• Users Online: 680
  • Print this page
  • Email this page


 
 
Table of Contents
CASE REPORT
Year : 2022  |  Volume : 10  |  Issue : 2  |  Page : 128-130

Posterior reversible encephalopathy syndrome in spotted fever group of rickettsial infection with brugada syndrome


Department of General Medicine, MVJ MC and RH, Hoskote, Karnataka, India

Date of Submission18-Dec-2020
Date of Decision31-Dec-2020
Date of Acceptance02-Jan-2021
Date of Web Publication05-Apr-2022

Correspondence Address:
Dr. Vasantha Kamath
Department of General Medicine, MVJ MC and RH, Hoskote - 562 114, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajim.ajim_106_20

Rights and Permissions
  Abstract 


Rickettsioses are one of the common causes of undifferentiated febrile illness. Patients present commonly with fever, rash, and eschar. Posterior Reversible Encephalopathy Syndrome (PRES) also known as reversible posterior encephalopathy syndrome is a rare condition, in which parts of the brain are affected by swelling as a result of an underlying cause such as infection. However, PRES caused by spotted fever group of rickettsiosis is rarely reported. We are here with reporting such a case. A 55-year-old male was admitted to MVJ Medical College and Research Hospital, Bengaluru, with complaints of fever for 3 days and one episode of Generalized Tonic Clonic Seizures (GTCS) following which he was in altered sensorium. On examination, there was hepatosplenomegaly. Weil-Felix OX19 and OX2 were 1:320. Lumbar puncture was done which showed 15 cells 98% lymphocytes and 2% neutrophils, proteins-119 mg/dl, and sugar- 153 mg/dl. Magnetic resonance imaging of the brain showed bilateral symmetric hyperintensities on T2-weighted imaging in the parietal and occipital lobes. The calcarine and occipital lobes were spared suggestive of posterior reversible encephalopathy syndrome. Electrocardiogram showed ST elevation in V1 and V2 along with T-wave inversion (Brugada sign). Posterior reversible encephalopathy syndrome is usually commonly seen in hypertensive encephalopathy and in infections such as dengue and few cases of scrub typhus. This is one of the rare cases where spotted fever group of rickettsia has caused posterior reversible encephalopathy syndrome and Brugada syndrome.

Keywords: Brugada syndrome, posterior reversible encephalopathy syndrome, rickettsial infection


How to cite this article:
Palleti SK, Kolla V, Kamath V. Posterior reversible encephalopathy syndrome in spotted fever group of rickettsial infection with brugada syndrome. APIK J Int Med 2022;10:128-30

How to cite this URL:
Palleti SK, Kolla V, Kamath V. Posterior reversible encephalopathy syndrome in spotted fever group of rickettsial infection with brugada syndrome. APIK J Int Med [serial online] 2022 [cited 2022 May 26];10:128-30. Available from: https://www.ajim.in/text.asp?2022/10/2/128/342535




  Introduction Top


Rickettsioses are one of the common causes of undifferentiated febrile illness. Patients present commonly with fever, rash, and eschar. Neurological manifestations include encephalitis, aseptic meningitis, and meningoencephalitis.[1] Posterior reversible leukoencephalopathy syndrome(PRES) also known as reversible posterior encephalopathy syndrome is a rare condition in which parts of the brain are affected by swelling as a result of an underlying cause such as infection. However, PRES caused by spotted fever group of rickettsiosis is rarely reported. We are herewith reporting such a case.


  Case Report Top


A 55-year-old male was admitted to MVJ Medical College and Research Hospital, Bengaluru with complaints of fever for 3 days and one episode of GTCS following which he was in altered sensorium. There was no history of weakness of limbs, blurring of vision, or headache. There was no history of syncope, palpitations, or history of sudden cardiac death in the family. He was newly detected to have type-2 Diabetes mellitus not on treatment. Not a known case of hypertension.

On examination patients pulse was 80/min, blood pressure was-124/72 mm Hg. Lungs were clear. Cardia was normal. There was hepatosplenomegaly and neurological examination showed no focal deficit.

At the time of admission patients Hb was13.5 g/dl, TC-5300 cells/cubic mm, platelet-39,000/cubic mm, GRBS-320 mg/dl total bilirubin–4.3 mg/dl, direct bilirubin-2.6 mg/dl, serum glutamic oxaloacetic transaminase-240 IU/L, serum glutamic-pyruvate transaminase-77 IU/L, alkaline phosphatase-154 IU/L, sodium-141 meq/L, potassium-3.2 meq/L, and calcium-8.8 mg/dl. Weil Felix OX19 and OX2 were 1:320. Lumbar puncture was done which showed 15 cells 98% lymphocytes and 2% neutrophils, proteins-119 mg/dl, and sugar-153 mg/dl.

