Case Report: Dystonic Storm Following Japanese Encephalitis Virus Infection

Abstract

Dystonic storm (also called status dystonicus) is a neurological emergency characterized by sustained/intermittent involuntary generalized muscle contractions resulting in repetitive painful twisting movements and abnormal postures. It is commonly documented in patients with diagnosed primary dystonic syndromes or secondary dystonic states (i.e., patients with inborn errors of metabolism, dystonic cerebral palsy, Wilson’s disease, pantothenate kinase-associated neurodegeneration, and exposure to drugs). However, viral-induced dystonic storm cases have rarely been reported. We describe the case of an 11-year-old girl from rural West Bengal (India) with a dystonic storm after Japanese encephalitis. Generalized dystonic spasms lasted for about 10–20 minutes and occurred 20–30 times/day. They were associated with extreme pain, fever, exhaustion, sweating, tachycardia, tachypnea, pupillary dilatation, arterial hypertension, and mutism and were precipitated by a full bladder and relieved somewhat during sleep. When dystonic spasms abated, she had high-grade generalized rigidity of all four limbs and fixed cervical and truncal dystonia. She was put on invasive ventilation and deep intravenous sedation with continuous midazolam infusion and other supportive measures and had a good clinical recovery. During the 12 months of follow-up, she did not have any other episode of a dystonic storm. However, axial rigidity and intermittent appendicular (upper limb) dystonic posturing were observed. The authors also have briefly discussed the differential diagnoses and treatment plans for such a neurological emergency.

An Intriguing Case of Expanded Dengue Syndrome With Co-existing Encephalitis, Pancreatitis, and Hepatitis: The Classic Thalamic “Double-Doughnut” Sign Revisited

“…"Double-doughnut," a radiological biomarker of dengue encephalitis, can help identify this life-threatening otherwise treatable condition to prompt and adequately identify and treat these patients, preventing further clinical and neurological sequelae. However, similar bilateral symmetrical thalamic involvement with clinical presentation of acute encephalitis syndrome or comatose state can also be seen particularly in Japanese B encephalitis (which is quite prevalent in India) 21,22 and rarely in autoimmune encephalitis, 23 Wernicke's encephalopathy, 24 subacute sclerosing pan-encephalitis, 25 bilateral thalamic infarction caused by top of basilar/Percheron's artery occlusion and deep cerebral venous sinus thrombosis, 26,27 and also in neuromyelitis optica spectrum disorders (NMOSD) 28 In closing, the "double-doughnut" sign, though not exclusive to dengue encephalitis, proved crucial in this case, aiding in differentiating from other causes of encephalitis. Recognition of this sign can be pivotal in diagnosing expanded dengue syndrome, facilitating timely and appropriate intervention, and improving patient outcomes..…”

Neuroinvasion of emerging and re-emerging arboviruses: A scoping review

“…GBS, transverse myelitis (<3 segments), longitudinally extensive transverse myelitis (⩾3 segments), acute disseminated encephalomyelitis, dystonic storm, have all been reported neurological complications in patients with JE. 95 – 99 Furthermore, a case of N-methyl d-aspartate receptor encephalitis secondary to JE has also been reported pointing toward the possibility of secondary autoimmune events. 100 A case of JE mimicking poliomyelitis and presenting with primary respiratory failure has also been reported.…”