciences, Dang, NepalAuthor ContributionsDr. Sagun Baral: Conceptualization, Data curation, Formal analysis, Methodology, Supervision, Validation, Visualization, Writing - original draft, Writing - review & editing Dr. Sushmita Bhattarai: Conceptualization, Formal analysis, Investigation, Supervision, Writing - review & editing Dr. Ajaya Raj Gautam, Dr. Prabesh Raghubanshi, Dr. Krishna KC: Methodology, Supervision, Visualization Writing - review & editing All authors reviewed and approved the final manuscript.Word CountApproximately 1800 wordsKey Clinical MessagePrimary Sjögren’s syndrome may rarely present with hypokalemic quadriparesis due to distal renal tubular acidosis. Clinicians should consider autoimmune causes in patients with unexplained non-anion gap metabolic acidosis and hypokalemia, even in the absence of sicca symptoms.Keywords: Autoimmune Disease; Distal renal tubular acidosis; Hypokalemic paralysis; Quadriparesis; Sjogren’s syndromeKey Clinical MessagePrimary Sjögren’s syndrome may rarely present as hypokalemic quadriparesis secondary to distal renal tubular acidosis (dRTA). Clinicians should consider autoimmune etiologies in patients with unexplained hypokalemia and non-anion gap metabolic acidosis, even in the absence of sicca symptoms, to prevent diagnostic delays and serious complications.Abstract:Primary Sjögren’s syndrome (PSS) is a chronic autoimmune disease primarily affecting the exocrine glands, leading to dryness of the eyes and mouth. Renal involvement, although uncommon, can occur in the form of tubulointerstitial nephritis and result in distal renal tubular acidosis (dRTA). Hypokalemic paralysis as the initial presentation of PSS is rare. We report the case of a 52-year-old woman presenting with acute flaccid quadriparesis due to severe hypokalemia. Laboratory investigations revealed a normal anion gap metabolic acidosis with features consistent with dRTA. Further evaluation showed positive antinuclear antibodies (ANA), anti-Ro/SSA, and anti-La/SSB antibodies, along with an abnormal Schirmer’s test, confirming the diagnosis of primary Sjögren’s syndrome despite the absence of sicca symptoms. The patient was treated with intravenous potassium and bicarbonate with rapid neurological improvement and was referred for long-term rheumatologic follow-up. This case highlights a rare but important manifestation of Sjögren’s syndrome. In patients with unexplained hypokalemia and metabolic acidosis, autoimmune causes such as PSS should be considered, even without classic symptoms. Early recognition and treatment of dRTA can result in full recovery and prevent complications such as life-threatening paralysis.IntroductionPrimary Sjögren’s syndrome (PSS) is a common autoimmune exocrinopathy, with peak incidence around 50 years of age and a female-to-male ratio of 9:1 [1]. Immune-mediated damage of glands results leads to xerophthalmia (dry eyes) and xerostomia (dry mouth), and may involve other mucosal surfaces such as airways, digestive tract and vagina leading to classical ‘‘sicca syndrome’’ [2]. Approximately 80% of patients present with the triad of dryness of mouth and eyes, fatigue, and joint pain, whereas 30-40% of patients experience systemic complications [1]. Renal involvement is a rare manifestation, occurring in fewer than 10% of cases [3]. Renal involvement varies over a wide spectrum from isolated electrolyte disturbances to nephrolithiasis, glomerulonephritis, and tubulointerstitial nephritis (TIN). TIN secondary to lymphocytic infiltration of the renal interstitium is the most common renal manifestation [3]. Renal tubular dysfunction leads to acid retention or bicarbonate loss, manifesting as renal tubular acidosis (RTA) [4]. RTA may be classified as distal (dRTA), when there is inadequate hydrogen ion excretion in the distal nephron, or proximal (Fanconi syndrome), when the proximal tubule is involved [5].Hypokalemic quadriparesis as the initial manifestation of dRTA secondary to PSS is rare. We report the case of a 52-year-old woman presenting with flaccid quadriparesis, diagnosed with dRTA secondary to PSS. This case highlights an uncommon presentation of Sjögren’s syndrome and underscores the importance of considering autoimmune etiologies in patients with unexplained electrolyte disturbances even in the absence of sicca symptoms to prevent diagnostic delay.Case PresentationA 52-year-old woman with no known comorbidities presented to the emergency Department with a two-day history of progressive, symmetric weakness in all four limbs, rendering her unable to rise from bed. The weakness was symmetric, flaccid and more prominent in proximal muscle groups. She denied fever, recent gastrointestinal losses (vomiting, diarrhea), reduced oral intake and use of diuretics or laxatives. There were no symptoms suggestive of cranial nerve involvement, sensory disturbance, or bladder or bowel dysfunction. Importantly, she denied joint pain, skin rash, oral or ocular dryness.On examination, she was alert and hemodynamically stable (Blood pressure 124/78 mmHg, Heart rate 86 bpm, SpO2: 98% on room air). Neurological examination revealed flaccid quadriparesis (Medical research council grade 1/5 in all limbs), and absent deep tendon reflexes. Cranial nerve function was intact, and sensory and cerebellar examinations were unremarkable.Initial laboratory studies revealed severe hypokalemia and normal anion gap metabolic acidosis, prompting further workup. The key findings are summarized in Table 1.

Introduction:Neuroleptic malignant syndrome (NMS), first described by Delay et al, is a neurological emergency and a severe adverse reaction associated with the use of neuroleptic medications.1 Although rare, with an incidence of approximately 0.2% among neuroleptic users, it requires a high index of suspicion due to its potentially fatal course, with mortality rates ranging from 3.3% to 27.7%.1 It is typically characterized by severe muscular rigidity and hyperthermia following antipsychotic use, along with other symptoms such as profuse sweating, tremors, urinary incontinence, altered mental status, labile or elevated heart rate and blood pressure, leukocytosis, and elevated serum creatine phosphokinase (CPK) levels.2 Risk factors include use of high-potency first-generation antipsychotics over atypicals, dehydration, exhaustion, malnutrition, parenteral administration, rapid dose escalation, and especially a prior episode of NMS. 3 Although most cases develop within the first four weeks of initiating antipsychotics—two-thirds within the first week—NMS may also arise after long-term treatment with a stable dose.2 We report the case of a 62-year-old male with a long-standing psychiatric illness of unclear diagnosis, maintained on a stable psychotropic regimen that included trifluoperazine, who presented with fever, generalized rigidity, poor oral intake, and altered sensorium. Although initially suspected to have an infectious etiology such as meningoencephalitis or sepsis—given the presence of fever, thrombocytopenia, and leukopenia—he was later diagnosed with NMS. This case highlights that NMS can occur even in patients receiving chronic, stable-dose antipsychotic therapy, without recent dose escalation or parenteral administration. Early recognition and prompt treatment are essential and often require collaboration between psychiatry and internal medicine.