Magnetic resonance imaging (MRI) of the brain showed bilateral symmetric hyperintensities on T2 weighted imaging in the parietal and occipital lobes. The calcarine paramedian areas of occipital lobes were spared suggestive of posterior reversible encephalopathy syndrome [Figure 1] and [Figure 2].
Figure 1: MRI of the brain showed bilateral symmetric hyperintensities on T2 weighted imaging in the parietal and occipital lobes. The calcarine paramedian areas of occipital lobes were spared-suggestive of posterior reversible encephalopathy syndrome

Click here to view
Figure 2: MRI of the brain showed bilateral symmetric hyperintensities on T2 weighted imaging in the parietal and occipital lobes. The calcarine paramedian areas of occipital lobes were spared suggestive of posterior reversible encephalopathy syndrome

Click here to view


Ultrasonography of the abdomen and pelvis had shown hepatosplenomegaly. Chest X-ray was normal.

Electrocardiogram (ECG) showed ST elevation in V1 and V2 along with T-wave inversion (Brugada sign) [Figure 3].
Figure 3: ECG showed ST elevation in V1 and V2 along with T-wave inversion (Brugada sign)

Click here to view


Two-dimensional echocardiography was normal.

LDH, CK-MB, and troponin levels were normal.

The patient was intubated on arrival due to low Glasgow Coma Scale and was started on doxycycline and was treated conservatively. The patient became afebrile within 48 h after starting doxycycline and gained consciousness within 72 h. MRI and ECG were repeated after 2 weeks which were found to be normal.


  Discussion Top


Rickettsial neuroinvasion occurs during the systemic phase of the disease and typically follows bacterial dissemination via the bloodstream. Rickettsia enters the blood-brain barrier by the transcellular approach which is supported by the fact that viral replication takes place within endothelial cells. Rickettsioses have a wide range of clinical spectrum ranging from mild febrile illness to life-threatening complications. Incubation period is from 2 to 21 days. The most common clinical features are high-grade fever, muscle ache, apathy, drowsiness, photophobia, rash, eschar, generalized lymphadenopathy, and hepatosplenomegaly.[1] Common neurological complications seen are meningitis, encephalitis, and acute disseminated encephalomyelitis.[2] However, unilateral facial nerve palsy,[3] cerebral infarction,[4] or visual loss[5] have also been reported.

Weil-Felix test with a sensitivity of 46% and specificity of 100% can be used to detect rickettsial infection as it is cheap and available easily. Four-fold rises in titers or titers >1:320 are important in confirming acute infection. Cerebrospinal fluid analysis in patients with rickettsial meningitis shows pleocytosis with lymphocyte predominance, hypoglycorrhachia, and hyperproteinorrhachia.[6]

Fatality is up to 30% if left untreated.[7] Doxycycline 100 mg twice a day for 7–10 days is the treatment of choice. Azithromycin 500 mg once a day for 5 days can also be used.[8]

Vasogenic edema is typically primary process leading to neurological dysfunction in posterior reversible encephalopathy syndrome. Vasogenic edema is either due to increased capillary pressure or endothelial dysfunction. Increased capillary pressure is seen in hypertensive encephalopathy, preeclampsia/eclampsia, and postcarotid endarterectomy syndrome. Endothelial dysfunction is seen in infections, calcineurin inhibitors, chemotherapeutic agents, HELLP syndrome, and hemolytic-uremic syndrome. Patients usually present with headache, seizures, cortical visual loss, or focal neurological deficit. MRI classically exhibits high T2 signal edema primarily in bilateral parietal and occipital lobes not corresponding to any single vascular territory. Neyaz et al. in their case report mentioned high signal intensities in the right parietooccipital cortex and right corona radiata on fluid-attenuated inversion recovery (FLAIR) images with true diffusion restriction.[9]

Infections such as dengue are usually implicated in posterior reversible encephalopathy syndrome but spotted fever group of rickettsia causing posterior reversible encephalopathy syndrome are rarely reported. Chen et al. in their case report described scrub typhus-associated acute disseminated encephalomyelitis with MRI findings of progressively extensive areas of signal hyperintensity on conventional T2-weighted images.[10] Naveen et al. in their case report described The clinical course of a 40-year-old female patient, who presented with acute febrile illness due to scrub typhus fever became complicated by seizures and posterior reversible encephalopathy syndrome (PRES), leading to a fatal outcome.[11]

FLAIR sequences can be better at showing these abnormalities.[12] Some specific other rare patterns have also been described in MRI of PRES. The superior frontal sulcus watershed pattern, a watershed pattern involving the entire hemisphere (holo hemispheric) and a central pattern with vasogenic edema in the deep white matter, basal ganglia, thalami, brain stem, and pons.[13],[14] These distinct patterns do not generally correlate with nature, symptoms and their severity and severe edema may suggest a poor prognosis.

