Short CommunicationChronic encephalopathy and locked-in state due to scrub typhus related CNS vasculitis
Graphical abstract
Introduction
Scrub typhus, a potentially fatal acute febrile illness with widespread systemic features, is endemic to the tsutsugamushi triangle in Asia-Pacific region. Caused by mite-borne intracellular gram negative cocco-baccillus Orientia tsutsugamushi, it is prevalent in the shrub and forestlands of Sub-Himalayan belt and Southern states of India during the wet months (Khan et al., 2017; Kar et al., 2014). Scrub typhus is considered a re-emerging disease in India in the past decade with western Rajasthan showing a rising trend of cases (Sharma et al., 2014). Common manifestations include acute fever, headache, lymphadenopathy, inoculation eschar, facial and peripheral edema with systemic complications namely pneumonia, hepatitis, azotemia, pancreatitis, myocarditis, shock, hepatomegaly, splenomegaly, thrombocytopenia and gastrointestinal bleed within the first week itself (Khan et al., 2017; Kar et al., 2014; Mahajan and Mahajan, 2017).
A high rate of neurological symptoms are noted in scrub typhus, namely headache (80–95%), altered sensorium (20–30%), neck stiffness (15–20%), and seizures (5–25%) (Khan et al., 2017; Kar et al., 2014; Mahajan and Mahajan, 2017; Jamil et al., 2015). Meningitis and acute encephalitis syndrome (AES) have been reported with variable frequency (Kar et al., 2014; Mahajan and Mahajan, 2017; Jamil et al., 2015; Kim et al., 2013). Focal neurological deficits are rare and unless treated early, multiorgan dysfunction invariably leads to death (Yum et al., 2013; Kim et al., 2000). The case reported here highlights that scrub typhus may not always have a tumultuous downhill course and can present with locked-in state and static encephalopathy and certain radiological indicators.
Section snippets
Case report
A 20-year-old female, resident of Barmer in western Rajasthan, India presented with history of symmetrical persistent moderate intensity pain in bilateral calves with occasional cramps at onset. Two days later, she developed nausea, vomiting and dizziness, without tinnitus, diplopia or postural exacerbation. After 3–5 days, she developed right lower limb weakness which progressed to inability to walk without assistance. No dysarthria, dysphagia, upper limb weakness, seizure or alteration in
Discussion
Among neuropsychiatric manifestations of scrub typhus, AES accounts for 30–60% (Kar et al., 2014; Mahajan and Mahajan, 2017; Jamil et al., 2015; Kim et al., 2013) while rarer features are infarction, cerebellitis, myelitis, parkinsonism, intracranial hemorrhage, subdural hematoma, cerebral venous thrombosis and Guillain-Barre syndrome (Mahajan and Mahajan, 2017; Kim et al., 2000; Heo and Jeong, 2015; Phillips et al., 2018; Yun et al., 2017; Chen et al., 2006). Scrub encephalitis is
Conclusion
Scrub typhus is a re-emerging disease in India and is very often missed by clinicians due to the frequent absence of the characteristic eschar and overall low suspicion. Atypical manifestations as prolonged encephalopathy and lock-in state without fever or eschar can occur due to scrub typhus. The presence of white matter lesions involving subcortical, periventricular, callosal and infratentorial regions producing starry sky appearance with diffusion restriction with or without microhemorrhages
Financial support
Nil.
Declaration of Competing Interest
Nil.
References (16)
- et al.
Prolonged coma in a scrub typhus patient
Int. J. Infect. Dis.
(2018) - et al.
Scrub typhus meningoencephalitis with focal neurologic signs and associated brain MRI abnormal findings: literature review
Clin. Neurol. Neurosurg.
(2011) - et al.
Scrub typhus associated acute disseminated encephalomyelitis
Acta Neurol. Taiwan.
(2006) - et al.
Rocky Mountain spotted fever: ‘starry sky’ appearance with diffusion-weighted imaging in a child
Pediatr. Radiol.
(2012) - et al.
Uncommon manifestations of scrub typhus encephalitis in two cases: clinical and magnetic resonance imaging findings
J. Korean Soc. Radiol.
(2015) - et al.
Scrub typhus meningoencephalitis, a diagnostic challenge for clinicians: a hospital based study from north-East India
J. Neurosci. Rural Pract.
(2015) - et al.
Acute encephalitis syndrome following scrub typhus infection
Indian J. Crit. Care Med.
(2014) - et al.
Scrub typhus leading to acute encephalitis syndrome, Assam, India
Emerg. Infect. Dis.
(2017)