| CASE REPORT
|Year : 2020 | Volume
| Issue : 2 | Page : 132-134
Primary hypokalemic periodic paralysis: Long-term management and complications in a child
Indar K Sharawat1, Renu Suthar2, Naveen Sankhyan2, Pratibha Singhi3
1 Pediatric Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, India
2 Pediatric Neurology Unit, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Pediatric Neurology Unit, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India; Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Medanta, The medicity, Gurugram, Haryana, India
Hypokalemic periodic paralysis (HPP) is a rare genetically determined neuromuscular disorder caused by mutation in skeletal muscles calcium and sodium channels. It presents with recurrent episodes of flaccid paralysis. A 9-year-old girl presented with recurrent episodic flaccid quadriparesis with complete recovery in-between the episodes. Investigations during the acute episode revealed marked hypokalemia with electrocardiogram changes. Next-generation sequencing showed pathogenic missense mutation in CACNA1S gene. She responded well to oral potassium supplementation, acetazolamide, and spironolactone therapy. Muscle weakness in HPP is reversible, and long-term management reduces frequency of paralysis and prevents permanent weakness.
Dr. Renu Suthar
Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh.
Source of Support: None, Conflict of Interest: None
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