Brugada syndrome is a disorder characterized by sudden death and associated with ECG patterns such as incomplete RBBB and ST elevations in anterior precordial leads. Brugada is diagnosed by a prominent elevation of J point a coved ST elevation and inverted T in V1 and V2. Mostly patients are asymptomatic. Symptoms are syncope and cardiac arrest. It is associated with atrial fibrillation in 20% of patients. The ECG changes in Brugada syndrome are dynamic, often hidden, and may reveal themselves in the presence of triggers such as fever, intoxication (alcohol, cocaine, or cannabis), vagal stimulation, electrolyte imbalance, anesthetics (propofol and bupivacaine), psychotropic agents (amitriptyline andlithium), and sodium channel blockers.[15] There are also similar case reports where Brugada was seen in patients with fever and ECG had become normal after fever subsided.[16]


  Conclusion Top


Rickettsial infections manifest with varied presentations and difficult to diagnose sometimes due to its atypical presentations. Prognosis is usually good when diagnosed and treated early. Posterior reversible encephalopathy syndrome is commonly seen in hypertensive encephalopathy and in infections such as dengue and few cases of scrub typhus. This is one of the rare cases where spotted fever group of rickettsia has caused posterior reversible encephalopathy syndrome and Brugada syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kamath V, Ganguly S, Bhatia JK, Himabindu R. Rickettsial infections: Past and present perspectives. APIK J Int Med 2020;8:4-10.  Back to cited text no. 1
  [Full text]  
2.
Eldin C, Parola P. Rickettsioses as causes of CNS infection in southeast Asia. Lancet Glob Health 2015;3:e67-8.  Back to cited text no. 2
    
3.
Kularatne SA, Fernando R, Selvaratnam S, Narampanawa C, Weerakoon K, Wickramasinghe S, et al. Intra-aural tick bite causing unilateral facial nerve palsy in 29 cases over 16 years in Kandy, Sri Lanka: Is rickettsial aetiology possible? BMC Infect Dis 2018;18:418.  Back to cited text no. 3
    
4.
Boulahri T, Taous A, Berri MA, Traibi I, Elbenaye J, Rouimi A. Rickettsiosis associated with cerebral infarction: A new case study. Pan Afr Med J 2017;26:80.  Back to cited text no. 4
    
5.
Kahloun R, Gargouri S, Abroug N, Sellami D, Ben YS, Feki J, et al. Visual loss associated with rickettsial disease. Ocul Immunol Inflamm 2014;22:373-8.  Back to cited text no. 5
    
6.
Salva I, de Sousa R, Gouveia C. Rickettsial meningitis. BMJ Case Rep. 2014;2014:bcr20 1 3 2 0 3 2 8 3. Published 2014 Mar 10. doi:10.1136/bcr- 2013- 203283.  Back to cited text no. 6
    
7.
Kamath V, Bhatia JK, Ganguly S. Rickettsial infections: Spreading beyond boundaries. In: Narsimalu G, editor. Progress in Medicine. Vol. 29. Mumbai: Jaypee; 2019. p. 195-9.  Back to cited text no. 7
    
8.
Kamath V, Ganguly S, Karthik Reddy PS. Rickettsial infection. In: Kamlesh Tiwary editor. Medicine. Vol.31 Update. Mumbai: Jaypee; 2021.p.1132-36.  Back to cited text no. 8
    
9.
Neyaz Z, Bhattacharya V, Muzaffar N, Gurjar M. Brain MRI findings in a patient with scrub typhus infection. Neurol India 2016;64:788-92.  Back to cited text no. 9
[PUBMED]  [Full text]  
10.
Chen PH, Hung KH, Cheng SJ, Hsu KN. Scrub typhus-associated acute disseminated encephalomyelitis. Acta Neurol Taiwan 2006;15:251-4.  Back to cited text no. 10
    
11.
Naveen V, Gaba S, Gupta M, Kaur D. Posterior reversible encephalopathy syndrome in scrub typhus fever. BMJ Case Rep 2020;13:e237262.  Back to cited text no. 11
    
12.
Liman TG, Siebert E, Endres M. Posterior reversible encephalopathy syndrome. Curr Opinion Neurol 2019;32:25-35.  Back to cited text no. 12
    
13.
Fischer M, Schmutzhard E. Posterior reversible encephalopathy syndrome. J Neurol 2017;264:1608-16.  Back to cited text no. 13
    
14.
Tetsuka S, Ogawa T. Posterior reversible encephalopathy syndrome: A review with emphasis on neuroimaging characteristics. J Neurol Sci 2019;404:72-9.  Back to cited text no. 14
    
15.
Bayés de Luna A, Brugada J, Baranchuk A, Borggrefe M, Breithardt G, Goldwasser D, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: A consensus report. J Electrocardiol 2012;45:433-42.  Back to cited text no. 15
    
16.
Manohar S, Dahal BR, Gitler B. Fever-induced Brugada syndrome. J Investig Med High Impact Case Rep 2015;3:1-4.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed175    
    Printed10    
    Emailed0    
    PDF Downloaded16    
    Comments [Add]    

Recommend this journal